Children's Outreach Tips Flashcards
List THREE surgical indications for varicocele repair
- Symptomatic (pain, discomfort)
- Decreased testicular volume by >10% compared to contralateral side
- Infertility
- Impaired semen parameters /sperm quality
What are the features of carcinoid syndrome
Flushing, diarrhea, bronchoconstriction
Which feature makes a neuroblastoma automatically classified as high risk?
MYCN +amplification
Define Generalized lymphatic Anomaly
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
What are three common areas in which lymphatic malformations can form
Cystic lymphatic malformations occur in areas of major lymphatic channels: neck axilla groin mediastinum retroperitoneum
What are the three types of Congenital Hemagiomas?
- Rapidly involuting congenital hemangioma (RICH)
- Noninvoluting congenital hemangioma (NICH)
- Partially involuting congenital hemangioma (PICH)
Where are GISTs most commonly located in children?
Stomach (90%), remainder in small bowel and colon.
What is the hirschsprungs enterocolitis scale?
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.
Origins of GIST tumors
myenteric ganglion cells - interstitial cells of Cajal
What is the syndrome of a facial hemangioma?
PHACES: Posterior fossa malformations Facial Hemangioma Arterial Anomalies Cardiac Anomalies Eye Anomalies Sternal Cleft
Stages of Diaphragm Embryology and congenial anomalies that can result from failure of those stages.
Remember: Please Make Teachers Incorporate STEM
Pleuroperitoneal Membranes (Folds) - CDH
Thoracic Intercostal groups - Eventration
Septum Transversum - Morgagni
Esophageal Mesentary - Hiatal/Paraesophageal Hernia
What is Barrett Esophagus?
Metaplastic transformation of esophageal squamous epithelia to intestinal columnar epithelia as a result of prolonged esophageal acid exposure
What is Kasabach-Merritt Phenomenon?
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
Name 4 Histological Subtypes of Hepatoblastoma
- Embryonal (30%)
- Fetal (54%)
- Anaplastic/Small Cell Undifferentiated (6%)
- Macrotrabecular (10%)
Where should the tip of a UVC be?
IVC or IVC/atrial junction at the level of the diaphragm
What are the feared complications of Kasabach-Merritt Phenomenon?
intracranial, pleural, pulmonary, gastrointestinal, retroperitoneal hemorrhage
What is the Carney Triad
sporadic syndrome which includes two of the following three tumors: GIST (usually multifocal in antrum), pulmonary chondroma and extra-adrenal paraganglioma
List 3 factors that predispose a patient to HAEC
- Family history
- trisomy 21
- long-segment disease
- prior episodes of HAEC
- transition zone pull-through
- anastamotic stricture
Special considerations for an inguinal hernia repair in Ehlers Danlos Syndrome
Apart from high ligation with repair of back wall of canal (Bassini repair), consider mesh
List 2 possible etiologies of distal gas in a patient presenting with a ?Duodenal Atresia
- duodenal web
- duodenal stenosis
- anomalous biliary ducts (double and patent bile duct)
What is blue rubber bleb nevus syndrome?
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)
Name 2 incomplete Vascular Rings
- Right aortic arch with mirror image branching
- aberrant right subclavian
- innominate artery compression
- pulmonary artery sling
What FIVE features are the Neuroblastoma pre-treatment risk based on?
- INRG Stage
- Age
- MYCN amplification status
- 11q aberration status
- DNA index/Ploidy
What are the goals of ventilatory therapy for CDH?
pre-ductal SaO2
PaCO2
pH
Tissue Perfusion
pre-ductal SaO2 - >85%
PaCO2 - 45-60
pH - 7.2 -7.4
Tissue Perfusion - adequate
Name FOUR different variations of omphalomesenteric duct Remnants
- patent omphalomesenteric duct (omphalo-ileal connection) with or without prolapse
- Umbilical Polyp
- fibrous remnant connecting antimesenteric border of meckel diverticulum/bowel to abdominal wall at site of umbilicus
- Meckel Diverticulum
- blind ending sinus tract
- omphalomesenteric cyst
Name three options for treatment of hemangiomas
- Propranolol (1st line)
- Corticosteroids
- Vincristine
- The laser
- Surgical resection
- TACE for hepatic
What is CLOVES syndrome?
Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies (CLOVES) syndrome.
Also includes orthotopic or embryonic veins
Describe the path of a UAC catheter
enters umbilical artery, course is inferior/posterior to iliac artery, then superior/medial (in posterior position) to aorta
Most common site of carcinoid tumor in children
Appendix (80%)
The respiratory tract and GIT are remaining 20% but can be in liver and testes too
What are the 5 Tanner Stages of Breast Development?
- Papilla Elevation
- Small breast buds palpable and areolae enlarge
- Elevation of Breast Contour; areolae enlarge
- Areolae forms secondary mound on the breast
- Mature breast; nipple projects
Which organism is responsible for Necrotizing fasciitis and which antimicrobial medication is essential for its treatment
Group A betahemolytic Strep; clindamycin
List three complications of Hepatic Hemangiomas
- Transient anemia and thrombocytopenia
- Massive hepatomegaly leading to Abdominal compartment syndrome in diffuse form.
