Quan Dong-Wen, Li Peng-Gang, Xu Xiang-Hua, Liu Shi-Qi
The Second Clinical Medical School, Shaanxi University of Chinese Medicine, Xianyang 712000, Shaanxi Province, China.
The First School of Clinical Medicine, Shaanxi University of Chinese Medicine, Xianyang 712000, Shaanxi Province, China.
World J Clin Cases. 2024 Feb 26;12(6):1150-1156. doi: 10.12998/wjcc.v12.i6.1150.
Giant congenital biliary dilation (CBD) is a rare condition observed in clinical practice. Infants born with this condition often experience a poor overall health status, and the disease progresses rapidly, leading to severe biliary obstruction, infections, pressure exerted by the enlarged CBD on abdominal organs, disturbances in the internal environment, and multiple organ dysfunction. The treatment of giant CBD using laparoscopy is challenging due to the high degree of variation in the shape of the bile duct and other organs, making it difficult to separate the bile duct wall from adjacent tissues or to control bleeding.
Herein, we present the details of an 11-d-old male newborn who was diagnosed with giant CBD. The patient was admitted to the neonatal surgery department of our hospital due to a history of common bile duct cyst that was detected more than 3 mo ago, and also because the patient had been experiencing yellowish skin for the past 9 d. The abnormal echo in the fetal abdomen was first noticed by the patient's mother during a routine ultrasound examination at a local hospital, when the patient was at 24 wk + 6 d of pregnancy. This finding raised concerns about the possibility of congenital biliary dilatation (22 mm × 21 mm). Subsequent ultrasound examinations at different hospitals consistently confirmed the presence of a congenital biliary dilatation. No specific treatment was administered for biliary dilatation during this period. A computed tomography scan conducted during the hospitalization revealed a large cystic mass in the right upper quadrant and pelvis, measuring approximately 9.2 cm × 7.4 cm × 11.3 cm. Based on the scan, it was classified as a type I biliary dilatation.
The analysis reveals that prenatal imaging techniques, such as ultrasound and magnetic resonance imaging, play a crucial role in the early diagnosis, fetal prognosis, and treatment plan for giant CBD. Laparoscopic surgery for giant CBD presents certain challenges, including difficulties in separating the cyst wall, anastomosis, and hemostasis, as well as severe biliary system infection and ulceration. Consequently, there is a high likelihood of converting to laparotomy. The choice between surgical methods like hepaticojejunostomy (HJ) or hepaticoduodenostomy has not been standardized yet. However, we have achieved favorable outcomes using HJ. Preoperative management of inflammation, biliary drainage, liver function protection, and supportive treatment are particularly vital in improving children's prognosis. After discharge, it is essential to conduct timely reexamination and close follow-up to identify potential complications.
巨大先天性胆管扩张（CBD）是临床实践中罕见的病症。患有这种病症的婴儿通常整体健康状况较差，疾病进展迅速，导致严重胆管梗阻、感染、扩大的CBD对腹部器官施加压力、内环境紊乱和多器官功能障碍。由于胆管和其他器官形状的高度变异，使用腹腔镜治疗巨大CBD具有挑战性，使得难以将胆管壁与相邻组织分离或控制出血。
在此，我们介绍一名11日龄男性新生儿被诊断为巨大CBD的详细情况。该患者因3个多月前被检测出胆总管囊肿病史，且在过去9天一直出现皮肤发黄，入住我院新生儿外科。胎儿腹部的异常回声最早由患者母亲在当地医院的常规超声检查中发现，当时患者处于妊娠24周+6天。这一发现引发了对先天性胆管扩张（22毫米×21毫米）可能性的担忧。随后在不同医院的超声检查一致证实存在先天性胆管扩张。在此期间未对胆管扩张进行特殊治疗。住院期间进行的计算机断层扫描显示右上腹和盆腔有一个大的囊性肿块，大小约为9.2厘米×7.4厘米×11.3厘米。根据扫描结果，其被分类为I型胆管扩张。
分析表明，产前成像技术，如超声和磁共振成像，在巨大CBD的早期诊断、胎儿预后和治疗方案中起着关键作用。巨大CBD的腹腔镜手术存在一定挑战，包括囊肿壁分离困难、吻合和止血困难，以及严重的胆道系统感染和溃疡。因此，转为开腹手术的可能性很高。肝空肠吻合术（HJ）或肝十二指肠吻合术等手术方法之间的选择尚未标准化。然而，我们使用HJ取得了良好的效果。术前炎症管理、胆道引流、肝功能保护和支持治疗对于改善儿童预后尤为重要。出院后，必须及时进行复查和密切随访，以发现潜在并发症。
