Case report: Cholecystoduodenostomy for cholestatic liver disease in a premature infant with cystic fibrosis and short gut syndrome

Abstract

BackgroundCholecystoduodenostomy is a surgical procedure that bypasses the extrahepatic biliary tree and connects the gallbladder directly to the duodenum. This case describes the successful use of this procedure in a novel situation.Case presentationA premature (34 weeks gestation) female infant with cystic fibrosis required a laparotomy on day 1 of life due to an intrauterine small bowel perforation. Resection of small bowel and ileostomy formation resulted in short gut syndrome, with 82 cm residual small bowel and intact ileocaecal valve. Post-ileostomy reversal at 2 months old, she developed conjugated hyperbilirubinaemia. Despite conservative management including increased enteral feeding, ursodeoxycholic acid, cholecystostomy drain insertion and flushes, her cholestatic jaundice persisted. A liver biopsy revealed an “obstructive/cholestatic” picture with fibrosis. To avoid further shortening her gut with an hepatoportoenterostomy, cholecystoduodenostomy was performed at 3 months of age with subsequent post-operative improvement and eventual normalisation of her clinical jaundice and liver biochemistry.ConclusionsThis is the first reported case of a cholecystoduodenostomy being used successfully to treat an infant with persistent conjugated hyperbilirubinemia, cystic fibrosis and short gut syndrome. Cholecystoduodenostomy is a treatment option that with further study, may be considered for obstruction of the common bile duct in patients with short gut and/or where a shorter operating time with minimal intervention is preferred.

Cystic fibrosis screening, evaluation, and management of hepatobiliary disease consensus recommendations

“…Biliary tract abnormalities can be present at birth, at diagnosis, or subsequently develop in infants and adults 122–125 . Biliary colic may occur in a small number of PwCF on initiation of CFTR modulator therapies 126 .…”

“…Biliary tract abnormalities can be present at birth, at diagnosis, or subsequently develop in infants and adults. [122][123][124][125] Biliary colic may occur in a small number of PwCF on initiation of CFTR modulator therapies. [126] Cholecystectomy is generally safe, with conservative preoperative and postoperative pulmonary management.…”

Severe obstructive biliopathy mimicking biliary atresia in an infant with cystic fibrosis

“…The Kasai portoenterostomy has been shown to have successfully reversed the hyperbilirubinaemia in two children with CF at 12 and 16 weeks with both patients free from further hepatobiliary disease at 30 and 40-months post-operation, respectively. 9 Furthermore, Fawcett et al 11 reported a case of a 3-month-old child with persistent conjugated hyperbilirubinaemia, CF and short gut syndrome in which a cholecystoduodenostomy (done in preference to a Roux-en Y due to short gut) successfully achieved bile drainage and normalised liver functions.…”