Case report: Cholecystoduodenostomy for cholestatic liver disease in a premature infant with cystic fibrosis and short gut syndrome
Abstract Background 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. Case presentation A premature (34 weeks gestation) fema...
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| Format: | Article |
| Language: | English |
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BMC
2019-03-01
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| Series: | BMC Pediatrics |
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| Online Access: | http://link.springer.com/article/10.1186/s12887-019-1443-5 |
| _version_ | 1811330849480638464 |
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| author | Laura K. Fawcett John Widger Guy M. Henry Chee Y. Ooi |
| author_facet | Laura K. Fawcett John Widger Guy M. Henry Chee Y. Ooi |
| author_sort | Laura K. Fawcett |
| collection | DOAJ |
| description | Abstract Background 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. Case presentation 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. Conclusions 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. |
| first_indexed | 2024-04-13T16:08:59Z |
| format | Article |
| id | doaj.art-7b399fc1a7224c9691e4e9e34514e9be |
| institution | Directory Open Access Journal |
| issn | 1471-2431 |
| language | English |
| last_indexed | 2024-04-13T16:08:59Z |
| publishDate | 2019-03-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pediatrics |
| spelling | doaj.art-7b399fc1a7224c9691e4e9e34514e9be2022-12-22T02:40:18ZengBMCBMC Pediatrics1471-24312019-03-011911410.1186/s12887-019-1443-5Case report: Cholecystoduodenostomy for cholestatic liver disease in a premature infant with cystic fibrosis and short gut syndromeLaura K. Fawcett0John Widger1Guy M. Henry2Chee Y. Ooi3Department of Respiratory Medicine, Sydney Children’s HospitalDepartment of Respiratory Medicine, Sydney Children’s HospitalDepartment of General Surgery, Sydney Children’s HospitalDiscipline of Paediatrics, School of Women’s and Children’s Health, Medicine, University of New South WalesAbstract Background 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. Case presentation 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. Conclusions 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.http://link.springer.com/article/10.1186/s12887-019-1443-5Cystic fibrosisConjugated hyperbilirubinaemiaPrematurityShort gut syndromeCholestasis |
| spellingShingle | Laura K. Fawcett John Widger Guy M. Henry Chee Y. Ooi Case report: Cholecystoduodenostomy for cholestatic liver disease in a premature infant with cystic fibrosis and short gut syndrome BMC Pediatrics Cystic fibrosis Conjugated hyperbilirubinaemia Prematurity Short gut syndrome Cholestasis |
| title | Case report: Cholecystoduodenostomy for cholestatic liver disease in a premature infant with cystic fibrosis and short gut syndrome |
| title_full | Case report: Cholecystoduodenostomy for cholestatic liver disease in a premature infant with cystic fibrosis and short gut syndrome |
| title_fullStr | Case report: Cholecystoduodenostomy for cholestatic liver disease in a premature infant with cystic fibrosis and short gut syndrome |
| title_full_unstemmed | Case report: Cholecystoduodenostomy for cholestatic liver disease in a premature infant with cystic fibrosis and short gut syndrome |
| title_short | Case report: Cholecystoduodenostomy for cholestatic liver disease in a premature infant with cystic fibrosis and short gut syndrome |
| title_sort | case report cholecystoduodenostomy for cholestatic liver disease in a premature infant with cystic fibrosis and short gut syndrome |
| topic | Cystic fibrosis Conjugated hyperbilirubinaemia Prematurity Short gut syndrome Cholestasis |
| url | http://link.springer.com/article/10.1186/s12887-019-1443-5 |
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