PDF(Pubmed)
Abstract：
Choledochal cysts (CCs) are quite rare and are characterized by anomalous dilations of the biliary tree, mostly due to anomalous pancreaticobiliary junction (APBJ). A less frequent congenital anomaly due to incomplete fusion of pancreatic ducts, pancreas divisum (PD) can complicate the clinical course of CC. Although rare, the coexistence of CC and PD presents significant clinical challenges. With very few documented cases globally, our experience with this case adds to our understanding of this unique condition. This report aims to highlight the complex relationship between these anomalies and underscores the need for heightened clinical awareness and comprehensive management strategies to improve patient outcomes. We present the case of a 27-year-old female patient who was diagnosed with type 1 CC with concomitant PD after recurrent pancreatitis and multiple biliary interventions. Her choledochal cyst was excised with Roux-en-Y hepaticojejunostomy (RYHJ). Histopathological examination confirmed CC with evidence of chronic inflammatory changes but no malignancy. The preoperative hospital stay was three days with an operative duration of 150 minutes and intraoperative blood loss of 210 mL. Postoperatively, the patient was discharged on day 5. The pain score as per the Visual Analog Scale (VAS) was 2 on the day of discharge. The patient was started on diet on postoperative day (POD) 3. The abdominal drains were removed on POD 4 (subhepatic) and POD 5 (pelvic). Sutures were removed on POD 10, with follow-up for two years with no recurrence of similar complaints. This case illustrates the diagnostic challenge of synchronous CC and PD and elaborates on the role of extensive imaging modalities in guiding management decisions. The surgical approach remains the foremost for CC; preventing complications in the form of cholangitis and malignancy is the mainstay of treatment. The present report is an addition to the existing literature on the management of complex biliary anomalies and places special emphasis on the need for a multidisciplinary approach with individualized treatment strategies in such rare clinical scenarios. Further studies are required to clarify pathophysiological mechanisms linking CC and PD, with the need for better therapeutic strategies toward the optimization of patient outcomes. More studies with robust data are necessary to draw better conclusions.
摘要：
胆总管囊肿（CC）非常罕见，其特征是胆道异常扩张，主要是由于胰胆管连接异常（APBJ）。由于胰管不完全融合引起的较不常见的先天性异常，胰腺分裂（PD）可使CC的临床过程复杂化。虽然罕见，CC和PD的共存提出了重大的临床挑战。全球记录的案例很少，我们在这个案例中的经验增加了我们对这个独特条件的理解。本报告旨在强调这些异常之间的复杂关系，并强调需要提高临床意识和综合管理策略以改善患者预后。我们介绍了一名27岁的女性患者，该患者在复发性胰腺炎和多次胆道干预后被诊断为1型CC并伴有PD。她的胆总管囊肿用Roux-en-Y肝空肠吻合术（RYHJ）切除。组织病理学检查证实CC有慢性炎症变化的证据，但没有恶性肿瘤。术前住院时间为3天，手术时间为150分钟，术中出血量为210mL。术后,患者在第5天出院。根据视觉模拟量表（VAS），出院当天的疼痛评分为2。患者在术后第3天(POD)开始饮食。在POD4（肝下）和POD5（骨盆）上切除腹部引流管。在POD10上去除缝线，随访两年，没有类似的投诉复发。此案例说明了同步CC和PD的诊断挑战，并阐述了广泛的成像方式在指导管理决策中的作用。手术方法仍然是CC的首要方法；预防胆管炎和恶性肿瘤形式的并发症是治疗的主要手段。本报告是对现有有关复杂胆道异常管理的文献的补充，并特别强调在这种罕见的临床情况下需要采用多学科方法和个性化治疗策略。需要进一步的研究来阐明连接CC和PD的病理生理机制。需要更好的治疗策略来优化患者的预后。需要更多具有可靠数据的研究才能得出更好的结论。
