Choledochal cysts: must excise
- Leaving cyst:
- 25% cancer
- 30% pancreatitis
- Type I: (>90%) whole CBD involved.
- Excise, do hepatico-jejunostomy
- Type II: diverticulum.
- Do diverticulectomy
- Type III: Choledochocele involving sphincter.
- Excise, sphincteroplasty
- Type IV: Intra- and Extrahepatic cysts (Caroli's disease)
- Type V: Intrahepatic cysts.
- Extralobar has systemic artery and vein
- Intraloabar has aorta in and pulmonary vein out.
- Resection is treatment for both.
- #1 presentation = infection (not as respiratory distress in newborn)
Congenital lobar emphysema:
- Massive hyperinflation of a single lobe, usually upper/middle
- 1/3 have respiratory distress at birth, only 5% present after age 6 months
- M:F ratio is 2:1
- CXR: radiolucency of affected lobe, compression of other lobe
- Severely symptomatic: lobectomy, excellent Prognostic
Cystic hygroma: lymphangioma è resect
- Infection is #1 complication
Sistrunk procedure: Excision of thyroglossal duct cyst (midline) with hyoid bone
CHF in children: 1st sign is hepatomegaly
Strawberry hemagioma: appear in 1st few weeks of life
Tx: Leave alone since most involute by age 7.
Neuroblastoma: #1 solid pediatric malignancy
- 90% have increased VMA
- High HVA (homovanillic acid) è worse prognosis
- From neural crest
- Only 30% cure
- Associated with N-myc
#1 peds malignancy overall is leukemia
Wilm's tumor: Nephroblastoma.
- 80% cure with nephrectomy
Biliary atresia: Need Kasi procedure (before age 3 months) = hepatoportoenterostomy
- A true diverticulum
- Embryology = persistent omphalomesenteric duct
- On anti-mesenteric border.
- 2 feet from ileocecal valve
- 2% population
- 2% symptomatic
- 2 types of tissue (pancreatic, gastric)
- 2 common presentations (diverticulitis, GIB).
- #1 GIB in children.
- Obstruction mc presentation in adults
- Dx: Meckel scan (99Tc)
- Uncomplicated: diverticulectomy
- Complicated: segmental resection
- Reduce with air/contrast enema.
- IV glucagon can help (relaxes smooth muscle).
- Usually < 3 yo. To OR if peritonitis, free air.
- Adult w/intussusception goes to OR since high likelihood of malignancy at lead point
- Are secondary to intra-uterine vascular events.
- Mother may have polyhydramnios.
- 10% of atresias are multiple.
- Presents w/bilious vomiting
- #1 neonatal duodenal obstruction
- Associated with trisomy 21 (Down's)
- 1/3 have cardiac defects
- 90% are type C as in "Common"
- Blind esophagus, distal TEF
- Spit up feeds
- NGT won't pass
- 5% are type A
- Blind esophagus, no fistula
- No air in entire GI tract
- Associated with: VATER =
- Vertebral, anorectal (imperforate anus in 10%). TEF, radial, renal anomalies.
Ladd's procedure: (for mal-rotation)
- Take down bands
- Counterclockwise rotation.
Meconium ileus (cystic fibrosis):
- Try gastrograffin enema (Dx and Tx)
Hirschsprung's: #1 cause of colon obstruction
- No BM in 1st 24 hours
- Dx with rectal bx)
NEC: Presents after initiating feeds in neonate (preemie) with blood in stool.
- OR for free air, peritonitis, acidosis, thrombocytopenia, clinical deterioration (resect, ostomies)
- Must do contrast evaluation before reconnecting bowel weeks later (20% will have stenosis)
- If high, have meconium in urine (fistula to bladder, vagina or urethra) need colostomy.
- Intrauterine rupture of umbilical cord
- No associated defects
- Lateral (right) defect, no sac
- Midline defect
- May contain liver or other non-bowel contents
- Frequent anomalies (cardiac, pericardium, sternum, diaphragm = Cantell pentology)
- Has peritoneal sac.