Department of Surgical Gastroenterology, Sheri-Kashmir Institute of Medical Sciences, Srinagar, Kashmir, India.
World J Surg. 2009 Nov;33(11):2403-11. doi: 10.1007/s00268-009-0184-2.
Choledochal cyst is a surgical problem usually related to infancy and childhood. Despite advancements in management, a large number of cases still present during adulthood. The clinical course and outcome in children varies from that in adults. This study focuses on these variations in terms of presentation, management, histopathology, and outcome.
An in-depth retrospective analysis was carried out on 79 patients presenting with choledochal cysts between December 2007 and January 1997. The patients were segregated into two groups: group A comprising 32 children and group B with 47 adults. The presentation, clinical evaluation, radiologic, and biochemical findings; operative details; pathologic findings; and early and long-term complications in the two groups were studied on a comparative basis.
The male/female ratios were 1:3 and 1.0:2.3 in groups A and B, respectively. A history of previous biliary surgery, pancreatitis, cholangitis, peroperative difficulties, and early and late postoperative complications were 5.1, 5.4, 6.4, 5.4, 2.0, and 3.3 times more common in group B than in group A. However, the classic triad of jaundice, abdominal pain, and a mass was 6.7 times more common in group A than in group B. The classic triad and cholangitis were the only parameters that were statistically significant. Rare presentations of spontaneous perforation of the cyst or cachexia manifested only in group A patients. The methods of detection and operative treatment were identical for both groups. Histologically, fibrosis of the cyst wall was a feature peculiar to group A, whereas signs of inflammation and hyperplasia were predominantly seen in group B. In the group B series, one patient had cholangiocarcinoma and another gallbladder carcinoma; one more patient developed malignancy during follow-up. Long-term complications were seen in 29.7% of patients in group B versus 9.3% in group A; the most rampant complication was a type IVa cyst, seen in 68.7% of patients.
Choledochal cysts present differently in adults and children; whereas children present with the classic triad, adults present with common biliary or infective complications. Although the methods of detection and surgical treatment are similar for the two groups, the type IVa cyst typically seen in the adult group creates a marked deviation with respect to long-term complications such as ascending cholangitis, anastomotic strictures, stone formation, and development of cholangiocarcinoma. These are areas of grave concern that can be addressed to a large extent by providing an access loop during the initial surgery especially for type IVa cysts. The glaring differences in terms of presentation, histologic picture, and outcome urges us to consider choledochal cysts in children as a separate entity.
胆总管囊肿是一种常见于婴儿和儿童的外科问题。尽管在管理方面取得了进展，但仍有大量病例在成年期出现。儿童的临床病程和结局与成人不同。本研究重点关注这些差异在表现、治疗、组织病理学和结果方面的表现。
对 2007 年 12 月至 1997 年 1 月期间出现的 79 例胆总管囊肿患者进行了深入的回顾性分析。患者分为两组：A 组 32 例儿童，B 组 47 例成人。对两组患者的临床表现、临床评估、影像学和生化发现、手术细节、病理发现以及早期和长期并发症进行了比较研究。
A 组男女比例为 1:3，B 组为 1.0:2.3。在 B 组中，有 5.1、5.4、6.4、5.4、2.0 和 3.3 倍的患者有既往胆道手术、胰腺炎、胆管炎、手术困难和早期和晚期术后并发症史。然而，在 A 组中，经典三联征（黄疸、腹痛和肿块）的发生率是 B 组的 6.7 倍。经典三联征和胆管炎是唯一具有统计学意义的参数。仅在 A 组患者中出现自发性囊肿穿孔或恶病质的罕见表现。两组的检测和手术治疗方法相同。组织学上，囊肿壁纤维化是 A 组的特征，而炎症和增生迹象在 B 组中更为常见。在 B 组系列中，有 1 例患者发生胆管癌，另 1 例患者发生胆囊癌；另有 1 例患者在随访中发生恶性肿瘤。B 组有 29.7%的患者出现长期并发症，A 组有 9.3%；最猖獗的并发症是 IVa 型囊肿，见于 68.7%的患者。
胆总管囊肿在儿童和成人中的表现不同；儿童表现为经典三联征，而成人表现为常见的胆道或感染性并发症。尽管两组的检测和手术治疗方法相似，但成人组中典型的 IVa 型囊肿会导致长期并发症（如上行性胆管炎、吻合口狭窄、结石形成和胆管癌发展）出现明显偏差。这些都是严重关注的领域，可以通过在初次手术中提供一个接入回路来在很大程度上解决，特别是对于 IVa 型囊肿。在表现、组织学图像和结果方面的明显差异促使我们将儿童的胆总管囊肿视为一个单独的实体。
