Biliary atresia with rare associations: a case report and literature review

Basel A. Zaben, Ahmad M. Abualrub, Waleed M. Malhes, Anas M. Barabrah, Anas R. Tuqan, Ibrahem A. Tahhan, Wael Amro
{"title":"Biliary atresia with rare associations: a case report and literature review","authors":"Basel A. Zaben, Ahmad M. Abualrub, Waleed M. Malhes, Anas M. Barabrah, Anas R. Tuqan, Ibrahem A. Tahhan, Wael Amro","doi":"10.1097/ms9.0000000000002173","DOIUrl":null,"url":null,"abstract":"\n \n Biliary atresia is a rare, progressive cholangiopathy that affects newborns, causing jaundice and other manifestations of hyperbilirubinemia. The incidence is higher in Asia than in Europe. The only available treatment is a surgical operation called Kasai portoenterostomy. In our case, we highlighted rare congenital anomalies that came with biliary atresia.\n \n \n \n A 10-day-old male infant was admitted to the hospital due to recurrent vomiting, yellowish skin, and scleral icterus. Laboratory investigations revealed elevated total serum and direct bilirubin levels. An atrophic gallbladder was observed on ultrasound. Intrahepatic cholangiography confirmed the diagnosis of biliary atresia, leading to the performance of a Kasai procedure. Additionally, the patient had intestinal malrotation and volvulus, which were managed with a Ladd’s procedure. Following surgery, there was notable improvement in liver enzymes and bilirubin levels, and the patient was discharged after 7 days. The infant has been initiated on oral vitamins, ursodeoxycholic acid, and antibiotics.\n \n \n \n Biliary atresia is a challenging condition characterized by progressive narrowing and fibrosis of the biliary tree. It is rarely associated with rare congenital anomalies like situs inversus totalis, intestinal malrotation, and volvulus. Diagnosis involves abdominal ultrasound and MRCG. The biliary atresia was managed by the kasai procedure and the intestinal malrotation, and volvulus were managed by Ladd’s procedure.\n \n \n \n This case report highlights the importance of considering rare associations such as situs inversus, intestinal malrotation and volvulus in the diagnosis of biliary atresia in newborn. Early diagnosis and prompt intervention are crucial for optimal outcomes.\n","PeriodicalId":503882,"journal":{"name":"Annals of Medicine & Surgery","volume":"12 5","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2024-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Annals of Medicine & Surgery","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1097/ms9.0000000000002173","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0

Abstract

Biliary atresia is a rare, progressive cholangiopathy that affects newborns, causing jaundice and other manifestations of hyperbilirubinemia. The incidence is higher in Asia than in Europe. The only available treatment is a surgical operation called Kasai portoenterostomy. In our case, we highlighted rare congenital anomalies that came with biliary atresia. A 10-day-old male infant was admitted to the hospital due to recurrent vomiting, yellowish skin, and scleral icterus. Laboratory investigations revealed elevated total serum and direct bilirubin levels. An atrophic gallbladder was observed on ultrasound. Intrahepatic cholangiography confirmed the diagnosis of biliary atresia, leading to the performance of a Kasai procedure. Additionally, the patient had intestinal malrotation and volvulus, which were managed with a Ladd’s procedure. Following surgery, there was notable improvement in liver enzymes and bilirubin levels, and the patient was discharged after 7 days. The infant has been initiated on oral vitamins, ursodeoxycholic acid, and antibiotics. Biliary atresia is a challenging condition characterized by progressive narrowing and fibrosis of the biliary tree. It is rarely associated with rare congenital anomalies like situs inversus totalis, intestinal malrotation, and volvulus. Diagnosis involves abdominal ultrasound and MRCG. The biliary atresia was managed by the kasai procedure and the intestinal malrotation, and volvulus were managed by Ladd’s procedure. This case report highlights the importance of considering rare associations such as situs inversus, intestinal malrotation and volvulus in the diagnosis of biliary atresia in newborn. Early diagnosis and prompt intervention are crucial for optimal outcomes.
罕见的胆道闭锁:病例报告和文献综述
胆道闭锁是一种罕见的进行性胆管病变,会影响新生儿,导致黄疸和其他高胆红素血症表现。亚洲的发病率高于欧洲。唯一的治疗方法是外科手术,即 "葛西肠管造口术"。在我们的病例中,我们强调了胆道闭锁的罕见先天性畸形。 一名出生 10 天的男婴因反复呕吐、皮肤发黄和巩膜黄疸入院。实验室检查显示血清总胆红素和直接胆红素水平升高。超声波检查发现胆囊萎缩。肝内胆管造影证实了胆道闭锁的诊断,因此进行了卡萨伊手术。此外,患者还伴有肠旋转不良和肠道内卷,通过拉德手术进行了处理。手术后,患者的肝酶和胆红素水平明显改善,7 天后出院。婴儿开始口服维生素、熊去氧胆酸和抗生素。 胆道闭锁是一种具有挑战性的疾病,其特点是胆管逐渐狭窄和纤维化。它很少伴有罕见的先天性畸形,如全坐位、肠旋转不良和肠卷。诊断方法包括腹部超声和 MRCG。胆道闭锁通过卡萨伊手术进行了处理,肠旋转不良和肠卷通过拉德手术进行了处理。 本病例报告强调了在诊断新生儿胆道闭锁时考虑坐位倒置、肠旋转不良和肠旋转等罕见并发症的重要性。早期诊断和及时干预对获得最佳治疗效果至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 求助全文
来源期刊
自引率
0.00%
发文量
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信