Pediatric Flashcards
Foregut, midgut, hindgut
Foregut – esophagus to 2nd portion of duo including ampulla. Gallbladder, liver, lungs, pancreas, bile duct – celiac artery
Midgut – 3rd portion of duo, first 2/3 of transverse colon. – SMA
Hindgut – last 1/3 of TV colon to anus – IMA and internal iliac
Umbilical blood supply
There are 2 umbilical arteries, one umbilical vein
Down’s syndrome associations
Down’s syndrome – Duodenal atresia, Hirshsprung’s, omphalocele, ASD -> ostium primum (endocardial cushion defect), annular pancreas
VACTERL associations
tracheoesophageal fistula, imperforate anus
Malrotation associations
Congenital diaphragmatic hernia, omphalocele, duodenal atresia
High associations with congenital anomlies
Duodenal atresia – Cardiac anomalies Need echo prior to surgery
Congenital diaphragmatic hernia – Malrotation, cardiac and neural tube defect Need echo before surgery
TEF – Cardiac abnormalities – get echo before surgery
Pediatric fluid resuscitation
Maintenance fluid = D5 0.9% NS with 10-20K
If Severe dehydration (mottled, lethargy, decreased cap refill) start with 20cc/kg of NS
The use of hypotonic or hypertonic crystalloid solutions for the purpose of emergent volume resuscitation is never recommended in pediatric patients – except pyloric stenosis
Oral rehydration therapy is the preferred treatment for children with mild to moderate hypovolemia or after severe dehydration and resuscitated
Infants with moderate or severe dehydration 2/2 to pyloric stenosis: D5 ½ (0.45) NS + 20K (1.5 to 2 times maintenance rate)
- If there is ANY renal dysfunction (rise in Cr or low UOP) DO NOT GIVE potassium until patient has UOP
- Alkalosis must be fixed prior to going to OR because it increases risk of post op apnea
Neonate and infant UOP should be 2-3 cc/kg/hr
Maintenance fluid for all pediatric patients = D5 ½ NS +/- potassium
- Avoid potassium in infants and toddlers until making urine
- All children need glucose in maintenance fluid
Congenital lobar hyperinflation (emphysema)
RESPIRATORY DISTRESS AND HYPOTESNION (Can look like a tension PTX) (SHIFT IN MEDIASTINUM)
CXR: shows hyperinflation of a lobe (hyper-lucent area); compression of other structures
Bronchus Cartilage fails to develop
Leads to air trapping with expiration
Vascular supply and lung tissues are normal lungs are just compressed by hyperinflation
LUL MC affected
Tx: Lobectomy
Pulmonary Sequestration
Pulmonary Sequestration
Sx: infections MC, resp distress, hemorrhage
Have a Continuous murmur
Dx: CT angio – shows lung mass with anomalous blood supply (key issue here)
MC LLL
Lung tissue is not connected to the airway, and has a SYSTEMIC ARTERIAL SUPPLY
- MC from thoracic aorta
- Can also be from abdominal aorta through inferior pulmonary ligament
Intra-lobar sequestration– remains in the native pleura viscera, pulmonary venous connection usual
Extra-lobar sequestration – enveloped by separate pleural lining, systemic venous drainage usual (MC Azygous)
Risk of CA
Tx: Ligate the systemic arterial supply FIRST (can bleed). Then perform segmentectomy vs lobectomy
Congenital cystic adenoid malformation (CPAM)
Sx: newborns respiratory distress, older children infections
Rapid decompensation can occur with ventilator needs emergent resection of cystic structure
Lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue
Risk of malignancy
Communicates with airway bronchus unlike pulmonary sequestration
This is secondary to bronchiole tissue overgrowth!
Dx: in utero can measure CAM volume ration (CVR) = volume of lung/head circumference. If > 1.6 high risk for fetal hydrops and will need biweekly US and will need to give steroids to decrease size
Tx: Lobectomy
Choledochal cyst
Pediatric presentation → abdominal mass + obstructive jaundice
Adult presentation→ biliary or pancreatic symptoms AND symptomatic cholelithiasis
ASIANS common.
