Department of Pediatric Surgery, Chiba Children's Hospital, Japan.
Department of Pediatric Surgery, Aichi Prefectural Colony, Japan.
J Pediatr Surg. 2024 Feb;59(2):240-246. doi: 10.1016/j.jpedsurg.2023.10.037. Epub 2023 Oct 21.
The aim of the study is to clarify the clinicopathological and biliary morphological characteristics in reported cases of diverticular congenital biliary dilatation (CBD).
Using PubMed and the Japan Medical Abstracts Society, articles on possible diverticular CBD were extracted and the clinical pictures examined. We also sought evidence for definitions of diverticular CBD and the associated condition of pancreaticobiliary maljunction (PBM) using the original articles by Alonso-Lej and Todani. The characteristic biliary morphologies of cases with images were also investigated.
Analyses of 211 possible cases superficially demonstrated multiple diverticula in 12 (12%) and single diverticulum in 89 (88%), with diverticula located in the upper (n = 38, 38%), middle (n = 32, 32%), or lower (n = 26, 26%) biliary tract in and presence of intra-diverticular stones, PBM, and biliary carcinoma in 23% (n = 18), 39% (n = 25), and 11% (n = 14), respectively. However, evidence defining diverticular CBD or justifying the lack of associated PBM was not demonstrated even in the original articles. Scrutiny of the biliary anatomy in 59 cases with images showed incorrect inclusions of types I or IV-A with an irregular biliary duct wall or dilated cystic duct, periampullary choledochal diverticula, or even solitary biliary cysts. Authentic diverticular CBD, representing the diverticulum connected to the middle of the common bile duct via a thin, patent stalk was seen in only 6 cases.
Real diverticular CBD appears extremely rare. The lack of an objective definition allows wide interpretations of clinical pictures, creating inconsistencies in the diagnosis and treatment of CBD and raising questions regarding the utility of conventional classifications.
Level III.
本研究旨在阐明已报道的憩室性先天性胆管扩张症（CBD）病例的临床病理和胆系形态学特征。
利用 PubMed 和日本医学摘要协会，提取了可能存在憩室性 CBD 的文献，并对其临床图片进行了检查。我们还使用 Alonso-Lej 和 Todani 的原始文章，对憩室性 CBD 的定义和相关胰胆管合流异常（PBM）的证据进行了研究。我们还对有图像的病例的特征性胆道形态进行了研究。
对 211 例疑似病例的分析表明，12 例（12%）有多个憩室，89 例（88%）有单个憩室，憩室位于胆管的上段（n=38，38%）、中段（n=32，32%）或下段（n=26，26%），伴有腔内结石、PBM 和胆管癌的分别占 23%（n=18）、39%（n=25）和 11%（n=14）。然而，即使在原始文章中，也没有证据证明憩室性 CBD 的定义或 justifies PBM 的缺失。对 59 例有图像的胆道解剖结构进行审查后发现，有错误地将不规则胆管壁或扩张的胆囊管、胰胆管旁憩室或甚至单个胆管囊肿纳入 I 型或 IV-A 型，这些均不属于真正的憩室性 CBD。只有 6 例真正的憩室性 CBD 表现为通过一个薄而通畅的茎与胆总管中段相连的憩室。
真正的憩室性 CBD 极为罕见。缺乏客观定义使得对临床图片的解释变得宽泛，导致 CBD 的诊断和治疗不一致，并对传统分类的实用性提出质疑。
III 级。
