Children's Outreach Tips

Question 1

List THREE surgical indications for varicocele repair

Answer 1

1. Symptomatic (pain, discomfort)
2. Decreased testicular volume by >10% compared to contralateral side
3. Infertility
4. Impaired semen parameters /sperm quality

Question 2

What are the features of carcinoid syndrome

Answer 2

Flushing, diarrhea, bronchoconstriction

Question 3

Which feature makes a neuroblastoma automatically classified as high risk?

Answer 3

MYCN +amplification

Question 4

Define Generalized lymphatic Anomaly

Answer 4

a multisystem disorder that also commonly affects bone. Generalized lymphatic anomaly is characterized by discrete radiolucencies with increasing numbers of bones affected overtime. No evidence of progressive osteolysis

Question 5

What are three common areas in which lymphatic malformations can form

Answer 5

Cystic lymphatic malformations occur in areas of major lymphatic channels:
neck
axilla
groin
mediastinum
retroperitoneum

Question 6

What are the three types of Congenital Hemagiomas?

Answer 6

• Rapidly involuting congenital hemangioma (RICH)
• Noninvoluting congenital hemangioma (NICH)
• Partially involuting congenital hemangioma (PICH)

Question 7

Where are GISTs most commonly located in children?

Answer 7

Stomach (90%), remainder in small bowel and colon.

Question 8

What is the hirschsprungs enterocolitis scale?

Answer 8

Standardized scoring system used to establish diagnosis of HAEC. It has a maximum of 20 points with a score of 10 or more consistent with HAEC. History, Physical Exam findings, Radiologic features, and Laboratory findings are important for the score.

Question 9

Origins of GIST tumors

Answer 9

myenteric ganglion cells - interstitial cells of Cajal

Question 10

What is the syndrome of a facial hemangioma?

Answer 10

PHACES:
Posterior fossa malformations
Facial Hemangioma
Arterial Anomalies
Cardiac Anomalies
Eye Anomalies
Sternal Cleft

Question 11

Stages of Diaphragm Embryology and congenial anomalies that can result from failure of those stages.

Remember: Please Make Teachers Incorporate STEM

Answer 11

Pleuroperitoneal Membranes (Folds) - CDH
Thoracic Intercostal groups - Eventration
Septum Transversum - Morgagni
Esophageal Mesentary - Hiatal/Paraesophageal Hernia

Question 12

What is Barrett Esophagus?

Answer 12

Metaplastic transformation of esophageal squamous epithelia to intestinal columnar epithelia as a result of prolonged esophageal acid exposure

Question 13

What is Kasabach-Merritt Phenomenon?

Answer 13

Associated with Kaposiform Hemangioendothelioma (KHE)
The cardinal features are: enlarging vascular lesion, thrombocytopenia (can be profound and sustained <50), microangiopathic hemolytic anemia and a mild consumptive coagulopathy

Question 14

Name 4 Histological Subtypes of Hepatoblastoma

Answer 14

1. Embryonal (30%)
2. Fetal (54%)
3. Anaplastic/Small Cell Undifferentiated (6%)
4. Macrotrabecular (10%)

Question 15

Where should the tip of a UVC be?

Answer 15

IVC or IVC/atrial junction at the level of the diaphragm

Question 16

What are the feared complications of Kasabach-Merritt Phenomenon?

Answer 16

intracranial, pleural, pulmonary, gastrointestinal, retroperitoneal hemorrhage

Question 17

What is the Carney Triad

Answer 17

sporadic syndrome which includes two of the following three tumors: GIST (usually multifocal in antrum), pulmonary chondroma and extra-adrenal paraganglioma

Question 18

List 3 factors that predispose a patient to HAEC

Answer 18

1. Family history
2. trisomy 21
3. long-segment disease
4. prior episodes of HAEC
5. transition zone pull-through
6. anastamotic stricture

Question 19

Special considerations for an inguinal hernia repair in Ehlers Danlos Syndrome

Answer 19

Apart from high ligation with repair of back wall of canal (Bassini repair), consider mesh

Question 20

List 2 possible etiologies of distal gas in a patient presenting with a ?Duodenal Atresia

Answer 20

• duodenal web
• duodenal stenosis
• anomalous biliary ducts (double and patent bile duct)

Question 21

What is blue rubber bleb nevus syndrome?

Answer 21

A rare disorder of multifocal venous malformations (most commonly on hands and fee) that affect primarily the skin and gastrointestinal tract. GI lesions can present with anemia or GI bleeding or intussusception; about 50-60% of cases are associated with oral lesions (usually buccal mucosa, retromolar trigone, ventrolateral tongue)

Question 22

Name 2 incomplete Vascular Rings

Answer 22

1. Right aortic arch with mirror image branching
2. aberrant right subclavian
3. innominate artery compression
4. pulmonary artery sling

Question 23

What FIVE features are the Neuroblastoma pre-treatment risk based on?

Answer 23

1. INRG Stage
2. Age
3. MYCN amplification status
4. 11q aberration status
5. DNA index/Ploidy

Question 24

What are the goals of ventilatory therapy for CDH?

pre-ductal SaO2
PaCO2
pH
Tissue Perfusion

Answer 24

pre-ductal SaO2 - >85%
PaCO2 - 45-60
pH - 7.2 -7.4
Tissue Perfusion - adequate

Question 25

Name FOUR different variations of omphalomesenteric duct Remnants

Answer 25

1. patent omphalomesenteric duct (omphalo-ileal connection) with or without prolapse 2. Umbilical Polyp 3. fibrous remnant connecting antimesenteric border of meckel diverticulum/bowel to abdominal wall at site of umbilicus 4. Meckel Diverticulum 5. blind ending sinus tract 6. omphalomesenteric cyst

Question 26

Name three options for treatment of hemangiomas

Answer 26

1. Propranolol (1st line) 2. Corticosteroids 3. Vincristine 4. The laser 5. Surgical resection 6. TACE for hepatic

Question 27

What is CLOVES syndrome?

Answer 27

Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies (CLOVES) syndrome. Also includes orthotopic or embryonic veins

Question 28

Describe the path of a UAC catheter

Answer 28

enters umbilical artery, course is inferior/posterior to iliac artery, then superior/medial (in posterior position) to aorta

Question 29

Most common site of carcinoid tumor in children

Answer 29

Appendix (80%) | The respiratory tract and GIT are remaining 20% but can be in liver and testes too

Question 30

What are the 5 Tanner Stages of Breast Development?

Answer 30

1. Papilla Elevation 2. Small breast buds palpable and areolae enlarge 3. Elevation of Breast Contour; areolae enlarge 4. Areolae forms secondary mound on the breast 5. Mature breast; nipple projects

Question 31

Which organism is responsible for Necrotizing fasciitis and which antimicrobial medication is essential for its treatment

Answer 31

Group A betahemolytic Strep; clindamycin

Question 32

List three complications of Hepatic Hemangiomas

Answer 32

1. Transient anemia and thrombocytopenia 2. Massive hepatomegaly leading to Abdominal compartment syndrome in diffuse form. 3. Hypothyroidism 4. High Output Cardiac Failure

Question 33

What is the grading system for varicocele

Answer 33

I – palpable only with Valsalva maneuver II – palpable only on standing III – palpable/visualized all the time

Question 34

ATA Classes of a Duodenal Injury

Answer 34

I – hematoma of single portion of duodenum or partial thickness laceration II - < 50 % circumference laceration of duodenum; hematoma involving more than 1 portion III - > 50% circumference laceration of duodenum (D1, D3, D4) or 50-75% circumference at D2 IV - >75% circumference at D2 or involves ampulla V – total devascularization of duodenum; massive disruption of duodenopancreatic complex

Question 35

Describe the path of a UVC catheter

Answer 35

enters the umbilical vein, course is superior/anterior in abdomen, through left PV through ductus venosus, through to IVC

Question 36

Name FIVE GI manifestations of Ehlers Danlos Syndrome

Answer 36

1. Rectal Prolapse 2. Megacolon 3. Recurrent Hernias 4. Spontaneous Rupture of Large vessels and bowel/GI Bleed 5. Spontaneous colonic perforation 6. GERD 7. Functional GI disorders

Question 37

Where can pre-ductal sats be measured?

Answer 37

Right arm, ear lobes

Question 38

How does hypothyroidism develop in children with hemangiomas?

Answer 38

Infantile hemangiomas express type 3 iodothyronine deiodinase which inactivates biologically active thyroid hormone; severity related to tumor burden

Question 39

GI Tumors that arise from the mesentery of the bowel (THREE) and their management

Answer 39

1. Fibromas 2. leiomyomas 3. neurofibromas Mgt - resect without bowel resection, segmental bowel resection if tumour involving the bowel wall.

Question 40

Name THREE primary Gastrointestinal malignancies in childhood in order of frequency

Answer 40

``` NHL (74%) Carcinoid tumors (16%) Colonic adenocarcinomas (5%) Gastric carcinoma (3%) ```

Question 41

List the FOUR diagnostic criteria for diagnosis of eosinophilic esophagitis

Answer 41

1. Clinical Sx of esophageal dysfunction 2. Biopsy with 15+ eosinophils/hpf 3. Failed trial of PPI (8 weeks) 4. Other causes of eosinophilia excluded

Question 42

List THREE things on your differential for a small mass above the eyebrow

Answer 42

1. Dermoid cyst 2. Epidermal inclusion cyst 3. Pilomatrixoma 4. Neurofibroma 5. Granular Cell Tumor

Question 43

What are three subclassifications of infantile hemangioma?

Answer 43

1. focal (solitary in 80%, ulceration in 10-15%), 2. multifocal (multiple) 3. regional (segmental or territorial, very prone to ulceration; if in the lower face ass. w/ upper airway involvement)

Question 44

What is Ehlers Danlos Syndrome?

Answer 44

inherited connective tissue disorder characterized by joint hypermobility, tissue hyperextensibility (skin stretches further than normal), tissue fragility

Question 45

Name the THREE imaging findings that indicate the presence of an IDRF

Answer 45

1. Encasement - surround structure by >50%, or vessel flattened by tumor 2. Infiltration - no margin between tumor and structure 3. Compression - only refers to airways

Question 46

Name THREE anomalies associated with duodenal atresia

Answer 46

1. Trisomy 21 2. Congenital heart defect 3. Malrotation 4. Annular Pancreas 5. EA/TEF

Question 47

Indications for surgery in Burkitt Lymphoma

Answer 47

Intestinal Obstruction Evidence of Threatened Bowel Intussusception Biopsy (pre or post chemo)

Question 48

List THREE non-ECMO options for CDH patients failing conventional ventilation

Answer 48

1. iNO 2. HFOV 3. Jet Ventilation

Question 49

Which neuroblastomas can be managed with observation alone?

Answer 49

1. Cystic neuroblastoma <5cm (cystic) or <3.1cm (solid) 2. L1 <18 months with tumor <5cm (cystic) or <3.1cm (solid) 3. MS tumor 4. Confirmed GN or GNB intermixed (consider if asymptomatic)

Question 50

List the two most common benign vascular tumors

Answer 50

Infantile Hemangioma | Congenital Hemangioma

Question 51

List FOUR complications of an infantile hemangioma

Answer 51

1. Astigmatism if located in periorbital region 2. Bleeding, including from GIT 3. Ulceration 4. Cardiac compromise d/t shunting through the lesion 5. Hypothyroidism 6. Acute Compartment Syndrome

Question 52

What are the THREE phases of High Risk Neuroblastoma treatment?

Answer 52

1. Induction - chemotherapy (+stem cell harvest) and surgery 2. Consolidation - myoablative chemotherapy (+ tandem BMT) and radiation therapy 3. Post-Consolidation - Immunotherapy, cytokines, and isotretinoin

Question 53

What is the management of Alagille Syndrome?

Answer 53

Conservative Mgt – Treat pruritis (ursodiol, rifampin, naltrexone, bile sequestrants – cholestyramine, colesevelam) and malabsorption (ADEK supplementation and high calorie feeds). Transplant if refractory puritis and portal HTN

Question 54

Why do GISTs behave differently in kids compared to adults

Answer 54

Kids typically lack KIT mutations making the use of imatinib and sunitinib unclear.

Question 55

What is the overarching goal of ventilatory management in CDH patients and what initial ventilatory setting will you use?

Answer 55

Overarching goal - Pressure-limited, lung protective strategy with permissive hypercapnia. ``` Initial Settings: FIO2 : 100% PIP: 18-22 PEEP: 5 RR: 30-50 TV: 3-5 ```

Question 56

THREE options for surgical management of FAP

Answer 56

1. Total colectomy with end ileostomy (need rectal surveillance, may do for fertility preservation) 2. Total colectomy with IRA (need rectal surveillance) 3. Total proctocolectomy with IPAA 4. Total proctocolectomy with end ileostomy

Question 57

List three indications for surgery in infantile hemangiomas

Answer 57

1. bleeding (including GI) 2. ulceration 3. threatens vital function 4. risk permanent disfigurement 5. ACS

Question 58

Describe the INRG stages for Neuroblastoma

Answer 58

L1 - locoregional tumor without IDRF L2 - locoregional tumor with IDRF M - Distant metastatic disease MS - L1 or L2 in patient less than 18 months with metastasis isolated to liver, skin, and <10% bone marrow

Question 59

Name 4 types of simple Vascular Malformations

Answer 59

Capillary Malformations (aka port wine stain) Venous Malformations Lymphatic Malformations Arteriovenous Malformations

Question 60

Most common location of leiomyosarcoma in children

Answer 60

Jejunum and colon

Question 61

Histology of a dermoid cyst

Answer 61

arise from elements trapped during embryonal fusion; composed of ectodermal and mesodermal elements (no endodermal); lined by squamous epithelium

Question 62

How to treat vascular rings (very basic)

Answer 62

Essentially involves ligation of aberrant vessel. - ligamentum/ductus divided - for double arch- nondominant arch is divided to preserve brachiocephalic flow - if atretic portion exists, divide at this site

Question 63

List FOUR syndromes associated with Hepatoblastoma

Answer 63

1. Beckwith Wiedamann 2. FAP 3. Prematurity 4. Li Fraumeni 5. Glycogen storage disease 6. Trisomy 18

Question 64

What is the calculation for Oxygen Index? At what oxygen index is ECLS/ECMO indicated?

Answer 64

OI = (mean airway pressure x FiO2 x100) / PaO2 An OI >25 and sustained is an indication to consider ECLS. At OI = 40 ECLS is essentially only option

Question 65

What are the initial settings for HFOV?

Answer 65

Hertz- 10 Mean Arterial Pressure - 15 Amplitude - 30 FiO2 - 100%

Question 66

What is the surgical management for leiomyosarcoma?

Answer 66

complete surgical resection, no lymph nodes required.

Question 67

FOUR subtypes of NHL

Answer 67

Burkitt Diffuse Large B cell Lymphoblastic Anaplastic large cell

Question 68

For which type of vascular anomaly should an EXIT procedure be considered if identified prenatally, and why?

Answer 68

Large cervicofacial lymphatic malformation; potential for postnatal airway obstruction

Question 69

Which neuroblastomas qualify as very low risk (THREE)?

Answer 69

1. Any GN or GNB intermixed 2. L1, MYCN -ve 2. MS, MYCN -ve

Question 70

Which chemotherapeutic agents are used in the treatment of Neuroblastoma? Remember to get the: "View on the Top DECC"

Answer 70

``` Vincristine Topotecan Doxorubicin Etoposide Cisplatin Cyclophosphamide ```

Question 71

Management of Barrett Esophagus with low-grade dysplasia

Answer 71

Treat underlying condition if present, Annual EGD, PPI, +/-fundoplication, +/- RFA

Question 72

Most common location of abdominal Burkitts

Answer 72

ileocecal

Question 73

Which imaging study should be performed if a baby is found to have 5 or more cutaneous infantile hemangiomas?

Answer 73

Liver ultrasound to screen for concurrent hepatic hemangioma

Question 74

Name FIVE hormones that functional carcinoid tumors secrete

Answer 74

1. serotonin 2. 5-HAA 3. dopamine 4. Norepinephrine 5. Histamine 6. Bradykinin 7. Prostaglandins

Question 75

Which Hepatoblastoma patients require neoadjuvant chemotherapy?

Answer 75

Any PRETEXT if unable to do upfront resection (ie 1 cm margin on PV/hepatic veins), extra hepatic, metastatic, PRETEXT III or IV (pre-transplant)

Question 76

In general, what is the goal of surgical resection in neuroblastoma?

Answer 76

Removal of >90% of tumor without contiguous organ resection.

Question 77

What are the 5 ASA classes

Answer 77

I - Normal healthy patient II - Patient with mild systemic disease III - Patient with severe systemic disease IV - Patient with severe systemic disease that is a constant threat to life V - Moribund patient not expected to survive without operation E - Emergent procedure

Question 78

DEFINE chronic eosinophilic esophagitis

Answer 78

A clinicopathologic disease characterized by chronic esophageal dysfunction caused by eosinophil-mediated inflammation of the esophagus

Question 79

What are the features of the LUMBAR association?

Answer 79

``` Lower body infantile hemangioma Urogenital anomalies and Ulceration Myelopathy Body deformities Anorectal malformations Arterial malformations ```

Question 80

What is the goal of resection in a GIST

Answer 80

Excision with free microscopic margins. In the bowel, segmental resection or wide excision with lymph nodes (especially pathologic appearing) is recommended.

Question 81

What is the Ann Arbor Classification for Lymphoma?

Answer 81

I- limited to one LN region II - 2 regions or organs on same side of the diaphragm III - both sides of the diaphragm IV - disseminated disease (w/ one or more extra lymphatic organ) Modifiers: A or B - absence or presence of B Symptoms S - Spleen E - extranodal X - >10cm

Question 82

List FOUR indications for Infective Endocarditis Prophylaxis

Answer 82

1. Prior history of endocarditis 2. Cardiac Valve disease in a transplanted heart 3. Unrepaired cyanotic congenital heart disease or incompletely repaired congenital heart disease 4. Congenital heart disease repaired using prosthetic material 5. A prosthetic heart valve 6. Valve repair using material prosthetic.

Question 83

What are the options for non-operative management in rectal prolapse?

Answer 83

- Teach parents how to reduce, treat electrolyte abnormalities - Lifestyle modifications: no withholding behaviour, no prolonged sitting on the toilet, no reading on the toilet, stool for feet - Diet modifications and bowel management: increase fluid, increase fibre, decrease constipating foods, PEG, enema/suppositories

Question 84

In what organ transplant is PTLD most common?

Answer 84

Intestinal (8%) Thoracic Organs (3-5%) Liver and Kidney (2%)

Question 85

Name TWO surgical procedures that may be required for a patient with Alagille Syndrome

Answer 85

1. Cholangiogram and biopsy | 2. Liver Transplant

Question 86

List FOUR benign gastrointestinal tumors

Answer 86

1. Ganglioneuroma 2. Neurofibroma (NF1) 3. Leiomyoma 4. Lipoblastoma 5. Teratoma 6. Hemangioma 7. Inflammatory Myofibroblastic Tumor

Question 87

What are the Annotation Factors for Hepatoblastoma Remember: Very Poor Eager Freshmen Really Need Cash Money!

Answer 87

``` V = Venous: all 3 1st-order hepatic veins or intrahepatic IVC P = Portal: both 1st-order portal veins or main portal vein E = Extra-hepatic spread F = Multi-Focality: 2 or more tumors w/ normal tissue b/w R = Rupture: (at diagnosis) C = Caudate involvement N = Lymph Node metastases M = Distant Metastases: 1 calcified pulmonary nodule ≥5mm or 2+ non-calcified pulmonary nodules ≥3mm ```

Question 88

How do the majority of children with GIST present

Answer 88

Syncope and generalized weakness due to chronic anemia (86%); palpable tumor (12%); range of asymptomatic to nausea, discomfort, diarrhea, intestinal obstruction, vomiting, constipation

Question 89

What kind of cells are seen in a PTLD biopsy

Answer 89

monoclonal, polyclonal, or monomorphous

Question 90

What is Gorham Stout Disease?

Answer 90

aka vanishing bone disease - progressive osteolysis with resorption of bone and cortical loss associated w/ cutaneous lymphatic malformations. When rib involvement extends beyond the bone a large volume chylous pleural effusion may develop that is very difficult to treat.

Question 91

Management for Carcinoid tumors of the appendix <2cm Management for Carcinoid tumors of the appendix >2cm

Answer 91

< 2 cm - simple appendectomy | >2cm - Right Hemicolectomy (new studies say only needed for positive margins or local lymph node involvement)

Question 92

What are the Features of Crohn's Disease? | Remember: A fat trans granny skips along a cobblestone path to visit the crypts of her pious aunt