- Hypothyroidism
- High Output Cardiac Failure
What is the grading system for varicocele
I – palpable only with Valsalva maneuver
II – palpable only on standing
III – palpable/visualized all the time
ATA Classes of a Duodenal Injury
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
Describe the path of a UVC catheter
enters the umbilical vein, course is superior/anterior in abdomen, through left PV through ductus venosus, through to IVC
Name FIVE GI manifestations of Ehlers Danlos Syndrome
- Rectal Prolapse
- Megacolon
- Recurrent Hernias
- Spontaneous Rupture of Large vessels and bowel/GI Bleed
- Spontaneous colonic perforation
- GERD
- Functional GI disorders
Where can pre-ductal sats be measured?
Right arm, ear lobes
How does hypothyroidism develop in children with hemangiomas?
Infantile hemangiomas express type 3 iodothyronine deiodinase which inactivates biologically active thyroid hormone; severity related to tumor burden
GI Tumors that arise from the mesentery of the bowel (THREE) and their management
- Fibromas
- leiomyomas
- neurofibromas
Mgt - resect without bowel resection, segmental bowel resection if tumour involving the bowel wall.
Name THREE primary Gastrointestinal malignancies in childhood in order of frequency
NHL (74%) Carcinoid tumors (16%) Colonic adenocarcinomas (5%) Gastric carcinoma (3%)
List the FOUR diagnostic criteria for diagnosis of eosinophilic esophagitis
- Clinical Sx of esophageal dysfunction
- Biopsy with 15+ eosinophils/hpf
- Failed trial of PPI (8 weeks)
- Other causes of eosinophilia excluded
List THREE things on your differential for a small mass above the eyebrow
- Dermoid cyst
- Epidermal inclusion cyst
- Pilomatrixoma
- Neurofibroma
- Granular Cell Tumor
What are three subclassifications of infantile hemangioma?
- focal (solitary in 80%, ulceration in 10-15%),
- multifocal (multiple)
- regional (segmental or territorial, very prone to ulceration; if in the lower face ass. w/ upper airway involvement)
What is Ehlers Danlos Syndrome?
inherited connective tissue disorder characterized by joint hypermobility, tissue hyperextensibility (skin stretches further than normal), tissue fragility
Name the THREE imaging findings that indicate the presence of an IDRF
- Encasement - surround structure by >50%, or vessel flattened by tumor
- Infiltration - no margin between tumor and structure
- Compression - only refers to airways
Name THREE anomalies associated with duodenal atresia
- Trisomy 21
- Congenital heart defect
- Malrotation
- Annular Pancreas
- EA/TEF
Indications for surgery in Burkitt Lymphoma
Intestinal Obstruction
Evidence of Threatened Bowel
Intussusception
Biopsy (pre or post chemo)
List THREE non-ECMO options for CDH patients failing conventional ventilation
- iNO
- HFOV
- Jet Ventilation
Which neuroblastomas can be managed with observation alone?
- Cystic neuroblastoma <5cm (cystic) or <3.1cm (solid)
- L1 <18 months with tumor <5cm (cystic) or <3.1cm (solid)
- MS tumor
- Confirmed GN or GNB intermixed (consider if asymptomatic)
List the two most common benign vascular tumors
Infantile Hemangioma
Congenital Hemangioma
List FOUR complications of an infantile hemangioma
- Astigmatism if located in periorbital region
- Bleeding, including from GIT
- Ulceration
- Cardiac compromise d/t shunting through the lesion
- Hypothyroidism
- Acute Compartment Syndrome
What are the THREE phases of High Risk Neuroblastoma treatment?
- Induction - chemotherapy (+stem cell harvest) and surgery
- Consolidation - myoablative chemotherapy (+ tandem BMT) and radiation therapy
- Post-Consolidation - Immunotherapy, cytokines, and isotretinoin
What is the management of Alagille Syndrome?
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
Why do GISTs behave differently in kids compared to adults
Kids typically lack KIT mutations making the use of imatinib and sunitinib unclear.
What is the overarching goal of ventilatory management in CDH patients and what initial ventilatory setting will you use?
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
THREE options for surgical management of FAP
- Total colectomy with end ileostomy (need rectal surveillance, may do for fertility preservation)
- Total colectomy with IRA (need rectal surveillance)
- Total proctocolectomy with IPAA
- Total proctocolectomy with end ileostomy
List three indications for surgery in infantile hemangiomas
- bleeding (including GI)
- ulceration
- threatens vital function
- risk permanent disfigurement
- ACS
Describe the INRG stages for Neuroblastoma
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
Name 4 types of simple Vascular Malformations
Capillary Malformations (aka port wine stain)
Venous Malformations
Lymphatic Malformations
Arteriovenous Malformations
Most common location of leiomyosarcoma in children
Jejunum and colon
Histology of a dermoid cyst
arise from elements trapped during embryonal fusion; composed of ectodermal and mesodermal elements (no endodermal); lined by squamous epithelium
How to treat vascular rings (very basic)
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
List FOUR syndromes associated with Hepatoblastoma
- Beckwith Wiedamann
- FAP
- Prematurity
- Li Fraumeni
- Glycogen storage disease
- Trisomy 18
What is the calculation for Oxygen Index?
At what oxygen index is ECLS/ECMO indicated?
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
What are the initial settings for HFOV?
Hertz- 10
Mean Arterial Pressure - 15
Amplitude - 30
FiO2 - 100%
What is the surgical management for leiomyosarcoma?
complete surgical resection, no lymph nodes required.
FOUR subtypes of NHL
Burkitt
Diffuse Large B cell
Lymphoblastic
Anaplastic large cell
For which type of vascular anomaly should an EXIT procedure be considered if identified prenatally, and why?
