Department of Paediatric Surgery, Kings College Hospital, London SE5 9RS.
J Pediatr Surg. 2020 Dec;55(12):2642-2646. doi: 10.1016/j.jpedsurg.2020.05.017. Epub 2020 May 23.
Type 4 choledochal malformations (CMs) can be defined as extra- and intrahepatic biliary dilatation. They are of uncertain etiology but make up about 20% of most series. The aim of this study was to investigate the pathophysiology and their natural history following surgical intervention.
Ambispective review of a single-center series of type 4 CM between 1996 and 2017. Perioperative imaging, intraoperative pressure monitoring, bile amylase, and long-term follow-up data were analyzed based on extrahepatic morphology [(cystic (C), fusiform (F)]. Data were expressed as median (range). Statistical analysis was performed with nonparametric tests. P < 0.05 was considered significant.
37 children had type 4 CM in the study period [age at surgery 4 (0.2-16) years] and could be further subdivided into cystic (4C) (n = 22) or fusiform (4F) (n = 15). There was significantly greater dilatation of the extrahepatic component in the cystic group [30 (11-94) versus 15 (8-90) mm; P = 0.0002] though there was no difference in left duct diameter [cystic 7.5 (0-17) mm versus fusiform 7.5 (3-16) mm; P = 0.86]. There was a trend to higher choledochal pressure in the cystic group [19 (4-40) versus 9 (6-25) mmHg; P = 0.09] and those in the fusiform group had higher bile amylase [8650 (3-890,000) versus 592 (1-123,000) IU/L; P = 0.01] and were older [4.1 (0.92-16.43) versus 2.4 (0.15-15.48) years; P = 0.03]. Children with type 4 CM were then separated simply on the basis of bile amylase into LOW (characterized by high pressure, cystic morphology and young age at surgery) and HIGH bile amylase (low pressure, fusiform morphology and older at time of surgery).
We propose further division of type 4 CM into 4C and 4F on clinical and pathophysiological grounds.
II (prospective cohort).
4 型胆总管畸形（CM）可定义为肝内外胆管扩张。其病因不确定，但构成大多数系列的约 20%。本研究旨在探讨手术干预后的病理生理学及其自然史。
对 1996 年至 2017 年间单中心 4 型 CM 系列进行前瞻性回顾。根据肝外形态（囊性（C）、梭形（F））分析围手术期影像学、术中压力监测、胆汁淀粉酶和长期随访数据。数据表示为中位数（范围）。采用非参数检验进行统计分析。P<0.05 被认为具有统计学意义。
研究期间有 37 例儿童患有 4 型 CM[手术时年龄 4（0.2-16）岁]，可进一步分为囊性（4C）（n=22）或梭形（4F）（n=15）。囊性组的肝外成分扩张明显更大[30（11-94）比 15（8-90）mm；P=0.0002]，而左胆管直径无差异[囊性 7.5（0-17）mm 与梭形 7.5（3-16）mm；P=0.86]。囊性组胆管压力有升高趋势[19（4-40）比 9（6-25）mmHg；P=0.09]，梭形组胆汁淀粉酶较高[8650（3-890000）比 592（1-123000）IU/L；P=0.01]，年龄较大[4.1（0.92-16.43）比 2.4（0.15-15.48）岁；P=0.03]。然后，根据胆汁淀粉酶简单地将 4 型 CM 患儿分为 LOW（特征为高压、囊性形态和手术时年龄较小）和 HIGH 胆汁淀粉酶（低压、梭形形态和手术时年龄较大）。
我们建议根据临床和病理生理学依据进一步将 4 型 CM 分为 4C 和 4F。
II（前瞻性队列）。