Answer 92

``` Creeping Fat Transmural Granulomas Skip Lesions Cobblestoning Crypt Abscesses Pyoderma Granulosum Apthous Ulcers ```

Question 93

List FOUR potential causes of congenital chylothorax

Answer 93

1. atresia of the thoracic duct 2. obstruction of thoracic duct from tumor 3. congenital pulmonary lymphangiectasia/Diffuse lymphangiectasia 4. lymphangiomatosis 5. birth trauma 6. idiopathic 7. associated with syndromes (Noonan, Turner, Down, Gorham-Stout) 8. Central Venous thrombosis 9. Extralobar BPS

Question 94

List three general categories of lymphatic malformations

Answer 94

1. Macrocystic 2. Microcystic 3. Mixed

Question 95

What are the most common symptoms at presentation for eosinophilic esophagitis

Answer 95

In newborns: vomiting, dysphagia, feeding intolerance | Other: food impaction d/t dysmotility +/- stricture, GERD

Question 96

What is required to make the diagnosis of Barrett Esophagus?

Answer 96

Metaplastic columnar mucosa above the GEJ on endoscopy. Goblet cells indicating intestinal metaplasia on histology.

Question 97

What is Alagille Syndrome and what genetic mutation is responsible?

Answer 97

an autosomal dominant genetic disorder caused by mutation in JAG1; bile duct paucity – decreased number and narrow/malformed ducts cause cholestasis, scarring, fibrosis

Question 98

Name TWO options for surgical management of rectal prolapse. Which is preferred in children?

Answer 98

1. rectopexy +/- anterior resection 2. Sclerotherapy (preferred) 3. altemeier or perineal rectosigmoidectomy

Question 99

What are the three stages of the life cycle of an infantile hemangioma?

Answer 99

1. Proliferation - takes 6-12 months; 80% of final size by 3 months 2. Plateau 3. Involution - can continue until 5-7 years old

Question 100

List THREE complications of omphalomesenteric duct remnant

Answer 100

1. intussusception of Meckel diverticulum 2. volvulus around fibrous remnant 3. Meckel diverticulitis inflammation/infection 4. lower gi bleed from ectopic gastric mucosa in Meckel diverticulum 5. omphalitis

Question 101

What is the name for for the Inguinal canal in females?

Answer 101

Canal of Nuck; | Gubernaculum – upper portion = ovarian ligament, lower portion = round ligament

Question 102

What is a pilomatrixoma, and what are the most common presenting features?

Answer 102

Benign, solitary califying appendageal tumor with differentiation toward hair follicles. They most commonly present as a subcentimeter, irregular, firm, non-tender "pebble" with bluish discolouration. 60-70% are found in the head and neck.

Question 103

What is the mainstay of treatment for Eosinophilic Esophagitis?

Answer 103

1. Elimination Diet 2. PO Fluticasone/budesonide x 8 weeks with oral prednisone if failure 3. Dilation of strictures

Question 104

List THREE diseases/clinical symptoms that make children more susceptible to rectal prolapse

Answer 104

1. CF 2. Hypothyroidism 3. Ehlers-Danlos 4. Hypercalcemia 5. Chronic Constipation 6. Diarrheal illness 7. Previous surgery for ARM/HD 8. Malnutrition

Question 105

Common presenting signs/symptoms of leiomyosarcoma

Answer 105

bleeding, intussusception, obstruction (late presentation since they grow outward from serosa), prolapse if in rectum.

Question 106

What is the major risk associated with the identification of a high flow vascular malformation prenatally

Answer 106

Can cause high output cardiac failure and fetal hydrops/demise. These babies need to be closely followed with frequent ultrasound and fetal echo assessment.

Question 107

What are the most common sites of infantile hemangiomas?

Answer 107

head and neck (60%) trunk (25%) extremities (15%) Other - airway (consider especially if seen on the mandible), perineum, and viscera

Question 108

List THREE specific complications of inguinal hernia repair in children

Answer 108

1. recurrence 2. iatrogenic undescended testicle 3. post operative hydrocele 4. testicular atrophy 5. injury to vas 6. bleeding - hematoma 7. chronic pain

Question 109

Name 2 complete vascular rings

Answer 109

1. Double aortic arch 2. Right aortic arch with aberrant left subclavian artery and LDA 3. Right arch with retroesophageal left ligamentum/ductus

Question 110

Name 3 treaments for PTLD

Answer 110

1. Reduce Immunosuppresion 2. Chemotherapy 3. Rituximab 4. Radiation (rescue)

Question 111

What is reverse ratio ventilation, when would you use it, and what is the mechanism?

Answer 111

- Reverse ratio ventilation is having an I:E time ratio greater than 1 (normal ratio is 1:2 to 1:5) - Used in ARDS/ALI to improve oxygenation while minimizing volutrauma or barotrauma - maintains inspiratory plateau pressure for longer (increases MAP) WITHOUT decreasing PIP or TV (minimizes volu/barotrauma)

Question 112

Three types of intestinal ganglioneuromas

Answer 112

1) solitary polypoid - most common; 2) Ganglioneuromatosis polyposis – similar in appearance to FAP; associated with cutaneous lipomas. 3) Diffuse ganglioneuromatosis - Part of MEN2B or Cowden

Question 113

Name three absolute contraindications for a UVC

Answer 113

1. omphalitis 2. omphalocele 3. NEC 4. peritonitis 5. portal venous hypertension

Question 114

Where should the tip of a UAC be?

Answer 114

The aorta between T6-T9, above the celiac take off

Question 115

What are the 5 Stages of Lung Development? What are the congenital anomalies that occur with the stage (if applicable). Remember: Every Peds Consult Sucks Ass!

Answer 115

1. Embryonic 2. Pseudoglandular - Pulmonary/Lobar agenesis, CPAM (1,2,3), BPS, Chaos 3. Canalicular 4. Saccular - Pulmonary Hypoplasia 5. Alveolar (CPAM Type 4)

Question 116

What is the chromosomal translocation associated with Rhabdomyosarcoma

Answer 116

t2;13 -PAX3/FOXO(fusion positive, BAD), t1;13 PAX7/FOXO (good)

Question 117

List THREE Absolute and TWO relative contraindications for ECLS

Answer 117

* Significant congenital anomalies. * Lethal chromosomal anomalies. * IVH grade 2+. * Weight <2kg (relative). * GA <34 weeks (relative).

Question 118

What are the Canadian Readiness for Surgery Consensus Guidelines in CDH?

Answer 118

* Urine output >1ml/kg/hr * FiO2 <50% * Pre-ductal SaO2 >85% * Normal MAP for GA * Lactate <3 * Estimated PA pressure < systemic pressure * (Failure to meet these criteria <2 weeks should prompt consideration of repair vs palliative approach)

Question 119

What are the FOUR most common locations of a Dermoid cysts

Answer 119

1. Glabella 2. Submental 3. Anterior Fontanelle (do U/S or MRI for intracranial extension) 4. Lateral 1/3 of eyebrow

Question 120

What are the indications to image a dermoid cyst?

Answer 120

Any dermoid that is in a precarious location should be imaged due to the propensity for bony/intracranial involvement

Question 121

List TWO indications for ECLS in a baby with CDH

Answer 121

* Preductal hypoxia (SaO2 <85%). * Inadequate oxygen delivery with metabolic acidosis (lactate >5 and/or pH <7.20). * Hypercarbia (>70mmHg with resulting respiratory acidosis— pH less than 7.20) . * Hypotension resistant to fluids and inotropic support with suboptimal tissue perfusion.

Question 122

Define Torticollis and Describe the typical position and findings of the head and neck

Answer 122

Torticollis is an abnormal contracture of the neck musculature resulting in rotation, flexion, and tilting of the neck. It is associated with a palpable unilateral mass within inferior third of SCM with contracture of the SCM leading to ipsilateral tilting with rotation of head & chin to contralateral side (restricted active/passive ROM).

Question 123

Name ONE non-paroxysmal and TWO paroxysmal causes of Torticollis and BRIEFLY describe their mechanism

Answer 123

1. Congenital Muscular Torticolis (CMT, non-paroxysmal) - SCM injury during delivery OR abnormal in utero positioning 2. Benign Paroxysmal Torticollis - Episodic neck wringing, self limiting by age 3 3. Sandifer Syndrome - Severe GERD causing episodic neck wringing, especially in children with epilepsy

Question 124

Name THREE conditions associated with torticollis

Answer 124

1. Hip Dysplasia 2. Plagiocephaly 3. Scoliosis 4. Craniofacial asymmetry/hypoplasia

Question 125

Name THREE options for treatment of Torticollis

Answer 125

1. Physical Therapy - most effective in patients diagnosed <3 months 2. Botox - 75% effective, but can cause dysphagia and weakness 3. Surgery - Unipolar or Bipolar SCM release - for failed physio x6 months or Dx >1yr

Question 126

List TWO risks and TWO benefits of a Primary pull-through for Hirschsprung

Answer 126

Risks: • inadequate rectal irrigations leading to preop enterocolitis • higher rates of postop enterocolitis Benefits: • one surgery • lower rates of anastomotic dehiscence • avoids stoma & complications (fluid losses, prolapse, stenosis)

Question 127

In which Hirschsprung patients is it indicated to perform a multi-stage procedure?

Answer 127

• sick pts with enterocolitis, significant comorbidity (CHD) • dx >6 mos (marked distension of proximal bowel, some component of malnutrition) • total colonic HD or small bowel involvement; liquid stool will cause excoriation & should never determine site of pullthrough on frozen section • pts who cannot maintain enteral nutrition or adequate decompression with rectal irrigations

Question 128

List TWO risks and TWO benefits of a multi-stage pull-through for Hirschsprung

Answer 128

RISKS: • 2 or 3 surgeries • stoma & complications (fluid losses, prolapse, stenosis) BENEFITS: • no rectal irrigations • will have final pathology from stoma site

Question 129

Neurofibromatosis 1 is an autosomal dominant disorder. On what chromosome is the defect? And list THREE findings in NF1

Answer 129

Chromosome 17 - cafe au lait spots - axillary/inguinal freckling - Multiple neurofibromas - pheochromocytoma - optic glioma

Question 130

Neurofibromatosis 2 is an autosomal dominant disorder. On what chromosome is the defect? And list TWO findings in NF2

Answer 130

Chromosome 22 - vestibular schwanoma - meningioma - ependymoma

Question 131

Name THREE things that you should include on your differential diagnosis for Torticollis

Answer 131

1. Sandifer 2. Infection 3. Trauma 4. Posterior fossa tumor 5. spinal cord tumor

Question 132

Name the THREE most commonly performed pull-through procedures for Hirschsprung. In which of the procedures is all aganglionic bowel removed?

Answer 132

1. Duhamel 2. Soave 3. Swenson (all aganglionic bowel removed)

Question 133

What are the FOUR IVH Grades?

Answer 133

I - Hemorrhage in germinal matrix only II - ventricular hemorrhage without ventricular dilation III - ventricular hemorrhage with ventricular dilation IV - brain parenchymal hemorrhage

Question 134

What are the THREE strategies to avoid IVH?

Answer 134

1. avoid prematurity 2. correct coagulopathies 3. antenatal steroids 4. avoid hypoxia/hypercarbia 5. avoid hypotension/fluctuating CBF

Question 135

What is the treatment of choice if a baby with IVH develops severe hydrocephalus

Answer 135

acetazolamide to decrease CSF production

Question 136

What is the mainstay of treatment for IVH?

Answer 136

Supportive therapy, maintenance of cerebral perfusion with cautious BP control, anticonvulsant therapy for seizures, transfusion if indicated.

Question 137

What is the Modified Bell Criteria for NEC

Answer 137

IA - suspected, normal xray, occult blood IB - suspected, normal xray, gross blood in stool IIA - definite, mild, pneumatosis on xray IIB - definite, moderate, ascites, acidosis, thrombocytopenia IIIA - advanced, very ill, bowel intact, DIC, neutropenia, acidosis, hypotension IIIB - advanced, very ill, bowel perforation, pneumoperitoneum

Question 138

List TWO indications for peritoneal drainage in a NEC

Answer 138

* Very ill pts who cannot be safely moved to OR * Premies <1500g (vLBW) *NB - 30% require exlap soon after drain, 30% require exlap later; 30% never require exlap

Question 139

List TWO advantages and THREE disadvantages to contralateral exploration in hernia repairs

Answer 139

Advantages: • Identification of a patent processus vaginalis on the contralateral side in a significant number of pts • Avoidance of a second surgery & anesthetic and cost if the contralateral patent processus vaginalis were to become symptomatic. Disadvantages: • Potential injury to the vas deferens or testicular vessels. • Increased operating time for contralateral procedure. • May be unnecessary in as many as seventy percent of pts.

Question 140

On what side are inguinal hernias more common? On what side are varicoceles most common?

Answer 140

Hernia - Right (75%) Varicocele - Left (90%)

Question 141

What is the natural history of a patent processus?

Answer 141

The natural history of a patent processus vaginalis is closure within two months after birth in 40% and within two years in an additional 20%.

Question 142

What percentage of babies have metachronous hernias?

Answer 142

Incidence of metachronous hernia 5-12%, it is 15% in premies, and 28% in pts who present with incarcerated hernias.

Question 143

In what circumstances should a primary anastomosis be considered in NEC?

Answer 143

Primary anastomosis should be considered is with spontaneous intestinal perforation or single segment disease when excellent perfusion of the remaining bowel is assured in a physiologically stable pt

Question 144

List FOUR causes of inguinal swelling in children

Answer 144

1. Inguinal Hernia 2. Inguinal lymphadenopathy or lymphadenitis 3. Granuloma inguinale 4. Femoral Hernia 5. Amyand Hernia (appendicitis within hernia sac) 6. Benign or malignant tumor

Question 145

Which babies should initially have their TEF clipped (or ligated), distal esophagus tacked, and gastrostomy inserted (on lesser-curve)?

Answer 145

* Significant cardiac anomalies * Critically ill babies (or pts needing multiple other surgeries) * <1.3kg. * Long-gap EA (>3cm or >2 vertebral bodies)

Question 146

What is the embryologic explanation for TEF?

Answer 146

Failure of septation of ventral and dorsal foregut

Question 147

Why is an Echo necessary prior to a TEF repair?

Answer 147

50% of TEF have a concurrent cardiac anomaly and need to know the side and character of the aortic arch

Question 148

What are the cardinal symptoms of Henoch-Schonlein Purpura (IgA Vasculitis)

Answer 148

* Palpable purpura in pts with neither thrombocytopenia nor coagulopathy. * Abdominal pain (precedes rash ~25%) * Renal disease (proteinuria, hematuria) * Arthritis or arthralgias * Scrotal symptoms (presents like testicular torsion)

Question 149

What complications of Henoch-Schonlein Purpura (IgA Vasculitis) bring children to the attention of Pediatric Surgery?

Answer 149

•Abdominal pain (precedes rash ~25%) • Nausea, vomiting, and colicky abdominal pain. • Chronic or recurrent abdominal pain from IgA deposition affecting the visceral vasculature is a common feature (old lady mesenteric ischemia). • Small bowel intussusception—lead point is from sub-mucosal hemorrhage and edema; infarction, ischemia, necrosis, late stricture, appendicitis, obstruction perforation; GI hemorrhage can be severe.

Question 150

What is the management of Henoch-Schonlein Purpura (IgA Vasculitis)

Answer 150

• Self-limited in majority of cases so most pts cared for as outpt with Tylenol and NSAIDS. • Steroids if symptoms are severe (studies have shown these will decrease duration of abdominal pain). • Taper off steroids 4-8 weeks to prevent rebound. • Intussusception in kids with HSP almost always needs surgery

Question 151

What is Hemolytic Uremic Syndrome and what is the most common causative organism?

Answer 151

HUS is a clinical syndrome characterized by progressive renal failure that is associated with microangiopathic hemolytic anemia and thrombocytopenia. It is most commonly caused by E. Coli O157:H7 (>90% cases, Shiga toxin-producing strain) or Shigella

Question 152

What is the classic triad of Hemolytic Uremic Syndrome?

Answer 152

acute hemolytic anemia, thrombocytopenia, AKI.

Question 153

What GI complications are associated with Hemolytic Uremic Syndrome?

Answer 153

Severe LGIB causing anemia, hemoperitoneum, severe colitis with megacolon, necrosis and perforation.

Question 154

Name the THREE types of histiocytosis

Answer 154

* Langerhans cell histiocytosis * Hemophagocytic lymphohistiocytosis (HLH) * Malignant histiocytosis

Question 155

What is Langerhan's Cell Histiocytosis?

Answer 155

A rare histiocytic disorder characterized by single or multiple osteolytic bone lesions with infiltration of histiocytes. It can involve lymph nodes, bone marrow, liver, spleen, lungs, CNS.

Question 156

What is the management for Langerhan's Cell Histiocytosis

Answer 156

* Prednisone, vinblastine, radiation for bony lesions at risk of collapse (spine, femur) * Do not perform wide excision as bone lesions will have complete healing with chemo.

Question 157

Name FOUR things on the differential diagnosis for an umbilical granuloma?

Answer 157

1. Urachal duct remnants 2. Omphalomesenteric duct remnants 3. Omphalitis 4. Ectopic intestinal mucosa 5. Umbilical Hernia

Question 158

List the FOUR types of urachal anomalies

Answer 158

Urachal Cyst Urachal Sinus Urachal Diverticulum Patent Urachus

Question 159

Name THREE Omphalomesenteric duct remnants

Answer 159

``` Meckel Diverticulum Meckel with Fibrous cord Umbilicoileal fistula Vitelline cyst, umbilical cyst Fibrous cord (with or without artery). ```

Question 160

List FOUR complications that are common to both UACs and UVCs

Answer 160

* Tip migration * Sepsis/omphalitis * Thrombosis * Hemorrhage

Question 161

List THREE complications of UVCs

Answer 161

* Perforation of the IVC * Extravasation of insufflate into the peritoneal cavity * Portal vein thrombosis * Omphalitis/sepsis * Perforation of intrahepatic vascular wall

Question 162

List FOUR complications of UACs

Answer 162

* Omphalitis * Aortic injuries * Thromboembolism of aortic branches * Aneurysms of the iliac artery or aorta * Paraplegia * Gluteal ischemia with possible necrosis

Question 163

What is Tuberous Sclerosis?

Answer 163

Autosomal dominant disorder characterized by Ash leaf spots, renal angiomyolipoma, giant cell astrocytoma, cardiac Rhabdo, infantile spasms

Question 164

What are the features of Mayer-Rokitansky-Küster-Hauser (MRKH)

Answer 164

• 46XX. • Absence of uterus, fallopian tubes, and upper 2/3 of vagina (Müllerian structures); can have very hypoplastic uterus. • Normal development of ovaries and normal secondary sexual characteristics. Type 1 = no other anomalies Type 2 = renal, skeletal, hearing, and/or cardiac anomalies.

Question 165

What are the THREE most important initial questions to ask in a patient presenting with a disorder of sexual development?

Answer 165

* Is there an anus on physical exam? (no anus, no DSD) * What is the karyotype (XX, XY, or other)? * Are gonads symmetric or asymmetric by PEx or laparoscopy?

Question 166

If a patient with a disorder of sexual development has symmetrical gonads, what are the TWO possible diagnoses?

Answer 166

* Congenital adrenal hyperplasia (CAH) = 46XX DSD | * Androgen insensitivity syndrome (AIS) = 46XY DSD

Question 167

If a patient with a disorder of sexual development has asymmetrical gonads, what are the THREE possible diagnoses?

Answer 167

* Pure gonadal dysgenesis = 46XX (2/3) or 46XY (1/3) * Mixed gonadal dysgenesis = 45XO/46XY or 46XY * Ovotesticular DSD = 46 XX or 46XY

Question 168

What is the initial workup of a patient born with a DSD?

Answer 168

* Karyotype * Serum electrolytes * EKG * Consult Endocrinology * Imaging (U/S +/- MRI) +/- EUA & laparoscopy

Question 169

What are the THREE most common genetic anomalies implicated in Congenital Adrenal Hyperplasia

Answer 169

- 21-hydroxylase deficiency most common (90%) - 11-β hydroxylase deficiency - 3-β hydroxysteroid deficiency

Question 170

Androgen insensitivity syndrome can be complete, partial, or mild. What are the mechanisms that can lead to androgen insensitivity?

Answer 170

* Deficiency of androgen production. * Androgen receptor deficiency. * Inability to convert testosterone to dihydroxytestosterone (5 alpha reductase deficiency).

Question 171

What are the processes responsible for gonadal development?

Answer 171

The default human setting is female. 1. The SRY protein makes the gonad a testical (no SRY = ovaries) 2. Testosterone turns the wolffian duct into a seminal vesicle (degenerates if no testosterone). 3. Anti-Mullerian hormone makes mullerian ducts disappear (otherwise you get a fallopian tube, uterus, and upper vagina)

Question 172

What is the processes are responsible for the development of external genitalia?

Answer 172

In the absence of androgens, the external genitalia become feminized

Question 173

What are the features of the classical presentation of congenital adrenal hyperplasia.

Answer 173

Decreased glucocorticoids and mineralocorticoids with excess androgens and adrenal hyperplasia. This constellation leads to sodium loss from the kidneys, hypovolemia, and hyperkalemia that can result in cardiac arrest and hypovolemic shock.

Question 174

What is Poland Syndrome?

Answer 174

unilateral chest wall anomaly resulting in the absence/hypoplasia of pectoralis muscles (especially pectoralis major), absence/deformity of ribs, chest wall depression, amastia, athelia, upper extremity abnormalities (syndactaly brachydactaly), absence of axillary hair & limited subcutaneous fat.

Question 175

Name THREE genetic disorders that are associated with Pectus Excavatum

Answer 175

1. Poland Syndrome 2. Marfan 3. Ehler-Danlos 4. Noonan

Question 176

What is the management of Poland Syndrome?

Answer 176

Combined surgical correction with plastic surgery; latissimus dorsi flaps for muscular hypoplasias +/- breast implants; pectus deformities can be addressed by Nuss +/- Ravitch.

Question 177

Beckwith-Wiedemann Syndrome (BWS) is an overgrowth syndrome with a range of phenotypes. Name FOUR associated features of BWS (not oncologic)

Answer 177

1. macroglossia 2. macrosomia (growth slows by age 8) 3. visceromegaly 4. hemihyperplasia 5. hyperinsulinemic hypogylcemia 6. Omphalocele 7. preauricular pits/sinuses

Question 178

Name FOUR tumors that patients with Beckwith Wiedemann can develop

Answer 178

* Wilms = 43% * Hepatoblastoma = 12%; usually develops by age 4. * Rhabdomyosarcoma * Neuroblastoma * Adrenal cortical carcinoma * Congenital mesoblastic nephroma

Question 179

What is the surveillance for children with BWS?

Answer 179

need glucose monitoring as neonate; need surveillance US q3 months until age 4 then renal/adrenal US q3months until 7, and AFP level q 3 months until 8 years old. After 8 until adolescence, annual or biannual renal US.

Question 180

Describe the theory of pathogenesis and the presentation of spontaneous perforation of the common bile duct in an infant.

Answer 180

The exact cause is unknown, but theories include: congenital mural weakness of CBD, ischemia, distal biliary obstruction, PBM. It most commonly presents with abdo distension, jaundice, acholic stools, +/- fever, vomiting, enlarging hernias, abdo mass

Question 181

List THREE conditions on the differential diagnosis of a biliary ascites in a baby

Answer 181

1. Blunt Liver injury 2. NEC 3. Distal Biliary stenosis-->CBD perforation 4. Perforated choledochal cyst 5. Pancreatitis 6. CMV

Question 182

What is the mechanism of action of cyclosporine A (CSA)?

Answer 182

A calcineurin inhibitor used as an immunosuppressant; works by decreasing function of lymphocytes by decreasing the production of inflammatory cytokines (reversible). No effect on phagocytic cells therefore no BM suppression

Question 183

What is OEIS complex?

Answer 183

* Omphalocele * Exstrophy of bladder * Imperforate anus * Spinal defects (dysraphism)

Question 184

Describe Classic Bladder Exstrophy

Answer 184

An open, inside-out bladder on the surface of the lower abdominal wall and an open exposed dorsal urethra. It is associated with epispadias, low set umbilicus, umbilical hernia/omphalocele, pubic diastasis, anterior anus, and inguinal hernias.

Question 185

What is he pathognomonic finding of cloacal exstrophy on a prenatal ultrasound

Answer 185

elephant trunk–like mass | (representing the prolapsed ileum) — found in 33%.

Question 186

Describe the classic findings of cloacal exstrophy

Answer 186

• 2 exstrophied bladder halves separated by a strip of exstrophied cecum (“cecal plate”) and imperforate anus. • Cecal plate typically accompanied by a prolapsed ileal segment and foreshortened colon. • Bifid phallus/vagina; females with 2 uteri. • Patulous bladder neck, widened pubic symphysis, lateral displacement of the rectus musculature, widened and attenuated linea alba, low set umbilicus. • +/- omphalocele (>90%).

Question 187

Describe a single-stage procedure for the repair of cloacal exstrophy

Answer 187

Involves closure of the abdominal wall, bladder, and phallic halves with end colostomy +/-osteotomy. Ureteral stents, urethral catheter, cystotomy generally placed; monitor kidneys for hydronephrosis postop

Question 188

Briefly describe the three options for colostomy creation in a patient with cloacal exstrophy

Answer 188

* Dissect cecal plate off hemibladders, tubularize, and create proximal end colostomy from the tubularized cecum * Dissect cecal plate off the hemibladders, tubularize, anastomose to hindgut, create end colostomy with distal-most portion of the hindgut * leave cecal plate attached to the hemibladders and detach hindgut and terminal ileum from the cecal plate; anastomose hindgut and terminal ileum and create end colostomy from the distal hindgut

Question 189

Why is a colostomy and not an ileostomy needed for cloacal exstrophy?

Answer 189

preserve bowel length, expose hindgut to fecal stream, minimize fluid/E+ problems, solid stools

Question 190

Describe the three-stage repair of bladder extrophy

Answer 190

1. At birth - convert to complete epispadias – close bladder, posterior urethra and abdo wall, use suprapubic cath, ureteral catheters 2. At 6-12 months, repair of epispadias 3. Before school age, bladder neck repair for continence and ureteral reimplantation for VUR

Question 191

For each branchial arch list THREE derivatives

Answer 191

1st - External auditory canal, Tympanic membrane, Muscles of mastication 2nd - Tonsil (tonsillar fossa), Facial nerve, Muscles of facial expression 3rd - Inferior parathyroids, Thymus, Hyoid bone 4th - Superior parathyroids, Superior LN, Pharyngeal muscles 5th - regresses 6th - Pulmonary arteries, Recurrent LN, Vagus.

Question 192

What is the Embryologic pathogenesis of Branchial cleft anomalies?

Answer 192

The fetal branchial apparatus of paired pharyngeal arches and clefts gives rise to mature head and neck structures. They usually obliterate after these structures are formed. Failure to obliterate leads to the spectrum of branchial anomalies seen in children, including cysts, sinuses, fistulae, and ectodermal remnants

Question 193

What is BOR Syndrome?

Answer 193

Branchio-Oto-Renal syndrome—branchial arch anomalies, hearing impairment (malformations of the auricle with pre-auricular pits, hearing impairment), and renal malformations (urinary tree malformation, renal hypoplasia/agenesis, renal dysplasia, renal cysts).

Question 194

What is the presentation and Management of a 1st branchial anomaly?

Answer 194

The external opening is found just inferior to the angle of the mandible. Typically presents as parotiditis/abscess (palpate tract, express fluid, feel for cyst/mass in parotid). Surgical management: excision essentially like superficial parotidectomy via pre-auricular incision; insert probe in tract and dissect all the way to external auditory canal (carefully preserve all facial nerve branches).

Question 195

Where would you expect to find a 2nd Branchial anomaly? And where does the fistula communicate to?

Answer 195

external opening anterior to SCM in lower neck; fistula communicates with pharynx at the tonsillar fossa with the tract potentially traveling between the internal & external carotid arteries

Question 196

Which structures should be protected/avoided in the excision of a 2nd Branchial anomaly?

Answer 196

beware damage hypoglossal (intrinsic muscles of the tongue), glossopharyngeal (taste posterior 1/3 tongue), vagus (parasympathetic function). Also watch for carotid, IJ, pharynx

Question 197

Describe the surgical Management of a 2nd Branchial anomaly

Answer 197

insert probe in tract, elliptical incision around fistula, step incisions to dissect proximally to maximal extent, ligate as close to pharynx as possible at the tonsillar fossa (ask anesthesia to push down on the oropharynx).

Question 198

50% 3rd and 4th Branchial anomalies have openings in the ______ sinus

Answer 198

piriform

Question 199

Describe the classic presentation of a 3rd/4th Branchial anomaly in a Neonate and in an older child

Answer 199

Neonatal: enlarging neck mass that compromises spontaneous breathing Older child: neck abscess within or in vicinity of left thyroid lobe; suppurative thyroiditis/thyroid cyst

Question 200

Outline the surgical management of a 3rd/4th Branchial anomaly

Answer 200

Left thyroid lobectomy with excision of the tract (risk of RLN injury) or endoscopic procedure to cauterize tract.

Question 201

85% of Biliary Atresia is Sporadic. Describe the TWO Syndromic groups

Answer 201

Syndromic group 1 (5%): occurs in association with other anomalies that do not involve laterality defects. • GU: cystic kidneys, hydronephrosis • GI: intestinal atresia, EA, ARM * Syndromic group 2 (10%): biliary atresia-splenic malformation (BASM) syndrome * Laterality defects (malrotation and/or situs inversus) * Polysplenia * Interrupted vena cava * Pre-duodenal portal vein

