Pediatric Surgery Flashcards
Paraneoplastic syndrome associated with Wilm’s tumor
Some Wilm tumors produce renin, causing hypertension through the RAAS axis
Wilm’s tumor
- Occurs in kids age 1-5
- Genetic risk factors (WT1, BW syndrome, Drash syndrome)
- Generally good prognosis
- Does not cause ESRD except with low likelihood in a subset of individuals with bilateral WT or with other unassociated renal conditions
- Tx:
- Most cases require surgery with adjuvant radiation and/or chemotherapy
- For a patient < 2 years of age, stage I disease, favorable histology, surgical resection and close surveillance is sufficient
Neuroblastoma
- Most common solid extra-cranial tumor in childhood
- 90% of cases prior to age 6
- Paraneoplastic syndromes:
- Opsoclonus-myoclonus syndrome
- VIPoma syndrome
- Pheochromocytoma/paraganglioma syndrome
-
Originate from neural crest cells of the SNS
- Hence, common locations: adrenals, posterior mediastinum, retroperitoneum, pelvis, neck (adrenals and along the sympthathetic chain)
- Abdominal NBL tends to be most aggressive subtype
- Staging is done with MRI (good for evaluation of bone marrow involvement)
Imaging study of choice for evaluating pediatric abdominal masses
Start with KUB.
Then, ultrasound.
If ultrasound shows a solid tumor, CT is indicated for imaging characteristics, surgical planning, and staging.
Nephroblastomatosis
- Immature metanephric tissue or nephrogenic nests
- Considered a precursor to Wilm’s tumor when identified
- If found in a kidney containing a Wilm’s tumor, recurrence in the contralateral kidney is 20%
Image-defined risk factors
- Major topic for neuroblastomas
- CT assessment of degree of involvement with other structures
- Tumor with absence of IDRFs can achieve more complete resection, and thus has better prognosis
Pediatric abdominal mass with associated calcifications in the intestinal tract
Meconium ileus
Pediatric abdominal mass with associated calcifications within the mass
Neuroblastoma
Neuroblastoma is associated with . . .
- Hirschprung’s disease
- Neurofibromatosis type I
- Beckwith-Wiedemann syndrome
- DiGeorge syndrome
“Stage V” Wilm’s tumor
Bilateral Wilm’s tumors
The most common cause of enlarged renal mass in a neonate is ___
The most common cause of enlarged renal mass in a neonate is hydronephrosis
Most common retroperitoneal mass in a child older than 1 year
Neuroblastoma
Management of persistent jaundice in infants (table)
Correction of biliary atresia should be performed before ___ of age
Correction of biliary atresia should be performed before 8 weeks of age
Lab findings of biliary atresia in a newborn
- GGT > 100 IU/L
- Alk Phos > 600 IU/L
- AST/ALT of 80-200 U/L
DDx of obstructive-pattern jaundice in a newborn
- Type I biliary atresia: Atresia of common bile duct
- Type II biliary atresia: Atresia of the common hepatic duct
- Type III biliary atresia: Atresia of the most proximal portion of the bile ducts (95% of cases)
- Obstructive choledochal cyst
- Alagille syndrome
- Caroli disease
- TPN-induced gall bladder stasis
Things that should always be considered for jaundice in a newborn
- Biliary disease
- Hemolytic disease or hematoma
- TORCH infection
- Sepsis
- Inborn error of metabolism
The average full-term newborn has a peak serum bilirubin of ___.
Any bilirubin greater than ___ should be regarded as pathologic.
The average full-term newborn has a peak serum bilirubin of 5-6 mg/dL.
Any bilirubin greater than 17 mg/dL should be regarded as pathologic.
Modes of pathology of hyperbilirubinemia
- Kernicterus (deposition of unconjugated bilirubin in basal ganglia, and other locations)
- General cellular toxicity of bilirubin (inhibits mithcondrial enzymes, interferes with DNA synthesis, induces DNA-strand breaks via intercalation, inhibits protein synthesis and phosphorylation)
Recommended threshold for exchange transfusion in hyperbilirubinemia of the neonate
> 20 mg/dL
Four phenotypes of biliary atresia (different from type I-III)
- Isolated biliary atresia: Biliary atresia is the only anomaly
- Cystic obliteration of the biliary tree: Biliary atresia + cystic changes
- Perinatal CMV infection: Biliary atresia associated with CMV infection (a potential etiology of biliary atresia)
- Syndromic biliary atresia: Biliary atresia in the context of other syndromic malformations
Kasai Portoenterostomy
- aka the Kasai procedure
- Surgery to correct biliary atresia
- Optimally done before 8 weeks of age and before kernicterus-range hyperbilirubinemia develops
- Optimally performed on a patient with preserved liver function and without cirrhosis
- Perioperative considerations:
- Ensure sufficient coagulation status prior to surgery
- High-energy and high-protein formulas recommended perioperatively to prophylax against malabsorption
- High suspicion for cholangitis in the perioperative period
- Complications:
- Post-operative portoenterostomy cholangitis (common)
- Most will develop progressive biliary obstruction later in life
- Still likely to need liver transplantation for prolonged survival down the road
How long does it take for bilirubin to normalize after a Kasai procedure for biliary atresia?
~6 months
Definitive diagnosis of biliary atresia can really only be made. . .
. . . intraoperatively with intra-op cholangiography
In infants older than ___, liver transplant may be a better initial treatment than Kasai procedure for biliary atresia.
In infants older than 120 days, liver transplant may be a better initial treatment than Kasai procedure for biliary atresia.
BASM syndrome
- Biliary atresia splenic malformation syndrome
- Common syndromic presentation of biliary atresia
Infantile hemangioma
- Simple hemangiomas may be observed without intervention, but those with concerning features require workup
Toddler’s fractures
- Spiral fractures of the distal tibia
- Seen in ambulatory children age <3
- Result from a twisting injury during a fall
For splenectomy, you give the Hib vaccine. . .
. . . EVEN if they are ALREADY vaccinated against Hib
Young kid (~2 years old) presents with failure to thrive and multiple episodes of solid food impaction. EGD shows an impression on the posterior esophagus. What is the likely diagnosis?
Vascular ring
