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双重罕见:完全性内脏反位患者的恶性伪装性胆道狭窄。

Double rarity: malignant masquerade biliary stricture in a situs inversus totalis patient.

机构信息

Department of Transplantation and Surgery, Semmelweis University, VIII. Baross u.23, Budapest, 1082, Hungary.

1st. Department of Surgery and Interventional Gastroenterology, Semmelweis University, Budapest, Hungary.

出版信息

BMC Surg. 2021 Mar 21;21(1):153. doi: 10.1186/s12893-021-01155-w.

DOI:10.1186/s12893-021-01155-w
PMID:33743673
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7981884/
Abstract

BACKGROUND

Situs inversus totalis is a rare anatomical variation of both the thoracic and the abdominal organs. Common bile duct strictures can be caused by malignant and benign diseases as well. 7-18% of the latter ones are 'malignant masquerade' cases, as pre-operative differentiation is difficult.

CASE PRESENTATION

We present the case of a 68y male patient with known situs inversus totalis and a recent onset of obstructive jaundice caused by a malignant behaving common bile duct stricture. Technically difficult endoscopic retrograde cholangiopancreatography, brush cytology, magnetic resonance cholangiopancreatography, endoscopic ultrasound, and percutaneous transhepatic drainage with stent implantation were performed for proper diagnosis. Cholecystectomy, common bile duct resection with hilar lymphadenectomy, and hepatico-jejunostomy have been performed following multidisciplinary consultation. The final histology report did not confirm any clear malignancy, the patient is doing well.

CONCLUSION

In situs inversus patients, both diagnostic and therapeutic procedures can lead to various difficulties. Benign biliary strictures are frequently misdiagnosed preoperatively as cholangiocellular carcinoma. Surgery is usually unavoidable, involving a significant risk of complications. The co-existence of these two difficult diagnostic and therapeutic features made our case challenging.

摘要

背景

全内脏反位是一种罕见的胸腹部器官解剖变异。胆总管狭窄可由恶性和良性疾病引起。后者的 7-18%为“恶性伪装”病例,因为术前难以区分。

病例介绍

我们报告了一例 68 岁男性患者,已知存在全内脏反位,近期因恶性行为的胆总管狭窄导致阻塞性黄疸。为了进行适当的诊断,我们进行了技术难度较大的内镜逆行胰胆管造影术、刷检细胞学检查、磁共振胰胆管造影术、内镜超声检查和经皮经肝穿刺引流并植入支架。经过多学科咨询,进行了胆囊切除术、胆总管切除和肝门淋巴结清扫术以及肝肠吻合术。最终的组织学报告并未证实任何明确的恶性肿瘤,患者情况良好。

结论

在全内脏反位患者中,诊断和治疗过程可能会带来各种困难。良性胆管狭窄常被误诊为胆管细胞癌。手术通常是不可避免的,涉及到较高的并发症风险。这两种困难的诊断和治疗特征并存使得我们的病例具有挑战性。

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