Sretenović Aleksandar, Slavković Milan, Vujović Dragana, Pavićević Polina, Zdujić Nenad, Budimir Dražen, Pogorelić Zenon
Department of Pediatric Surgery, University Children's Hospital Belgrade, 11000 Belgrade, Serbia.
School of Medicine, University of Belgrade, 11000 Belgrade, Serbia.
Children (Basel). 2025 May 28;12(6):689. doi: 10.3390/children12060689.
: Forme fruste choledochal cyst (FFCC) is a choledochal cyst with minimal or no dilatation of the extrahepatic bile duct (EHBD) and is usually associated with an anomalous pancreaticobiliary junction (APBJ). While sharing similar symptoms, inflammation, and malignant potential with classic biliary cysts, FFCC is often overlooked on ultrasound. This paper aims to present the experience of two tertiary pediatric centers in managing FFCC. In this retrospective study, the clinical data of pediatric patients treated for FFCC at two tertiary pediatric surgical centers between 1 January 2008 and 31 December 2023 were analyzed. The primary outcome was the clinical success of the surgical procedure, defined by the resolution of symptoms and the absence of major complications. Secondary outcomes included postoperative complications, type and duration of surgical procedures, and length of hospital stay. All patients underwent biliary reconstruction via either Roux-en-Y hepatico-jejunostomy or hepatico-duodenostomy. Clinical outcomes, including postoperative complications and patient follow-up, were evaluated. : Fourteen children (9 girls, 5 boys; aged 18 months to 12 years) underwent surgical treatment of FFCC. The mean age at surgery was 5.3 ± 3.8 years, and the mean diameter of the common bile duct was 7.9 ± 1.2 mm. Thirteen patients underwent Roux-en-Y hepatico-jejunostomy, and one underwent hepatico-duodenostomy. Over a mean follow-up period of 6.2 ± 3.6 years, no cholangitis or anastomotic stricture cases were observed. Two patients (14.3%) experienced minor wound infections managed conservatively. FFCC remains a diagnostic challenge due to its subtle imaging findings and non-specific clinical presentation. However, once identified, surgical excision with biliary reconstruction, most commonly via Roux-en-Y hepatico-jejunostomy, is a safe and effective treatment with excellent long-term outcomes. Given the potential for serious complications if left untreated, FFCC should be actively considered in pediatric patients with unexplained pancreatitis or biliary symptoms, even in the absence of overt ductal dilatation.
隐匿型胆总管囊肿（FFCC）是一种肝外胆管（EHBD）扩张极小或无扩张的胆总管囊肿，通常与胰胆管异常汇合（APBJ）有关。虽然与典型胆管囊肿有相似的症状、炎症和恶变潜能，但FFCC在超声检查中常被忽视。本文旨在介绍两家三级儿科中心处理FFCC的经验。在这项回顾性研究中，分析了2008年1月1日至2023年12月31日期间在两家三级儿科外科中心接受FFCC治疗的儿科患者的临床数据。主要结局是手术的临床成功，定义为症状缓解且无重大并发症。次要结局包括术后并发症、手术类型和持续时间以及住院时间。所有患者均通过 Roux-en-Y 肝空肠吻合术或肝十二指肠吻合术进行胆道重建。评估了临床结局，包括术后并发症和患者随访情况。14名儿童（9名女孩，5名男孩；年龄18个月至12岁）接受了FFCC手术治疗。手术时的平均年龄为5.3±3.8岁，胆总管平均直径为7.9±1.2毫米。13例患者接受了Roux-en-Y肝空肠吻合术，1例接受了肝十二指肠吻合术。在平均6.2±3.6年的随访期内，未观察到胆管炎或吻合口狭窄病例。2例患者（14.3%）发生轻微伤口感染，经保守治疗。由于其细微的影像学表现和非特异性的临床表现，FFCC仍然是一个诊断挑战。然而，一旦确诊，手术切除并进行胆道重建，最常见的是通过Roux-en-Y肝空肠吻合术，是一种安全有效的治疗方法，长期效果良好。鉴于如果不治疗可能会出现严重并发症，对于患有不明原因胰腺炎或胆道症状的儿科患者，即使没有明显的胆管扩张，也应积极考虑FFCC。
