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成功分离具有单一肝外胆道系统的胸部联体双胞胎。

Successful separation of thoracopagus conjoined twins with a single extra-hepatic biliary system.

作者信息

Saranrittichai Surachai, Niramis Rangsan, Siripornadulsilp Surachai, Thianjaruwatthana Wichian, Hompleum Tanin, Tipsunthonsak Nakhon, Pulnitiporn Aksorn, Vasanawathana Sirijitt, Jarupoonphol Vithya

机构信息

Department of Surgery, Khon Kaen Regional Hospital, Khon Kaen 40000, Thailand.

出版信息

J Med Assoc Thai. 2007 May;90(5):994-1000.

Abstract

A pair of thoracopagus conjoined twins were separated at the age of 3 months at Khon Kaen Regional Hospital, Thailand on November 19, 2004. Pre-operative investigations showed separate hearts, joined duodenum, and fusion of the livers. Separation of the extra-hepatic biliary systems was suspected. Operative findings revealed fusion of the intestines from the second part of the duodenum to the terminal ileum with two normal colons. An intussusception was found at the terminal ileum. Fusion of the livers with only one extra-hepatic biliary system was noted In one of the twins, the gastrointestinal tract was anastomosed with Roux-en- Y enteric loop to one area of good bile drainage at the cut surface of liver Post operative course was hectic but both twins recovered satisfactorily. Both are doing well at present, two years after the separation. This was the first reported case of thoracopagus conjoined twins with complex biliary tract anomalies in Thailand. From the literature, pre-operative investigations in most cases of these conjoined twins failed to define the precise anatomy of the biliary system and may be misleading as in the presented case. The mortality rate remains high. Meticulous pre-operative planning, decision-making in the operative field and postoperative management as well as a multidisciplinary team are very important for a successful separation.

摘要

2004年11月19日,一对胸腹联体双胞胎在泰国孔敬地区医院3个月大时接受了分离手术。术前检查显示心脏各自独立,十二指肠相连,肝脏融合。怀疑肝外胆道系统分离。手术结果显示,从十二指肠第二部到回肠末端的肠道融合,有两个正常的结肠。在回肠末端发现了肠套叠。注意到肝脏融合且只有一个肝外胆道系统。在其中一个双胞胎中,胃肠道与Roux-en-Y肠袢吻合至肝脏切面胆汁引流良好的一个区域。术后过程很艰难,但两个双胞胎都恢复得令人满意。分离两年后的现在,两人情况良好。这是泰国首例报道的胸腹联体双胞胎合并复杂胆道异常的病例。从文献来看,这些联体双胞胎大多数病例的术前检查未能明确胆道系统的确切解剖结构,可能会像本病例一样产生误导。死亡率仍然很高。精心的术前规划、手术中的决策、术后管理以及多学科团队对于成功分离非常重要。

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