Large cervicofacial lymphatic malformation; potential for postnatal airway obstruction
Which neuroblastomas qualify as very low risk (THREE)?
- Any GN or GNB intermixed
- L1, MYCN -ve
- MS, MYCN -ve
Which chemotherapeutic agents are used in the treatment of Neuroblastoma?
Remember to get the: “View on the Top DECC”
Vincristine Topotecan Doxorubicin Etoposide Cisplatin Cyclophosphamide
Management of Barrett Esophagus with low-grade dysplasia
Treat underlying condition if present, Annual EGD, PPI, +/-fundoplication, +/- RFA
Most common location of abdominal Burkitts
ileocecal
Which imaging study should be performed if a baby is found to have 5 or more cutaneous infantile hemangiomas?
Liver ultrasound to screen for concurrent hepatic hemangioma
Name FIVE hormones that functional carcinoid tumors secrete
- serotonin
- 5-HAA
- dopamine
- Norepinephrine
- Histamine
- Bradykinin
- Prostaglandins
Which Hepatoblastoma patients require neoadjuvant chemotherapy?
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)
In general, what is the goal of surgical resection in neuroblastoma?
Removal of >90% of tumor without contiguous organ resection.
What are the 5 ASA classes
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
DEFINE chronic eosinophilic esophagitis
A clinicopathologic disease characterized by chronic esophageal dysfunction caused by eosinophil-mediated inflammation of the esophagus
What are the features of the LUMBAR association?
Lower body infantile hemangioma Urogenital anomalies and Ulceration Myelopathy Body deformities Anorectal malformations Arterial malformations
What is the goal of resection in a GIST
Excision with free microscopic margins. In the bowel, segmental resection or wide excision with lymph nodes (especially pathologic appearing) is recommended.
What is the Ann Arbor Classification for Lymphoma?
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
List FOUR indications for Infective Endocarditis Prophylaxis
- Prior history of endocarditis
- Cardiac Valve disease in a transplanted heart
- Unrepaired cyanotic congenital heart disease or incompletely repaired congenital heart disease
- Congenital heart disease repaired using prosthetic material
- A prosthetic heart valve
- Valve repair using material prosthetic.
What are the options for non-operative management in rectal prolapse?
- 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
In what organ transplant is PTLD most common?
Intestinal (8%)
Thoracic Organs (3-5%)
Liver and Kidney (2%)
Name TWO surgical procedures that may be required for a patient with Alagille Syndrome
- Cholangiogram and biopsy
2. Liver Transplant
List FOUR benign gastrointestinal tumors
- Ganglioneuroma
- Neurofibroma (NF1)
- Leiomyoma
- Lipoblastoma
- Teratoma
- Hemangioma
- Inflammatory Myofibroblastic Tumor
What are the Annotation Factors for Hepatoblastoma
Remember: Very Poor Eager Freshmen Really Need Cash Money!
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
How do the majority of children with GIST present
Syncope and generalized weakness due to chronic anemia (86%); palpable tumor (12%); range of asymptomatic to nausea, discomfort, diarrhea, intestinal obstruction, vomiting, constipation
What kind of cells are seen in a PTLD biopsy
monoclonal, polyclonal, or monomorphous
What is Gorham Stout Disease?
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.
Management for Carcinoid tumors of the appendix <2cm
Management for Carcinoid tumors of the appendix >2cm
< 2 cm - simple appendectomy
>2cm - Right Hemicolectomy (new studies say only needed for positive margins or local lymph node involvement)
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
Creeping Fat Transmural Granulomas Skip Lesions Cobblestoning Crypt Abscesses Pyoderma Granulosum Apthous Ulcers
List FOUR potential causes of congenital chylothorax
- atresia of the thoracic duct
- obstruction of thoracic duct from tumor
- congenital pulmonary lymphangiectasia/Diffuse lymphangiectasia
- lymphangiomatosis
- birth trauma
- idiopathic
- associated with syndromes (Noonan, Turner, Down, Gorham-Stout)
- Central Venous thrombosis
- Extralobar BPS
List three general categories of lymphatic malformations
- Macrocystic
- Microcystic
- Mixed
What are the most common symptoms at presentation for eosinophilic esophagitis
In newborns: vomiting, dysphagia, feeding intolerance
Other: food impaction d/t dysmotility +/- stricture, GERD
What is required to make the diagnosis of Barrett Esophagus?
Metaplastic columnar mucosa above the GEJ on endoscopy. Goblet cells indicating intestinal metaplasia on histology.
What is Alagille Syndrome and what genetic mutation is responsible?
an autosomal dominant genetic disorder caused by mutation in JAG1; bile duct paucity – decreased number and narrow/malformed ducts cause cholestasis, scarring, fibrosis
Name TWO options for surgical management of rectal prolapse. Which is preferred in children?
- rectopexy +/- anterior resection
- Sclerotherapy (preferred)
- altemeier or perineal rectosigmoidectomy
What are the three stages of the life cycle of an infantile hemangioma?
- Proliferation - takes 6-12 months; 80% of final size by 3 months
- Plateau
- Involution - can continue until 5-7 years old
List THREE complications of omphalomesenteric duct remnant
- intussusception of Meckel diverticulum
- volvulus around fibrous remnant
- Meckel diverticulitis inflammation/infection
- lower gi bleed from ectopic gastric mucosa in Meckel diverticulum
- omphalitis
What is the name for for the Inguinal canal in females?
Canal of Nuck;
Gubernaculum – upper portion = ovarian ligament, lower portion = round ligament
What is a pilomatrixoma, and what are the most common presenting features?
