Case report: Cholecystoduodenostomy for cholestatic liver disease in a premature infant with cystic fibrosis and short gut syndrome
Cholecystoduodenostomy 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.
A 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.
This 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.
KeywordsCystic fibrosis Conjugated hyperbilirubinaemia Prematurity Short gut syndrome Cholestasis
Liver Function Test
Cystic fibrosis (CF) is an autosomal recessive disease of inflammation, obstruction and infection caused by mutations in the CF transmembrane conductance regulator gene. Mutations in this gene lead to an abnormality of, or reduction in, Chloride channels in the apical membranes of cells present in a variety of organs including the lung, pancreas and liver. The chloride channel abnormality results in thick sticky mucous in the airways and pancreas . Neonatal cholestasis and meconium ileus are recognised complications [1, 2, 3, 4]. Obstruction of the biliary tract (intra and extrahepatic) occurs in cystic fibrosis due to inspissated bile. Hepatocyte damage is thought to result from the subsequent accumulation of toxic bile acids and depletion of hepatic antioxidants [2, 4]. Extrahepatic biliary tract obstruction can be overcome with bypass surgery or drainage procedures. The Kasai procedure for infants with biliary atresia is a common example of bypass surgery. Cholecystostomy, where a drain is inserted into the gallbladder to allow bile to drain externally overcomes the acute obstruction but is not a definitive procedure. Cholecystoduodenostomy is a less frequently used surgical procedure that bypasses the extrahepatic biliary tree and connects the gallbladder directly to the duodenum. Here we report a rare and unusual case which required an infrequently used intervention. We discuss the challenges in managing this infant and the successful use of cholecystoduodenostomy to bypass the biliary obstruction.
Discussion and conclusions
Hepatoportoenterostomy (Kasai procedure) is the preferred treatment for biliary atresia and may be used to bypass other causes of cholestasis due to bile duct obstruction . However, this procedure requires mobilisation of a portion of the small bowel and would lead to further shortening of the gut. It involves removal of the extrahepatic biliary tract and the formation of a Roux-en-Y loop. We were concerned that the consequences of further shortening the gut would result in a longer period of dependence on parenteral nutrition, which would impact further on an already fibrotic liver. Portoduodenostomy is another biliary bypass procedure which does not require removal of any further small bowel. In cases where this has used the ileocaecal appendix to bypass biliary atresia there is a high risk for stone formation and obstruction because bile can stagnate in the ileocecum . When this procedure is compared with the Kasai, improvement of hyperbilirubinaemia is less successful, with the portoduodenostomy group having a higher number of patients proceeding to liver transplantation . With the viscous bile which is present in CF, failure of biliary drainage could be hypothesised to be of increased risk in our patient’s case. Cholecystoduodenostomy is an alternative procedure that attaches the gallbladder to the duodenum, which leaves the small bowel and extrahepatic biliary tree intact. It is a less invasive and shorter operation than the Kasai. Complications of cholecystoduodenostomy include cholangitis  as in this reported case of the procedure being used to manage a case of extra hepatic biliary atresia. There may secondary benefit of antibiotic prophylaxis in our patient, reducing her risk of cholangitis.
There was an urgency to manage the obstruction in our case, which required surgical intervention following failure of conservative management. The patient was small (3.9 kg), had poor weight gain (contributed to by short gut and malabsorption due to CF), has had previous abdominal surgeries and had adhesions from her meconium peritonitis. To the best of our knowledge, this is the first reported case of an infant with CF and short gut syndrome successfully undergoing cholecystoduodenostomy for neonatal cholestasis from extrahepatic biliary obstruction. Cholecystoduodenostomy may be a surgical option for obstruction of the common bile duct in affected infants with short gut syndrome and/or other factors where a shorter, less invasive surgery is required. Further experience and evaluation of cholecystoduodenostomy in this patient population is recommended to determine its efficacy and long term outcomes.
Dr. Albert Shun and Dr. Gordon Thomas from the Department of Surgery, Children’s hospital Westmead, Sydney, contributed to the case discussion and management of our patient.
This case was presented at the 41st ECFS conference in Belgrade, Serbia.
Graphical Image courtesy of David Fawcett – Redfawn Photographic + Visual arts.
No funding was provided for this study.
Availability of data and materials
Data sharing is not applicable to this article.
LF drafted the document with the supervision and guidance of CO, JW and GH. CO, JW and GH were the lead clinicians for this case, who reviewed the treatment options, formulated and carried out the management plan. All authors read and approved the final manuscript.
Ethics approval and consent to participate
Ethics approval was obtained from Sydney Children’s Hospital Network Human Research Ethics committee. CCR2018/03.
Consent for publication
Signed consent was obtained from our patient’s parents to publish this case report.
There are no competing interest related to the case to declare. CO is a consultant for Vertex pharmaceuticals.
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