A case report of intrahepatic bile duct confluence anomalies in VACTERL syndrome
DC Field | Value | Language |
---|---|---|
dc.contributor.author | Yoon, Yoonsun | - |
dc.contributor.author | Kim, Kyungju | - |
dc.contributor.author | Yeom, Suk Keu | - |
dc.contributor.author | Lee, JeeHyun | - |
dc.contributor.author | Lee, Yoon | - |
dc.date.accessioned | 2021-09-02T07:32:20Z | - |
dc.date.available | 2021-09-02T07:32:20Z | - |
dc.date.created | 2021-06-16 | - |
dc.date.issued | 2018-09 | - |
dc.identifier.issn | 0025-7974 | - |
dc.identifier.uri | https://scholar.korea.ac.kr/handle/2021.sw.korea/73694 | - |
dc.description.abstract | Rationale: The clinical manifestations of VACTERL association include vertebral anomalies, anal atresia, congenital heart diseases, tracheoesophageal fistula, renal dysplasia, and limb abnormalities. The association of intrahepatic anomalies and VACTERL syndrome is a rare coincidence. VACTER syndrome and intrahepatic bile drainage anomalies might be genetically related. Patient concerns: A 12-year-old girl presented with episodic colicky abdominal pain, nausea, and vomiting for several years. The individual episodes resolved spontaneously within a few days. She had a history of VACTERL syndrome, including a butterfly shape of the L3 vertebra, anal atresia, and an atrial septal defect. Diagnoses: On laboratory findings, abnormal liver function tests included elevated total bilirubin, alanine aminotransferase, aspartate aminotransferase, and gamma-glutamyltransferase. There was no significant abnormal finding in hepatobiliary system sonography except mild gallbladder wall thickening. We performed magnetic resonance cholangiopancreatography and demonstrated an abnormal intrahepatic bile duct confluence, which showed 3 bile ducts draining directly into the neck of the gallbladder. Intervention: Her symptoms related to bile reflux during gallbladder contraction. Cholecystectomy with choledochojejunostomy was undertaken because segments of the bile drainage were intertwined. Outcomes: After surgery, her symptoms decreased, but abdominal discomfort remained due to uncorrected left intrahepatic anomalies. Lessons: Although hepatobiliary anomalies are not included in VACTERL association diagnostic criteria, detailed hepatobiliary work up is needed when gastrointestinal symptoms are present in VACTERL association patients. | - |
dc.language | English | - |
dc.language.iso | en | - |
dc.publisher | LIPPINCOTT WILLIAMS & WILKINS | - |
dc.subject | ANATOMIC VARIATIONS | - |
dc.subject | ASSOCIATION | - |
dc.title | A case report of intrahepatic bile duct confluence anomalies in VACTERL syndrome | - |
dc.type | Article | - |
dc.contributor.affiliatedAuthor | Yeom, Suk Keu | - |
dc.contributor.affiliatedAuthor | Lee, Yoon | - |
dc.identifier.doi | 10.1097/MD.0000000000012411 | - |
dc.identifier.scopusid | 2-s2.0-85054418069 | - |
dc.identifier.wosid | 000449373500038 | - |
dc.identifier.bibliographicCitation | MEDICINE, v.97, no.39 | - |
dc.relation.isPartOf | MEDICINE | - |
dc.citation.title | MEDICINE | - |
dc.citation.volume | 97 | - |
dc.citation.number | 39 | - |
dc.type.rims | ART | - |
dc.type.docType | Article | - |
dc.description.journalClass | 1 | - |
dc.description.journalRegisteredClass | scie | - |
dc.description.journalRegisteredClass | scopus | - |
dc.relation.journalResearchArea | General & Internal Medicine | - |
dc.relation.journalWebOfScienceCategory | Medicine, General & Internal | - |
dc.subject.keywordPlus | ANATOMIC VARIATIONS | - |
dc.subject.keywordPlus | ASSOCIATION | - |
dc.subject.keywordAuthor | gallbladder | - |
dc.subject.keywordAuthor | intrahepatic bile duct | - |
dc.subject.keywordAuthor | VACTERL syndrome | - |
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