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.
What is the mainstay of treatment for Eosinophilic Esophagitis?
- Elimination Diet
- PO Fluticasone/budesonide x 8 weeks with oral prednisone if failure
- Dilation of strictures
List THREE diseases/clinical symptoms that make children more susceptible to rectal prolapse
- CF
- Hypothyroidism
- Ehlers-Danlos
- Hypercalcemia
- Chronic Constipation
- Diarrheal illness
- Previous surgery for ARM/HD
- Malnutrition
Common presenting signs/symptoms of leiomyosarcoma
bleeding, intussusception, obstruction (late presentation since they grow outward from serosa), prolapse if in rectum.
What is the major risk associated with the identification of a high flow vascular malformation prenatally
Can cause high output cardiac failure and fetal hydrops/demise.
These babies need to be closely followed with frequent ultrasound and fetal echo assessment.
What are the most common sites of infantile hemangiomas?
head and neck (60%)
trunk (25%)
extremities (15%)
Other - airway (consider especially if seen on the mandible), perineum, and viscera
List THREE specific complications of inguinal hernia repair in children
- recurrence
- iatrogenic undescended testicle
- post operative hydrocele
- testicular atrophy
- injury to vas
- bleeding - hematoma
- chronic pain
Name 2 complete vascular rings
- Double aortic arch
- Right aortic arch with aberrant left subclavian artery and LDA
- Right arch with retroesophageal left ligamentum/ductus
Name 3 treaments for PTLD
- Reduce Immunosuppresion
- Chemotherapy
- Rituximab
- Radiation (rescue)
What is reverse ratio ventilation, when would you use it, and what is the mechanism?
- 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)
Three types of intestinal ganglioneuromas
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
Name three absolute contraindications for a UVC
- omphalitis
- omphalocele
- NEC
- peritonitis
- portal venous hypertension
Where should the tip of a UAC be?
The aorta between T6-T9, above the celiac take off
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!
- Embryonic
- Pseudoglandular - Pulmonary/Lobar agenesis, CPAM (1,2,3), BPS, Chaos
- Canalicular
- Saccular - Pulmonary Hypoplasia
- Alveolar (CPAM Type 4)
What is the chromosomal translocation associated with Rhabdomyosarcoma
t2;13 -PAX3/FOXO(fusion positive, BAD), t1;13 PAX7/FOXO (good)
List THREE Absolute and TWO relative contraindications for ECLS
- Significant congenital anomalies.
- Lethal chromosomal anomalies.
- IVH grade 2+.
- Weight <2kg (relative).
- GA <34 weeks (relative).
What are the Canadian Readiness for Surgery Consensus Guidelines in CDH?
- 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)
What are the FOUR most common locations of a Dermoid cysts
- Glabella
- Submental
- Anterior Fontanelle (do U/S or MRI for intracranial extension)
- Lateral 1/3 of eyebrow
What are the indications to image a dermoid cyst?
Any dermoid that is in a precarious location should be imaged due to the propensity for bony/intracranial involvement
List TWO indications for ECLS in a baby with CDH
- 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.
Define Torticollis and Describe the typical position and findings of the head and neck
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).
Name ONE non-paroxysmal and TWO paroxysmal causes of Torticollis and BRIEFLY describe their mechanism
- Congenital Muscular Torticolis (CMT, non-paroxysmal) - SCM injury during delivery OR abnormal in utero positioning
- Benign Paroxysmal Torticollis - Episodic neck wringing, self limiting by age 3
- Sandifer Syndrome - Severe GERD causing episodic neck wringing, especially in children with epilepsy
Name THREE conditions associated with torticollis
- Hip Dysplasia
- Plagiocephaly
- Scoliosis
- Craniofacial asymmetry/hypoplasia
Name THREE options for treatment of Torticollis
- Physical Therapy - most effective in patients diagnosed <3 months
- Botox - 75% effective, but can cause dysphagia and weakness
- Surgery - Unipolar or Bipolar SCM release - for failed physio x6 months or Dx >1yr
List TWO risks and TWO benefits of a Primary pull-through for Hirschsprung
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)
In which Hirschsprung patients is it indicated to perform a multi-stage procedure?
• 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
List TWO risks and TWO benefits of a multi-stage pull-through for Hirschsprung
RISKS:
• 2 or 3 surgeries
• stoma & complications (fluid losses, prolapse, stenosis)
BENEFITS:
• no rectal irrigations
• will have final pathology from stoma site
Neurofibromatosis 1 is an autosomal dominant disorder. On what chromosome is the defect? And list THREE findings in NF1
Chromosome 17
- cafe au lait spots
- axillary/inguinal freckling
- Multiple neurofibromas
- pheochromocytoma
- optic glioma
Neurofibromatosis 2 is an autosomal dominant disorder. On what chromosome is the defect? And list TWO findings in NF2
Chromosome 22
- vestibular schwanoma
- meningioma
- ependymoma
Name THREE things that you should include on your differential diagnosis for Torticollis
- Sandifer
- Infection
- Trauma
- Posterior fossa tumor
- spinal cord tumor
Name the THREE most commonly performed pull-through procedures for Hirschsprung. In which of the procedures is all aganglionic bowel removed?
- Duhamel
- Soave
- Swenson (all aganglionic bowel removed)
What are the FOUR IVH Grades?
