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多发性翼状胬肉综合征:麻醉医生面临的挑战。

Multiple pterygium syndrome: Challenge for anesthesiologist.

作者信息

Sethi P, Bhatia P K, Gupta N, Singh K

机构信息

Department of Anesthesia and Critical Care, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India.

Department of Pediatrics, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India.

出版信息

Saudi J Anaesth. 2016 Jul-Sep;10(3):350-2. doi: 10.4103/1658-354X.174901.

Abstract

Multiple pterygium syndrome (MPS) is a very rare autosomal recessive disorder characterized by flexion of joint and digit contractures, skin webbing, cleft palate, deformity of the spine, and cervical spine fusion. Difficult airway is associated mainly due to micrognathia, retrognathia, webbing of the neck, and limitation of the mouth opening and neck extension. We are reporting a case of a 5-year-old female diagnosed with MPS and exhibiting a bilateral club foot and congenital vertical talus. The patient was posted for manipulation and above the knee casting under general anesthesia.

摘要

多发性翼状胬肉综合征(MPS)是一种非常罕见的常染色体隐性疾病,其特征为关节和手指挛缩、皮肤蹼状、腭裂、脊柱畸形以及颈椎融合。主要由于小颌畸形、下颌后缩、颈部蹼状以及张口和颈部伸展受限导致气道困难。我们报告一例5岁女性诊断为MPS,伴有双侧马蹄内翻足和先天性垂直距骨。该患者拟在全身麻醉下进行手法复位及膝上石膏固定。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a1b/4916826/9e395310dc69/SJA-10-350-g001.jpg

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