Background

The present study was aimed to update the classification of hepatobiliary malformations and study the clinicopathological profile of pediatric choledochal cyst (CDC) and pediatric giant choledochal cyst (GCC) patients undergone surgery.

Methods

We have retrospectively analysed the data of 57 consecutive cases of CDCs in paediatric patients from a time period spanning from 2016 to 2020.

Results

Revised classification of hepatobiliary malformations was proposed and these were divided into 2 broad headings, choledochal (congenital and acquired) and extra-choledochal spectrum. 57 pediatric patients were diagnosed as having CDC with average age 4.615 years and female to male ratio of 3.7:1. We have also reported one of the largest GCC measuring 23 × 10 × 9 cm size. The classical triad was known to be more common and seen in 60% GCCs as opposed to 14.5% in CDCs. Values of serum bilirubin, SGOT, SGPT, PT/INR were elevated in CDC series and normal in GCC patients. 55 patients (96.5%) underwent cyst excision and Roux-en-Y hepaticojejunostomy. Mortality was seen in 7.01% patients (n = 4) following surgery.

Conclusion

Simplified and broader classification system for CDCs has been proposed. Clinical studies found that GCC differs considerably from classical CDCs.

中文翻译：

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肝胆畸形：分类系统、临床病理学特征和最大儿科巨大胆总管囊肿报告的建议更新

背景

本研究旨在更新肝胆畸形的分类，并研究接受手术的小儿胆总管囊肿（CDC）和小儿巨大胆总管囊肿（GCC）患者的临床病理学特征。

方法

我们回顾性分析了 2016 年至 2020 年期间儿科患者中 57 例连续 CDC 病例的数据。

结果

提出了修订的肝胆畸形分类，并将其分为 2 个广泛的标题，胆总管（先天性和获得性）和胆总管外谱。57 名儿科患者被诊断为 CDC，平均年龄 4.615 岁，男女比例为 3.7:1。我们还报道了最大的 GCC 之一，尺寸为 23 × 10 × 9 厘米。众所周知，经典三联征更为常见，在 60% 的 GCC 中可见，而在 CDC 中则为 14.5%。CDC系列患者血清胆红素、SGOT、SGPT、PT/INR升高，GCC患者正常。55 名患者 (96.5%) 接受了囊肿切除术和 Roux-en-Y 肝空肠吻合术。术后死亡率为 7.01%（n = 4）。

结论

已经提出了简化和更广泛的 CDC 分类系统。临床研究发现，GCC 与经典 CDC 有很大不同。