Question 202

``` Disorders of the parotid can be divided into several categories. List TWO things on the differential for each of the following categories: Infectious Inflammatory Neoplastic Vascular ```

Answer 202

Infectious: mumps, CMV, scrofula, bacterial infection Inflammatory: juvenile Sjögren, idiopathic chronic recurrent juvenile sialadenitis Neoplastic: pleomorphic adenoma (benign), mucoepidermoid carcinoma, acinic and adenoid cystic carcinoma Vascular: juvenile capillary hemangioma, lymphangioma, vascular malformation

Question 203

Detail the Presentation and Management of Atypical Mycobacterial Lymphadenitis

Answer 203

Presentation: increasing mass with overlying skin changes —> fistulization in sub-mandibular, parotid, pre-auricular, cervical LN basins; blue/purple discolouration with shiny skin overlying; not associated with fever, or systemic symptoms. Management: gold standard is surgical resection—excise all gross disease, including skin, subcutaneous tissue, lymph nodes, and involved salivary glands

Question 204

Detail the Presentation and Management of a thyroglossal duct cyst

Answer 204

Presentation: mass in midline (note that in reality, 1/3 off midline), above level of thyroid cartilage; often moves with tongue protrusion; may rupture and present with overlying inflammation. Management: Sistrunk procedure, involving resection of the entire lesion en bloc (involved skin, core of strap muscles, cystic lesion, anterior portion of hyoid bone) with ligation of the tract as high as possible in the muscles of the base of the tongue

Question 205

Name FOUR infectious causes of a neck mass in children

Answer 205

1. Reactive LN hyperplasia 2. Cervical Suppurative Lymphadenitis 3. Scrofula (TB) 4. Atypical Mycobacterium 5. Bartonella (cat-scratch)

Question 206

Name FOUR Non-infectious causes of a neck mass in children

Answer 206

1. Thyroglossal duct cyst 2. Dermoid cyst 3. Epidermoid cyst 4. Ectopic Thyroid Tissue 5. Lymphoma 6. Hemangioma 7. Teratoma 8. Branchial Cleft Remnant 9. Cervical Thymic Cyst

Question 207

What is the embryological explanation for thyroglossal duct cysts?

Answer 207

persistence of a tract during the descent of the thyroid from the foramen cecum (at the base of the tongue) to the neck; this occurs while the hyoid bone in forming, which explains association with hyoid.

Question 208

What is the embryological explanation for Prune Belly?

Answer 208

urethra obstructed, leads to enormous bladder dilatation, prevents development of abdominal wall musculature.

Question 209

What are the THREE features of Triad Syndrome (Prune Belly)?

Answer 209

Deficient or absent abdominal wall, Intra-abdominal undescended testes, ectasia of urinary system (Dilated distal genitourinary tract) BONUS: Pulmonary Hypoplasia due to Oligohydramnios

Question 210

Where are Accessory Spleens typically found? Name THREE potential complications of Accessory Spleens

Answer 210

75% of accessory spleens are found in the splenic hilum. The remaining are nearly all near the tail of the pancreas and rarely found in the splenic ligaments, splenic artery, wall of the stomach, omentum or pelvis (or scrotum!) Complications: Torsion, Infarction, Rupture, Abscess

Question 211

Name FOUR Developmental Splenic Anomalies

Answer 211

- Heterotaxy - Spleno-gonadal fusion - Spleno-pancreatic fusion - Wandering Spleen

Question 212

Define Wandering Spleen and describe its management

Answer 212

Abnormal development of splenic ligaments (or older person with lax ligaments over time); spleen at risk for torsion, ischemia, infarction Treatment is splenopexy

Question 213

Describe the THREE mechanisms that lead to Tumor Lysis Syndrome

Answer 213

1. DNA breakdown--> hyperuricemia —> renal failure 2. Protein breakdown —> hyperphosphatemia —> hypocalcemia —> cardiac arrhythmia—> renal failure 3. Cytosol breakdown —> hyperkalemia —> cardiac arrhythmia —> renal failure

Question 214

Which tumors are at higher risk of Tumor Lysis Syndrome?

Answer 214

Typically for Burkett’s and ALL, but can occur in any tumour that has a high cell proliferation rate

Question 215

Management of Tumor Lysis Syndrome

Answer 215

Prevent with prophylaxis (IV hydration), hypouricemic agents (allopurinol + rasburicase), urinary alkalinization (acetazolamide or NaHCO3)

Question 216

Detail the embryology of Cloaca

Answer 216

The cloaca is present in the developing embryo by the 21st day of gestation and over a six-week process separates into a separate anterior urogenital cavity and posterior anorectal cavity. Inadequate separation results in 3 systems remaining in communication

Question 217

What criteria are used to determine if a PSARP and Total Urogenital Mobilization can be performed for Cloaca?

Answer 217

If Common Channel <3cm and urethra >1.5cm

Question 218

If the common channel is >3cm and urethra <1.5cm in a cloaca, what procedure is required for repair?

Answer 218

1. Help from a friend named Mark or Alberto 2. Urogenital Separation 3. Posterior Sagittal Anorectal Vaginal Urethroplasty

Question 219

An abdominal mass in a patient with cloaca is _____ until proven otherwise

Answer 219

Hydrocolpos

Question 220

What are THREE features of a covered exstrophy?

Answer 220

- wide public symphysis - low-lying umbilicus - open bladder neck

Question 221

List THREE options for drainage of Hydrocolpos

Answer 221

1. Intermittent drainage via perineal orifice 2. Tube Vaginostomy 3. Cutaneous Vaginostomy

Question 222

What is the Workup in VACTERL Screening

Answer 222

* CXR + AXR with NG in place * AP/lateral sacral x-ray * Abdominal, pelvic, renal U/S * Spinal U/S +/- MRI * Echocardiogram * CBC, CMP, coags, cross-match

Question 223

What does VACTERL stand for?

Answer 223

If you don't know the answer to this question after 5 years of residency and 2 years of Peds Fellowship, only God can help you now! :)

Question 224

How should a colostomy be made in Cloaca? (classic answer)

Answer 224

High sigmoid colostomy (i.e. top of left colon) to make sure you leave enough length for reconstruction later —> try not to divide marginal artery. Other options: Turnbull, Transverse colostomy

Question 225

Describe the cardinal steps in a Total Urogenital Mobilization

Answer 225

1. Separate the rectum from the vagina 2. Keep vagina and urethra together as UG sinus 3. Free UG sinus anteriorly by dividing suspensory ligaments 4. Mobilize UG sinus to perineum 5. Sew new position of urethra anteriorly 6. Introitoplasty and PSARP

Question 226

Describe the very basic steps of a Urogenital Separation

Answer 226

1. Separate the rectum from the vagina 2. Separate vagina from urinary tract 3. Close bladder neck-vaginal fistula 4. Common channel becomes the neourethra 5. Vaginal pull-through to perineum

Question 227

Renal Trauma Grades

Answer 227

I – contusion (microscopic/gross hematuria); hematoma (subcapsular, non expanding, w/o parenchymal laceration II – hematoma – non expanding, perirenal- confined to RP; laceration < 1 cm depth of cortex w/o urinary extravasation III – laceration > 1 cm depth of cortex w/o collecting system rupture or urinary extravasation IV – laceration – extends through renal cortex, medulla and collecting system; vascular – main renal artery or vein injury w/ contained hemorrhage V – laceration- completely shattered kidney; avulsion of renal hilum

Question 228

What is Page Kidney?

Answer 228

Hypertension develops secondary to extrinsic compression of the kidney by a subcapsular collection (hematoma, seroma, urinoma), which can constrict the kidney. It can occur with ANY injury grade

Question 229

What is the most common kidney tumor in patients with Tuberous Sclerosis, and why do we care?

Answer 229

Angiomyolipoma (AML) is the most common renal lesion observed among patients w/ TSC. The majority have few or no symptoms and lesions are detected on surveillance imaging. These are benign lesions but if >4cm have high risk for catastrophic hemorrhage spontaneously or with minor trauma. Treatment for AML is partial nephrectomy. AML are fat-rich; if renal mass without typical appearance of AML on U/S or CT, pt should have MRI and biopsy to rule-out renal cell carcinoma.

Question 230

What is the appropriate follow up for patients with Sacrococcygeal teratoma?

Answer 230

Q3 mos PEx (with DRE) + alpha fetoprotein (αFP) levels x 1 year then Q6 mos thereafter x 3 years (all recurrences have been diagnosed in that time). Additional studies such as MRI or serum βHCG may be obtained.

Question 231

What are Posterior Urethral Valves (PUV)

Answer 231

PUV are the most common congenital urethral obstruction in male newborns; most common cause of CKD in kids. It is caused by obstructing membranous folds within the lumen of the posterior urethra.

Question 232

Describe the prenatal findings of Posterior Urethral Valves (PUV)

Answer 232

1/3 diagnosed prenatally with hydronephrosis or | distended thick-walled bladder and dilated posterior urethra.

Question 233

What are the presenting symptoms of a newborn with Posterior Urethral Valves (PUV)? What are the presenting symptoms of a child with PUV?

Answer 233

Newborn: UTI, urosepsis, abdominal distension, poor stream, grunting while voiding; also clinical manifestations of pulmonary hypoplasia. Children: UTIs, incontinence, enuresis, frequency, poor stream.

Question 234

What is the initial treatment, endoscopic treatment, and surgical treatment for Posterior Urethral Valves?

Answer 234

Initial treatment: drain bladder with 8Fr feeding tube (avoid foley balloon because may cause bladder irritation), monitor fluids and electrolytes, prophylactic antibiotics Cystoscopic treatment: STING (Sub-urethral Teflon Injection) - valve leaflets are ablated at the 5 and 7 o’clock positions Surgical treatment: cutaneous vesicostomy if cystoscopic treatment fails.

Question 235

What are the FIVE grades of Vesico-ureteral reflux on VCUG?

Answer 235

1: reflux into ureter without ureteral dilation 2: reflux into pelvis + calyces without ureteral dilation 3: reflux into pelvis + calyces + mild dilation ureter/pelvic/calyces 4: reflux into pelvis + calyces + mod dilation ureter/pelvic/calyces + ureteral tortuosity 5: reflux into pelvis + calyces + gross dilation ureter/pelvic/calyces + ureteral tortuosity

Question 236

Name FOUR indications for VCUG

Answer 236

1. Recurrent UTIs 2. UTI before toilet training 3. UTI with pathogen other than E coli 4. Concern with parental compliance for follow-up (i.e. after episode of UTI) 5. Any boy with UTI? 6. Renal anomalies on U/S 7. Systemic signs of chronic kidney disease

Question 237

What is a TART?

Answer 237

Marriam-Webster: a woman who wears very sexy clothing and has sex with many men. Too many "sleepovers" Coran: Testicular Adrenal Rest Tumours (TART); Classically present in males with CAH. If CAH or any syndrome with elevated ACTH, can undergo hyperplasia and malignant transformation

Question 238

What is the significance of an Adrenal Rest?

Answer 238

Adrenal rests can be found along spermatic cord or anywhere in RP, including ovaries or surrounding structures (anywhere along tract of descent of gonads). Association with undescended testes. Hard yellow nodules. Remove if you see them.

Question 239

Name the FIVE key vessels in fetal circulation and their adult vestiges

Answer 239

Umbilical Vein - Ligamentum Teres Umbilical Arteries - Medial umbilical ligaments Ductus Venosus - Ligamentum Venosum Foramen Ovale - interatrial septum or Fossa Ovalis Ductus Arteriosus - Ligamentum Arteriosum

Question 240

Define Assent

Answer 240

term used to express willingness to participate in research or accept medical/surgical treatment for persons who are by definition too young to give informed consent but old enough to understand the proposed research or medical/surgical treatment in general, including risk, benefits, and expected activities;

Question 241

Define Consent

Answer 241

must include 3 major elements: (1) disclosure of information, (2) competency of the patient (or surrogate) to make a decision, and (3) voluntary nature of the decision.

Question 242

Define Beneficence

Answer 242

refers to actions that promote the well being of others; in the medical context, this means taking actions that serve the best interests of patients and their families.

Question 243

Define non-maleficence

Answer 243

do no harm; refers to an intention to avoid needless harm or injury that can arise through acts of commission or omission; in common language, it can be considered “negligence” if you impose a careless or unreasonable risk of harm upon another.

Question 244

Define the term "Standard quality of life"

Answer 244

standard level of health, comfort, or happiness, or general well being that can be expected or strived for

Question 245

Describe the embryology of Pancreatic Divisum

Answer 245

The pancreas is formed from fusion of the dorsal and ventral anlages, which develop from the embryologic foregut. Fusion of the ductal system occurs in just over 90 percent of individuals, although variations in patency of the accessory duct (of Santorini) occur. Failure of fusion of the ventral and dorsal duct system results in pancreas divisum. Pts with pancreas divisum have a dominant dorsal duct, therefore most pancreatic enzymes enter the duodenum via the duct of Santorini (minor papilla).

Question 246

What is the natural history of pancreatic divisum?

Answer 246

95% of pts with pancreas divisum never have problems associated with it, but 5% develop pancreatitis. The hypothesis is that pancreatitis develops because pts cannot adequately drain their pancreatic secretions into the duodenum.

Question 247

What are the THREE histologic subtypes of congenital mesoblastic nephroma

Answer 247

classic (65%), cellular (25%), mixed (10%)

Question 248

List THREE things on the differential for a renal mass in a 5 month old.

Answer 248

1. Wilms 2. CMN 3. rhabdoid of the kidney 4. clear cell sarcoma 5. renal cell carcinoma, 6. angiomyelolipoma 7. ossifying renal tumour

Question 249

Describe the Stocker Classification and association with each type.

Answer 249

0 - Tracheobronchial - Death I - Bronchial - Large cysts - Bronchoalveolar carcinoma II - Bronchiolar - Small cysts - renal agenesis or dysgenesis, CDH, BPS III - Alveolar Duct - Microcystic - no association IV - Acinar/alveoli - Large Cysts - PPB

Question 250

Name THREE tumors are associated with DICER1

Answer 250

1. PPB 2. RMS 3. Ovarian sex-cord stromal tumors 4. cystic nephroma 5. thyroid nodules

Question 251

List THREE neonatal problems that could require ECMO

Answer 251

1. persistent pulmonary hypertension of the newborn 2. meconium aspiration syndrome 3. respiratory distress syndrome 4. sepsis/pneumonia 5. air leak syndrome 6. CDH

Question 252

Name ONE advantages and ONE disadvantages of VV ECMO

Answer 252

Advantages - normal lung blood flow, carotid not ligated, oxygenated lung blood to pulmonary vasculature and myocardium, pulsatile blood pressure, minimal embolism risk, little effect on preload and afterload Disadvantages - vein size prohibits insertion of larger catheters, no cardiac support, ongoing support more tenuous

Question 253

Name ONE advantages and ONE disadvantages of VA ECMO

Answer 253

Advantages - provides hemodynamic support, more straight forward, initiation not limited by vasculature Disadvantages - relative lung ischaemia, non-pulsatile blood flow, possible poor perfusion of coronaries and cerebral vessels, distal limb ischaemia, risk of lung overventilation

Question 254

Define Meconium Aspiration Syndrome

Answer 254

Respiratory distress in an infant born through meconium-stained amniotic fluid (MSAF) whose symptoms cannot otherwise be explained. The presence of meconium in the amniotic fluid is typically due to fetal distress around the time of birth including pre-eclampsia, maternal infections, and placental insufficiency among other conditions

Question 255

What is the pathophysiology of pulmonary hypertension in Meconium Aspiration Syndrorme?

Answer 255

When meconium is aspirated into the lungs, the sticky material can block the airways causing a problem in air reaching the alveolar capillary surface. In addition, meconium incites a severe chemical pneumonitis and inactivates surfactant production

Question 256

Describe the presentation of a baby with meconium aspiration syndrome.

Answer 256

Pts present with respiratory distress with marked tachypnea, cyanosis, intercostal and subxiphoid retractions, abdominal breathing, grunting, and nasal flaring. The chest appears barrel-shaped (increased AP diameter) due to overinflation

Question 257

Describe the Radiologic findings of a baby with meconium aspiration syndrome.

Answer 257

Initially streaky, linear densities, followed by hyperinflation, and alternating diffuse patchy densities with areas of expansion. May also see pneumothorax.

Question 258

List TWO ventilatory strategies for babies with Meconium Aspiration Syndrome

Answer 258

1. increase O2 2. increase PEEP to improve end-expiratory lung volume 3. increase PIP to recruit atelectatic lung 4. HFO to recruit atelectatic lung 5. ECLS.

Question 259

What is WAGR and on what mutation(s) is responsible for it?

Answer 259

Wilms, Aniridia, Genitourinary anomalies / Gonadoblastoma, Retardation Caused by mutation on chromosome 11p13 region leading to deletions of WT1 are responsible for Wilms tumour and PAX6 are responsible for the aniridia

Question 260

Name THREE ways in which children with WAGR are different from sporadic Wilms

Answer 260

1. Picked up earlier due to screening (like BWS) 2. Earlier age at diagnosis (<3 years) 3. More frequently bilateral 4. Higher risk of metachronous tumour 5. Higher rate ESRD after resection 6. Nephron-sparing approach preferred for operative mgt 7. Increased risk of gonadoblastoma d/t streak gonads

Question 261

What is Nephroblastomatosis and how does it relate to Wilms Tumor?

Answer 261

Nephroblastomatosis is diffuse overgrowth of rests which usually disappear by 36 weeks gestation. Nephrogenic rests may appear indistinguishable from Wilms on biopsy and have been noted to undergo malignant transformation

Question 262

List FIVE favourable sites for rhabdomyosarcoma

Answer 262

1. Orbit 2. Head and neck (not parameningeal) 3. Biliary 4. Paratesticular 5. Vaginal, vulvar, uterine

Question 263

List FIVE UNfavourable sites for rhabdomyosarcoma

Answer 263

1. Cranial/parameningeal 2. Extremities 3. Trunk 4. Retroperitoneal 5. Bladder, prostate, perineal

Question 264

What are the FOUR postoperative groups for Rhabdomyosarcoma?

Answer 264

1 - Localized disease, completely resected 2. Total gross resection with evidence of residual disease 3. Incomplete resection with gross residual disease 4. Metastatic disease present at onset.

Question 265

In what instances should lymph nodes be removed in rhabdomyosarcoma?

Answer 265

sample clinically positive nodes; SLNB trunk and extremity; and paratestticular SLND for boys >10yrs with clinically negative nodes or boys <10yrs with CT (+) nodes

Question 266

List FOUR histological subtypes of rhabdomyosarcoma

Answer 266

1. Embryonal (Fusion negative, good prognosis) 2. Alveolar (75% Fusion positive) 3. Anaplastic 4. Undifferentiated

Question 267

What are the principles of surgical management of a rhabdomyosarcoma?

Answer 267

Maintain form & function; wide and complete resection with confirmed adequate margin (0.5cm) as long as there is no functional impairment or disfigurement; debulking is no better than a biopsy (only possible exception is >50% debulking for pelvic/RP RMS). Place clips in tumor bed for XRT

Question 268

Discuss Pre-treatment RE-excision or Primary-site RE-excision (PRE) and Delayed Primary Excision (DPE) as it pertains to rhabdomyosarcoma

Answer 268

PRE: pts who undergo biopsy only, have initial non-oncologic resections, or have positive margins may undergo resection with negative margins should prior to chemotherapy and radiation if the mass is felt to be resectable without undue morbidity. DPE: evaluate response to therapy at 12 weeks; consider DPE in pts with residual disease after chemo if a complete resection can be achieved without significant morbidity

Question 269

Which Chemotherapy agents are used in the treatment of rhabdomyosarcoma?

Answer 269

VAC - vincristine, actinomycin-D, cyclophosphamide

Question 270

List THREE finding on history that are concerning for non-accidental trauma

Answer 270

1. Lack of or vague explanation for significant injury 2. Lack of reported trauma in a child with obvious injury 3. Discrepant histories over time / between persons interviewed 4. Explanation that is not consistent with the injury pattern or severity or child’s developmental capability 5. Delay in seeking care 6. History of multiple injuries, ER visits, hospitalizations

Question 271

List THREE bruising patterns that are consistent with non-accidental trauma

Answer 271

1. Any bruising in nonambulatory infant or child 2. Bruises on the face, cheeks, ears, neck, genitalia or hands in any aged child 3. Located on the buttocks and front trunk in early mobile and premobile children 4. Defensive in location (e.g. outer arm) 5. Clustered bruising or larger number of bruises present at the time of diagnosis 6. Patterned bruises that match an identifiable object (e.g. loop mark from a cord)

Question 272

Describe a bruising pattern consistent with ACCIDENTAL trauma

Answer 272

Ambulatory children, especially if located over bony prominences (including the forehead, nose, knees and shins, especially on the front of the body)

Question 273

Describe a fracture pattern consistent with ACCIDENTAL trauma

Answer 273

Fractures explained by a known history of severe trauma or underlying bone disorder…

Question 274

List THREE burn findings consistent with ACCIDENTAL trauma

Answer 274

1. Spill or flowing water injury (more rarely hot beverages or hot tap water) 2. Irregular margins 3. Varying burn depth 4. Asymmetric involvement of lower limbs, head, neck, trunk, face and upper body

Question 275

List THREE burn patterns that are consistent with non-accidental trauma

Answer 275

1. Immersion burns, especially with hot tap water 2. Clear upper limits 3. Uniform depth 4. Symmetrical involvement of lower limbs—especially with skin fold sparing ("zebra striping") or central sparing of buttocks ("doughnut pattern") or soles of the feet 5. Isolated scald to buttocks/perineum 6. Glove and stocking appearance

Question 276

List THREE fracture patterns that are consistent with non-accidental trauma

Answer 276

1. Fracture(s) in nonambulatory infants 2. Multiple fractures 3. Fractures of different ages 4. Infants and children with rib fractures, midshaft humerus, or femur fractures, or unusual fractures, including those of the scapula, classic metaphyseal lesions (CMLs) of the long bones, vertebrae, and sternum, 5. History of trauma does not explain the resultant fracture

Question 277

Name FOUR potential causes of cirrhosis in children

Answer 277

1. Biliary Atresia 2. Autoimmune Hepatitis 3. Cystic Fibrosis 4. alpha-1 antitrypsin deficiency 5. Wilson disease 6. Fatty Liver disease 7. Chronic viral hepatitis or hepatitis C 8. Primary sclerosing cholangitis 9. Alagille

Question 278

In general, how does portal hypertension develop?

Answer 278

Portal hypertension develops when there is resistance to portal blood flow and is aggravated by increased portal collateral blood flow. The resistance most often occurs within the liver (as is the case in cirrhosis), but it can also be prehepatic (eg, portal vein thrombosis) or posthepatic (eg, Budd-Chiari syndrome)

Question 279

Name TWO consequences of portal hypertension for which medical or surgical management may be required

Answer 279

1. Esophageal and gastric varices 2. Hemorrhoids 3. Ascites 4. Anemia, thrombocytopenia, leukopenia 5. Splenomegally 6. Gastrointestinal hemorrhage from portal hypertensive gastropathy, gastric antral vascular ectasia or isolated gastric, duodenal, peristomal or rectal varices.

Question 280

Detail the MEDICAL treatment for bleeding esophageal varices

Answer 280

IV fluids, PRBC, correct coagulopathy with IV Vitamin K, plasma, cryoprecipitate. IF cirrhosis, should also give antibiotics (cipro/cephalosporin), NG placement and gastric lavage, vasoactive drugs (e.g. somatostatin, octreotide or terlipressin for 5 days)

Question 281

Why is endoscopic variceal band ligation favoured over sclerotherapy in the management of varices in children?

Answer 281

Sclerotherapy has been associated with a higher complication rate than banding - esophageal stricture (16%), recurrent esophageal varices and bleeding before eradication (25%), esophageal dysmotility and gastroesophageal reflux, esophageal perforation and mediastinitis

Question 282

Describe Managment for a variceal bleed in order of escalating intervention

Answer 282

1. Resuscitation with IV Fluids and PRBCs and Antibiotic prophylaxis 2. Vasoactive drugs eg. octreotide 3. Varicele banding or sclerotherapy 4. Sengstaken-Blakemore (S-B) tube 5. TIPS

Question 283

Name THREE surgical options for the treatment of portal hypertension? Not endoscopic

Answer 283

1. meso-Rex bypass (MRB) 2. selective portosystemic shunts -distal splenorenal shunt (DSRS) 3. nonselective portosystemic shunts - meso-caval shunt (MCS) 4. esophagogastric devascularization (Sugiura’s procedure) 5. liver transplantation

Question 284

List FOUR factors that are used to determine the type of bypass/shunt/transplant procedure for portal hypertension

Answer 284

1. cause of portal hypertension 2. presence of pulmonary hypertension 3. presence of cirrhosis (compensated or uncompensated) 4. presence of intrinsic liver disease on liver biopsy 5. hepatic venous gradient (difference between free hepatic venous pressure and wedged hepatic vein pressure) 6. age of the patient 7. appearance of intrahepatic portal vein on venography

Question 285

THREE absolute contraindicaiton for a mesocaval shunt

Answer 285

1. hepatopulmonary syndrome 2. hepatic encephalopathy 3. uncompensated cirrhosis

Question 286

What are the THREE most common presentations of labial fusion

Answer 286

1. Asymptomatic - discovered by a parent or during routine medical examination 2. Urinary symptoms - dysuria, urinary frequency, refusal to urinate, or post-void dribbling 3. Vaginal discharge due to pooling of urine in the vulval vestibule or vagina

Question 287

Define labial fusion and decribe the causes

Answer 287

Labial fusion (or labial adhesion) is when the labia minora become sealed together, typically due to a lack of estrogen. It can also be caused by vaginitis. It is common in girls under 7 and rarely seen in girls after they begin puberty because of estrogen production.

Question 288

Describe the treatment of labial fusion

Answer 288

1. Usually separates naturally without treatment when estrogen level start to rise 2. Estrogen creams applied daily to the central line of the fusion for MAX 4-6 weeks. Continue to apply an emollient for a few months after separation. 3. Surgery - rarely needed

Question 289

What is a laryngotracheal cleft?

Answer 289

Laryngotracheal clefts are a posterior midline defect in separation between larynx/trachea and hypopharynx/esophagus; commonly occurs with EA/TEF

Question 290

What are the FOUR grades of laryngotracheal clefts?

Answer 290

I – defect of interarytenoid to above or at the level of vocal cords II – defect extends partially to cricoid plate III – defect extends completely through cricoid plate IV – defect extends into cervical or intrathoracic trachea (can extend all the way to carina)

Question 291

What is the management of laryngotracheal clefts?

Answer 291

I - usually don’t need intervention unless swallowing or respiratory issues - Tx medically for GERD or endoscopically if medication fails. II-IV - all require surgery – commonly do anterior approach through laryngofissure – allows for good exposure w/o risk to laryngeal innervation, if long defect (IV) may require posterolateral approach for 2 layer closure

Question 292

What is a ranula?

Answer 292

Ranulas are pseudocysts associated with the sublingual glands and submandibular ducts. They can be congenital, probably from improper drainage of sublingual glands, or acquired after oral trauma. They appear as blue, fluctuant swellings lateral to the midline in the lower mouth

Question 293

Describe the surgical management of a ranula

Answer 293

Ranulas lack a true cyst wall. As a result, complete excision of the pseudocyst is not necessary. Transoral removal of the sublingual gland is sufficient, even for those presenting as a neck mass. During excision, need to be careful about the Wharton duct and lingual nerve.

Question 294

List the THREE management options for patients with lymphatic malformation

Answer 294

1. Non-operative - Intralesional bleeding is treated with pain medication, rest, and prophylactic antibiotics. An infected LM is controlled with IV antibiotics 2. Sclerotherapy - first-line management for large or problematic macrocystic/combined LM. Involves aspiration of the cysts followed by injection of an inflammatory substance, which causes scarring of the cyst walls to each other 3. Resection - site dependant

Question 295

Altman Classification of sacrococcygeal teratoma

Answer 295

I – predominately external (~50%) II – external w/ intrapelvic extension (~35%) III – visible externally but predominately pelvic and abdominal (~9%) IV – entirely presacral (~10%)

Question 296

What are the THREE types of Biliary Atresia?

Answer 296

Type I: Atresia is restricted to the common bile duct. Type II: Atresia of the common hepatic duct. Type III: Atresia involves the most proximal part of the bile ducts (>95% of all cases).

Question 297

List THREE genetic and THREE environmental factors that may contribute to pyloric stenosis

Answer 297

Genetic: Caucasian, male, first born child, family history, Environmental: C-section, prematurity, primiparity, young maternal age, smoking

Question 298

What is the pathogenesis and management of a simple appendiceal mucocele?

Answer 298

Simple mucoceles are characterized by degenerative epithelial changes due to obstruction and distention, without any evidence of mucosal hyperplasia or neoplasia. Surgical resection of all localized appendiceal mucinous lesions for both diagnostic and therapeutic purposes. Because there are no reliable criteria to exclude malignant lesions, surgery should be pursued even for a benign-appearing appendiceal mucocele on imaging studies

Question 299

List THREE potential finding of the presentation of an appendiceal mucocele in children with cystic fibrosis?

Answer 299

1. Asymptomatic or non-specific symptoms 2. Acute or chronic right lower quadrant abdominal pain 3. Abdominal mass 4. Gl bleed associated with intussusception of mucocele 5. Intestinal obstruction from mass effect 6. GU symptoms due to obstruction of the right 7. Acute abdomen from mucocele rupture

Question 300

What is a preuricular pit and where does it terminate?

Answer 300

Small ectodermal inclusions located anterior to the helix and superior to the tragus of the ear (bilateral in 25 to 50%). 1% white, 5% black, 10% Asian children. Sinuses often short and end blindly (never have internal connection; end in thin strands that blend w/ periosteum of external auditory canal)

Question 301

What should be screened for in a child presenting with a pre-auricular cyst?

Answer 301

Patients should have formal audiologic evaluation because the risk of permanent hearing loss in children with preauricular pits or tags is five times that of the general population.

Question 302

What should be screened for in a child presenting with a pre-auricular cyst?

Answer 302

Patients should have formal audiologic evaluation because the risk of permanent hearing loss in children with preauricular pits or tags is five times that of the general population. Preauricular pits may also be the first indication of branchio-oto-renal (BOR) syndrome.

Question 303

List THREE complications of pre-auricular pits

Answer 303

1. Drainage 2. Infection 3. Recurrence

Question 304

Describe the surgical management of pre-auricular pits

Answer 304

Surgery involves excision of the pit; the squamous-lined cyst usually present beneath the skin and the cartilage at the root of the helix en bloc to avoid recurrence.

Question 305

What is scrofula?

Answer 305

Tuberculous lymphadenopathy in the cervical region

Question 306

What are the classic FOUR indications for intestinal transplant?

Answer 306

1. Impending or overt liver failure due to PN-induced liver injury 2. Thrombosis of 2+ central veins 3. Single episode of line-related fungemia, septic shock or acute respiratory distress syndrome 4. Frequent episodes of severe dehydration despite intravenous fluid supplementation in addition to PN

Question 307

What are the updated THREE indications for intestinal transplant? Hint: Having 2/3 of these indicate 98% likelihood of needing intestinal transplant

Answer 307

1. 2+ intensive care unit admissions 2. Persistent direct bilirubin of greater than 75 mmol/L despite optimized lipid strategies 3. Loss of 4+ central venous access sites

Question 308

Name FOUR small round blue cell tumors of childhood

Answer 308

1. Non-Hodgkins Lymphoma 2. Neuroblastoma 3. Rhabdomyosarcoma 4. Ewing's sarcoma 5. Wilm's Tumor 6. Hepatoblastoma

Question 309

List TWO reasons pediatric airways are more challenging than adults

Answer 309

1. Big ass head/occiput 2. Big ass tongue 3. Big ass floppy epiglottis 4. larynx higher + more anterior

Question 310

Describe the modified mallampati score

Answer 310

Class I – soft palate, uvula, pillars visible Class II – soft palate and major part of uvula visible Class III – soft palate and only base of uvula visible Class IV – only hard palate visible

Question 311

How do you determine endotracheal tube size in children?

Answer 311

Uncuffed tube - (Age/4) + 4 Cuffed tube - (Age/4) + 3 -uncuffed tubes for kids <8 to avoid subglottic stenosis

Question 312

In which children is cricothyroidotomy contraindicated and why?

Answer 312

cricothyroidotomy contraindicated <8-12 due to potential for significant airway damage

Question 313

What is malignant hyperthermia, what are the signs and symptoms, and how is it treated?

Answer 313

MH is an autosomnal dominant with incomplete pentrance triggered by succinylcholine and inhalational anaesthetics. It causes an uncontrolled release of Ca2+ sustained muscle contraction/rigidity The earliest sign is rapid rise in ETCO2. Other signs are hyperthermia, respiratory+metabolic acidosis, rhabdomyolysis, tachycardia Treatment = stop the agent, IV dantrolene, supportive care

Question 314

In terms of respiratory physiology, decribe Inspiration and Expiration

Answer 314

Inspiration: active process; - phrenic nerve activates the diaphragm which flattens - external intercostal muscles contract which lifts ribs and pulls chest wall out - increased volume in chest causes decreased intra-alveolar pressure and air enters the lungs Expiration: passive process - relaxation of the diaphragm and intercostal muscles - lung recoils due to elastic connective tissue and alveolar surface tension - intra-alveolar pressure rises and air leaves the lungs

Question 315

How does gas exchange occur?

Answer 315

- CO2 = product of metabolism - exchange of CO2 between alveoli and blood occurs by simple diffusion - oxygen transported in the blood bound to Hb - oxyhemoglobin dissociation affected by pH, temperature, CO2

Question 316

List FOUR causes of hypoxemia

Answer 316

1. Global hypoventilation - newborns have apneas due to immature nervous system. Treat with stimulation, positive pressure ventilation and increased FiO2. 2. Diffusion disequilibrium - thickening of the interface b/w alveoli and bloodstream ie. pulmonary edema, interstitial diseases of the lung 3. Pulmonary shunt - failed ventilation to perfused regions ie. atelectasis, aspiration, pneumonia, foreign bodies 4. Ventilation/perfusion mismatch - less blood flow to superior segments of the lung and dead space ventilation ie. pulmonary embolus

Question 317

What are the FIVE potential conventional ventilator modes?

Answer 317

1. Controlled Mandatory Ventilation (CMV) - ventilator initiates and delivers set of mandatory breaths at equal intervals 2. Intermittent Mandatory Ventilation (IMV) - ventilator delivers set number of breaths, but breaths can be taken in between but they are not supported by the ventilator and therefore asynchrony can result 3. Assist Control (AC) - all spontaneous breaths that exceed the trigger sensitivity result in a delivery of a fully assisted mechanical breath. If the patient fails to breath, ventilator will trigger a mandatory breath. 4. Synchronized Intermittent Mandatory Ventilation (SIMV) - ventilator synchronizes mandatory breaths with patient effort. Patient can breathe above the set rate but not below. 5. Pressure Support Ventilation - decreases the work of breathing by providing flow during inspiration for patient triggered breaths. Comfortable mode that is good for weaning.

Question 318

Which THREE elements determine oxygen delivery?

Answer 318

1. Cardiac output = HR x SV 2. Hemoglobin content 3. Oxygen saturation

Question 319

How is malnourishment defined in children?

Answer 319

Malnutrition definitions: • 1-2 SD < mean for age → mildly malnourished • 2-3 SD - moderate • >3 SD - severe

Question 320

What are the normal growth parameters for preterm babies, term babies, and infants? What about 3-6 months? When should babies double and triple?

Answer 320

