presentation: progressive jaundice, pale stools, enlarged liver, elevated conj. Bilirubin, alk phos, and other liver enzymes
may do hepatic scintiscan after pretreatment with Phenobarbital to exclude biliary atresia if radionuclide shows up in intestine (shows bile duct patency)
treatment: laparotomy to inspect hilum of liver; if intraoperative cholangiogram shows patent bile ducts then dx is probably neonatal hepatitis
Kasai portoenterostomy – drain hepatic ductules at porta hepatis to halt disease process; cut across tissue and anastamose to intestinal tract before child is 8 wks old. 30% do well after this operation. Rest are candidates for liver transplant.
Prognosis: depends on diameter of hepatic ductules and absence of hepatic fibrosis. Recurrent postop cholangitis is common, give long-term abx prophylactically. Portal hypertension with esophageal varices is also common.
choledochal cysts – dilation of the common bile duct with distal obstruction
presentation: presents similarly to biliary atresia
identify with US
treatment: excise and reconstruct biliary system by anastamosing defunctionalized bowel loop to proximal hepatic ducts
Abdominal Wall Defects: Omphalocele and Gastroschisis
omphalocele and gastroschisis are congenital defects of the abdominal wall through which the abdominal contents protrude externally to a varying degree
embryology: four folds form abdominal wall: cephalic, caudal, and two lateral folds converge to form a large umbilical ring surrounding umb. Cord and yolk sac. Between weeks 5-10, intestine is extruded out of the umbilical ring and into the yolk sac. It then returns to abdominal cavity, where it undergoes rotation.
omphalocele results when lateral folds do not close, extruded viscera remain in yolk sac; omphaloceles are always covered by a translucent membrane
gastroschisis probably results from an in utero perforation of developing abdominal wall at pt where one of the paired umbilical veins undergoes atrophy (pt of weakness)
Omphalocele – opening in abd. wall with protruding viscera covered by a translucent membrane. High rate of associated congenital anomalies.
Gastroschisis – opening in abd. is lateral to umbilical cord, usu. to the R. Viscera are not covered by a membrane and may be inflamed and edematous. May have intestinal atresia caused by in utero compression and vascular compromise.
High risk for fluid and heat loss from exposed viscera – keep viscera moist with saline, wrap abdomen with plastic, IV fluids, broad spectrum abx, and NG decompression
Gastroschisis – keep viscera on top of or beside baby to avoid kinking of vascular supply to bowel. Emergency surgery is required to replace viscera in abdomen and close defect. If abdominal cavity is too small to allow primary closure, place “silo” over defect and manually compress daily to reduce viscera and expand abdominal cavity.