Department of Paediatric Surgery, King's College Hospital, Denmark Hill, London, SE5 9RS, UK.
J Pediatr Surg. 2011 Feb;46(2):299-303. doi: 10.1016/j.jpedsurg.2010.11.008.
Type 4 choledochal malformations (CMs) may be defined as those with both intrahepatic and extrahepatic bile duct dilatation. The aims of this study were to investigate possible causes of intrahepatic duct (IHD) dilatation in CM and to define the effect of surgery over time.
This study was a single-center retrospective review of a database of all children with CM undergoing surgery (excision of extrahepatic bile duct dilatation and hepaticojejunostomy) and identified as type 4 (on imaging and at surgery). Data included intraoperative choledochal pressure measurements and biliary amylase content and were expressed as median (interquartile range [IQR]). All comparisons used nonparametric statistical tests. P ≤.05 was regarded as significant.
Twenty children were identified as type 4 CM (age, 4.3 years; range, 2.7-10.4 years) with preoperative IHD dilatation (right duct: diameter [range], 8.5 [4.5-14] mm; left: 8 [4-14.5] mm). Median intraoperative choledochal pressure was 17 (8-27) mm Hg (normal, <5 mm Hg), and intraoperative bile amylase was 3647 (range, 500-58,000) IU/L (normal, <100 IU/L). Preoperative IHD diameter correlated with choledochal pressure (right: r(s)=0.46, P = .03; left: r(s)=0.34, P = .07) but not with biliary amylase (P = .28 and P = .39, respectively). At 1 year postsurgery, median (range) IHD diameter had decreased to 1 (1-2.5) mm for right duct (P = .0002) and 1.5 (1-3) mm for left duct (P = .0006) and remained stable for up to a 10-year follow-up.
Our data suggest that IHD dilatation is related to sustained increased intrabiliary pressure rather than any intrinsic intrahepatic CM. Effective surgery invariably reduces measured IHD toward normal values.
4 型胆总管畸形（CM）可定义为同时存在肝内和肝外胆管扩张的病变。本研究旨在探讨 CM 肝内胆管（IHD）扩张的可能原因，并明确手术随时间的影响。
这是一项单中心回顾性研究，对所有接受手术（切除肝外胆管扩张和胆肠吻合术）并被诊断为 4 型 CM（影像学和手术）的患儿的数据库进行了回顾。数据包括术中胆总管压力测量和胆汁淀粉酶含量，并以中位数（四分位距 [IQR]）表示。所有比较均采用非参数统计检验。P≤.05 被认为有统计学意义。
共确定 20 例 4 型 CM 患儿（年龄 4.3 岁；范围 2.7-10.4 岁）术前存在 IHD 扩张（右胆管：直径 [范围]，8.5 [4.5-14] mm；左胆管：8 [4-14.5] mm）。术中胆总管压力中位数为 17（8-27）mmHg（正常<5 mmHg），术中胆汁淀粉酶为 3647（500-58000）IU/L（正常<100 IU/L）。术前 IHD 直径与胆总管压力相关（右胆管：r(s)=0.46，P =.03；左胆管：r(s)=0.34，P =.07），但与胆汁淀粉酶无关（P =.28 和 P =.39）。术后 1 年，右胆管 IHD 直径中位数（范围）降至 1（1-2.5）mm（P =.0002），左胆管降至 1.5（1-3）mm（P =.0006），并在 10 年随访中保持稳定。
我们的数据表明，IHD 扩张与持续升高的胆道内压有关，而与肝内 CM 无关。有效的手术可使 IHD 测量值降低至正常范围。