``` Preterm infants (24-30 wks) = 12-25g/day Preterm infants (30-40wks) = 25-35g/day Term infant – 3 mos = 25-30g/day 3-6 mos = 12-20g/day Baby should double birth wt by 4-5 mos, triple birth wt by 1 year ```

Question 321

What is the body compostition (Water, Protein, Fat) of preterm, full term, and 1 year olds?

Answer 321

Prem baby = 80% water, 11% protein, 8% fat Full term baby = 75% water, 11% fat 1-year old = 60% water, 15% protein, 20% fat

Question 322

How much energy comes from 1 gram of: Fat Protein Glucose

Answer 322

1 gram fat = 9kcal energy 1 gram protein = 4 kcal energy 1 gram glucose = 4 kcal energy

Question 323

How do you calculate the Nutritional needs of: Newborn baby Older child

Answer 323

Newborn baby: 100-135 kcal/kg/day 2.5-3 g/kg of protein/day 2-3 g/kg of fat/day Older child: 30% calories from fat 45-60% calories from carb 10-20% calories from protein

Question 324

List FOUR ways to assess the nutritional status of a child

Answer 324

1. physical appearance (muscle wasting, edema, ascites) 2. anthropometric measures (wt and ht velocity, mid upper arm circumference, triceps skinfold) 3. screening tools 4. albumin (long ½ life so not great acutely), prealbumin 5. transferrin 6. retinol binding protein 7. CBC (lymphopenia)

Question 325

You are called to ward at 10 pm because the charge nurse reminds you that a child requires TPN. Your resident forgot to put in a dietician referral when you asked him to at 7:30 am that morning. Inexplicably, despite the fact that this is a non-emergency and you could just wait until the morning to call the dietician at your tertiarty care pediatric hospital, you feel compelled to calculate the TPN orders by yourself. What parameters would you used to initiate TPN and what are the caloric goals for preterm infants, term infants, and children?

