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一名进行性骨化性纤维发育不良患者所采用的两种手术及麻醉管理技术的描述。

A description of two surgical and anesthetic management techniques used for a patient with fibrodysplasia ossificans progressiva.

作者信息

Wadenya Rose, Fulcher Megan, Grunwald Tal, Nussbaum Burton, Grunwald Zvi

机构信息

University of Pennsylvania School of Dental Medicine, Philadelphia, Attending Dentist, The Children's Hospital, Philadelphia, Pennsylvania, USA.

出版信息

Spec Care Dentist. 2010 May-Jun;30(3):106-9. doi: 10.1111/j.1754-4505.2010.00133.x.

Abstract

Fibrodysplasia ossificans progressiva (FOP) is a rare and debilitating genetic disorder of skeletal malformations and progressive heterotopic ossification. Flare-ups are episodic, with bone formation in skeletal muscle and connective tissue leading to ankylosis of major joints of the axial and appendicular skeleton. This report outlines the management of a patient with FOP who had ankylosis of the temporomandibular joint and progressive ossification of the neck structures. The patient underwent two different surgical and anesthetic procedures within a 10-year period to manage his oral pain. The authors compare the surgical techniques, osteotomy versus the more conservative buccal approach, anesthesia techniques, and conventional intubation versus sedated fiberoptic intubation. This report emphasizes the importance of a less invasive surgical technique and an appropriate anesthetic management that reduces the risks, cost, and morbidity associated with routine surgical management of patients with FOP.

摘要

进行性骨化性纤维发育不良(FOP)是一种罕见且使人衰弱的遗传性骨骼畸形和进行性异位骨化疾病。病情发作呈间歇性,骨骼肌和结缔组织中形成骨骼,导致中轴骨骼和附属骨骼的主要关节发生强直。本报告概述了一名患有颞下颌关节强直和颈部结构进行性骨化的FOP患者的治疗情况。该患者在10年内接受了两种不同的手术和麻醉程序以治疗口腔疼痛。作者比较了手术技术(截骨术与更保守的颊侧入路)、麻醉技术以及传统插管与镇静纤维支气管镜插管。本报告强调了采用侵入性较小的手术技术和适当的麻醉管理的重要性,这可降低与FOP患者常规手术治疗相关的风险、成本和发病率。

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