I - Hemorrhage in germinal matrix only
II - ventricular hemorrhage without ventricular dilation
III - ventricular hemorrhage with ventricular dilation
IV - brain parenchymal hemorrhage
What are the THREE strategies to avoid IVH?
- avoid prematurity
- correct coagulopathies
- antenatal steroids
- avoid hypoxia/hypercarbia
- avoid hypotension/fluctuating CBF
What is the treatment of choice if a baby with IVH develops severe hydrocephalus
acetazolamide to decrease CSF production
What is the mainstay of treatment for IVH?
Supportive therapy, maintenance of cerebral perfusion with cautious BP control, anticonvulsant therapy for seizures, transfusion if indicated.
What is the Modified Bell Criteria for NEC
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
List TWO indications for peritoneal drainage in a NEC
- 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
List TWO advantages and THREE disadvantages to contralateral exploration in hernia repairs
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.
On what side are inguinal hernias more common?
On what side are varicoceles most common?
Hernia - Right (75%)
Varicocele - Left (90%)
What is the natural history of a patent processus?
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%.
What percentage of babies have metachronous hernias?
Incidence of metachronous hernia 5-12%, it is 15% in premies, and 28% in pts who present with incarcerated hernias.
In what circumstances should a primary anastomosis be considered in NEC?
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
List FOUR causes of inguinal swelling in children
- Inguinal Hernia
- Inguinal lymphadenopathy or lymphadenitis
- Granuloma inguinale
- Femoral Hernia
- Amyand Hernia (appendicitis within hernia sac)
- Benign or malignant tumor
Which babies should initially have their TEF clipped (or ligated), distal esophagus tacked, and gastrostomy inserted (on lesser-curve)?
- Significant cardiac anomalies
- Critically ill babies (or pts needing multiple other surgeries)
- <1.3kg.
- Long-gap EA (>3cm or >2 vertebral bodies)
What is the embryologic explanation for TEF?
Failure of septation of ventral and dorsal foregut
Why is an Echo necessary prior to a TEF repair?
50% of TEF have a concurrent cardiac anomaly and need to know the side and character of the aortic arch
What are the cardinal symptoms of Henoch-Schonlein Purpura (IgA Vasculitis)
- 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)
What complications of Henoch-Schonlein Purpura (IgA Vasculitis) bring children to the attention of Pediatric Surgery?
•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.
What is the management of Henoch-Schonlein Purpura (IgA Vasculitis)
• 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
What is Hemolytic Uremic Syndrome and what is the most common causative organism?
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
What is the classic triad of Hemolytic Uremic Syndrome?
acute hemolytic anemia, thrombocytopenia, AKI.
What GI complications are associated with Hemolytic Uremic Syndrome?
Severe LGIB causing anemia, hemoperitoneum, severe colitis with megacolon, necrosis and perforation.
Name the THREE types of histiocytosis
- Langerhans cell histiocytosis
- Hemophagocytic lymphohistiocytosis (HLH)
- Malignant histiocytosis
What is Langerhan’s Cell Histiocytosis?
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.
What is the management for Langerhan’s Cell Histiocytosis
- 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.
Name FOUR things on the differential diagnosis for an umbilical granuloma?
- Urachal duct remnants
- Omphalomesenteric duct remnants
- Omphalitis
- Ectopic intestinal mucosa
- Umbilical Hernia
List the FOUR types of urachal anomalies
Urachal Cyst
Urachal Sinus
Urachal Diverticulum
Patent Urachus
Name THREE Omphalomesenteric duct remnants
Meckel Diverticulum Meckel with Fibrous cord Umbilicoileal fistula Vitelline cyst, umbilical cyst Fibrous cord (with or without artery).
List FOUR complications that are common to both UACs and UVCs
- Tip migration
- Sepsis/omphalitis
- Thrombosis
- Hemorrhage
List THREE complications of UVCs
- Perforation of the IVC
- Extravasation of insufflate into the peritoneal cavity
- Portal vein thrombosis
- Omphalitis/sepsis
- Perforation of intrahepatic vascular wall
List FOUR complications of UACs
- Omphalitis
- Aortic injuries
- Thromboembolism of aortic branches
- Aneurysms of the iliac artery or aorta
- Paraplegia
- Gluteal ischemia with possible necrosis
What is Tuberous Sclerosis?
Autosomal dominant disorder characterized by Ash leaf spots, renal angiomyolipoma, giant cell astrocytoma, cardiac Rhabdo, infantile spasms
What are the features of Mayer-Rokitansky-Küster-Hauser (MRKH)
• 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.
What are the THREE most important initial questions to ask in a patient presenting with a disorder of sexual development?
- 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?
If a patient with a disorder of sexual development has symmetrical gonads, what are the TWO possible diagnoses?
- Congenital adrenal hyperplasia (CAH) = 46XX DSD
* Androgen insensitivity syndrome (AIS) = 46XY DSD
If a patient with a disorder of sexual development has asymmetrical gonads, what are the THREE possible diagnoses?
- Pure gonadal dysgenesis = 46XX (2/3) or 46XY (1/3)
- Mixed gonadal dysgenesis = 45XO/46XY or 46XY
- Ovotesticular DSD = 46 XX or 46XY
What is the initial workup of a patient born with a DSD?
- Karyotype
- Serum electrolytes
- EKG
- Consult Endocrinology
- Imaging (U/S +/- MRI) +/- EUA & laparoscopy
What are the THREE most common genetic anomalies implicated in Congenital Adrenal Hyperplasia
- 21-hydroxylase deficiency most common (90%)
- 11-β hydroxylase deficiency
- 3-β hydroxysteroid deficiency
Androgen insensitivity syndrome can be complete, partial, or mild. What are the mechanisms that can lead to androgen insensitivity?