Answer 325

``` Glucose = 4-6 mg/kg/minute Fat emulsion = 1 g/kg/day if <2kg, 2g/kg/day if >2kg Protein = 2.5-3 g/day/day Calcium 1-5 mEq/kg/day Phosphorus 1.3-2 mM/kg/day Magnesium 0.5-1 mEq/kg/day ``` Preterm infant = 90-110 kcal/kg/day Term infant = 80-100 kcal/kg/day Child = 25-30 kcal/kg/day

Question 326

What fats are in intralipid, omegavan, and SMOF?

Answer 326

Intralipid - soybean oil Omegavan - fish oil emulsion SMOF - 30% soybean oil, 30% MCT, 25% olive oil, 15% fish oil

Question 327

Why are most varicoceles left sided?

Answer 327

90% on left side because the left spermatic vein enters the left renal vein at a 90° angle, whereas the right spermatic vein drains at a more obtuse angle directly into the IVC

Question 328

What is a varicocele and what is the difference between a primary and secondary varicocele?

Answer 328

A varicocele is a collection of dilated and tortuous veins in the pampiniform plexus surrounding the spermatic cord in the scrotum. The cause of primary varicoceles is unknown (increased venous pressure and incompetent valves?); The cause of secondary varicoceles is obstruction of the IVC.

Question 329

10-25% of all adolescent males and up to 1/3 of males examined at an infertility clinic have a varicocele BUT only 10-15% percent of males with varicoceles have fertility problems Does the grade of varicocele correlate with serum analysis or fertility?

Answer 329

NOPE!

Question 330

Which findings on exam would prompt you to consider a secondary varicocele? What are the THREE most common causes of a secondary varicocele?

Answer 330

If the varicocele persists in the supine position, has acute onset, or is right-sided, bilateral, or occurs in a pre-pubertal male, a secondary cause must be ruled out. The THREE causes are: 1. Abdominal mass—retroperitoneal tumours (Wilms, other renal tumours) or lymphadenopathy) 2. IVC thrombus 3. Right renal vein thrombosis with clot propagation down the IVC

Question 331

What are the treatment options for varicocele?

Answer 331

1. Observation 2. IR testicular vein ligation 3. Surgical testicular vein ligation 4.

Question 332

What is the difference between Extravaginal and Intravaginal Torsion of the testis?

Answer 332

Extravaginal torsion: perinatal event during testicular descent before tunica vaginalis and cord structures form; torsion proximal to tunica vaginalis investment. Intravaginal torsion: more common & occurs in adolescence; cord twists within the tunica vaginalis.

Question 333

What FOUR conditions (congenital or otherwise) predispose a child to testicular torsion?

Answer 333

1. bell clapper deformity (failure of normal posterior anchoring of gubernaculum, epididymis, and testis) 2. long mesorchium 3. cryptorchidism (10x more frequent) 4. larger testicle (normal variation or tumour)

Question 334

What are the classic finding on presentation of a testicular torsion?

Answer 334

Sudden onset, unilateral scrotal or lower abdominal pain without precipitating event +/- nausea/vomiting; intermittent testicular pain may represent intermittent torsion; on exam, high-riding testis with transverse lie of affected testis (anterior position of epididymis), loss of cremasteric reflex, pain relief with scrotal elevation, +/- edema and erythema

Question 335

What is the classic presentation and management of a torsion of of the appendix testis/epididymis

Answer 335

Can present with pain very similar to testicular torsion; however, pain is usually more localized; there may be a blue dot sign on scrotum (visual transmittance of the appendage through the skin); age generally younger (prepubertal boys age 7-10); Doppler U/S will differentiate from testicular torsion; treat with NSAIDS, elevation, warm compresses, rest.

Question 336

Describe the presentation of Epididymitis and Orchitis. What is the treatment?

Answer 336

Epididymitis – pain and swelling, could have urethral discharge. Usually d/t reflux of urine or UTI, can be STDs in older kids; urine culture will confirm source, Tx = NSAIDs, compresses, Abx Orchitis – unilateral pain and swelling, more gradual onset, usually viral source (mumps aka paramyxovirus), TB, syphilis, mono, varicella; Tx – pain control, compresses, Abx if bacterial source

Question 337

Describe the embryological descent of the testes

Answer 337

Testes begin developing at 3 weeks —> labioscrotal swellings fuse to become scrotum at 9 weeks —> processus vaginalis develops at 13 weeks —> epididymis precedes testes into processus vaginalis, then both descend into scrotum and fuse with posterior layers of scrotum between 26-36 weeks —> processus vaginalis closes at 37-40 weeks.

Question 338

What circumstances would prompt orchiectomy in a child presenting with testicular torsion?

Answer 338

If symptoms >10 hours, absence of flow on Doppler, and lack of bleeding when incised = orchiectomy; why not just leave the testicle in place? Concern for auto-immunization >10 years old

Question 339

What is Autoimmunization as it relates to testicular torsion?

Answer 339

Development of anti-sperm antibodies, believed to occur when there is disruption of the hemato-testicular barrier leading to development of antibodies against the native spermatogonia; presence of an ischemic or necrotic testicle after torsion is one potential cause of disruption of this blood-testis barrier

Question 340

What are the TWO most common locations of osteosarcoma and how do they typically present?

Answer 340

Most common locations: distal femur + proximal tibia Presentation: often present with a sport injury; pain at rest and with movement, enlarging mass at the area of the injury; **a mass that does not resolve w/in a few weeks of the injury needs to be investigated** 20% present with mets

Question 341

Which patients with osteosarcoma lung metastasis require surgery and what are the surgical considerations?

Answer 341

- Patients with synchronous pulmonary mets should undergo resection of the mets after treatment of the primary tumour; complete resection of mets improves OS - If >10 lung mets = no resection, MIS discouraged (need to palpate lungs) - 20-25% relapse in the lungs Surgical considerations - posterolateral thoracotomy (muscle-sparing), single lung ventilation, if lesion <1cm from primary bronchus - lobectomy rather than wedge

Question 342

List TWO findings that signify poor prognosis in osteosarcoma

Answer 342

1. <99% necorosis of tumor | 2. metachronous metastases w/in 18 mos

Question 343

FOUR most common locations of Ewing's Sarcoma

Answer 343

1. femur 2. humerus 3. pelvis 4. rib

Question 344

Detail the management of Ewings

Answer 344

- neoadjuvant chemo then definitive resection OR radiation - less defined role for metastectomy than in osteosarcoma, can consider after completion of chemotherapy - total lung radiation if metastases not resectable

Question 345

Detail the potential presentations of simple ovarian cysts

Answer 345

Presentation: • Neonates (up to 90% neonates) and adolescents (up to 20%). • Associations with precocious puberty, McCune-Albright syndrome, or severe hypothyroidism. • Menstrual irregularities, pelvic pain, urinary frequency; may hemorrhage, rupture, or undergo torsion and cause acute pain, n/v, fever, leukocytosis.

Question 346

Detail the presentation, tumor markers and management of ovarian teratoma

Answer 346

Presentation: torsion <10%; Bilateral in 10%; Predominantly cystic but may have solid components on imaging; calcifications (70%), teeth, hair possible. Markers: Benign (mature): no yolk sac component, normal AFP. Malignant (immature): yolk sac component, elevated AFP. Management: ovarian sparing resection unless AFP is elevated. Chemo is PEB. Follow-up with U/S Q 6mos x 2 then yearly until adulthood.

Question 347

Detail the presentation, tumor markers and management of dysgerminoma

Answer 347

Presentation: Mostly in dysgenic gonads (streak gonads); >1/3 germ cell tumours; Bilateral in 15-30%; Solid on imaging; May rupture at presentation. Markers: Elevated CA-125, βHCG, LDH. Management: oophorectomy. Chemo is PEB.

Question 348

Detail the presentation, tumor markers and management of ovarian yolk sac tumors

Answer 348

Presentation: Solid and cystic on imaging. Markers: Elevated AFP, βHCG, LDH. Management: oophorectomy. Chemo is PEB.

Question 349

Detail the presentation, tumor markers and management of embryonal cell ovarian tumors and choriocarcinoma.

Answer 349

Presentation: precocious puberty; Solid on imaging. Markers: Elevated AFP, βHCG. Management: oophorectomy. Chemo is PEB.

Question 350

Detail the presentation, tumor markers and management of gonadoblastoma

Answer 350

Presentation: primary amenorrhea or virilization; Streak or dysgeneic gonad, intra-abdominal “testicle” in phenotypic female with XY. Markers: none elevated Management: oophorectomy. Chemo is PEB.

Question 351

Name FIVE types of ovarian germ cell tumors

Answer 351

1. Teratoma 2. Dysgerminoma 3. Yolk Sac 4. Embyronal Cell 5. Choriocarcinoma 6. Gonadoblastoma 7. Mixed Germ Cell

Question 352

Name the TWO Sex-cord stromal ovarian tumors

Answer 352

1. Granulosa-Stromal | 2. Sertoli- Leydig

Question 353

Name TWO epithelial ovarian tumors

Answer 353

1. Mucinous cystadenocarcinoma | 2. Serous cystadenocarcinoma

Question 354

Granulosa-Stromal ovarian tumors often present with isosexual precocious puberty with early vaginal bleeding as a hallmark symptom. What are the tumor markers of interest and what is the usual management of these tumors

Answer 354

Markers: Elevated inhibin A & B. ``` Management: • Oophorectomy • Platinum-based chemotherapy for any tumour rupture or tumour outside ovaries. • Chemo = PEB (carboplatin, etoposide, bleomycin). ```

Question 355

Tumor markers for : 1. Mucinous cystadenocarcinoma 2. Serous cystadenocarcinoma

Answer 355

1. Mucinous cystadenocarcinoma - CA 125, CA 19-9 | 2. Serous cystadenocarcinoma - CA 125

Question 356

Tumor Markers for sertoli-leydig tumors

Answer 356

Elevated CA 125, AFP, +/- LDH.

Question 357

What are the FOUR stages of ovarian germ cell tumors?

Answer 357

I - limited to ovary II - microscopic residual tumor III - lymph node involvement, gross residual disease or biopsy only; contiguous visceral involvement, peritoneal evaluation positive (NB gliomatosis peritonei does NOT count as peritoneal positive) IV- distant metastases including liver.

Question 358

List SIX things on the differential diagnosis of a cystic mass on prenatal ultrasound

Answer 358

1. Ovarian Cyst 2. Hydrocolpos (cloaca or UG sinus) 3. Bladder outlet obstruction 4. Ureteropelvic junction (UPJ) obstruction 5. Ureterovesicular junction (UVJ) obstruction 6. Posterior urethral valves (PUV) 7. Ureterocele 8. Urethral stenosis 9. Duplicated collecting systems 10. Multi-cystic kidney disease 11. Hepatic cyst 12. Choledochal cyst 13. Splenic cyst 14. Pancreatic cyst 15. Enteric duplication cyst 16. Mesenteric cysts

Question 359

How frequently does ovarian cyst torsion occur prenatally?

Answer 359

40-50% ovarian cysts will undergo torsion prenatally; U/S finding of a cyst with a fluid/fluid level (debris from the ischemic ovary will become dependent); ovary usually has multiple twists at the level of the fallopian tube and may even auto-amputate from the adnexal structures.

Question 360

Describe the pathophysiology of neonatal ovarian cysts

Answer 360

Maternal hormones (estrogen & gonadotropin) cross the placenta and cause maturation of the follicle, which can lead to the development of a follicular cyst; prenatal cysts generally regress postnatally after cessation of exposure to maternal hormones. • More common in pregnancies complicated by maternal DM, toxemia, and Rh isoimmunization.

Question 361

Detail the postnatal management of neonatal ovarian cysts

Answer 361

- If ovarian cyst <5cm on postnatal U/S, continue to follow. - If not resolved by 6 months and >5cm, drain or resect cyst via laparoscopy to reduce the risk of torsion - If solid component, further imaging, tumour markers, +/- resection; if prenatal torsion has occurred, most will auto-amputate and absorb;

Question 362

What are the TWO subtypes of Hepatocellular carcinoma

Answer 362

1. Classical | 2. Fibrolamellar

Question 363

Define Fibrolamellar HCC

Answer 363

Fibrolamellar HCC is a distinct histologically defined subtype of HCC commonly arising in the setting of normal hepatic parenchyma without evidence of cirrhosis Adults with fibrolamellar have better prognosis, but this isn’t the case for children.

Question 364

List the TWO Benign Mesenchymal Liver Tumors

Answer 364

1. Hemangioma | 2. Hamartoma

Question 365

List THREE Benign Epithelial Liver Tumors

Answer 365

1. Focal Nodular Hyperplasia 2. Hepatic Adenoma 3. Inflammatory Myofibroblastic Tumor

Question 366

Describe the presentation and treatment of Hepatic Hemangiomas

Answer 366

Can be Focal vs. Multifocal vs. Diffuse morphologic classes; ½ also have cutaneous hemangiomas and can be identified on screening ultrasound. **May Present with hypothyroid state d/t tumor expression of iodothyronine deiodinase which inactivates Thyroid hormone. Treatment - usually observation. IF symptomatic then propranolol +/- vincristine +/- angioembolization +/- transplant; ACS may mandate decompressive laparotomy

Question 367

Describe the presentation and treatment of Hepatic Hamartoma

Answer 367

Usually present before age 2 or noted on prenatal ultrasound; they collect fluid and form large cyst; Symptoms range from abdominal pain to ACS. Treatment - can involute and therefore can observe if asymptomatic; Resect if symptomatic, but also okay to enucleate or marsupialized largest cysts if unresectable; there is potential for malignant transformation so resection preferred.

Question 368

Describe the presentation and treatment of Focal Nodular Hyperplasia

Answer 368

Presentation usually incidental; symptoms include abdominal pain from mass effect; Classic central stellate scar. Treatment - can observe if asymptomatic, US surveillance in younger kids d/t risk of rapidly growing FNH, resect if symptoms of mass effect

Question 369

Describe the presentation and treatment of Inflammatory Myofibroblastic Tumor

Answer 369

Presentation - Benign but can be locally aggressive; fever and abdo pain in 40%. Treatment - generally surgical resection, they may need transplant.

Question 370

What is the bimodal age distribution of Hepatocellular Carcinoma in children. Is it more predominant in males or females

Answer 370

Bimodal 4-5 and 12-14 yrs age M>F 50% of kids have pre-existing cirrhosis

Question 371

How is Hepatocellular carcinoma staged in children?

Answer 371

Using PRETEXT staging.

Question 372

Describe the PRETEXT Classification

Answer 372

PRETEXT I - Three contiguous sections free of tumor PRETEXT II - Two contiguous sections free of tumor (or caudate) PRETEXT III - One contiguous sections free of tumor PRETEXT IV - Zero contiguous sections free of tumor

Question 373

What is the management of PRETEXT I Hepatoblastoma?

Answer 373

Tumour involves only right posterior or left lateral sections Management: Upfront resection with right or left segmentectomy or hemi-hepatectomy if ≥1cm clear margin from middle hepatic vein & portal bifurcation and if annotation factors (-)

Question 374

What is the management of PRETEXT II Hepatoblastoma?

Answer 374

Generally involve only left or right lobes [except multi-focal & involves caudate] Management: -Upfront resection with right or left hemi-hepatectomy if ≥1cm clear margin from middle hepatic vein & portal bifurcation and if annotation factors (-)

Question 375

What is the management of PRETEXT III Hepatoblastoma?

Answer 375

Management: Biopsy, Neo-adjuvant cisplatin based chemo x 2-6 cycles; Repeat CT scan Q2 cycles and examine POST-TEXT; upfront transplant referral. After 2 cycles chemo: • If POST-TEXT I or II —> resect • If POST-TEXT III —> continue chemo to 4 cycles After 4 cycles chemo: • If POST-TEXT III annotation factors (-) —> resect • If POST-TEXT III annotation factors (+) —> refer to liver transplantation centre for consideration of complex resection or transplant Pulmonary metastases: • Pts with mets will always be treated like PRETEXT III • Resect pulmonary mets if primary tumour has been removed and all pulmonary disease can be resected

Question 376

Which histology indicates a better prognosis in hepatoblastoma?

Answer 376

Fetal

Question 377

What is the management of PRETEXT III Hepatoblastoma?

Answer 377

Management: Biopsy; Neo-adjuvant cisplatin based chemo and early referral to liver transplant centre Indications for transplant: Unresectable by conventional approach (proximity/involvement of major vessels); Bilateral lobar disease

Question 378

List FOUR things on the differential diagnosis for an anterior mediastinal lesion

Answer 378

1. Lymphoma (most common malignancy of mediastinum = 50% tumours) 2. Thymic hyperplasia (can be physiologic; observe or Rx steroids if resp sx) 3. Thymic cyst 4. Thymoma & carcinoma (rare in children, 40% paraneoplastic syndrome, resect +/- RT) 5. Teratomas & other germ cell tumours (<2y generally benign, >2y often malignant) 6. Thyroid goiter

Question 379

List FOUR things on the differential diagnosis for an middle mediastinal lesion

Answer 379

1. Lymphoma (most common malignancy of mediastinum = 50% tumours) 2. Vascular tumours 3. Cardiac tumours 4. Bronchogenic cysts 5. Metastases

Question 380

List FOUR things on the differential diagnosis for an posterior mediastinal lesion

Answer 380

1. Neuroblastoma 2. Neurogenic (neurofibromas (NF1), Schwannomas, sympathetic ganglion tumours) 3. Neuroenteric cysts (GI epithelium but communicate with CNS) 4. Bronchogenic cysts 5. Esophageal duplication cysts

Question 381

Name THREE common symptoms of Anterior Mediastinal lesions

Answer 381

1. Dyspnea + respiratory distress 2. Retrosternal chest pain 3. Orthopnea 4. SVC syndrome

Question 382

Name THREE common symptoms of Posterior Mediastinal lesions

Answer 382

1. Dysphagia 2. Horner’s syndrome 3. Paraplegia 4. Other neurologic findings

Question 383

List THREE common sites of Teratomas

Answer 383

1. Ovarian 2. Retroperitoneal 3. Mediastinal 4. Sacrococcygeal

Question 384

What is a Chamberlain Procedure?

Answer 384

awake anterior or parasternal mediastinotomy used to obtain | tissue from an anterior mediastinal mass when pt not safe for GA

Question 385

In which patients with anterior mediastinal masses is it considered dangerous to do a general anesthetic.

Answer 385

if >50% compression of trachea or severe postural | symptoms (orthopnea), cannot get a GA and intubation

Question 386

What is your differential diagnosis for a patient born with hypospadias and bilateral undescended testes?

Answer 386

46 XY DSD: - testosterone deficiency - 5 alpha reductase deficiency - incomplete androgen insensitivity

Question 387

# Define the following terms: 1. Undescended testis or congenital cryptorchidism 2. Retractile testis 3. Ectopic testis 4. Absent, vanishing or severely dysgenetic testis 5. Ascended testis or acquired cryptorchidism 6. Entrapped testis

Answer 387

1. Undescended testis or congenital cryptorchidism -testicular descent is arrested during passage from its retroperitoneal origin into the scrotum 2. Retractile testis - contraction of the cremaster muscle pulls the normally descended testis out of the scrotum 3. Ectopic testis - the testis is visible and/or palpable in a location that is out of its normal path of descent 4. Absent, vanishing or severely dysgenetic testis - no visible or palpable testis - often as a result of in utero event 5. Ascended testis or acquired cryptorchidism - though currently located above the scrotum, the testis was previously confirmed to be in the scrotum 6. Entrapped testis - testicular descent into the scrotum is blocked by scar tissue after a prior operation

Question 388

How does hypospadias develop?

Answer 388

Hypospadias is associated with: a) failure of the urethral meatus to be located on the tip of the glans, and failed ventral fusion of the prepuce, causing a ‘dorsal hood’ b) inadequate growth of the ventral shaft around the urethra, leading to bend, known as chordae

Question 389

What are the indications/reasons for orchidopexy to be performed? Remember: HIS TESTIS

Answer 389

Hernia - 60-90% have patent processus; 2.5% present with hernia Injury - increased risk of injury with testical in the groin Symmetry - cosmesis Tumor - increased risk of malignancy in cryptorchid testes (highest if intra-abdominal) Epididymo-orchitis - higher risk d/t repeated trauma Sterility - need cool location to make sperm Torsion Intersexuality - DSD pSycholical reasons

Question 390

Classic cell on histology of Hodgkin Lymphoma

Answer 390

Presence of multi-nucleated giant cells -Reed-Sternberg

Question 391

FOUR complications of TEF repair

Answer 391

1. Leak 2. Stricture — 15-60%, wide range bc no consistent definition 3. Recurrent fistula — 5-15%; rare if no leak; may consider endoscopic treatment (Bugbee cautery & Deflux) 4. Missed fistula — think about this if early and no leak 5. RLN injury 6. Tracheal Injury

Question 392

Differential diagnosis for post-op "spells" following EA TEF repair. List FIVE and management

Answer 392

1. Stricture — pneumatic dilation. 2. Recurrent fistula — endoscopic treatment or operative ligation. 3. Missed fistula — operative ligation; consider whether can be approached from neck if high. 4. Tracheal diverticulum — consult ENT. 5. Laryngeal nerve palsy — consult ENT; usually watchful waiting with NG/gastrostomy feeds. 6. Vascular ring or aberrant subclavian artery — consult cardiac surgery; may consider ligation. 7. Tracheomalacia — consult ENT; may ultimately require aortopexy. 8. GERD — PPI +/- NJ feeds; may ultimately require Nissen.

Question 393

What is the study of choice for the diagnosis of a missed or recurrent fistula?

Answer 393

Prone pull-back esophagram.

Question 394

What are the TWO classification types of Hodgkin Lymphoma?

Answer 394

1. Classic HL | 2. Nodular lymphocyte-predominant HL (NLPHL)

Question 395

What are the FOUR subclassifications of Classic Hodgkin Lymphoma. Which is the most common?

Answer 395

1. Nodular Sclerosis (most common) 2. Mixed Cellularity 3. Lymphocyte rich 4. Lymphocyte depleted

Question 396

Which THREE findings are needed to diagnose Achalasia on manometry?

Answer 396

1. Absence of peristalsis 2. High resting pressure of LES 3. Failure of receptive relaxation of the LES

Question 397

What are the THREE subtypes of Achalasia?

Answer 397

* Type I (classic) with minimal contractility in the esophageal body. * Type II with intermittent periods of pan-esophageal pressurization. (70% of children, best response to Mgt) * Type III (spastic) with premature or spastic distal esophageal contractions.

Question 398

What are the finding on a plain CXR of Achalasia?

Answer 398

- convex opacity overlapping right mediastinum (dilated esophagus) - air-fluid level in thoracic esophagus (retained secretions/food) - small/absent gastric bubble - anterior displacement of trachea on lateral - patchy infiltrates

Question 399

Describe the surgical management of Achalasia (Not POEM)

Answer 399

myotomy at the LES (+/- partial fundoplication, typically a Toupet) or esophagectomy for end-stage disease (“megaesophagus”) Recall that a sufficient myotomy should extends from 4-8cm above the GEJ to 2-3cm below the GEJ.

Question 400

Pancreatic Trauma Grades

Answer 400

I - minor contusion or laceration with no duct injury II - major contusion or laceration with no duct injury III - distal transection or parenchymal injury with duct injury IV - proximal transection or parenchymal injury involving ampulla V - massive disruption of the pancreatic head

Question 401

List FOUR advantages of operative intervention for pancreatic trauma

Answer 401

1. lower rate of pseudocyst formation 2. shorter time to oral feeds and less need for parenteral nutrition 3. shorter length of stay 4. evaluation and possible management of associated injuries

Question 402

List FIVE potential causes of distal intestinal obstruction in a neonate

Answer 402

1. Hirschsprung 2. meconium ileus 3. meconium plug 4. ileal/colonic atresia 5. ileal/colonic web 6. small left colon syndrome 7. intestinal neuronal dysplasia 8. internal hernia or congenital/ Ladd’s band 9. inguinal hernia

Question 403

List FOUR possible Brain injuries caused by traumatic birth

Answer 403

1. cerebral hemorrhage 2. IVH 3. SAH 4. SDH 5. cephalohematoma 6. hypoxic ischemic encephalopathy (HIE)

Question 404

List FIVE anatomical areas that could be affected by birth trauma and give one example of each

Answer 404

1. Brain and soft tissue injury - SAH 2. Peripheral nerve injury - Erb’s palsy 3. Cranial nerve/spinal cord injury - facial nerve paralysis 4. Bone injury: shoulder dystocia-->clavicle # 5. Intra-abdominal injury - liver

Question 405

What are the THREE ultrasound criteria required to diagnose pyloric stenosis?

Answer 405

1. Pyloric muscle thickness >3mm on transverse image 2. Pyloric muscle length >14 on longitudinal image 3. Hyperactive peristalsis against an obstruction at the pylorus (place patient right side down and observe that pylorus doesn’t open and fluid doesn’t pass through)

Question 406

What is the mechanism of paradoxic aciduria in pyloric stenosis?

Answer 406

The body’s primary homeostatic set point is volume, rather than potassium or pH. In the presence of severe dehydration, aldosterone levels are elevated in order to retain sodium. The high aldosterone level stimulates the sodium/potassium and sodium/hydrogen exchange pumps in the distal renal tubule, retaining sodium and secreting potassium and hydrogen in the urine. This further exacerbates the serum alkalosis and hypokalemia.

Question 407

Define Toxic Shock Syndrome

Answer 407

Staphylococcal toxic shock syndrome (TSS) rapid onset of fever, rash, hypotension, and multiorgan system involvement.

Question 408

Describe the criteria for diagnosis of Toxic Shock Syndrome

Answer 408

fever <38.9, hypotension, rash, desquamation, multi-system involvement, lab (culture, serologic tests for alternative pathogens).

Question 409

Describe the management of Toxic Shock Syndrome

Answer 409

Management includes treatment of shock, surgical debridement (if warranted), and antibiotic therapy. Surgical wounds may not appear to be infected because of diminished inflammatory response; nonetheless, wound exploration is indicated for TSS pts if recent surgery —> remove all packing from wound +/- vaginal canal (tampon, contraceptive sponge, or IUD)

Question 410

How does Meconium Plug Syndrome typically present and what is the diagnostic and therapeutic strategy?

Answer 410

Typically presents in term neonate whose abdomen is soft but distended and has not passed meconium; AXR shows distended loops; contrast enema is diagnostic and therapeutic—filling defect at TZ with no microcolon of disuse; plug characteristically tenacious, forest green with a pale head; pts should improve following enema when plug is expelled.

Question 411

Describe the typical findings of a contrast enema in a neonate with meconium ileus.

Answer 411

microcolon of disuse +/- meconium pellets in right colon + TI; distal 15–30cm TI filled with inspissated meconium pellets, which are adherent to the bowel wall; proximal ileum fills with thick, putty-like meconium and dilates.

Question 412

Describe the typical findings of an xray in a neonate with meconium ileus

Answer 412

‘soap bubble’ / ‘ground glass’ appearance in RLQ (air mixing w thick meconium) with no air fluid levels (meconium doesn’t succumb to gravity)

Question 413

What genetic anomaly is associated Meconium Ileus?

Answer 413

occurs in 10-20% infants with CF; due to abnormal meconium (mucoviscidosis) secondary to deficient pancreatic and intestinal secretions and abnormal concentration of the meconium within the duodenum and proximal jejunum. 90% of Meconium ileus is related to cystic fibrosis.

Question 414

List FOUR complications of Meconium Ileus

Answer 414

1. volvulus 2. bowel perforation 3. atresia 4. cystic meconium peritonitis

Question 415

List FOUR causes of constipation after repair for anorectal malformation

Answer 415

1. stricture 2. poor baseline functional prognosis. 3. misplaces anorectoplasty 4. sacral anomaly 5. idiopathic- diet related 6. Rectal prolapse

Question 416

How is the sacral index calculated for anorectal malformation and what is the prognosis based on the result?

Answer 416

BC/AB | >0.7=good // 0.4-0.7=moderate // <0.4=poor

Question 417

List FOUR causes of constipation after pull-through for Hirschsprung Disease

Answer 417

1. Mechanical Obstruction - twist in pull-through, soave cuff, duhamen spur 2. Persistent or acquired aganglionosis, hypoganglionosis, or TZ pull-through 3. Internal sphincter achalasia 4. Motility disorder 5. Functional megacolon

Question 418

What is the difference between GER and GERD

Answer 418

GER is defined as the passage of content from the stomach retrograde into the esophagus caused by inappropriate transient LES relaxation. It is considered a normal and physiologic process in a newborn — 70% infants spit up daily, 25% QID. Frequency of GER increases over the first 4 mos then eventually tapers over the first year of life. GERD is defined as the presence of GER that is associated with troublesome symptoms/complications.

Question 419

What is Sandifer Syndrome?

Answer 419

spasmodic torsional dystonia with arching of the back and rigid posturing involving the neck and back.

Question 420

LIst FOUR potential modalities to work up GERD

Answer 420

1. 24 hour pH monitoring 2. Combined impedance/pH monitoring - capture acidic + non-acidic reflux 3. EGD - can see reflux esophagitis, biopsy can r/o eosinophilic esophagitis 4. Manometry - r/o achalasia and motility disorders 5. UGI series - r/o anatomical problems (eg. hiatal hernia) 6. Gastric emptying scan (nuclear scintigraphy) - r/o gastroparesis

Question 421

Why is 24 hour pH monitoring a useful test for GERD?

Answer 421

Measure esophageal acid exposure; degree of acid exposure does not necessarily correlate with the severity of symptoms or the presence of complications; summary scores such as DeMeester index. Measures: • Reflux index (% time pH <4), total # reflux events (pH<4), total # events >5mins, longest episode pH<4.

Question 422

Discuss the non-surgical option for the management of GERD

Answer 422

Basic measures: keep babies in upright position after feeds, dietary changes for older children. Medical treatment: H2 blockers, PPIs, antacids, prokinetic agents; No surgery for pts who are able to achieve good symptom control, obtain resolution of esophageal mucosal damage, maintain appropriate weight gain and thrive developmentally on medical therapy

Question 423

Describe your work-up in a child presenting with caustic ingestion

Answer 423

* Call poison control. * Asymptomatic and no signs of oral cavity tissue injury may be monitored; if they tolerate oral fluids they may be sent home with instructions to return if symptoms worsen or new symptoms occur. * Symptomatic and/or signs of tissue injury from ingestion need to be admitted for close monitoring and further work-up: CXR + AXR; Endoscopy within 24-48hrs; Esophagram.

Question 424

Discuss the medical management of a child with a high grade esophageal injury following caustic ingestion

Answer 424

Pharmacologic management with antacids, acid suppressant, and antibiotics. Esophagram 3 weeks later to evaluate for any stricture. Stricture Management - esophageal dilation or in more clinically significant cases, placement of an enteral feeding tube, resection, or esophageal replacement. Periodic endoscopy to monitor for carcinoma

Question 425

Name FOUR potential options for esophageal replacement and list ONE downside of each

Answer 425

1. Reverse gastric tube (++ GERD, poor emptying) 2. Gastric transposition = gastric pull-up (++ GERD, poor emptying) 3. Jejunal interposition (technically challenging) 4. Colonic interposition (precarious blood supply, redundant over time)

Question 426

List the FOUR most common causes of short bowel syndrome from most to least common

Answer 426

1. NEC = 35% 2. Intestinal atresia = 25% 3. Gastroschisis = 18% 4. Malrotation with volvulus = 14% Other: Total colonic or small bowel Hirschsprung, congenital short bowel

Question 427

Define Intestinal Failure

Answer 427

intrinsic bowel disease resulting in an inability to sustain growth, hydration, or electrolyte homeostasis.

Question 428

What are FIVE principles or options for the management of intestinal failure?

Answer 428

1. During initial OR (s), maintain maximal intestinal length 2. Prevention of PN-related complications 3. Continuous then slow change to bolus allows intestinal adaptation 4. Breast milk over formula 5. Promotility agents (domperidone, cisapride) if gastroschisis-associated motility 6. Fluid and electrolyte management 7. Monitoring of nutritional deficiencies (vitamin, mineral, trace elements) 8. Surgical management (tapering enteroplasty, STEP, LILT/Bianchi) 9. Small bowel transplant

Question 429

What is D-lactic acidosis?

Answer 429

D-lactic acidosis occurs secondary to alterations in colonic pH and growth inhibition of Bacteroides species, thereby fostering the growth of acid-resistant anaerobes capable of producing D-lactate; presents like drunk child.

Question 430

What is the embryological hypothesis for the development of choledocal cysts?

Answer 430

Anomalous Pancreaticobiliary duct union = terminal common bile duct and pancreatic duct unite to form a common channel well outside the duodenal wall. Since this common channel is not surrounded by the normal sphincter mechanism, pancreatic juice refluxes into the biliary tree and high concentrations of pancreatic amylase and/or lipase are typically present in the bile.

Question 431

Describe the classification of choledocal cysts

Answer 431

Ia – cystic dilation of entire CBD Ib – cystic dilation of portion of CBD Ic – fusiform dilation of CBD II – diverticulum of CBD with no dilation of CBD/extra or intrahepatic ducts III – choledochocele- normal CBD and pancreatic duct with cystic dilation of CBD either intraduodenal or intrapancreatic in location IVa – multiple cysts intra and extra hepatic IVb – multiple cysts extrahepatic V – single/multiple cysts confined to intrahepatic duct (Caroli - w/ hepatic fibrosis)

Question 432

Describe the management of each Type of choledocal cyst

Answer 432

Ia - Excision of cyst and REY hepaticojejunostomy or hepaticoduodenostomy. Ib - Excision of cyst and REY hepaticojejunostomy or hepaticoduodenostomy. II - Complete excision of the cyst with REY HJ or HD OR Complete excision of the cyst with closure of CBD III - ERCP + sphincterotomy; Resection of cyst and sphincteroplasty; Whipple’s if malignancy is suspected IV - Extrahepatic cyst excision and REY HJ or HD; Hepatectomy if unilobular V - Drainage, stone extraction, antibiotics, ursodiol; Hepatectomy if unilobular; if severe pHTN, consider OLTx

Question 433

A 3-month old boy is found to have an absence of the vas deferens during a hernia repair. What are the TWO (and a half!) associations and significance of this?

Answer 433

1. Cystic fibrosis — most have azoospermia and congenital bilateral absence or obstruction of the vas deferens. 2. Ipsilateral renal agenesis — arises because the vas deferens originates from the ureteral bud from the mesonephric duct. 0.5 - SD: CAH – with ovary in inguinal canal or ovotesticular DSD

Question 434

Define Pancreatic Pseudocyst

Answer 434

Cystic collection adjacent to pancreas include pseudocysts (no necrosis) and walled off pancreatic necrosis (WOPN). Pseudocysts by definition occur >4 weeks into disease course, lack epithelial layer, and develop secondary to duct disruption, either due to acute/chronic pancreatitis or trauma. Fluid contained high amylase, low CEA, no mucin.

Question 435

What are the management strategies for if planning intervention for pancreatic pseudocyst?

Answer 435

Key to management is to delineate ductal anatomy prior to intervention with MRCP. If communication with the main duct, proceed with ERCP & stent. If no communication with main duct, proceed with endoscopic or open cystgastrostomy. Avoid percutaneous drainage due to high risk of external fistula and recurrence. Watchful waiting is an appropriate option in pts with minimal or no symptoms and no evidence of pseudoaneurysm.

Question 436

Describe the classic presentation of a H-type TEF

Answer 436

coughing, choking, and cyanosis due to recurrent aspiration through the fistula; occasionally diagnosed as a newborn with aforementioned symptoms or persistent abdominal distension and increased gas on AXR; in older children, suspect in the setting of recurrent pneumonias, often involving the RUL.

Question 437

Which diagnostic modalities can be used to diagnose an H-type fistula?

Answer 437

prone pull-back esophagram, bronchoscopy, "triple endoscopy" (rigid bronch, flexible bronch, EGD) +/- methylene blue injection

Question 438

What is the in utero function of the ductus arteriosus?

Answer 438

Shunts blood from PA trunk to aorta (to avoid lungs, which are not participating in gas exchange). It remains dilated in a hypoxic an environment or under influence of prostaglandin E2

Question 439

What would you expect to occur with a PDA when systemic pressure is greater than pulmonary pressure?

Answer 439

Left to right shunting of blood from the aorta into the pulmonary artery causing decreased blood flow to the body and increased blood flow to the lungs —> can lead to poor oxygenation and perfusion of the organs in the abdomen (kidneys, bowel), which can lead to NEC; in addition, increased flow to lungs means increased flow to heart, which can lead to heart failure.

Question 440

What would you expect to occur with PDA when systemic pressure is less than pulmonary pressure?

Answer 440

Right to left shunting of blood from the pulmonary artery into the descending aorta meaning that de-oxygenated blood mixes with oxygenated blood is in the descending aorta —> poor oxygenation of end organs.

Question 441

What are the physical exam findings that may be present in a neonate with a PDA?

Answer 441

asymptomatic pansystolic murmur & widened pulse pressure —> CHF (respiratory compromise, tachypnea, FTT, diaphoresis);

Question 442

What are the potential complications of surgical closure of a PDA?

Answer 442

incomplete ductal occlusion, vocal cord paralysis, inadvertent occlusion of a nearby structure (left pulmonary artery, left main stem bronchus, aorta), pneumothorax, hemorrhage, diaphragmatic paralysis, chylothorax, and scoliosis.

Question 443

For which patients with a PDA is surgical closure necessary?

Answer 443

reserve mechanical closure for pts with failure to wean from mechanical ventilation and/or hemodynamic significance plus medical failure and/or inability to tolerate medical treatment (SIP/NEC).

Question 444

Describe FOUR differences between VV and VA ECMO

Answer 444

1. Indications – VV for resp failure only; VA for cardioresp failure 2. Bypass – VA bypasses heart and yields reduced pulsatile flow, VV does not 3. Cannulas – VV is single cannula with double lumen; VA usually IJ and carotid and is two separate cannulas 4. Weaning – VV disconnect gas exchange and can do longer wean; VA wean flows then clamp lines, need a quick removal of cannulas 5. Complications – VV flow goes through heart so less risk of air embolus stroke as compared to VA. VA has risk of myocardial shunting, ligation of carotid, and higher risk of thromboembolic to systemic and cerebral circulation.

Question 445

Describe THREE ways to prevent complications during an EA/TEF repair

Answer 445

1. Set up – Bronchoscopy and placement of catheter in fistula to help with identification 2. Intra-operative – careful dissection, ensure not dissecting too deep on contralateral side to avoid picking up other RLN, interposition patch between trachea and repair. 3. Post-op – no trans-anastamotic feeding tube to prevent stricture.

Question 446

List FOUR structures that can be injured during resection of type II branchial cleft sinus

Answer 446

1. common carotid 2. external and internal carotid arteries 3. hypoglossal nerve 4. glossopharyngeal nerve 5. pharynx 6. vagus

Question 447

Define ALTE/BRUE

Answer 447

ALTE (apparent life-threatening event) and BRUE (brief, resolved, unexplained event) are not specific disorders but terms for a group of alarming symptoms that can occur in infants. They involve the sudden appearance of respiratory symptoms (apnea), change in colour or muscle tone, and/or altered responsiveness. The caregiver may fear that the child is dead or that his or her life is in jeopardy. Events typically occur in children < 1 year with peak incidence at 12 weeks

Question 448

What are TWO lengthening procedures for short gut?

Answer 448

1. STEP - Serial transverse enteroplasty | 2. LILT - Longitudinal intestinal lengthening and tailoring

Question 449

Describe Bronchopulmonary Sequestration

Answer 449

Predisposition to lower lobes, non-functioning tissue Presence of a systemic arterial blood supply from lower thoracic or upper abdominal aorta-->can result in steal of blood flow-->heart failure and hydrops; Intralobar = 75%; extralobar=35%

Question 450

Describe Congenital Lobar Emphysema

Answer 450

Parenchymal abnormalities secondary to over inflation during respiration. LUL most frequently affected followed by RML

Question 451

A mass in left lobe of liver of teenage boy. U/S and axial imaging show no other anomalies. Describe TWO important issues to consider in your operative planning. Recall: approximately 2/3 of peds liver tumors are malignant

Answer 451

1. Resectability – can it be removed with negative 1 cm margins on critical structures (PV, HV/IVC) 2. Residual liver function- amount of residual functioning liver as well as underlying liver disease that may decrease function of remaining liver 3. Is this patient better off with transplant?

Question 452

You perform an esophagoscopy for a child who had a caustic ingestion 24 hours ago and has been stable on the floor since admission. Describe the grading of esophageal caustic injury and the management of each grade.

Answer 452

Grade 0 – normal; no treatment Grade I – edema and hyperemia of mucosa; no treatment, start liquid oral intake and transition to solids, discharge if tolerated. ``` Grade IIa (moderate)– friability: hemorrhage, erosion blisters, exudates, superficial ulcers; need treatment to prevent strictures; oral sucralfate, mycostatin, PPI; NG feed until tolerate oral feeds Grade IIb (severe)– grade IIa plus deep, discrete or circumferential ulcers. Mgt as above ``` Grade IIIa – small scattered areas of nerosis, brownish black or gray discoloration; Mgt as above + Abx Grade IIIb – extensive necrosis; Mgt as above +Abx + be ready for other intervention - chest tube, OR, etc

Question 453

What is the appropriate follow up for a child following discharge for caustic ingestion?

Answer 453

follow up in 2-3 weeks and do UGIS; earlier if clinical symptoms of dysphagia.

Question 454

In which patients with caustic injury is esophagoscopy contraindicated?

Answer 454

Esophagoscopy is contraindicated when evidence of pharyngeal burn w/ stridor d/t risk of aggravating airway – do upper laryngoscopy to assess upper airway, if need intubation then can do EGD, otherwise hold off until airway edema resolved if fever, systemic sepsis – could mean perforation – can do upper GI series to assess w/ water soluble contrast

Question 455

A 5 year old boy with a lye ingestion returns for follow up and is found to have a 3x2cm stricture and a luminal diameter of 3mm. What is your management and end points for this patient?

Answer 455

Management involves a program of serial esophageal dilations every 2 weeks to start, if improving can decrease frequency; if not improving can do every week; if still not improving, can trial intralesional steroid injection or topical MMC; if still not improving, assess extent of reflux contributing to problem w/ biopsies (ensure on PPI throughout course); May require fundoplication; If all else fails and continues to have persistent stricture, may need resection w/ anastomosis vs replacement depending on length/extent of stricture.

Question 456

A 3 year old presents with a left varicocele. What is the significance of this finding?

Answer 456

Uncommon to have hydrocele in young children (<10 yrs), need to ensure doesn’t have a Wilms, neuroblastoma or hydronephrosis causing obstruction of venous return from testis; most varicoceles occur on left, if occurs on right – need to r/o RP tumor.

Question 457

You come across an adrenal rest while doing a hernia repair in a 3 year old boy. What is the significance of this finding?

Answer 457

Minimal significance. Common incidental finding based on embryology – gonads develop just lateral to mesoderm that forms into adrenal cortex, attachment of adrenal cells onto testis before descent from RP to scrotum can bring adrenal rest down into inguinal canal; does not require any further investigation or treatment

Question 458

Indicate THREE common causes of ALTE/BRUE

Answer 458

1. GERD 2. Seizure 3. Tracheomalacia 4. Brain, nerve, or muscular disorder 5. Non-accidental trauma

Question 459

Describe some of the common physical exam findings in patients with Turner syndrome

Answer 459

Short stature, short and webbed neck, “shield chest,” low-set ears, swollen hands and feet at birth, low hairline at the back of the neck. Turner syndrome may present with primary amenorrhea due to early ovarian failure, but a minority of girls with Turner syndrome begin pubertal development and a few may achieve regular menses and even fertility.

Question 460

What are the cardiovascular associations of Turner Syndrome?

Answer 460

cardiovascular malformations include aortic valvular disease (mainly a bicuspid aortic valve), aortic arch anomalies (primarily coarctation), pulmonary or systemic venous abnormalities, ventricular septal defects, and hypoplastic left heart syndrome.

Question 461

Turner syndrome is associated with renal abnormalities (60% horseshoe kidneys!) and hypertension. What endocrine abnormalities are associated with Turner?

Answer 461

Increased risk of obesity, DM-2, hypothyroidism

Question 462

Discuss the management of a patient with Turner Syndrome

Answer 462

cardiology consult; recombinant human growth hormone for short stature; low-dose estrogen for primary hypogonadism (ovarian failure); consider prophylactic gonadectomy in 45X/45XY mosaics given risk of gonadoblastoma

Question 463

List FOUR diagnostic tests in the work up for Hirschsprung

Answer 463

1. AXR: dilated loops, absence of gas in rectum. 2. Contrast enema: sensitivity 80%; transition zone where aganglionic bowel transitions into dilated ganglionic bowel; Abnormal rectosigmoid ratio <0.9 in short-segment disease; Total colonic may have “question mark sign.” 3. Rectal biopsy: (-) ganglion cells, (-) calretinin, nerve hypertrophy >40 microns, hyperactivity of acetylcholinesterase 4. Anorectal manometry: HD pts will have absence of RAIR (recto-anal inhibitory reflex) because they have internal anal sphincter achalasia

Question 464

Where should a suction rectal biopsy be take for Hirschsprung?

Answer 464

biopsy must be 1cm+ higher than dentate line (normal zone of aganglionosis below dentate; pathology may see squamous epithelial cells rather than columnar epithelial cells).

Question 465

Name THREE risk factors for Hirschsprung associated enterocolitis

Answer 465

1. Previous episode of HAEC 2. T21 3. long-segment disease

Question 466

List the THREE characteristics of an intestinal duplication

Answer 466

(1) well developed coat of smooth muscle (2) epithelial lining representing some portion of the GIT (3) intimately attached to some portion of the GIT.

Question 467

Describe the classification of intestinal duplications

Answer 467

most commonly cystic or tubular having an architecture that resembles the normal GIT; occur on mesenteric side and commonly share muscular and serosal layers as well as a blood supply with the associated segment; may communicate with the lumen of the involved normal GIT

Question 468

What are the FOUR embryologic hypotheses for intestinal duplication?

Answer 468

1. Split notochord: due to failed separation of endoderm from notochord causing traction diverticulum 2. Defects resulting from failed or incomplete canalization: vacuolization in two places leading to intestine development in two places 3. Abortive twinning: complete doubling due to split in primitive streak 4. Environmental factors: hypoxic or traumatic events, could induce attempts at twinning that might result in alimentary duplications.

Question 469

List FOUR clinical scenarios in which a rectal biopsy to rule out Hirschsprung is appropriate

Answer 469

1. Neonate with feeding intolerance, failure to pass meconium in 24 hours, and bilious vomiting 2. Neonate with cecal or appendiceal perforation 3. Neonate with meconium plug syndrome 4. Older child with chronic constipation 5. Colonic atresia

Question 470

List FOUR areas of the body where Nitric Oxide is important

Answer 470

1. Lung 2. Penis 3. Colon - pathologic absence of nitric-oxide mediated relaxation responsible for Hirschsprung 4. Esophagus - as above with Achalasia (theory) 5. Pylorus - as above with pyloric stenosis (theory)

Question 471

Describe the embryological explanation for Hirschsprung

Answer 471

Ganglion progenitor cells develop from embryonal neural crest and migrate from proximal to distal. Ganglions are normally found in the rectum by 12 wks GA. Ganglions produce local nitric oxide with relaxes enteric smooth muscle and is critical for effective peristalsis

Question 472

Thoracic Duplications account for 20% or intestinal duplications. What is the management of these anomalies?

Answer 472

Resect them! Generally in posterior mediastinum; associated with CDH, BPS, EA-TEF, vertebral anomalies; may communicate with neural canal —> if suspected, do preop MRI and involve neurosurgeon; thoracoabdominal often traverse through right crus and can require staged or 2 body compartment approach

Question 473

Foregut Duplications account for 15% or intestinal duplications. What is the management of these anomalies?

Answer 473

Resect them! Often located in greater curve or medial/posterior portions of D2/D3; often communicate with lumen of stomach/duodenum; can communicate with the biliary/pancreatic ducts—if suspected, do IOC; rarely, need internal drainage w REY.

Question 474

Midgut Duplications account for 50% or intestinal duplications. What is the management of these anomalies?

Answer 474

Often a very long duplication, either (1) attempt procedure to separate mesentery (like LILT) then remove duplication, (2) marsupialize using long stapler to create anastomosis between lumens, or (3) strip mucosa (“Soave”) with series of incisions.

Question 475

Hindgut Duplications account for 15% or intestinal duplications. What is the management of these anomalies?

Answer 475

can be associated with myelomeningocele, GU anomalies, and perineal/vaginal fistulas; preop MRI, contrast studies, +/- endoscopy paramount; rectal duplications typically cystic in presacral space (think Currarino); long tubular colonic duplications typically side-to-side—resect, fenestrate with stapler, or strip mucosa.

Question 476

Describe the classification of jejuno-ileal atresia

Answer 476

Type I – intact bowel wall and mesentery with mucosal web/atresia Type II – intact mesentery, distinct proximal and distal blind ends with fibrous cord Type IIIa – atretic ends separated by V-shaped mesenteric gap Type IIb – apple peel atresia Type IV – multiple atresias

Question 477

Which finding on prenatal ultrasound are concerning for jejuno-ileal atresia?

Answer 477

Considered when dilated >14-17mm, or echogenic bowel seen. Identifies 66% jejunal, 26% ileal atresia; more proximal = polyhdramnios, Fetal MRI may help narrow Dx (eg, seeing microcolon, external mass, etc)

Question 478

Pentalogy of Cantrell (FIVE)

Answer 478

1. epigastric abdominal wall omphalocele 2. anterior diaphragm w/ diaphragmatic hernia 3. sternal cleft (ectopia cordis) 4. pericardium 5. intracardiac defect

Question 479

Detail the embryology of the anterior abdominal wall

Answer 479

Begins in the 4th week with the simultaneous longitudinal and transverse folding of the flat embryonic disk into a 3D cylindrical embryo; body wall folding is the result of the differential growth of the embryonic disk in which there is faster growth centrally than peripherally as well as dorsally than ventrally; body wall folds must successfully meet and fuse to form a solid, ventral abdominal wall.

Question 480

What is the likely etiology of omphalocele?

Answer 480

likely results as a combination of abnormal migration + incomplete fusion of the lateral body wall folds around the umbilicus and failure of the normally herniated midgut to return to the abdominal cavity; in some cases there may be associated failure of the superior/inferior longitudinal folding.

Question 481

What are the THREE layers of the omphalocele membrane?

Answer 481

(1) inner = peritoneum (2) middle = Wharton jelly (3) outer = amnion that normally covers umbilical cord

Question 482

Differentiate Pentalogy of Cantrell, traditional omphalocele and cloacal/bladder exstrophy by where the folding defect occurs

Answer 482

Pentalogy of Cantrell = epigastric omphalocele —> cephalic folding defect. Traditional omphalocele = central omphalocele —> lateral folding defect. Cloacal & bladder exstrophy = caudal omphalocele —> caudal folding defect. BONUS: Body stalk anomalies = severe, frequently lethal defect with failure of folding of all three (cephalic, lateral, and caudal) folds.

Question 483

What are the FIVE most common associated anomalies of omphalocele?

Answer 483

1. Congenital heart disease 2. Chromosomal anomalies (especially if central) — T13, T18 (90% have omphalocele), T21 3. BWS (6% of omphalocele) 4. CNS defects — spina bifida, anencephaly. 5. Pulmonary hypoplasia

Question 484

Name FOUR predisposing anomalies or genetic syndromes for Wilms Tumor

Answer 484

1. BWS 2. WAGR 3. Denys-Drash 4. hemihypertrophy 5. familial Wilms.

Question 485

What is Denys-Drash?

Answer 485

Wilms, congenital nephropathy, DSD—>gonadal dysgenesis

Question 486

What are the FIVE contraindications to upfront resection in Wilms?

Answer 486

1. Bilateral disease 2. solitary kidney 3. extension into retrohepatic IVC 4. tumor involves contiguous structures necessitating their removal 5. pulmonary compromise from +++mets

Question 487

What is the COG Staging for Wilms Tumor?

Answer 487

I - completely excised limited to kidney II - completely excised but extends beyond capsule or into vasculature III - retroperitoneal lymph node involvement or gross residual tumor or margin +/rupture/transected tumor thrombus IV - hematogenous mets or extra-abdominal LN involvement V - bilateral at diagnosis

Question 488

Vhat chemo is used for Vilms?

Answer 488

Stage I and II - VA - vincristine and dActinomycin Stage III and IV - VAD - vincristine, dActinomycin and doxorubicin. Also need flank radiation or full abdomen radiation if large rupture or peritoneal mets Stage V - VAD x 6 weeks - vincristine, dActinomycin and doxorubicin.; both kidneys biopsied if 50% shrinkage. Continue chemo x 6 weeks and OR 12 weeks post initiation

Question 489

A 12 year old boy comes to your office. He has been freaking out because he thinks he's growing boobs like his older sister did. "I think the other boys will make fun of me", he says as his voice cracks. What do you tell him? And what organs do you need to examine.

Answer 489

You respond, "2/3 of your friends will get these temporary breasts. It's the 1/3 that DON'T get breasts that will feel left out! Usually it goes away after 1-2 years" You ask him if he smokes "the reefer" or is on any steroids, HCG, spironolactone, etc. Now that he has calmed down, you examine the breasts and axilla for any abnormality, testicles (Kleinfelter syndrome, Testicular injury or tumor), thyroid (Hypothyroidism), liver for signs of cirrhosis, adrenal glands for tumors.

Question 490

How is Gynecomastia graded?

Answer 490

