Cholecystoappendicostomy for progressive familial intrahepatic cholestasis.
We report a rare case of progressive familial intrahepatic cholestasis type 2 from India. The diagnosis was confirmed on the basis of gene mutation analysis. The child had intense pruritus refractory to conventional medical management. As liver biopsy did not reveal any cirrhosis, partial external biliary diversion was considered as an alternative to liver transplant. We performed cholecystoappendicostomy rather than the conventional method of using an ileal loop as a conduit between the gall bladder and abdominal wall. Child recovered completely.
Sharma
Deepa
D
Division of Pediatric Gastroenterology and Pediatric Surgery, Apollo Centre for Advanced Pediatrics, Indraprastha Apollo Hospitals, New Delhi 110 076, India.
Shah
Ubaid Hameed
UH
Sibal
Anupam
A
Chowdhary
Sujit K
SK
D001065:Appendix / Q000601:surgery*; D002675:Child, Preschool; D002763:Cholecystectomy / Q000379:methods*; D002780:Cholestasis, Intrahepatic / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D011537:Pruritus