Introduction

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.

Methods

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.

Results

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).

Conclusions

We propose further division of type 4 CM into 4C and 4F on clinical and pathophysiological grounds.

Level of evidence

II (prospective cohort).

中文翻译：

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深入了解4型胆总管畸形的病理生理和分类。

介绍

4型胆总管畸形（CMs）可定义为肝外和肝内胆管扩张。它们的病因不确定，但约占大多数序列的20％。这项研究的目的是调查手术干预后的病理生理及其自然史。

方法

对1996年至2017年间单中心4型CM系列进行了回顾性回顾。根据肝外形态分析了围手术期影像学，术中压力监测，胆汁淀粉酶和长期随访数据[（囊性（C），梭形（ F）]的数据以中位数（范围）表示，并通过非参数检验进行统计分析，P <0.05被认为是显着的。

结果

在研究期间[手术4（0.2–16）岁的年龄]中有37名儿童患有4型CM，可以进一步分为囊性（4C）（n  = 22）或梭形（4F）（n = 15）。囊性组肝外成分的扩张明显更大[30（11–94）mm对15（8–90）mm；P = 0.0002]，尽管左管直径[囊状7.5（0–17）mm与梭形7.5（3–16）mm]无差异。P = 0.86]。囊性组胆总管压力有升高趋势[19（4–40）vs 9（6–25）mmHg；P = 0.09]，梭形组的胆汁淀粉酶水平较高[8650（3–890,000），而IU / L为592（1–123,000）；P = 0.01]，年龄较大[4.1（0.92–16.43）岁，而2.4（0.15–15.48）岁；P = 0.03]。然后简单地根据胆汁淀粉酶将4型CM儿童分为LOW（以高压，囊性形态和手术年龄为特征）和HIGH胆汁淀粉酶（以低压，梭形形态以及手术时年龄较大）。

结论

我们建议根据临床和病理生理基础将4型CM进一步分为4C和4F。

证据水平

II（预期队列）。