``` 1 = small amount of breast enlargement restricted to the subareolar space. 2 = moderate breast enlargement extending beyond the borders of the areola. 3 = moderate breast enlargement extending beyond the borders of the areola with some skin redundancy. 4 = marked breast enlargement with skin redundancy and feminization of the breast. ```

Question 491

Describe the management of gynecomastia.

Answer 491

* Lifestyle changes (diet, exercise) and reassurance. * Stop marijuana, steroids, or other offending drugs. * Medications: SERMs (tamoxifen, raloxifene, clomifene) or aromatase inhibitors —> but off-label. * Surgery: (1) persistent and bothersome or (2) rated as grade 2 or above once a systemic condition has been excluded

Question 492

Peutz–Jeghers syndrome (PJS) can present with bleeding or intussusception outside of the usual age. What is PJS, and what is it's associated carcinoma risk?

Answer 492

PJS is an autosomal dominant disorder characterized by FH + benign hamartomatous polyps (2+) + mucocutaneous pigmentation —> hyperpigmented macules on lips/oral mucosa/hands/feet/genitals; While the hamartomatous polyps themselves only have a small malignant potential (<3%), pts have an increased risk of cancer: colorectal (40% lifetime), gastric (30% lifetime), liver, lungs, breast, ovaries, uterus, testes.

Question 493

What is a Fibroadenoma (breast)?

Answer 493

benign neoplastic stroma, hormone responsive; 60% of palpable breast masses in teenagers; >5 cm =giant. 10-15% have more than one. Rare to have ++ fibroadenomas (think of underlying conditions and exposure, Carney Complex – cutaneous spotting, endocrinopathies and myxoid lesion in heart, breast, skin). Involute in perimenopausal period.

Question 494

List THREE disorders of breast involution

Answer 494

1. Cyst formation 2. ductal ectasia 3. sclerosing adenosis

Question 495

Describe the pathophysiology of Neonatal breast hypertrophy.

Answer 495

circulating hormones in late pregnancy cross into fetal circulation and stimulate fetal breast growth -->prime fetal vesicles for milk production -->after birth, sex steroid hormones decrease but prolactin maintained -->engorgement and milky secretions in up to 90%; Most resolve by two months but take up to two years.

Question 496

Neonatal mastitis -presentation and management

Answer 496

– w/in first 2 months of life. Can be unilat or bilat. Staph aureus then e. coli most causative. Avoid I&D to prevent damage tot breast. Warm compresses, Abx and needle aspiration instead

Question 497

Explain the definition, extraintestinal findings, malignancy risk, and genetic origin of Juvenile polyposis syndrome

Answer 497

Definition - any of the following: four or more polyps, family history of JPS or GI malignancy at young age with any number of polyps, extraintestinal manifestations with any number of polyps Extraintestinal findings - cardiac (mitral valve prolapse), urologic, gynecologic Malignancy risk - colorectal, small bowel, stomach Genetics - SMAD4

Question 498

What mutation is responsible for both cowden and bannayan-riley-ruvalcaba syndrome?

Answer 498

PTEN

Question 499

Is there a cancer risk associated with isolated juvenile polyps?

Answer 499

No. 1-4 polyps and no family history defines juvenile polyps.

Question 500

What is a lymphoid polyp?

Answer 500

benign lesions which clinically resemble a polyp; pathophysiology is reactive hyperplasia of mucosa associated lymphoid tissue; associated with adenovirus infection, immunodeficiency (low IgA and IgM) +/- secondary to EBV; most common in colon around the ileocecal valve due to the presence of abundant lymphoid tissue (prominent Peyer's patches); rectal and anal lymphoid follicles may be associated with inflammatory bowel disease or hemorrhoids; multiple lymphoid polyps can be associated with FAP and Gardner syndrome; in children, larger lymphoid polyps may cause intussusception or luminal obstruction.

Question 501

What is Gardner syndrome?

Answer 501

multiple adenomatous polyps with benign extraintestinal tumors; extraintestinal findings are the same as FAP but also include epidermoid cysts, fibromas, desmoid tumors, osteomas

Question 502

What is Turcot syndrome?

Answer 502

multiple adenomatous polyps with intracranial neoplasm (medulloblastoma, glioblastoma)

Question 503

Define familial adenomatous polyposis (FAP). What are the extra-colonic manifestations?

Answer 503

Germline mutation in APC (tumor suppressor gene) ``` sparse type (100s of polyps) profuse type (1000s of polyps) If 20 to 100 polyps and later in life, consider Attenuated FAP (AFAP) ``` Extra-colonic findings: duodenal adenomas (may be microscopic), gastric adenomas/fundic gland polyps, congenital hypertrophy of the retinal pigment epithelium, nasopharyngeal angiofibromas, dental/jaw lesions, lipomas

Question 504

Describe the characteristics and management of cystic neuroblastoma

Answer 504

Rare and exclusively located in adrenal gland. Adrenal masses are typically found during U/S examinations performed after 32 weeks GA with the earliest mass observed reported at 20 weeks. Vast majority are localized tumours with favourable biological features that correlate with an excellent survival (>95%). 50% of these tumours are solid, 25% cystic, and 25% are complex. Management: L1 < 5cm <18 mos = observe (solid <3.1cm or cystic <5cm) —> spares 80% children surgery

Question 505

List FOUR things on the differential diagnosis for a cystic neuroblastoma

Answer 505

1. adrenal hemorrhage 2. adrenal cyst 3. adrenal adenoma 4. adrenal abscess 5. ACC 6. CPAM 7. extra-lobar BPS

Question 506

What is the classic presentation fo Kawasaki disease and what is the most feared consequence of Kawasaki?

Answer 506

Presentation: young children; characteristic clinical features are fever x5 days with bilateral non-purulent conjunctivitis, rash, changes in lips and oral cavity (strawberry tongue), changes in peripheral extremities (erythema palms/soles), and cervical lymphadenopathy; usually at least one unilateral cervical nonpurulent lymph node that measures greater than 1.5cm —> potential for surgical biopsy; may have GI involvement with abdominal pain, diarrhea, and pseudo-obstruction. It has become the leading cause of acquired pediatric heart disease in the United States

Question 507

What are the options for management of Kawasaki disease?

Answer 507

* IVIG + ASA | * Corticosteroids, infliximab, cyclosporine A as adjunct therapy for disease refractory to initial treatment.

Question 508

What is ITP?

Answer 508

Immune thrombocytopenia (ITP), previously called idiopathic thrombocytopenia, is a relatively rare autoimmune disorder that is characterized by isolated thrombocytopenia; as a result of the humoral antibody response against platelets, platelets are destroyed in a matter of hours instead of eight to ten days.

Question 509

What is the classic presentation and time course of ITP?

Answer 509

Acute onset of bruising, petechiae, and purpura without other symptoms; degree of bruising can be enough to consider NAT; often history of viral illness; these are well children —> if unwell or multiple cell lines affected, think leukemia; time course: (1) newly diagnosed x 3 months; (2) persistent 3-12 months, (3) chronic >12 months —> only 30% will resolve ITP.

Question 510

What are the options for medical management of ITP?

Answer 510

- IVIG + IV anti-D therapy (WinRho) if Rh-positive —> steroids no longer first line - Rituximab

Question 511

What is the mature minor doctrine?

Answer 511

The mature minor doctrine allows children <18 years who are sufficiently mature to make their own treatment decisions. Most legislation does not identify an age at which minors may exercise independent consent for health care but recognizes that the level of the patient's understanding of the nature and consequences of the treatment have determinants beyond age. This allows physicians to make a determination of capacity to consent for a child just as they would for an adult.

Question 512

What THREE criteria allow for a minor to provide consent for themselves? (Royal College)

Answer 512

1. Be informed of his or her condition, prognosis, proposed treatments, and alternatives 2. Understands the risks and potential benefits of each alternative and the consequences of choosing a particular alternative 3. Has the ability to relate a choice to a stable set of values.

Question 513

What is post transplant lymphoproliferative disorder and who is most at risk?

Answer 513

(PTLD) is an unusual entity that has many features of an immune system malignancy. It is characterized by uncontrolled proliferation of lymphoid lineage cells (typically B cells, rarely non-B lineage) in the context of post-transplant immunosuppression. Up to 15% of pediatric transplant recipients develop PTLD and 20% of those will die from the disease. There is potential for graft loss due to disease itself or the need to reduce immunosuppression which increases the risk of rejection. Highest risk in recipients who are EBV seronegative at the time of transplant. Intestinal >thoracic > liver > kidney.

Question 514

Describe the presentation of a patient with PTLD

Answer 514

Sudden onset of a lymphoid mass or swelling, either externally (e.g. cervical lymph nodes) or internally (e.g. abdominal or intracranial mass); can mimic cat scratch disease and manifest with fever + LAD. PTLD occurring early after transplant often involves the allograft organ and is nearly always EBV related

Question 515

Name FOUR options for management of PTLD

Answer 515

1. Reduction of immunosuppression 2. Immunotherapy with rituximab (CD20 monoclonal antibody) 3. Chemotherapy 4. Radiation therapy

Question 516

Describe the THREE groups of extra-intestinal manifestations in ulcerative colitis

Answer 516

1. Group 1 parallels disease activity: aphthous ulcers, erythema nodosum, large joint arthropathy, episcleritis. 2. Group 2 independent from disease activity: ankylosing spondylitis, uveitis. 3. Group 3 unclear relationship: pyoderma gangrenosum, primary sclerosing cholangitis.

Question 517

List FOUR risks/complications of IPAA

Answer 517

1. pouchitis (70% lifetime risk) 2. fertility problems (pelvic dissection) 3. anastomotic leak 4. strictures, bowel obstructions 5. perianal or vaginal fistulas —> query Crohn’s

Question 518

How frequently does overwhelming post-splenectomy infection (OPSI) occur?

Answer 518

These infections occur with an estimated incidence of ~0.5% per year and lifetime risk is 5%. Highest risk in the first 2 years after OR. The highest rates of infection are observed following splenectomy in children under five years of age and in patients with sickle cell and thalassemia. Despite its rare occurrence, the mortality rate of sepsis associated with asplenia remains significant.

Question 519

What are the THREE most common pathogens associated with OPSI?

Answer 519

1. Streptococcus pneumoniae > 2. Hemophilus influenzae > 3. Neisseria meningitidis

Question 520

List the THREE types of duodenal atresia

Answer 520

Type 1: thin membrane separates the proximal and distal portions of the duodenum (92% of patients). Type 2: fibrous cord connects the two atretic ends (1%). Type 3: complete separation of the segments with a mesenteric defect between the two (7%)

Question 521

What proportion of duodenal atresia is pre-ampullary vs. post-ampullary?

Answer 521

80% are at ampulla or just post-ampulla —> bilious emesis; if distal air, rule-out malrotation but if not could be a bifid CBD (rare). 20% are pre-ampulla —> babies with T21 are more likely to be pre-ampullary

Question 522

List FOUR congenital anomalies associated with duodenal atresia

Answer 522

1. T21 (30%) 2. CHD (25%) esp. endocardial cushion 3. annular pancreas (25%) 4. malrotation (25%) 5. EA-TEF (15%) 6. ARM (15%) 7. intestinal atresia (<3%) 8. VACTERL (5%)

Question 523

What is BASM?

Answer 523

Biliary atresia-splenic malformation (BASM) syndrome • Biliary atresia • Laterality defects (malrotation and/or situs inversus) • Polysplenia • Interrupted vena cava • Pre-duodenal portal vein

Question 524

Describe the presentation of early onset and late onset GBS infection

Answer 524

Early onset (<7 days) is acquired in utero or during passage through the vagina. Presents as generalized sepsis and less commonly as pneumonia or meningitis, generally within 24 hours of birth. Late onset (<90 days) can be acquired vertically at birth or horizontally in household and community settings. Presents as bacteremia without a focus or meningitis; less common but well-described late-onset GBS focal infections include septic arthritis, osteomyelitis, and cellulitis-adenitis.

Question 525

Name FOUR risk factors for GBS in neonates

Answer 525

1. Delivery at <37 weeks 2. Maternal GBS bacteriuria during the current pregnancy 3. Heavy maternal GBS colonization 4. Prior delivery of an infant with GBS disease 5. Chorioamnionitis 6. maternal temperature ≥38°C during labor 7. Premature rupture of membranes or rupture of membranes for ≥18 hours before delivery

Question 526

Describe the management of GBS in a neonate

Answer 526

Empiric antibiotics = ampicillin + gentamicin (cover for GBS, other strep, gram negatives, and Listeria) plus cefotaxime if suspicion of meningitis —> definitive therapy is penicillin G once GBS confirmed.

Question 527

Name TWO absolute and FOUR relative indications for resection in ulcerative colitis

Answer 527

Absolute indications: 1. exsanguinating hemorrhage 2. perforation 3. Toxic megacolon with ongoing progression despite optimal medical Tx. Relative: 1. refractory to medical treatment 2. intractable disease 3. steroid dependence 4. poor quality of life 5. growth restriction 6. patient preference

Question 528

List FIVE potential ways that a Meckel Diverticulum can cause obstruction

Answer 528

1. intussusception 2. volvulus of diverticulum around own base 3. external compression from inflammation 4. internal hernia 5. obstruction from intestine kinked around fibrous vitelline remnant connecting Meckel to umbilicus 6. volvulus of intestine around fibrous vitelline remnant 7. self knotting diverticulum 8. littre hernia – indirect inguinal hernia

Question 529

Describe THREE indications for surgery for a Meckel Diverticulum

Answer 529

1. bleeding (from ulceration) 2. obstruction (from intussusception, volvulus, or inflammation and usually mechanical in origin) 3. infection/inflammation (also from ulceration). 4. tumors (carcinoid is the most common) 5. impacted foreign bodies and parasitic infections 6. incidental Meckel diverticulectomy ? - controversial.

Question 530

Name THREE premedicationos that make a meckel scan more sensitive

Answer 530

1. Pentagastrin 2. Glucagon 3. H2 blockers

Question 531

What is hemorrhagic disease of the newborn?

Answer 531

Bleeding problem that occurs in a baby during the first few days of life. Babies are normally born with low levels of vitamin K, an essential factor in blood clotting. A deficiency in vitamin K is the main cause of hemorrhagic disease in newborn babies. This leads to deficiency/inadequate activity of factors 2, 7, 9, and 10; also, immature liver doesn’t utilize vitamin K well; treat by giving oral or IM vitamin K; if life-threatening hemorrhage, can transfuse FFP +/- PRBCs.

Question 532

What are THREE potential causes of post-circumcision bleeding?

Answer 532

1. Vitamin K deficiency - hemorrhagic disease of the newborn 2. Trauma 3. Clotting factor deficiency - Hemophilia A or B 4. DIC 5. Thrombocytopenia

Question 533

What is the definition of abdominal compartment syndrome?

Answer 533

For most critically ill babies and children, an intra-abdominal pressure of 4-9 is considered normal. Intraabdominal hypertension (IAH) in children is defined as a sustained intraabdominal pressure >10. For research purposes, ACS is defined as a sustained intraabdominal pressure >10 that is associated with new organ dysfunction. For clinical purposes, ACS is better defined as IAH-induced new organ dysfunction without a strict intraabdominal pressure threshold, since no intra abdominal pressure can predictably diagnose ACS in all patients.

Question 534

How would you measure intra abdominal pressure?

Answer 534

Get a kit. Read the instructions. Just like Ikea

Question 535

What is Tracheomalacia and how does it present?

Answer 535

Definition: typically defined as a greater than a 50% collapse of the trachea during spontaneous expiration. Presentation: suspected when an infant develops expiratory stridor on exertion, feeding, or crying; increasing difficulty with expiration leads to difficulty with feeding, cyanotic spells, and, in extreme cases, apnea and collapse (death attacks); symptoms become apparent after the first 2 weeks of life Post EA-TEF: pts may present with multiple failed attempts at extubation with episodes of desaturation, apnea, cyanosis and bradycardia

Question 536

What are the options for management of tracheomalcia?

Answer 536

1. Watchful waiting: recall that most cases resolve by 12-18 months; trachea tends to stiffen during infancy. 2. Supportive treatment with positive pressure ventilation 3. Aortopexy: indications for surgery in tracheomalacia are somewhat controversial; however, in cases where focal tracheomalacia adjacent to the aortic arch and innominate artery is identified, an aortopexy can be successful because it helps to relieve anterior external airway compression

Question 537

List FOUR manifestation of abdominal compartment syndrome

Answer 537

1. decreased urine output/kidney function 2. decreased perfusion/pulses to lower limbs 3. systemic hypotension 4. increased respiratory distress requiring increased peak pressures to maintain ventilation

Question 538

Name FOUR antenatal complication of meconium ileus

Answer 538

1. pseudocyst 2. perforation – adhesional meconium peritonitis 3. meconium ascites - translocation of intestinal organisms- bacterially infected ascites 4. volvulus 5. atresia

Question 539

List FOUR surgical options for meconium ileus

Answer 539

1. enterotomy/appendectomy with intestinal irrigations and flushing out meconium pellets with primary closure 2. intestinal resection with primary anastomosis 3. intestinal resection with bishop koop stoma 4. intestinal resection with santuli stoma If complicated: 5. pseudocyst resection and abdominal washout

Question 540

What is DIOS? Describe the management

Answer 540

Distal intestinal obstruction syndrome (DIOS) aka Mec ileus equivalent. Impaired secretory function of the pancreas, altered composition of enteric fluids, decreased bowel motility and intestinal mucosal glands with chronically inspissated secretions can cause chronic constipation, intussusception Treatment is aggressive oral rehydration, stool softeners and a bowel regimen including polyethylene glycol (GoLytely); Complete bowel obstruction may require surgical correction

Question 541

What are FOUR conditions found in MEN2B?

Answer 541

1. MTC 2. marfanoid body habitus 3. pheochromocytoma 4. multiple mucosal neuromas 5. intestinal ganglioneuromas

Question 542

What is the recommended treatment for a child with MEN2B

Answer 542

thyroidectomy optimally done at or before 1 year of age, post op physical exam, US, CEA/calcitonin level at 6 months, 1 year, annually, annual screening for pheo w/ US and labs at age 11

Question 543

What are FOUR conditions found in MEN2A?

Answer 543

1. medullary thyroid carcinoma 2. pheochromocytoma 3. hyperplasia or sometimes adenomas of the parathyroid glands (with resultant hyperparathyroidism) 4. cutaneous lichen amyloidosis 5. Hirschsprung

Question 544

Extra-intestinal manifestations of Crohn's may present years before any intestinal disease in children. What are FOUR extraintestinal manifestations of Crohn's disease?

Answer 544

1. Arthritis 2. osteopenia 3. osteoporosis 4. nephrolithiasis d/t Vit D and calcium malabsorption 5. uveitis/iritis/episcleritis 6. erythema nodosum 7. pyoderma gangrenosum

Question 545

What is the first line treatment for children with Crohn's to accomplish complete mucosal healing?

Answer 545

Exclusive enteral nutrition therapy is first line for induction and remission in children. Patient stops eating a regular diet and all intake is elemental and/or polymeric formula for 6-8 weeks. This Promotes mucosal healing, restores bone mineral density and improves growth in children with inflammatory intestinal luminal disease. Mechanism of action = alteration of the gut microflora and subsequent decreased inflammation. Remission rate is between 50 and 80% after four weeks.

Question 546

Name FIVE medical treatment options for Crohn's disease (not exclusive enteral nutrition)

Answer 546

1. Aminosalicylates - for mild CD, no benefit to mucosal healing 2. Oral corticosteroid - induction agent, 60 to 80% response 3. Oral budesonide - controlled ileal release (budesonide MMX is colonic release); remission induction. 4. Antibiotics - adjuvant - ciprofloxacin and metronidazole for fistulizing disease or intra-abdominal abscess; 5. Immunomodulators - azathioprine (AZA), 6-mercaptopurine (6-MP), Methotrexate, Cyclosporine (severe CD colitis refractory to steroids and biologics) 6. Biologic therapy - Anti-tumor necrosis factor (TNF) infliximab, adalimumab; achieve the highest degree of induction response and remission maintenance in CD. 7. anti-adhesion molecules - natalizumab, vedolizumab. Action is limited mainly to the gastrointestinal tract.

Question 547

What are the indications for surgical management of Crohn's disease?

Answer 547

1. Disease specific complications - stricture, abscess, fistula, obstruction, bleeding, sepsis 2. Failure of Medical management - steroid dependance, worsening symptoms despite max treatment, developing antibodies to biologic treatment, FTT, patient preference.