- Deficiency of androgen production.
- Androgen receptor deficiency.
- Inability to convert testosterone to dihydroxytestosterone (5 alpha reductase deficiency).
What are the processes responsible for gonadal development?
The default human setting is female.
- The SRY protein makes the gonad a testical (no SRY = ovaries)
- Testosterone turns the wolffian duct into a seminal vesicle (degenerates if no testosterone).
- Anti-Mullerian hormone makes mullerian ducts disappear (otherwise you get a fallopian tube, uterus, and upper vagina)
What is the processes are responsible for the development of external genitalia?
In the absence of androgens, the external genitalia become feminized
What are the features of the classical presentation of congenital adrenal hyperplasia.
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.
What is Poland Syndrome?
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.
Name THREE genetic disorders that are associated with Pectus Excavatum
- Poland Syndrome
- Marfan
- Ehler-Danlos
- Noonan
What is the management of Poland Syndrome?
Combined surgical correction with plastic surgery; latissimus dorsi flaps for muscular hypoplasias +/- breast implants; pectus deformities can be addressed by Nuss +/- Ravitch.
Beckwith-Wiedemann Syndrome (BWS) is an overgrowth syndrome with a range of phenotypes. Name FOUR associated features of BWS (not oncologic)
- macroglossia
- macrosomia (growth slows by age 8)
- visceromegaly
- hemihyperplasia
- hyperinsulinemic hypogylcemia
- Omphalocele
- preauricular pits/sinuses
Name FOUR tumors that patients with Beckwith Wiedemann can develop
- Wilms = 43%
- Hepatoblastoma = 12%; usually develops by age 4.
- Rhabdomyosarcoma
- Neuroblastoma
- Adrenal cortical carcinoma
- Congenital mesoblastic nephroma
What is the surveillance for children with BWS?
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.
Describe the theory of pathogenesis and the presentation of spontaneous perforation of the common bile duct in an infant.
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
List THREE conditions on the differential diagnosis of a biliary ascites in a baby
- Blunt Liver injury
- NEC
- Distal Biliary stenosis–>CBD perforation
- Perforated choledochal cyst
- Pancreatitis
- CMV
What is the mechanism of action of cyclosporine A (CSA)?
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
What is OEIS complex?
- Omphalocele
- Exstrophy of bladder
- Imperforate anus
- Spinal defects (dysraphism)
Describe Classic Bladder Exstrophy
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.
What is he pathognomonic finding of cloacal exstrophy on a prenatal ultrasound
elephant trunk–like mass
(representing the prolapsed ileum) — found in 33%.
Describe the classic findings of cloacal exstrophy
• 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%).
Describe a single-stage procedure for the repair of cloacal exstrophy
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
Briefly describe the three options for colostomy creation in a patient with cloacal exstrophy
- 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
Why is a colostomy and not an ileostomy needed for cloacal exstrophy?
preserve bowel length, expose hindgut to fecal stream, minimize fluid/E+ problems, solid stools
Describe the three-stage repair of bladder extrophy
- At birth - convert to complete epispadias – close bladder, posterior urethra and abdo wall, use suprapubic cath, ureteral catheters
- At 6-12 months, repair of epispadias
- Before school age, bladder neck repair for continence and ureteral reimplantation for VUR
For each branchial arch list THREE derivatives
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.
What is the Embryologic pathogenesis of Branchial cleft anomalies?
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
What is BOR Syndrome?
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).
What is the presentation and Management of a 1st branchial anomaly?
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).
Where would you expect to find a 2nd Branchial anomaly? And where does the fistula communicate to?
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
Which structures should be protected/avoided in the excision of a 2nd Branchial anomaly?
beware damage hypoglossal (intrinsic muscles of the tongue), glossopharyngeal (taste posterior 1/3 tongue), vagus (parasympathetic function).
Also watch for carotid, IJ, pharynx
Describe the surgical Management of a 2nd Branchial anomaly
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).
50% 3rd and 4th Branchial anomalies have openings in the ______ sinus
piriform
Describe the classic presentation of a 3rd/4th Branchial anomaly in a Neonate and in an older child
Neonatal: enlarging neck mass that compromises spontaneous breathing
Older child: neck abscess within or in vicinity of left thyroid lobe; suppurative thyroiditis/thyroid cyst
Outline the surgical management of a 3rd/4th Branchial anomaly
Left thyroid lobectomy with excision of the tract (risk of RLN injury) or endoscopic procedure to cauterize tract.
85% of Biliary Atresia is Sporadic. Describe the TWO Syndromic groups
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
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
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
Detail the Presentation and Management of Atypical Mycobacterial Lymphadenitis
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
Detail the Presentation and Management of a thyroglossal duct cyst
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
Name FOUR infectious causes of a neck mass in children
- Reactive LN hyperplasia
- Cervical Suppurative Lymphadenitis
- Scrofula (TB)
- Atypical Mycobacterium
- Bartonella (cat-scratch)
Name FOUR Non-infectious causes of a neck mass in children
- Thyroglossal duct cyst
- Dermoid cyst
- Epidermoid cyst
- Ectopic Thyroid Tissue
- Lymphoma
- Hemangioma
- Teratoma
- Branchial Cleft Remnant
- Cervical Thymic Cyst
What is the embryological explanation for thyroglossal duct cysts?
