Vila-Carbó J J, Ayuso L, Hernández E, Lluna J, Ibáñez V
Servicio de Cirugía Pediátrica, Hospital Universitario Infantil La Fe, Valencia.
Cir Pediatr. 2006 Jan;19(1):33-8.
The aim of the present study has been to systematize the clinical presentation of the entity named choledochal cyst, in relation with its probable etiopathology and the intraoperative findings as well as its evolution after surgery, based on the revision of the literature and of our experience in 29 cases.
29 cases of cystic dilatation of the biliar duct extra and/or intrahepatic are analyzed. In 4 cases the diagnosis was prenatal and two were excluded of the study after it has been confirmed they suffered biliary atresia type I. In the left 27 cases, 19 variables are analyzed retrospectively, like age, sex, weight, symptoms, ultrasonographic images, etc. Subsequently, intra-operative cholangiographic findings were correlated with the clinic presentation and the evolution of the patients after surgery.
Of the 27 cases analyzed 16 (59,25%) were cystic dilatations from which 14 had a neonatal or early clinic presentation (before 2 years), however the fusiform dilatations were presented later on. From the analyzed symptoms, in relation with the age only the pain and the jaundice showed significant differences, being the pain most frequent in later presentation ande the jaundice in the early form. The two cases of type 3 of Todani or choledochocele were of later presentation. An anomaly in the bilionpacreatric junction was detected in 15 patients; the majority had a later presentation, associated to pancreatitis in 4 cases. Primary cyst excision and biliary Roux-en-Y reconstruction was the treatment of election in the majority of cases. In 3 cases we used the appendix to replace the choledocus, but all three cases were reconverted two years later because of permanent elevation of ALT and GGT.
In favour of the literature and of our experience nowadays it would be possible to systematize this malformation and make a division in two groups, depending on the cholangiographic findings and clinical presentation: 1. Cystic dilatations with a clinical neonatal presentation or beneath 2 years. 2. Fusiform dilatations with a later clinical predominance and associated frequently to pancreatitis and anomalous pancreatobiliary junction. Choledochocele is an entity that must be considered not only for its etiology but for its clinical presentation and treatment. Primary cyst excision and biliary Roux-en-Y reconstruction is the treatment of election. Regular long-term review of these patients is mandatory in the surveillance of sub-clinic cholangitis and the risk of possible long-term malignance of this entity.
本研究的目的是，在复习文献及总结我们29例病例经验的基础上，根据胆总管囊肿可能的病因病理、术中所见以及术后演变情况，对其临床表现进行系统整理。
分析29例肝外和/或肝内胆管囊性扩张病例。4例产前诊断，其中2例确诊为I型胆道闭锁后被排除在研究之外。对其余27例病例回顾性分析19项变量，如年龄、性别、体重、症状、超声图像等。随后，将术中胆管造影结果与临床表现及患者术后演变情况进行关联分析。
在分析的27例病例中，16例（59.25%）为囊性扩张，其中14例有新生儿期或早期临床表现（2岁前），梭形扩张则出现较晚。在分析的症状中，仅疼痛和黄疸与年龄有关且存在显著差异，疼痛在较晚出现时更为常见，黄疸在早期更为常见。2例Todani 3型或胆总管囊肿病例出现较晚。15例患者检测到胆胰管连接异常；大多数出现较晚，4例伴有胰腺炎。多数病例首选一期囊肿切除及胆道Roux-en-Y重建术。3例使用阑尾替代胆总管，但3例均在两年后因谷丙转氨酶（ALT）和谷氨酰转肽酶（GGT）持续升高而再次手术。
结合文献及我们目前的经验，有可能根据胆管造影结果和临床表现对这种畸形进行系统整理并分为两组：1. 有新生儿期或2岁以下临床表现的囊性扩张。2. 以较晚临床症状为主且常与胰腺炎及胆胰管连接异常相关的梭形扩张。胆总管囊肿不仅因其病因，还因其临床表现和治疗都必须予以考虑。一期囊肿切除及胆道Roux-en-Y重建术是首选治疗方法。对这些患者进行定期长期复查对于监测亚临床胆管炎以及该疾病可能的长期恶变风险是必不可少的。