Question 548

List FIVE of the most common causes of persistent pulmonary hypertension in a newborn

Answer 548

1. Meconium aspiration 2. CDH 3. oligohydramnios 4. pulmonary hypoplasia 5. infection 6. respiratory distress syndrome 7. hypoventilation 8. hypothermia 9. idiopathic 10. perinatal asphyxia

Question 549

1) What is the intracellular enzyme that NO effects? | 2) What is the resultant mediator that effects smooth muscle relaxation?

Answer 549

1) Guanylate cyclase 2) Increase in intracellular cGMP causes decrease in calcium influx, and relaxation of smooth muscle cells by stimulating protein kinase

Question 550

Define pulmonary hypertension

Answer 550

Pulmonary hypertension is a physiologic state in which elevated pulmonary vascular pressures cause poor pulmonary blood flow and right ventricular dysfunction leading to gas-exchange problems and abnormal hemodynamics.

Question 551

What are the radiographic and physical exam findings of pulmonary hypertension?

Answer 551

Hypoxia in the absence of significant radiography signs of lung disease, pre/post ductal oxygen saturation difference >5% that improves with supplemental O2, right ventricular strain, increased intra- and extracardiac shunting, hypercarbia and poor left ventricular preload leading to hypotension. CXR - slight hyperinflation, paucity of vascular markings

Question 552

Describe chronic intestinal pseudo-obstruction

Answer 552

Chronic intestinal pseudo-obstruction (CIPO) is a syndrome that suggests mechanical bowel obstruction of the small or large bowel in the absence of an anatomic lesion that obstructs the flow of intestinal contents.

Question 553

Detail the medical treatment for CIPO

Answer 553

1. Nutritional support (Hypercaloric liquid formulations if low calorie intake); TPN for patients with severe dysmotility 2. Antibiotics - For patients with steatorrhea, vitamin B12 malabsorption, or folate excess (suggestive of bacterial overgrowth) 3. Prokinetic agents - erythromycin for acute exacerbations; chronic cisapride or prucalopride

Question 554

Detail the surgical options for CIPO

Answer 554

Surgery should be performed to provide access to the stomach or small bowel for venting and feeding (ileostomy, colostomy, cecostomy, endoscopic decompression). Resection of localized disease should be avoided. Intestinal transplantation is indicated in patients in whom long-term parenteral nutrition cannot be initiated or continued safely

Question 555

What are the zones of the adrenal gland and what does each excrete?

Answer 555

1. Glomerulosa – mineralocorticoids 2. Fasiculata – glucocorticoids 3. reticulata – sex steroid hormones Adrenal medulla - catecholamines

Question 556

Name TWO adrenal tumors, what they secrete, and associated syndrome

Answer 556

1. Pheochromocytoma – catecholamines, MEN2 2. Hyperaldosteronism – aldosterone, Conns 3. Adrenal cortex (fasiculata) tumor– glucocorticoids, cushing syndrome 4. Adrenocortical lesion – sex hormones – virilization with or without hypercortisolism; or feminizing tumor (very rare, usually malignant)

Question 557

Where is the organ of Zuckerkandl?

Answer 557

chromaffin mass at the origin of the inferior mesenteric artery or aortic bifurcation

Question 558

Describe the function of the adrenal medulla

Answer 558

synthesizes and releases catecholamines: dopamine, epinephrine, and norepinephrine

Question 559

Describe the function of the adrenal cortex

Answer 559

synthesizes three types of hormones: glucocorticoids, mineralocorticoids, and sex hormones. Regulation of these is accomplished by the hypothalamic-pituitary-adrenal axis

Question 560

Detail the presenting symptoms and the best diagnostic tests for pheochromocytoma

Answer 560

Symptoms - headaches, fever, palpitations, thirst, polyuria, sweating, nausea, and weight loss, sustained hypertension. Diagnosis - 24-hour urine catecholamines, metanephrine, and vanillylmandelic acid is the best diagnostic test. Can also do serum levels. CT, MRI, MIBG

Question 561

Detail the presenting symptoms and the best diagnostic tests for Cushing's

Answer 561

Present with generalized obesity and long bone growth retardation; hypertension, weakness, thin skin with striae and easy bruising, acne, menstrual irregularity, osteoporosis. Patients < 6 years, most likely adrenal tumor. Diagnosis - 24-hour urinary free cortisol test, midnight plasma cortisol or late-night salivary cortisol, low-dose dexamethasone suppression test

Question 562

Detail the presenting symptoms for sex hormone producing tumors

Answer 562

Virilization with or without hypercortisolism is the most common presentation. Boys - precocious puberty, including penile enlargement, acne, and premature development of pubic, axillary, and facial hair. Girls - clitoral hypertrophy, hirsutism, and acne. Feminizing adrenocortical tumors are rare BUT usually malignant. Present with precocious isosexual development in girls.

Question 563

b) What is the initial treatment for a mediastinal teratoma?

Answer 563

Needle biopsy, neoadjuvant chemotherapy with cisplatin, etoposide, bleomycin; then excise residual tumor

Question 564

What are the most common causes of esophageal perforation in children and infants?

Answer 564

The most common cause (60%) is iatrogenic injury secondary to instrumentation. Most perforations located in hypopharynx or upper esophagus d/t injury from either endotracheal tube or oro/nasogastric tube insertion just proximal to the most narrow portion of the esophagus - the cricopharyngeus muscle. In young children d/t from caustic ingestion and/or foreign objects (button batteries).

Question 565

Name FOUR possible causes of thymic masses in children and their management

Answer 565

1. Thymic hyperplasia - physiologic or following treatment for hematologic neoplasms, burns and cardiac surgery; treatment with steroids or RT 2. Thymic cysts - benign, usually asymptomatic unless hemorrhage causes mass effect; Surgical excision of solitary cysts when symptomatic. Multiloculated cysts = systemic condition like Sjogren’s. 3. Thymoma - rarely malignant; 40% present with a paraneoplastic syndrome (eg. myasthenia gravis). Treatment is complete surgical resection and RT for positive margins. 4. Thymic carcinoma – exceedingly rare; Surgical resection offers the best chance for cure

Question 566

Describe the presentation of esophageal perforation based on location

Answer 566

1. Pharyngeal perforations or perforations limited to upper mediastinum: respiratory distress, chest pain, neck pain, difficulty swallowing, excessive salivation, neck and upper chest emphysema, difficulty passing an OG/NG tube, fever, and tachycardia. 2. Perforations extending into the pleural space: acute respiratory distress, tachycardia, sepsis, chest wall emphysema, leukocytosis, shock and hydropneumothorax 3. Intra-abdominal esophageal perforations: dull epigastric pain that radiates to the back if the perforation is posterior and communicates to the lesser sac; anterior perforation typically presents with a sharp, relentless epigastric pain with peritonitis.

Question 567

Discuss the surgical approach for a cervical, mid, and distal esophageal perforation.

Answer 567

cervical - right neck incision as the thoracic duct is in the left neck and the RLN more prone to injury because not tight on the esophagus midesophagus = right thoracotomy distal esophagus = left thoracotomy

Question 568

Define Epidermoid cysts

Answer 568

congenital ectodermal cysts that arise from the trapped pouches of ectoderm during infolding of the head and neck or from failure of surface ectoderm to separate from the neural tube. Grossly, they are quite similar to dermoid cysts, containing thick caseous material, but histologically they contain only squamous epithelium and do not contain skin appendages

Question 569

Cold babies are such a RECC! List the FOUR ways that babies lose heat

Answer 569

1. Radiation 2. Evaporation 3. Conduction 4. Convection

Question 570

What is non-shivering thermogenesis?

Answer 570

brown fat → requires adequate oxygenation; babies at risk: premies, IUGR, poor oxygenation (sepsis, CHD). Brown adipose tissue (BAT) appears in the fetus and is located near organs of high perfusion, such as the kidneys. Preterm infants have less BAT than term infants, making the contribution of brown adipose tissue to to non-shivering thermogenesis more limited.

Question 571

What is Potter’s syndrome?

Answer 571

Syndrome associated w/ oligohydramnios usually secondary to bilateral renal agenesis (also prune belly syndrome, polycystic kidney disease, urinary tract obstruction); can have distinct facial features- flattened nose, recessed chin, skin folds covering corner of eyes (epicanthal folds), low set abnormal ears; pulmonary hypoplasia; eye malformations; cardiac defect

Question 572

What surgical procedures may be required in the first years of life for a child with PBS (List THREE)?

Answer 572

1. Abdominal wall reconstruction with resection of redundant skin/thinned abdo wall 2. orchidopexy 3. bladder domectomy to improve emptying and decrease postvoid residual urine 4. pyeloplasty for UPJ obstruction 5. urethroplasty for urethral atresia/stenosis

Question 573

Name THREE things that put babies at high risk of heat loss

Answer 573

1. Greater surface area to body ratio 2. Cannot shiver 3. Cannot put on warm sweater, touque, and gloves

Question 574

A child presents with dysphagia. A mobile mass is identified on UGI contrast study. What are your next investigations? Biopsy is performed and reveals interlaced smooth muscle fibers. What is the diagnosis?

Answer 574

Work-up for this mass could include EGD ± biopsy, EUS ± biopsy, and CT c/a/p. The biopsy finding of “interlaced smooth muscle fibers” is suggestive of leiomyoma or leiomyosarcoma. These tumours of smooth muscle derivation can occur anywhere along the gastrointestinal tract

Question 575

What is the treatment of choice for leiyomyoma?

Answer 575

Complete surgical resection is the treatment of choice. If there is concern for leiomyosarcoma, wide local excision with lymph node basin excision should be performed. Because mesenchymal tumours rarely spread through the lymphatic system, extensive lymphadenectomy is not an important tenant for operations of suspected mesenchymal tumours.

Question 576

16. Name the branchial arch origins of… a) Cranial nerve VII b) R subclavian artery right c) Common carotid artery d) Recurrent laryngeal nerve

Answer 576

a) second arch b) fourth arch c) third arch d) sixth arch

Question 577

Classic clinical presentation of choledocal cysts

Answer 577

Jaundice, abdominal pain, palpable abdominal mass in RUQ

Question 578

List FOUR complications following surgery for choledocal cysts

Answer 578

1. Bile leak (0-5%) 2. Cholangitis (2-3%) 3. Pancreatitis (2.5%) 4. Small bowel obstruction (0-5%) 5. Anastomotic stricture (1-2%) 6. Bile reflux gastritis (5% of patients with HD)

Question 579

List FOUR potential factors that put a baby with esophageal atresia at risk for GERD?

Answer 579

1. Esophageal motor dysfunction secondary to operative manipulation /ischemia 2. shortening of intra-abdominal esophagus 3. natural esophageal dysfunction from congenital anomaly 4. poor/absent esophageal peristaltic pump exposes esophagus to prolonged exposure to acid (non-propagating peristalsis, low distal wave amplitudes) 5. decreased lower esophageal sphincter pressures 6. change of angle of His

Question 580

List FOUR factors that must be considered in the planning of an anti-reflux procedure for a patient with EA/TEF

Answer 580

1. Need for feeding tube → gastrostomy, GJ 2. Type of wrap → Nissen, loose Nissen, Toupet, Dor 3. Need for pyloroplasty → if delayed gastric emptying but unclear if improved outcomes 4. Presence of hiatal hernia 5. Presence of rotational anomalies 6. Presence of other esophageal anomalies → web, CES, stricture, achalasia 7. Size of stomach → especially if type A 8. Existing gastrostomy → placement of gastrostomy and whether it precludes fundoplication without taking down gastrostomy

Question 581

Name FOUR complications of blunt hepatic injury and their treatments

Answer 581

1. Bleeding (ongoing or recurrent) → transfusion if HD unstable 2. Pseudoaneurysm 1-2% → angiography and coiling; up to 17% incidence, which warrants repeat imaging for grade 4/5 3. Hepatic cyst → laparoscopic aspiration and fenestration of the cyst 4. Bile leak or biloma → ERCP ± drainage (percutaneous or laparoscopic); exclusively in grades 3+ 5. Hepatic necrosis → conservative management ± liver resection 6. Gallbladder ischemia → cholecystectomy

Question 582

What are the AAST grades for Hepatic injury?

Answer 582

I – laceration or subcapsular hematoma <1 cm II – laceration or subcapsular or central hematoma 1-3 cm III – laceration or subcapsular or central hematoma 3-10 cm IV – laceration or subcapsular or central hematoma > 10 cm; lobar maceration or devitalization V – bilobar maceration or devitalization

Question 583

Regarding Tracheomalacia: a) What is the best way to diagnose? b) What is the pathognomonic finding? c) What are the surgical options for treatment (list 3)?

Answer 583

a) Bronchoscopy during spontaneous respiration b) anterior tracheal wall touches posterior wall c) Aortopexy, innominate artery suspension/repositioning, tracheostomy, correct vascular ring, stent

Question 584

Define Congenital pulmonary lymphangiectasia

Answer 584

a condition in which the interlobar and subpleural pulmonary lymphatic vessels are dilated and prone to leakage. This may occur due to the failure of pulmonary interstitial tissues to appropriately regress during fetal life. Lymphangiectasia may occur sporadically or in association with a variety of disorders including T21, Noonan, Turner syndromes. Requires biopsy to confirm diagnosis.

Question 585

How is chylothorax diagnosed?

Answer 585

Diagnosis based on analysis of the pleural fluid obtained by thoracentesis or tube thoracostomy. Chylous fluid appears straw coloured in chronically fasted pts, but turns milky with enteral feeding. A pleural fluid cell count greater than 1000 cells per microliter with more than 80% lymphocytes is diagnostic for a chylothorax. In older non-fasted children, chylomicrons and a triglyceride level of more than 110 mg/dL supports the diagnosis.

Question 586

A 15 year-old presents with a large 10x15cm chest wall mass with rib involvement, a pleural effusion, and a small nodule on the ipsilateral lung. What is the most likely diagnosis?

Answer 586

Diagnosis - Chest wall Ewing Sarcoma

Question 587

What percentage of total bilirubin does the direct need to be before it is considered cholestatic jaundice?

Answer 587

>20%

Question 588

List FOUR causes of indirect hyperbilirubinemia

Answer 588

1. Physiologic jaundice 2. RBC hemolysis 3. gilbert syndrome 4. Crigler Najjar syndrome 5. breast milk jaundice 6. G6PD deficiency

Question 589

Describe the histologic findings of biliary atresia on liver biopsy

Answer 589

4 Ps → portal expansion, portal fibrosis, ductal proliferation, and bile plugs.

Question 590

Name SIX entities on the differential diagnosis of biliary atresia

Answer 590

1. choledochal cyst 2. gallstones or biliary sludge 3. Alagille syndrome 4. CF 5. neonatal sclerosing cholangitis 6. congenital hepatic fibrosis/Caroli’s 7. idiopathic neonatal hepatitis (26%) 8. infection/sepsis (TORCH, HIV, UTI, Syphillis) 9. α1-antitrypsin deficiency (4.1%) 10. congenital portosystemic shunt

Question 591

What are the finding on ultrasound that indicate biliary atresia?

Answer 591

Absent gallbladder is highly specific for BA but presence of a gallbladder does not exclude BA(10 to 20% have a patent CBD). A triangular echogenic density seen just above the porta hepatis (triangular cord sign) is highly suggestive of BA

Question 592

What is the value of a HIDA scan in the work up of biliary atresia?

Answer 592

drainage of 99m technetium into intestine from the liver proves bile duct patency. Takes 3-5 days to premed w/ phenobarbital and has high NPV and low PPV (only helps exclude but can prevent negative laparotomy)

Question 593

Name TWO entities that could lead to a false positive interpretation on biopsy for biliary atresia and TWO factors that could lead to a false negative

Answer 593

False positive interpretation: parenteral nutrition associated liver disease, α1-antitrypsin deficiency False negative interpretation: early age at diagnosis (4 to 6 weeks), small/inadequate sample (5 to 6 portal tracts)

Question 594

Describe in words or pictures FIVE named stomas appropriate for complicated atresia

Answer 594

1. bishop koop – distal stoma 2. santuli – proximal stoma 3. double barrel – divided bowel placed side by side 4. Rehbein – tube anastomotic stoma 5. Mikulicz - anastomosis and double stoma

Question 595

What is the most common type of abdominal lymphoma? What virus is associated? In a patient with intussusception found to have a pathologic lead point consistent with lymphoma in the ileum, what is the surgical management?

Answer 595

Burkitt’s is the most common type of abdominal lymphoma. Caused by EBV. Surgical management is biopsy initially. If it found during a OR for intussusception, performa a primary resection with involved mesenteric lymph nodes. Send pathology fresh.

Question 596

What bacteria (list 3 in order of frequency) are most commonly found in a short bowel pt presenting with a line infection? What empiric antibiotics should be started? What are some indications for line removal?

Answer 596

Bacteria: Klebsiella, enterococcus coli, coag-negative staphylococcus (CONS). Empiric antibiotics: piptazo, vanco. Indications for removal: HD instability, fungal, staph aureus (makes a biofilm), 3 consecutive days of positive line cultures, inflammation over the line.

Question 597

What on FOUR things on history would make you suspect vWD?

Answer 597

1. Family history of bleeding disorder 2. mucosal bleeding 3. excessive nose bleeds 4. menorrhagia 5. blood in stool, hematuria 6. easy bruising/lumpy bruises 7. excessive bleeding after injury/surgery or dental work

Question 598

How do you diagnose vWD?

Answer 598

VWF antigen test (VWD typically have < 50% normal VWF in plasma), factor VIII clotting activity, Von Willebrand factor multimers – test to see which type, bleeding time

Question 599

What preoperative preparations need to be made for patients with vWD?

Answer 599

DDAVP with fluid restriction, replacement therapies – Humate-P - factor VIII and vWF, rvWF; clot stabilizing medications (TXA, aminocaproic acid)

Question 600

List and describe TWO mechanisms of intestinal adaptation

Answer 600

1. Structural adaptation- morphologic- increasing bowel diameter and length, lengthening of villi, deepening of crypts, increasing rate of enterocyte proliferation (hyperplasia) -> results in increased absorptive surface area and increased numbers of enterocytes 2. Functional adaptation- modifications of brush border membrane permeability and upregulation of carrier-mediated transport -> results in increased nutrient absorption by isolated enterocytes

Question 601

Which of the following are contraindicated when using mono-polar electrocautery? a) Cochlear implant b) Cardiac pacemaker c) Piercings d) Vagal stimulator for seizure control

Answer 601

a) Cochlear implant → contraindicated b) Cardiac pacemaker → contraindicated but can turn off before surgery and reprogram if pt not pace-dependent c) Piercings → not contraindicated as long as functional grounding pad; Burns can occur where cardiac monitor leads and body jewellery are located. If monopolar diathermy needs to be used, place the return electrode as far from the implanted device and as close to the surgical site as possible. d) Vagal stimulator for seizure control → contraindicated but can turn off before surgery and reprogram if pt not at risk of seizing during procedure, which in theory they should not be because of general anesthesia medication

Question 602

List THREE complications of surgical management in torticollis

Answer 602

1) damage to nerves (spinal accessory and brachial plexus) 2) hematoma if proper hemostasis not achieved 3) recurrence/persistence if incomplete division of both heads and cervical fascia

Question 603

Why does thermal injury occur in laparoscopy with non-touching instruments?

Answer 603

The most common inadvertent thermal injuries caused by monopolar surgical instruments during laparoscopic surgery arise because of inadvertent tissue contact, insulation failure, direct coupling, and capacitive coupling.

Question 604

Define direct coupling

Answer 604

Direct coupling: can occur if the tip of the active electrode comes in direct contact with (or very close to) another metal instrument or conductor within the surgical field. For example, if the active electrode accidentally touches or arcs to the laparoscope, the entire laparoscope becomes electrified

Question 605

Define capacitance coupling

Answer 605

Capacitive coupling: induction of stray current to a surrounding conductor through the intact insulation of an active electrode. For example, charge from active electrode → insulation on electrode → metal trocar sheath).

Question 606

History of inability to access port with image of a flipped port. Management?

Answer 606

Incise over chamber, dissect it out, and correct its position or replace it if impossible.

Question 607

History of initially being able to infuse Hickman but never being able to draw back. Subsequently, you lose the ability to infuse as well. You decide to remove the Hickman in the OR but when you remove the line it flushes normally. You image the vessel with an ultrasound right after removal and it looks like there is a valve or a false passage. Why was the line not working and how could you have saved it without replacing it?

Answer 607

This was likely caused by a fibrin sheath, which could have been managed with fibrinolysis (alteplase/TPA). A pro-coagulative state can lead to fibrin sheath formation around the catheter, which may result in increased flow resistance during infusions. Short infusions of thrombolytics restore tube patency with a high success rate. However, such a fibrin sheath is also the breeding ground for microorganisms and subsequent biofilm formation and infections

Question 608

Image of externally fractured catheter. Management

Answer 608

Use catheter repair kit (specific type for each manufacturer); fracture must be far away from the skin (~5cm)

Question 609

Image of tubing disconnected from reservoir on the lower chest. Management

Answer 609

Incise over neck, control the distal catheter, feed wire through and confirm position, then replace entire system (chamber and catheter). • Catheter fracture is often caused by the catheter being pinched off at thoracic inlet (between the clavicle and first rib).

Question 610

An 8 month old girl presents with an adrenal mass. She is found to have elevated cortisol pre-operatively. Pathology confirms an adrenocortical carcinoma (ACC). How is this tumour managed? What are the results of surgery compared to chemotherapy?

Answer 610

Surgery is the mainstay of treatment of ACC; complete surgical resection of the primary lesion and any extension to contiguous lymph nodes or organs provides the best chance at survival and should be undertaken even if there is locally advanced disease. If the tumour is stage 2 or higher, the pt will receive mitotane and chemo (PED→cisplatin, etoposide, doxorubicin). Mitotane is adrenolytic agent that may delay tumour progression; side effects = anorexia, n/v. Adjuvant chemotherapy for ACC is not curative and is only used for metastatic disease or residual disease not amenable to surgical resection. May prolong survival

Question 611

What is the difference between true precocious puberty and incomplete or pseudoprecocious puberty.

Answer 611

True precocious puberty is due to premature maturation of the hypothalamic–pituitary axis and results in gonadal enlargement and premature development of secondary sexual characteristics due to pituitary gonadotropin secretion; secondary sexual characteristics are appropriate for sex of the child and merely occur at a younger-than-appropriate age. Incomplete or pseudoprecocious puberty is due to production of human chorionic gonadotropin (hCG), luteinizing hormone (LH), follicles stimulating hormone (FSH), androgens or estrogens, or it is due to stimulation of their receptors by tumors; only one secondary sexual characteristic develops prematurely and this may or may not be appropriate for the pt’s gender

Question 612

What is Ollier Disease?

Answer 612

Disorder characterized by multiple enchondromas, which are benign tumours of cartilage; these enchondromas most commonly occur in the limb bones, especially in the bones of the hands and feet; however, they may also occur in the skull, ribs, and vertebrae; may result in severe bone deformities, limb discrepancy, and fractures; association with chondrosarcomas (especially of the skull), liver tumours, and ovarian neoplasms, particularly juvenile granulosa cell tumours

Question 613

8 year old girl with precocious puberty. No visual disturbance. Thyroid normal. Not on any meds. a. If you can only send two assays what would they be? b. What imaging studies would be appropriate in the work up

Answer 613

a. Serum hormone measurement, gonadotropin RH | b. US abdo/pelvis; brain MRI, bone age

Question 614

Describe the embryology of testicular descent and it’s relation to the development of an inguinal hernia

Answer 614

starts near kidney(peri-nephric area), Testes follow the path of the gubernaculum. Travels through processus vaginalis at internal ring at 7-9 months gestation, travels through inguinal canal through external ring and descends into scrotum. Causes processus to elongate, usually processus above testis obliterates and distal portion becomes tunica vaginalis. Failure of processus vaginalis to obliterate leaves an inguinal hernia

Question 615

List FOUR intraop complications and FOUR postop complications of inguinal hernia complications

Answer 615

Intraop: 1. Injury to vas deferens → if divided, call urology ± repair with 8-0 monofilament 2. Injury to testicular vessels → can lead to testicular atrophy 3. Injury to ilioiguinal nerve 4. Bleeding → control needle-hole to epigastric vessels or femoral vein by removing suture and placing pressure Postop: 1. Wound infection 2. Scrotal hematoma 3. Postoperative hydrocele → due to incomplete resection of distal sac 4. Recurrence → <1%

Question 616

Name FOUR clinical features Alagille syndrome

Answer 616

1. pulmonic stenosis (and tetralogy of Fallot) 2. distinctive facial features (broad, prominent forehead; deep set eyes; small, pointed chin) 3. butterfly vertebrae 4. bile duct paucity (narrow, malformed, reduced in number, causing cholestasis, scarring, fibrosis)

Question 617

Describe the management of Alagille Syndrome

Answer 617

Conservative Mgt – Treat pruritis (ursodiol, rifampin, naltrexone, bile sequestrants – cholestyramine, colesevelam) and malabsorption (ADEK supplementation and high calorie feeds). Transplant if refractory puritis and portal HTN

Question 618

From most to least frequent, list the causes of an acute scrotum

Answer 618

* Torsion of the appendix testis (Hydatid of Morgagni) or appendix epididymis * Epididymitis/orchitis * Testicular torsion * Hernia/hydrocele * Trauma/sexual abuse * Tumour * Idiopathic scrotal edema (dermatitis, insect bite) * Cellulitis * Vasculitis

Question 619

What are the ultrasound findings of testicular rupture?

Answer 619

* hematocele is usually present * disruption of the tunica albuginea, which is normally a smooth echogenic line (signs of disruption include loss of continuity, crinkling, or retraction) * extrusion of the seminiferous tubules can occur (and may mimic a complex hematocele)

Question 620

Ultrasound findings of Testicular ischemia/infarct

Answer 620

* a heterogenous testis suggests patchy testicular ischemia or infarct * ultrasound imaging features are similar to testicular torsion but the pathogenesis is different; post-traumatic infarct is due to increased intra-testicular pressure resulting in venous obstruction and venous infarction

Question 621

A 3 day old presents with frank hematemesis. List the THREE most common causes of neonatal hematemesis along with the appropriate diagnostic test and management of each problem. If you feel like it, list FIVE others!

Answer 621

1. Swallowing of maternal blood → alkali denaturation test (Apt test) used to differentiate fetal or neonatal blood from maternal blood found; supportive care. 2. Hemorrhagic disease of newborn/vitamin K deficiency → history of not receiving prophylactic vitamin K at birth; treat by giving oral or IM vitamin K ± transfusion FFP ± transfusion PRBCs. 3. Stress gastritis or ulcer (critically ill infant or CMV) → EGD; treat with PPI, supportive care ± transfusion PRBCs. 4. Esophagitis 5. Trauma (NG tube) 6. Vascular anomalies (hemangioma, telangiectasia, other) 7. Esophageal, gastric, or duodenal duplications 8. Coagulopathy (immune mediated or secondary to infection, liver failure, or congenital) 9. Cow’s milk protein allergy (CMPA)

Question 622

A patient is undergoing air reduction enema for ileocollic intussusception. a. What are the signs of abdominal tension pneumoperitontum? b. How would you manage it? c. What is the maximal pressure that should be used during the procedure?