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.
What is the embryological explanation for Prune Belly?
urethra obstructed, leads to enormous bladder dilatation, prevents development of
abdominal wall musculature.
What are the THREE features of Triad Syndrome (Prune Belly)?
Deficient or absent abdominal wall, Intra-abdominal undescended testes, ectasia of urinary system (Dilated distal genitourinary tract)
BONUS: Pulmonary Hypoplasia due to Oligohydramnios
Where are Accessory Spleens typically found?
Name THREE potential complications of Accessory Spleens
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
Name FOUR Developmental Splenic Anomalies
- Heterotaxy
- Spleno-gonadal fusion
- Spleno-pancreatic fusion
- Wandering Spleen
Define Wandering Spleen and describe its management
Abnormal development of splenic ligaments (or older person with lax ligaments over time); spleen at risk
for torsion, ischemia, infarction
Treatment is splenopexy
Describe the THREE mechanisms that lead to Tumor Lysis Syndrome
- DNA breakdown–> hyperuricemia —> renal failure
- Protein breakdown —> hyperphosphatemia —> hypocalcemia —> cardiac arrhythmia—> renal failure
- Cytosol breakdown —> hyperkalemia —> cardiac arrhythmia —> renal failure
Which tumors are at higher risk of Tumor Lysis Syndrome?
Typically for Burkett’s and ALL, but can occur in any tumour that has a high cell proliferation rate
Management of Tumor Lysis Syndrome
Prevent with prophylaxis (IV hydration), hypouricemic agents (allopurinol + rasburicase), urinary alkalinization (acetazolamide or NaHCO3)
Detail the embryology of Cloaca
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
What criteria are used to determine if a PSARP and Total Urogenital Mobilization can be performed for Cloaca?
If Common Channel <3cm and urethra >1.5cm
If the common channel is >3cm and urethra <1.5cm in a cloaca, what procedure is required for repair?
- Help from a friend named Mark or Alberto
- Urogenital Separation
- Posterior Sagittal Anorectal Vaginal Urethroplasty
An abdominal mass in a patient with cloaca is _____ until proven otherwise
Hydrocolpos
What are THREE features of a covered exstrophy?
- wide public symphysis
- low-lying umbilicus
- open bladder neck
List THREE options for drainage of Hydrocolpos
- Intermittent drainage via perineal orifice
- Tube Vaginostomy
- Cutaneous Vaginostomy
What is the Workup in VACTERL Screening
- 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
What does VACTERL stand for?
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! :)
How should a colostomy be made in Cloaca? (classic answer)
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
Describe the cardinal steps in a Total Urogenital Mobilization
- Separate the rectum from the vagina
- Keep vagina and urethra together as UG sinus
- Free UG sinus anteriorly by dividing suspensory ligaments
- Mobilize UG sinus to perineum
- Sew new position of urethra anteriorly
- Introitoplasty and PSARP
Describe the very basic steps of a Urogenital Separation
- Separate the rectum from the vagina
- Separate vagina from urinary tract
- Close bladder neck-vaginal fistula
- Common channel becomes the neourethra
- Vaginal pull-through to perineum
Renal Trauma Grades
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
What is Page Kidney?
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
What is the most common kidney tumor in patients with Tuberous Sclerosis, and why do we care?
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.
What is the appropriate follow up for patients with Sacrococcygeal teratoma?
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.
What are Posterior Urethral Valves (PUV)
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.
Describe the prenatal findings of Posterior Urethral Valves (PUV)
1/3 diagnosed prenatally with hydronephrosis or
distended thick-walled bladder and dilated posterior urethra.
What are the presenting symptoms of a newborn with Posterior Urethral Valves (PUV)?
What are the presenting symptoms of a child with PUV?
Newborn: UTI, urosepsis, abdominal distension, poor stream, grunting while voiding; also clinical manifestations of pulmonary hypoplasia.
Children: UTIs, incontinence, enuresis, frequency, poor stream.
What is the initial treatment, endoscopic treatment, and surgical treatment for Posterior Urethral Valves?
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.
What are the FIVE grades of Vesico-ureteral reflux on VCUG?
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
Name FOUR indications for VCUG
- Recurrent UTIs
- UTI before toilet training
- UTI with pathogen other than E coli
- Concern with parental compliance for follow-up (i.e. after episode of UTI)
- Any boy with UTI?
- Renal anomalies on U/S
- Systemic signs of chronic kidney disease
What is a TART?
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
What is the significance of an Adrenal Rest?
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.
Name the FIVE key vessels in fetal circulation and their adult vestiges
Umbilical Vein - Ligamentum Teres
Umbilical Arteries - Medial umbilical ligaments
Ductus Venosus - Ligamentum Venosum
Foramen Ovale - interatrial septum or Fossa Ovalis
Ductus Arteriosus - Ligamentum Arteriosum
Define Assent
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;
Define Consent
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.
Define Beneficence
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.
Define non-maleficence
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.
Define the term “Standard quality of life”
standard level of health, comfort, or happiness, or general well being that can be expected or strived for
Describe the embryology of Pancreatic Divisum
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).
What is the natural history of pancreatic divisum?
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.
What are the THREE histologic subtypes of congenital mesoblastic nephroma
classic (65%), cellular (25%), mixed (10%)
List THREE things on the differential for a renal mass in a 5 month old.
- Wilms
- CMN
- rhabdoid of the kidney
- clear cell sarcoma
- renal cell carcinoma,
- angiomyelolipoma
- ossifying renal tumour
Describe the Stocker Classification and association with each type.
