蝶翼发育不良3例报告。

Muhammad Umar Qayyum, Ehtesham Ul Haq, Syed Atif Mahmood Kazmi, Rubbab Zahra
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引用次数: 0

摘要

蝶骨发育不良是指没有完整或部分蝶骨,最常见的是蝶骨的大翅。它可以作为孤立的畸形或神经纤维瘤病-1 (NF1)发生。NF1的特征包括咖啡斑点、腹股沟或腋窝雀斑、神经纤维瘤、视神经胶质瘤、脊柱侧凸和胫骨畸形。我们的研究是回顾性的病例系列的3例蝶翼发育不良。孤立性骨缺损1例,NF-1 1例,手术颅面纤维发育不良累及蝶骨翼1例。主要手术2例,辅助手术1例。蝶骨、颞骨缺损患者搏动性眼球突出得到缓解。面部畸形NF1患者的减容令患者满意,但患者拒绝手术矫正蝶骨畸形。第三例患者为再做手术患者,原植入物被移除,脑脊液鼻漏,患者不同意矫正眼眶垂直异位。蝶翼发育不良是一种复杂的畸形,需要多专科护理和治疗计划。通过精心的计划和手术,可以取得良好的效果,正如我们的病例系列所示。
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Sphenoid Wing Dysplasia: Report Of 3 Cases.

Sphenoidal Dysplasia is the absence of complete or a part of sphenoid bone, most commonly the greater wing of sphenoid. It can occur as an isolated deformity or in Neurofibromatosis-1 (NF1). Features of NF1 include café au lait spots, inguinal or axillary freckling, neurofibromas, optic gliomas, scoliosis and tibial deformity. Our study is retrospective case series of 3 cases of Sphenoid wing dysplasia. There was 1 case of isolated bone defect, 1 case of NF-1 and 1 case of operated Craniofacial Fibrous Dysplasia involving the sphenoid wing. There were 2 primary operated cases while 1 was operated secondarily. There was resolution of pulsatile exophthalmos in patient with sphenoid and temporal bone defect. Patient with facial deformity NF1 was debulked to the satisfaction of the patient, the patient however declined surgery to correct the sphenoid bone deformity. The 3rd patient was a re-do surgery patient in which the previous implant material was removed and the CSF rhinorrhoea, the patient did not consent to the correction of vertical orbital dystopia. Sphenoid wing dysplasia is a complex deformity requiring multi speciality care and treatment planning. With meticulous planning and surgery, good results can be achieved as shown in our case series.

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