Answer 622

a. hypotension/decompensation, decreased lower limb perfusion, tense abdominal distension b. Percutaneous needle decompression w/ 18 gauge needle, enema discontinued and rectum decompressed w/ enema tube, need to go to OR for laparotomy, reduction, resection, anastomosis c. 120 mmHg

Question 623

Intussusceptum vs. Intussuscipiens

Answer 623

Intussusceptum - the bowel that telescopes IN | Intussuscipiens - the recipient bowel

Question 624

15F post cadaveric renal transplant. Comes to ED with fever, RLQ pain. On immunosuppression. U/A positive. WBC on CBC List FIVE things on the differential diagnosis

Answer 624

1. UTI - pyelonephritis 2. CMV infection 3. BK infection 4. graft failure/loss 5. renal vascular thrombosis 6. chronic rejection 7. recurrence of primary disease 8. acute rejection 9. discontinuation of immunosuppression 10. PTLD 11. appendicitis

Question 625

What are the THREE stages of Parapneumonic effusions and empyema

Answer 625

1. Exudative stage: characterized by a thin, sterile, pleural exudate. 2. Fibrinopurulent stage: fluid becomes turbid and loculated, with a fibrinous pleural peel. 3. Organizational stage: thick exudate and an organized, fibrous pleural peel encasing and trapping the lung and rendering it immobile.

Question 626

List FOUR complications of necrotizing pneumonia

Answer 626

1. Pleural effusion causing respiratory distress 2. Loculated effusion or empyema 3. Pneumatocele 4. Lung Abscess 5. Bronchopleural fistula

Question 627

Describe the medical treatment of empyema

Answer 627

Hydration, broad spectrum Abx, drainage of effusion and intrapleural fibrinolysis.

Question 628

What are the indications for surgery in patients with empyema?

Answer 628

Failure of intrapleural fibrinolytic therapy is the most commonly accepted indication for video assisted thoracic surgery in a child with parapneumonic effusion or empyema. VATS may also be indicated in a child with a contraindication to intrapleural fibrinolysis (e.g. necrotizing pneumonia, peripheral bronchopleural fistula, pyopneumothorax). Early tube thoracostomy insertion is recommended for patients in moderate to severe respiratory distress (e.g. increased work of breathing, tachypnea, hypoxia) even with smaller effusion

Question 629

What is a pleomorphic adenoma of the parotid? Discuss the management

Answer 629

Most common benign non vascular tumor in kids; firm rubbery mass; avg age at presentation 9.5 yrs; painless, slow growing; US variable echogenicity, increased echogenicity in larger lesions secondary to necrosis/cystic change. Management superficial parotidectomy with facial nerve identification & preservation; high recurrence rate, so need long term follow up; simple excisional biopsy have even higher recurrence rates

Question 630

What is the medical and surgical management of fecal incontinence in spina bifida.

Answer 630

Med management - behavior modification, biofeedback, daily bowel routine w/ enema/suppositories to effect clearance Surgical management - cecostomy for antegrade enemas to allow for clearance of colon; colostomy

Question 631

Why is High-frequency oscillatory ventilation (HFOV) used? and what is the mechanism of gas exchange?

Answer 631

High-frequency oscillatory ventilation (HFOV) is a lung protective ventilation & oxygenation strategy. HFOV uses a constant distending pressure (mean airway pressure = MAP) with pressure variations oscillating around the MAP at very high rates—up to 900 cycles per minute. This creates small tidal volumes (less than the dead space of the lung and the circuit). Mechanisms of gas exchange: radial mixing (Taylor dispersion), cardiogenic mixing, collateral ventilation, Pendelluft ventilation, and coaxial flow.

Question 632

What are the FOUR sub competencies of professionalism in CANMEDS?

Answer 632

Commitment to: 1. Patients 2. Society 3. Profession 4. Self

Question 633

What are the FOUR sub competencies of Scholar in CANMEDS?

Answer 633

1. Life-long learning 2. Teacher 3. Evidence-informed decision making 4. Research

Question 634

What are the CANMEDS competencies? Remember: Please correctly count little hens squawking merrily!

Answer 634

``` Medical Expert (the integrating role) Communicator Collaborator Leader Health Advocate Scholar Professional ```

Question 635

Indications of for transplant in Hepatoblastoma

Answer 635

1. PRE-TEXT 3 or 4 with extensive multifocal disease 2. POST-TEXT 3, annotation factors + 3. POST-TEXT 4 4. Unresectable by conventional approach (proximity/involvement of major vessels) 5. Unresectable local relapse 6. Underlying cirrhosis with insufficient liver volume to sustain post-resection liver function (especially relevant for familial hepatic cholestasis)

Question 636

Photo of a 3-week old baby with numerous blueish skin lesions on the head. The baby is lethargic, doesn’t feed well, and seems to have a tender and distended belly. List your differential diagnosis and indicate which is the most likely.

Answer 636

MS neuroblastoma (most likely), congenital leukemia, TORCH (rubella), hemolytic diseases, infantile hemangiomas, congenital hemangiomas.

Question 637

EIGHT pathologic lead points for intussusception

Answer 637

1. Meckel’s 2. Duplications 3. Vascular malformations (intramural hemangioma) 4. Ectopic gastric or pancreatic tissue 4. Polyps (hamartomatous/juvenile, adenomatous, lymphoid) 6. Foreign bodies (NJ tips) 7. Lymphoma and other intestinal tumours 8. HSP (bowel wall hematomas) 9. Appendix (mucocele) 10. Viscous intestinal contents in CF

Question 638

Classic presentation of intussusception

Answer 638

intermittent bouts of abdominal pain with intervening lethargic periods, vomiting, and currant-jelly stools (stool mixed with blood and mucous due to ischemic mucosal sloughing and mucous secretion → late sign); note that the intussusceptum suffers first as mesentery is incarcerated, leading to venous congestion and obstruction, arterial obstruction, and necrosis; increasing pressure on the intussuscipiens may also lead to necrosis

Question 639

Management of Intussusception

Answer 639

once diagnosed, treatment is pneumatic reduction as long as the pt is HD stable, does not have peritonitis, and AXR does not show free air; if unsuccessful, can proceed with second attempt after short waiting period or proceed to operative reduction; if lead point has been identified, pneumatic reduction can still be helpful if long intussuscepting segment in order to make OR easier; recurrent intussusception, which occurs in 10% and commonly in first 24 hours, can be treated with recurrent pneumatic reductions, but thought should be given to presence of a lead point.

Question 640

Vienna Classification for Crohn's

Answer 640

(1) inflammatory (most common, responsive to medications) (2) fibrostenotic (least responsive to medications) (3) penetrating (i.e. fistulizing)

Question 641

What are the advantages and disadvantages of Savary-Gilliard dilations

Answer 641

Basics: dilation performed over a guide wire with fluoroscopic guidance. Advantages: more gradual dilation, cheaper because reusable; may be helpful for pts who have chronic need for dilations. Disadvantages: tangential force, higher perforation rate? (depends where you look)

Question 642

What are the advantages and disadvantages of Pneumatic dilations

Answer 642

Advantages: radial force, decreased perforation rate? (depends where you look) Disadvantages: cost, requires technical expertise and equipment, increased fluoroscopy time and thus radiation exposure.

Question 643

Grades of Blunt Aortic Injury

Answer 643

Grade I – intimal tear (minimal aortic injury) Grade II – intramural hematoma or large intimal tear Grade III – aortic transection with pseudoaneurysm Grade IV – free rupture

Question 644

Management of Grade I blunt aortic injury

Answer 644

Type I injuries (intimal tear) - nonoperative management w/ aggressive management to maintain heart rate <100 beats per minute, blood pressure <100 mmHg, and serial imaging.

Question 645

Management of Grade II-IV blunt aortic injury

Answer 645

Repair with aggressive medical mgt while waiting. Although the traditional therapy for blunt traumatic rupture of the thoracic aorta is immediate repair, in some patients with concomitant head trauma, respiratory failure, cardiac dysfunction or sepsis, this injury can be managed conservatively with selective delayed operative repair without increasing the risk for exsanguinating hemorrhage

Question 646

List EIGHT GI Complications of Cystic Fibrosis

Answer 646

1. GERD 2. Malnutrition 3. Pancreatic Insufficiency 4. Meconium ileus 5. DIOS 6. Rectal Prolapse 7. Appendiceal Mucocele 8. Intussusception 9. Cirrhosis and Portal Hypertension 10. Biliary Perforation

Question 647

List the factors associated with improved outcomes in Biliary atresia

Answer 647

* Age at portoenterostomy → surrogate marker for extent of liver damage; Kasai <70-90 days optimal; consideration of primary liver transplant if >90 days. * Centralization → positive outcomes in the UK. * Cystic biliary atresia

Question 648

List the factors associated with worse outcomes in Biliary atresia

Answer 648

* >90 days at the time of portoenterostomy * BASM or other syndromic. * CMV-positive status. * Ascites or nodular liver at the time of Kasai.

Question 649

Discuss mechanisms to reduce postop cholangitis?

Answer 649

Consensus to continue IV antibiotics x 5-7 days ± discharge on TMP-SMX as prophylaxis (some concern for selection of resistant organisms). Steroid protocols are controversial; RCT showed no overall benefit but in infants operated <70 days, 25% improvement in jaundice clearance. Note that pts should be sent home on ADEK vitamins (increased risk of long-bone fractures due to vitamin D deficiency) ± ursodiol once bilirubin is coming down.

Question 650

What is congenital hyperinsulinism?

Answer 650

Congenital hyperinsulinism (HI) is the leading cause of persistent hypoglycemia in infants and children. HI results in severe and recurrent hypoglycemia, as well as an inability to make ketone bodies, a crucial alternative fuel for the brain.

Question 651

What are the TWO types of Congenital Hyperinsulinism? How do you diagnose it?

Answer 651

diffuse and focal How to you diagnose - fasting and postprandial hypoglycemia with unsuppressed hyperinsulinism in which neonatal hypoglycemia is defined as a glucose plasma concentration of less than 50 mg/dL after the first 24 hours of life with a simultaneous plasma insulin concentration greater than 36 pmol/L

Question 652

How can you differentiate focal vs. diffuse?

Answer 652

IF genetic testing confirms diffuse HI (one mutation on the maternal allele plus one mutation on the paternal allele SUR1/Kir6.2 complex) no imaging studies are necessary. IF genetic testing suggestive of focal HI -->Need 18F-DOPA PET/CT scan to confirm Dx of focal and localize the lesion (accuracy is ~100%) IF genetic test is inconclusive-->DOPA

Question 653

Regarding pectus carinatum : a) What is the success rate for bracing? b) What is the most frequent cause of bracing failure?

Answer 653

a) 80% | b) Non-compliance

Question 654

Chondrogladiolar Pectus Carinatum vs. Chondromanubrial

Answer 654

Chondrogladiolar PC is the more common type and refers to protrusion of the body of the sternum. This is often accompanied by lateral depression of the ribs, termed runnels or Harrison Grooves. Chondromanubrial PC refers to protrusion of the manubrium and superior costal cartilages with a relative depression of the body of the sternum and represents less than one percent of all patients with PC.

Question 655

Describe a bracing protocol for pectus carinatum

Answer 655

The brace is worn for 23 hours a day until corrected. (can take 2-6 months on average) The maintenance phase is then 8-14 hours a day while still growing; can be x 12-18 mos. While chest wall rigidity, age, and type of pectus carinatum (symmetric versus asymmetric) all influence outcomes, the most important factor is compliance.

Question 656

How are the Haller Index and Correction Index calculated?

Answer 656

Haller Index – ratio of transvers dimension of chest over AP distance from back of sternum to anterior edge of vertebrae. Dependent on transverse dimension being normal range. Ratio >3.2 suggests OR needed. Haller index = a/b Correction index- more reliable metric performed by drawing a horizontal line across the posterior aspect of corrected sternum. CI = [(A – B)/B] × 100. >10% to justify correction. More generalizable than Haller.

Question 657

FOUR complications of Nuss procedure

Answer 657

1. Bleeding – low risk overall, hemothorax rate =0.6%, cardiac perf rare. Intercostal or internal mammary bleeds are usually the source. 2. Bar Displacement – incidence is 3-5%; rates have improved w/ bar stabilizers. Mechanisms for displacement include bar flipping, hinge point disruption or a lateral sliding. Management includes multiple point fixation or bar removal. 3. Metal or nickel allergy - 2.2% to 6.4%. **Titanium bars need to be pre-bent in factory and are ++expensive** 4. Complication of bar removal - wound infection or hematoma. bleeding from internal mammary artery, cardiac laceration, sternal erosion have been reported

Question 658

Define what a giant congenital nevus is

Answer 658

large clusters pigment producing cells of melanocyte lineage clustered in dermis and produces pigment at birth; if compared to acquired nevus- they are larger, increased cellularity and lie deeper in dermis; giant congenital nevus is >20 cm

Question 659

Classification of Giant Congenital Nevus

Answer 659

classified by size: small: <1.5 cm, medium 1.5 – 19.9 cm, giant/large >20 cm

Question 660

FOUR reasons for false negative Meckel Scan

Answer 660

1. Meckel’s without ectopic gastric mucosa. 2. High bleeding rate. 3. Poor blood supply Meckel’s. 4. Proximity to bladder (which can obscure). 5. Aluminum hydroxide (found in some anti-ulcer medications, limits the mucosal localization of radiotracer). 6. Barium study.

Question 661

FOUR Reasons for false POSITIVE in a Meckel scan

Answer 661

1. intestinal gastric heterotopia 2. duplication cysts 3. obstructed loops of bowel 4. intussusception 5. inflammatory lesions 6. arteriovenous malformations 7. ulcers 8. some intestinal neoplasms

Question 662

FOUR things on the differential for a teenager with leukemia and neutropenia presenting with RLQ pain and fever

Answer 662

1. neutropenic enterocolitis 2. acute appendicitis 3. graft versus host disease 4. pseudomembranous colitis 5. infectious colitis 6. veno-occlusive disease 7. IBD

Question 663

Medical Treatment of Typhlitis

Answer 663

Medical treatment: broad-spectrum antibiotics, NPO, NG to suction, parenteral nutrition ± fluid resuscitation & vasopressors if shock; role of G-CSF unclear → current evidence supports its use in pts with profound neutropenia (ANC <100), uncontrolled disease, multisystem organ failure and invasive fungal infection; consider fungal coverage.

Question 664

FIVE indications for EXIT procedure

Answer 664

1. Large high risk congenital lung lesion that fail to respond to in utero maneuvers (steroids for microcystic and thoracoamniotic shunt for macrocystic) 2. High risk SCT (hydrops, AV shunting causing high output cardiac failure) 3. Cervical teratoma causing airway obstruction, high-output cardiac failure 4. Congenital high airway obstruction – overdistension by lung fluid; 5. High risk CDH who have had fetal surgery (tracheal occlusion);

Question 665

THREE features of a midline cervical clefts

Answer 665

1. Nipple-like skin tag in the cranial portion 2. Sinus tract or fistula in the caudal portion 3. Midline cleft resembling a skin defect in between.

Question 666

Management of midline cervical cleft

Answer 666

Excision is warranted for cosmesis and to avoid neck contractures. Simple excision and closure will invariably lead to contracture, therefore Z-plasties are required. Avoid neck extension. Wise to involve plastic surgery.

Question 667

Hemolytic Disease of the newborn - Define

Answer 667

most frequent cause is Rh incompatibility but can also happen with ABO incompatibility (usually less severe), or more rare blood types (Kell, Duff); destruction of red blood cells in the neonate or fetus by maternal IgG antibodies → happens when fetal erythrocytes gain access to maternal circulation; maternal sensitization occurs if previous pregnancy with Rh+ offspring or previous transfusion with Rh+ blood cells (creates antibodies against Rh+); women are now given RhoGam to prevent this from happening.

Question 668

Treatment of Hemolytic Disease of the newborn

Answer 668

Treat the anemia and hyperbilirubinemia caused by the hemolysis; if severe anemia or hyperbilirubinemia, may need exchange transfusion; mild anemia can be treated with EPO; late onset anemia treated with Fe, transfusion as needed; hydration and phototherapy; severe may need IVIG.

Question 669

Describe a Meso-Rex Bypass

Answer 669

Meso-Rex Bypass: treatment of choice for extra-hepatic portal vein obstruction but cannot be used with parenchymal liver disease; bypasses the obstructed extrahepatic portal vein and redirects flow to the intrahepatic portal venous system; vein graft (IJ) between SMV and intrahepatic portal vein.

Question 670

Describe Distal Spleno-Renal Shunt (selective)

Answer 670

treatment of choice for portal HTN from congenital hepatic fibrosis and compensated cirrhosis; ligate splenic vein close to the SMV and then anastomose splenic vein to renal vein; this allows for selective variceal decompression into the renal vein.

Question 671

Myasthenia Gravis neurotransmitter

Answer 671

acetylcholinesterase

Question 672

Symptoms of myasthenia gravis

Answer 672

There are two clinical forms of myasthenia gravis: ocular and generalized. In ocular myasthenia, the weakness is limited to the eyelids and extraocular muscles. In generalized disease, the weakness may also commonly affect ocular muscles, but it also involves a variable combination of bulbar, limb, and respiratory muscles. The most common symptoms of myasthenia gravis include ptosis and diplopia. Approximately 10-15% of those with myasthenia gravis have an underlying thymoma.

Question 673

Treatment options for myasthenia gravis

Answer 673

* Symptomatic therapy with an acetylcholinesterase inhibitor → pyridostigmine * Chronic immunosuppressive therapies * Rapid but transient immunomodulatory therapies (plasma exchange and IVIG) * Thymectomy

Question 674

A previously well 13 year old male has been experiencing bilious vomiting for weeks. He has an UGI which shows massive dilation of the duodenum but does have contrast distally. You take the pt to the OR but do not find any anatomic reason for the duodenal dilation. You take biopsies but these are non-diagnostic on pathology. (I think there was a part about the child being skinny and that you leave an NJ at the end of the surgery for feeds?) What is the most likely diagnosis?

Answer 674

This is mostly likely superior mesenteric artery (SMA) syndrome, an unusual cause of proximal intestinal obstruction. The syndrome is characterized by compression of the third portion of the duodenum due to narrowing of the space between the superior mesenteric artery and aorta and is primarily attributed to loss of the intervening mesenteric fat pad. There remains some controversy surrounding a diagnosis of SMA syndrome since symptoms do not always correlate well with abnormal anatomic findings on radiologic studies, and symptoms may not resolve completely following treatment. Furthermore, the diagnosis may be confused with other anatomic or motility-related causes of duodenal obstruction.

Question 675

Imaging Criteria for SMA syndrome

Answer 675

* Duodenal obstruction with an abrupt cutoff in the third portion and active peristalsis. * An aortomesenteric artery angle of ≤25° is the most sensitive measure of diagnosis, particularly if the aortomesenteric distance is ≤8 mm. * High fixation of the duodenum by the ligament of Treitz, the abnormally low origin of the superior mesenteric artery, or anomalies of the superior mesenteric artery.

Question 676

List FOUR things on the differential for SMA syndrome

Answer 676

1. Malrotation 2. Fenestrated duodenal web 3. Annular pancreas 4. Preduodenal portal vein 5. Duodenal dysmotility and "megaduodenum" due to DM, collagen vascular diseases, scleroderma, and chronic idiopathic intestinal pseudo-obstruction.

Question 677

What factors contribute to SMA syndrome?

Answer 677

The most common is significant weight loss leading to loss of the mesenteric fat pad as a consequence of medical disorders, psychological disorders, or surgery. In younger pts, most commonly described following corrective spinal surgery for scoliosis (cast syndrome). This procedure lengthens the spine cranially, displacing the superior mesenteric artery origin, decreasing the mesenteric artery's lateral mobility, and reducing the acuteness of the aortomesenteric angle.

Question 678

What is the medical and surgical management of SMA syndrome?

Answer 678

Medical: NG tube, correct electrolyte abnormalities, nutritional support with NJ feeds, ± psychiatric evaluation. Surgical: Strong's procedure (a division of LoT), gastrojejunostomy, or duodenojejunostomy ± division or resection of the fourth part of the duodenum.

Question 679

Risk Factors, Treatment, and prevention of MRSA

Answer 679

Risk factors: aboriginal communities, sports teams, child-care centres, crowding (detention centres). Antibiotics: vancomycin, linezolid, daptomycin, clindamycin, TMP-SMX. Prevention of transmission: adhere to infection control contact precautions (gown + gloves) for pts with known MRSA infection, routine hand-washing, decolonization regimes, surveillance cultures (routine practice is controversial), reducing broad-spectrum antibiotic use.

Question 680

List SIX causes of an ingrown toe nail

Answer 680

1. Cutting toenails incorrectly (cut straight across and not angulated because this can encourage the nail to grow into the skin). 2. Irregular, curved toenails. 3. Footwear that places a lot of pressure on the big toes. 4. Toenail injury, including stubbing your toe, dropping something heavy on your foot, or kicking a ball repeatedly. 5. Improper foot hygiene, such as not keeping your feet clean or dry. 6. Genetic predisposition

Question 681

List FOUR management options for ingrown toenail

Answer 681

1. Foot soaks TID with pushing the lateral nail fold away from the nail plate. 2. Toe taping. 3. Nail bracing (hook, adhesive, or composite). 4. Nail avulsion and chemical matricectomy (phenol) → lateral wedge excision or Vandenbos.

Question 682

What is pseudosubluxation of the cervical spine?

Answer 682

Pseudosubluxation of the cervical spine is the physiological anterior displacement of C2 on C3 in children. It is common in children <7 years (19% children 1-7 years). Can also be seen at C3 on C4. It is more pronounced in flexion and is of clinical significance as it can be confused with traumatic cervical injury. Immature lax ligaments are considered the cause of this appearance. Can be differentiated from true subluxation based on a normal relationship of the upper cervical spine using Swischuk’s line and the absence of prevertebral soft tissue swelling on a lateral c-spine x-ray with flexion/extension views.

Question 683

Structures going into umbilicus, specifically how does the allantois and urachus develop?

Answer 683

Structures going into umbilicus: • 1 umbilical vein → becomes ligamentum teres. • 2 umbilical arteries → become medial umbilical ligaments. • Allantois → fetal urachus → becomes median umbilical ligament. The allantois is an extension of the posterior wall of the yolk sac. Its exact role in development is unclear, though its blood vessels do become the umbilical blood vessels. It gives rise to a structure called the urachus which contributes to the superior wall of the urinary bladder

Question 684

A baby presents with a hyper-inflated RML. They are not intubated. What is the diagnosis?

Answer 684

This is most likely congenital lobar emphysema (CLE) with air-trapping through a ball-valve effect → air enters lobe during active inspiration but is trapped when the bronchus collapses or is obstructed during passive expiration, leading to significant hyperinflation. Etiology is intrinsic (underdeveloped or hypoplastic cartilage that collapses with expiration) or extrinsic (mediastinal masses such as bronchogenic or foregut duplication cysts or vascular anomalies that compress bronchus externally).

Question 685

What is the risk of intubating a patient with CLE?

Answer 685

Intubation and positive pressure ventilation can lead to severe clinical deterioration. This is due to a ball-valve effect that results in severe air-trapping in some pts with CLE. Contralateral bronchial mainstem intubation or HFO ventilation may be required.

Question 686

What are the zones of the neck?

Answer 686

* Zone 3: angle of the mandible to the base of the skull * Zone 2: cricoid to angle of the mandible to the clavicle * Zone 1: clavicles to the cricoid

Question 687

What conditions are associated with Morgagni hernia?

Answer 687

Morgagnis are related to Pentology of Cantrell: diaphragmatic defect, sternal defect ± ectopia cordis, pericardial defect, omphalocele, cardiac defect; T21; malrotation

Question 688

Chest xray findings of diaphragm rupture

Answer 688

XR findings include abnormal diaphragm contour, high-riding diaphragm, air-fluid levels in the thorax, and tip of NG/OG in thorax (can inject some contrast into NG/OG to help). CT can be performed but is not very sensitive. Laparoscopy helpful to confirm diagnosis.

Question 689

Hard Signs for exploration of penetrating neck injury

Answer 689

* Active bleeding * Expanding or pulsatile hematoma * SubQ emphysema or air bubbling from wound

Question 690

Indications for splenectomy

Answer 690

* Spherocytosis → hypersplenism, need for transfusions, or presence of jaundice ± pigmented gallstones. * ITP → significant chronic ITP or life-threatening complications in children unresponsive to medical therapy.s * Sickle cell disease → major episode of splenic sequestration. * Thalassemia major * Storage disease (Gaucher’s) * Trauma → hemodynamically unstable pt with splenic trauma (i.e. not a candidate for IR) * Splenic cyst → if symptomatic or >5cm (typically epidermoid cyst) * Splenic mass → often for diagnostic uncertainty; most common = hemangioma, hamartoma, lymphangioma. * Splenic abscesses → single can be drained; multiple may require splenectomy if do not resolve with antibiotics. * Sinistral hypertension → left-sided portal hypertension.

Question 691

Soft signs for exploration in penetrating neck trauma

Answer 691

* Wide mediastinum * Hemoptysis * Dysphonia (voice change) * Dysphagia

Question 692

What factors are associated with poor prognosis in CDH?

Answer 692

* LHR <1.0 particularly at late GA (<0.6 = death) → LHR >1.5 carries a favorable prognosis, particularly at an early GA. * O/E LHR <25% → 20% survival * Fetal lung volume <25% → 10-20% survival * Liver up * Right-sided (less certain) * Cardiac abnormality

Question 693

``` Breast Mass DDx. List TWO things in each of the following categories: Developmental Inflammatory Cystic Benign Malignant ```

Answer 693

* Developmental: neonatal hypertrophy, juvenile hypertrophy * Inflammatory: abscess, mastitis, fat necrosis * Cystic: single cyst, fibrocystic disease * Benign: fibroadenoma, phyllodes (benign), juvenile papillomatosis, tubular adenoma, neurofibroma * Malignant: phyllodes (malignant), adenocarcinoma, metastatic from lymphoma, leukemia, or RMS

Question 694

Regarding portal hypertension list TWO causes in each of the following categories: Pre-hepatic Intra-hepatic Post-hepatic

Answer 694

Pre-hepatic: Vascular anomalies, Portal vein thrombosis (can be related to UVC), Splenic vein thrombosis Intra-hepatic: (pre-sinusoidal, sinusoidal, post-sinusoidal): Cirrhosis (biliary atresia, ɑ1 anti-trypsin deficiency, cystic fibrosis, Alagille’s, hepatitis, auto-immune); Non-cirrhotic (congenital hepatic fibrosis, non cirrhotic portal hypertension, schistosomiasis) Post-hepatic: Budd-Chiari syndrome (hepatic vein thrombosis), right-sided congestive heart failure, IVC webs or thrombosis.