Caused by reflux of pancreatic enzymes 2/2 to abnormal biliopancreatic junction, having a long common channel
Dx: RUQ US 1st. 2nd MRCP next to define anatomy (diagnostic test of choice)f. Need to look all bile ducts intra-extrahepatic
Type I - MC 85% Saccular/fusiform dilation of CBD tx: CBD resection, Roux en y hepaticojejunostomy, and cholecystectomy
Type II - diverticulum off CBD tx: resection off CBD, and close primarily. If communicating with biliary tree you will need Roux en y hepaticojejunostomy
Type III or choledochocele: Dilation of duodenal CBD or where pancreas joins CBD. tx: Transduodenal approach with marsupialization or cyst resection may need sphincteroplasty
Type IVa fusiform both intra and extra hepatic tx: If only affecting one lobe partial hepatectomy with hepaticoJ. Or liver transplant
Type IVb multiple extra hepatic cysts – Tx: same as type I: CBD resection, Roux en y hepaticojejunostomy, and cholecystectomy
Type V (Caroli’s disease) - intrahepatic cyst only, get hepatic fibrosis, associated with congenital hepatic fibrosis and medullary sponge kidney tx: Partial liver resection or transplant
Lymphadenopathy in peds
-usually acute suppurative adenitis following URI or pharyngitis.
- If fluctuant and TENDER - FNA, culture, and abx. Recheck in 2 weeks.
- Chronic causes- cat scratch fever, atypical mycoplasma
Asymptomatic adenopathy in peds CNBx this is lymphoma until proven otherwise
Congenital Diaphragmatic hernia
!!50% survival!!!
Most die from congenital defects (neural tube and cardiac defects)
Usually on Left
Associated with vitamin A deficiency and exposure to drugs
Both lungs are dysfunctional
Ipsilateral lung (with hernia) severe pulmonary hypoplasia
Contralateral lung – severe pulmonary HTN, and some hypoplasia
Pulmonary HTN is the most important physiological affect causing hypoxia
50% will have life-long chronic pulmonary disease
80% have, malrotation, cardiac and neural tube defects (Most of them die due to the congenital defects not CDH)
Most devastating ECMO complication intracerebral bleeding
Dx:
- Prenatal dx can be made with MRI or US
- CXR for newborns to confirm will show bowel is in chest
Tx = medical optimization first
- Urgent surgical intervention IS NOT INDICATED HERE
- high frequency ventilation, NO, may need ECMO.
- intra-abdominal approach – reduce bowel and repair with mesh!
- Need to look for visceral anomalies (malrotation)
Bochdalek hernia
- MC hernia, located posteriorly on LEFT – Usually do not contain a hernia sac
Morgagni
More common in adults. Located anteriorly on the RIGHT, usually does contain hernia SAC.
Pectus excavatum
chest in, needs strut (Nuss Procedure) bar across mediastinum.
Repair if causing respiratory/cardiac sx or emotional distress with sternal osteotomy
All patients need echo and EKG preop. Have high cardiac abnormalities
Pectus carinatum
Pigeon chest. Strut not needed. First line treatment is a brace. Repair for emotional stress only
Cystic hygroma (lymphangioma)
Lymphatic malformation
Benign - Lateral to SCM in posterior neck triangle
Can get infected and forms sinuses
Tx: excision
Branchial cleft cyst
Leads to cyst, sinuses, or fistulas
1st branchial cleft cyst – Arise between angle of mandible to external auditory canal; facial nerve affected
2nd branchial cleft cyst (MC TYPE) - On the lower anterior border of middle SCM, goes through carotid bifurcation and into tonsillar pillar
* MC present as a cyst
3rd – Arise anterior to SCM in low neck into the piriform sinus, inside the pharynx!!
Tx: for all is resection of cyst
Thyroglossal duct cyst
Between hyoid bone and thyroid isthmus
Midline cervical mass, moves up with swallowing
Formed from descent of thyroid from foramen cecum
Risk of CA and infection
May be only thyroid tissue patient has
Tx: Sistrunk procedure: lateral neck incision excises cyst tract, hyoid bone
Hemangioma
MC tumor of childhood
Appears at birth or shortly after
Rapid growth during first 12 months then begins to involute
Most Resolves by age 8: tx: Observations unless, uncontrolled growth, impairs function (eyelid/ear canal), Bleed or ulcers, or persistent after age 8 → oral steroids. Laser or resection if not successful
Congenital AVM
-treatment is embolization.
-On lung: embolization.
