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累及颞骨的骨纤维发育不良:三例新病例报告。

Fibrous dysplasia involving the temporal bone: report of three new cases.

作者信息

Smouha E E, Edelstein D R, Parisier S C

出版信息

Am J Otol. 1987 Mar;8(2):103-7.

PMID:3591915
Abstract

Monostotic fibrous dysplasia, an unusual disease that can involve the temporal bone, will frequently cause an acquired stenosis of the external auditory canal. Three patients with this disorder who presented with occluded canals are described. Two individuals developed external canal cholesteatomas medial to the obstruction, one of whom eventually developed a postauricular abscess and infected draining sinus. The third patient presented with a restenosis nine months following canalplasty. The cases reported illustrate three surgical criteria necessary to manage these unusual cases successfully: removal of sufficient diseased bone to create a patulous canal; resurfacing denuded bony areas with thin split-thickness skin grafts to prevent soft tissue contractions; an adequate meatoplasty. Postoperatively, the reconstructed canals have remained patent and stable during follow-up periods ranging from one to four years. Clinical, radiographic, and pathologic features of fibrous dysplasia are discussed, the differential diagnosis is presented, and the relevant literature is reviewed.

摘要

单骨型纤维性发育不良是一种可累及颞骨的罕见疾病,常导致后天性外耳道狭窄。本文描述了3例因外耳道闭塞而就诊的该疾病患者。2例患者在梗阻内侧形成外耳道胆脂瘤,其中1例最终形成耳后脓肿和感染性引流窦。第3例患者在耳道成形术后9个月出现再狭窄。所报道的病例说明了成功处理这些罕见病例所需的三个手术标准:切除足够的病变骨质以形成通畅的外耳道;用薄断层皮片覆盖裸露的骨面以防止软组织收缩;进行充分的外耳道成形术。术后,在1至4年的随访期内,重建的外耳道保持通畅和稳定。本文讨论了纤维性发育不良的临床、影像学和病理学特征,给出了鉴别诊断,并回顾了相关文献。

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