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
Name THREE tumors are associated with DICER1
- PPB
- RMS
- Ovarian sex-cord stromal tumors
- cystic nephroma
- thyroid nodules
List THREE neonatal problems that could require ECMO
- persistent pulmonary hypertension of the newborn
- meconium aspiration syndrome
- respiratory distress syndrome
- sepsis/pneumonia
- air leak syndrome
- CDH
Name ONE advantages and ONE disadvantages of VV ECMO
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
Name ONE advantages and ONE disadvantages of VA ECMO
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
Define Meconium Aspiration Syndrome
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
What is the pathophysiology of pulmonary hypertension in Meconium Aspiration Syndrorme?
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
Describe the presentation of a baby with meconium aspiration syndrome.
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
Describe the Radiologic findings of a baby with meconium aspiration syndrome.
Initially streaky, linear densities, followed by hyperinflation, and alternating diffuse patchy densities with areas of expansion. May also see pneumothorax.
List TWO ventilatory strategies for babies with Meconium Aspiration Syndrome
- increase O2
- increase PEEP to improve end-expiratory lung volume
- increase PIP to recruit atelectatic lung
- HFO to recruit atelectatic lung
- ECLS.
What is WAGR and on what mutation(s) is responsible for it?
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
Name THREE ways in which children with WAGR are different from sporadic Wilms
- Picked up earlier due to screening (like BWS)
- Earlier age at diagnosis (<3 years)
- More frequently bilateral
- Higher risk of metachronous tumour
- Higher rate ESRD after resection
- Nephron-sparing approach preferred for operative mgt
- Increased risk of gonadoblastoma d/t streak gonads
What is Nephroblastomatosis and how does it relate to Wilms Tumor?
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
List FIVE favourable sites for rhabdomyosarcoma
- Orbit
- Head and neck (not parameningeal)
- Biliary
- Paratesticular
- Vaginal, vulvar, uterine
List FIVE UNfavourable sites for rhabdomyosarcoma
- Cranial/parameningeal
- Extremities
- Trunk
- Retroperitoneal
- Bladder, prostate, perineal
What are the FOUR postoperative groups for Rhabdomyosarcoma?
1 - Localized disease, completely resected
- Total gross resection with evidence of residual disease
- Incomplete resection with gross residual disease
- Metastatic disease present at onset.
In what instances should lymph nodes be removed in rhabdomyosarcoma?
sample clinically positive nodes; SLNB trunk and extremity; and paratestticular SLND for boys >10yrs with clinically negative nodes or boys <10yrs with CT (+) nodes
List FOUR histological subtypes of rhabdomyosarcoma
- Embryonal (Fusion negative, good prognosis)
- Alveolar (75% Fusion positive)
- Anaplastic
- Undifferentiated
What are the principles of surgical management of a rhabdomyosarcoma?
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
Discuss Pre-treatment RE-excision or Primary-site RE-excision (PRE) and Delayed Primary Excision (DPE) as it pertains to rhabdomyosarcoma
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
Which Chemotherapy agents are used in the treatment of rhabdomyosarcoma?
VAC - vincristine, actinomycin-D, cyclophosphamide
List THREE finding on history that are concerning for non-accidental trauma
- Lack of or vague explanation for significant injury
- Lack of reported trauma in a child with obvious injury
- Discrepant histories over time / between persons interviewed
- Explanation that is not consistent with the injury pattern or severity or child’s developmental capability
- Delay in seeking care
- History of multiple injuries, ER visits, hospitalizations
List THREE bruising patterns that are consistent with non-accidental trauma
- Any bruising in nonambulatory infant or child
- Bruises on the face, cheeks, ears, neck, genitalia or hands in any aged child
- Located on the buttocks and front trunk in early mobile and premobile children
- Defensive in location (e.g. outer arm)
- Clustered bruising or larger number of bruises present at the time of diagnosis
- Patterned bruises that match an identifiable object (e.g. loop mark from a cord)
Describe a bruising pattern consistent with ACCIDENTAL trauma
Ambulatory children, especially if located over bony prominences (including the forehead, nose, knees and shins, especially on the front of the body)
Describe a fracture pattern consistent with ACCIDENTAL trauma
Fractures explained by a known history of severe trauma or underlying bone disorder…
List THREE burn findings consistent with ACCIDENTAL trauma
- Spill or flowing water injury (more rarely hot beverages or hot tap water)
- Irregular margins
- Varying burn depth
- Asymmetric involvement of lower limbs, head, neck, trunk, face and upper body
List THREE burn patterns that are consistent with non-accidental trauma
- Immersion burns, especially with hot tap water
- Clear upper limits
- Uniform depth
- Symmetrical involvement of lower limbs—especially with skin fold sparing (“zebra striping”) or central sparing of buttocks (“doughnut pattern”) or soles of the feet
- Isolated scald to buttocks/perineum
- Glove and stocking appearance
List THREE fracture patterns that are consistent with non-accidental trauma
- Fracture(s) in nonambulatory infants
- Multiple fractures
- Fractures of different ages
- 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,
- History of trauma does not explain the resultant fracture
Name FOUR potential causes of cirrhosis in children
- Biliary Atresia
- Autoimmune Hepatitis
- Cystic Fibrosis
- alpha-1 antitrypsin deficiency
- Wilson disease
- Fatty Liver disease
- Chronic viral hepatitis or hepatitis C
- Primary sclerosing cholangitis
- Alagille