-In bowel: endoscopy therapy
Neuroblastoma
#1 solid abdominal malignancy in < 2 years old
MC malignancy in infancy
<1 y/o have best prognosis. Prognosis is based on AGE and tumor biology
Overall survival <30%. 50% have mets at presentation
Symptoms:
* Asymptomatic abdominal mass
* Diarrhea
* Raccoon eyes (periorbital metastasis) proptosis, ecchymosis
* HTN
* opsomyoclonus syndrome (uncontrolled fast eye movement) (unsteady gait)
Mets to skin = blue skin lesions (blue berry muffin)
MC mets is adrenals but can occur anywhere on sympathetic chain
90% secrete catecholamines, Check UA: VMA, HVA, metanephrines
Derives from neural crest cells - Path = small round blue cells in ROSETTE pattern
Disease spectrum:
-ganglioneuroma (benign, still need to resect this)
-ganglioblastoma (malignant)
-neuroblastoma
One of the few malignancies in humans that can spontaneously regress
Worse prognosis:
- Age > 18 months, high grade, NSE (neuron specific enolase), LDH, > 3 copies of N-myc
Dx: CT scan: Calcifications compresses renal parenchyma rather than invades
MIBG – locates tumors
Metastasizes to bone, bone marrow, and liver (hepatomegaly)
Initially unresectable tumors can become resectable with doxorubicin-based chemo
NSE increased in all pt with mets
Tx: 5YS 40%
- Low risk (stage I and stage II): Just surgery, resect adrenal gland and kidney only
- Mod/high risk: neoadjuvant chemo (DECC) doxorubicin, etoposide, cisplatin and cyclophosphamide THEN RETINOIC ACID
- HIGH RISK: can down stage tumors; resection; Post op XRT
Resect even if stage IV
Stage I localized tumor completely excised
Stage II incomplete excision but does not cross midline
Stage III crosses midline +/- regional nodes
Stage IV distant mets (distant nodes or solid organ)
IV-S localized tumor with distant mets but age < 1
Wilm’s tumor (nephroblastoma)
10% are bilateral!
Hematuria, HTN and large abdominal mass in > 2 years old.
Most important prognostic factor is tumor grade
Much better prognosis than neuroblastoma
CT: replacement, of renal parenchyma. NO calcifications
Associated with WAGR: Wilms, aniridia, GU abnormality (cryptochordism and hypospadias), mental retardation. hemihypertrophy
Associated with Beckwith-Wiedemann (macroglossia, visceromegaly, hyper insulinemic hypoglycemia)
Tx:
- nephrectomy (90% cured)
- Dactinomycin and vincristine in all except If very low risk: stage I, < 500 g tumor, and favorable histology
- Pulm mets gets whole lung radiation
-If venous extension in renal vein, can still be extracted from the vein
-Need to Examine contralateral kidney and look for peritoneal implants
-Avoid rupture with resection can increase stage
-Need nephron sparing surgery for stage V (BL)
Stage I Limited to kidney, no capsule involvement Completely excised
Stage II Beyond kidney. Completely excised
Stage III residual non-hematogenous tumor. + nodes, or not completely excised
Stage IV hematogenous spread. Mets to any other organ
Stage V BL renal involvement
Time of repairs
-Cleft lip – (primary palate) –involves lip or alveolus or both. Failure of fusion of maxillary and medial nasal processes. Rule of 10, Repair at 10 weeks when Hgb is > 10, > 10 lbs weight
-Cleft palate - involves hard and soft palate. Hole in roof of mouth. Causes speech and swallowing issues. Repair at 1 year
-VSD: 80% close by 6 months. Surgical Tx: Repair when large (>2.5 shunt) at one year, and medium (2-2.5 shunt) at 5 years, Failure to thrive is MC reason for early repair
-ASD: Repair when symptomatic or asymptomatic at 5 years
-Tetralogy of Fallot: Tx: repair at 3-6 months with RV outflow tract obstruction removal. RVOFT enlargement, and VSD repair. VSD, RVH, overriding aorta, pulm stenosis
-PDA: Indomethacin only works for premature. Term babies need surgical repair with left thoracotomy immediately if symptomatic or @ 1-2 years old if asymptomatic
-Hypospadias - ventral opening tx: repair at 6 months with penile skin. Don’t circumcise
-Undescended Tests – Wait 6 months to repair
-Attempt to avoid splenectomy in children <5 years old. If < 10 years old and had splenectomy, needs daily augmentin for 6 months.
-Umbilical hernia; Delay until 5 years old unless incarcerated or patient has a VP SHUNT
-Hemangioma in children - Usually resolved by age 8: tx: Observations unless, uncontrolled growth, impairs function (eyelid/ear canal), it persistent after age 8 → oral steroids
Hepatoblastoma
MC liver CA in kids
Elevated AFP 90%, and BHCG (fractures and precocious puberty)
Better prognosis than HCC
Can be pedunculated, vascular invasion common
Survival is related to resectability
Strongly associated with beckwith-weidman
Tx: Resection followed by platinum based chemo, neoadjuvant doxorubicin based chemo can downstage and help with resection. Liver transplant
Fetal histology has the best prognosis
1 child malignancy
leukemia ALL
MC tumor in newborn
sacrococcygeal teratoma
