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患有卡塔格内综合征和脊柱侧弯患者肺移植的陷阱与挑战

Pitfalls and Challenges of Lung Transplant in a Patient With Kartagener Syndrome and Scoliosis.

作者信息

Yazicioglu Alkin, Alici Ibrahim Onur, Karaoglanoglu Nurettin, Yekeler Erdal

机构信息

From the Turkiye Yuksek Ihtisas Training and Research Hospital, Thoracic Surgery and Lung Transplantation Clinic, Ankara, Turkey.

出版信息

Exp Clin Transplant. 2018 Apr;16(2):237-241. doi: 10.6002/ect.2015.0190. Epub 2016 Mar 14.

Abstract

We present a 22-year-old woman with Kartagener syndrome and scoliosis who died 112 days after single lung transplant. The classic thoracic involvement of situs inversus totalis and the asymmetric arrangement of the thoracic vascular structures might be a pitfall for surgeon. Anatomic obstacles have forced the surgeon to perform a single transplant. The period of primary graft dysfunction in a single transplanted lung patient was a challenge; supporting the patient with a high flow and long period of extracorporeal membrane oxygenation might lead to a vanishing bronchus. Immotile cilia, a feature of Kartagener syndrome, were another challenge and patient needed several daily aspiration bronchoscopies. Vanishing bronchus is a gradual process with high mortality rates; commonly, stenosis is at the non anastomotic bronchial tree because of insufficient nourishment of the bronchial cartilages. Several repeat bronchoscopic balloon dilatations accompanied with medical treatment were unsuccessful.

摘要

我们报告一名22岁患有卡塔格内综合征和脊柱侧弯的女性,她在单肺移植术后112天死亡。全内脏转位的典型胸部表现以及胸部血管结构的不对称排列可能给外科医生带来陷阱。解剖学上的障碍迫使外科医生进行单肺移植。单肺移植患者的原发性移植物功能障碍期是一个挑战;用高流量和长时间的体外膜肺氧合支持患者可能会导致支气管消失。不动纤毛是卡塔格内综合征的一个特征,这是另一个挑战,患者需要每天进行多次吸引支气管镜检查。支气管消失是一个死亡率高的渐进过程;通常,狭窄发生在非吻合支气管树,因为支气管软骨营养不足。多次重复支气管镜球囊扩张并辅以药物治疗均未成功。

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