Qayyum Muhammad Umar, Haq Ehtesham Ul, Mahmood Kazmi Syed Atif, Zahra Rubbab
Combined Military Hospital, Lahore.
Bahria International Hospital, Rawalpindi.
J Ayub Med Coll Abbottabad. 2022 Oct-Dec;34(Suppl 1)(4):S1013-S1020. doi: 10.55519/JAMC-04-S4-9327.
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.
蝶骨发育异常是蝶骨完全或部分缺失，最常见的是蝶骨大翼。它可作为一种孤立的畸形出现，也可发生于神经纤维瘤病1型（NF1）。NF1的特征包括牛奶咖啡斑、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3例蝶骨翼发育异常的回顾性病例系列。其中1例为孤立性骨缺损，1例为NF-1，1例为累及蝶骨翼的手术治疗的颅面骨纤维发育不良。有2例为初次手术病例，1例为二次手术。蝶骨和颞骨缺损患者的搏动性眼球突出得到缓解。面部畸形NF1患者的肿物切除至患者满意，但患者拒绝手术矫正蝶骨畸形。第3例患者是再次手术患者，术中取出了先前的植入材料，患者存在脑脊液鼻漏，不同意矫正垂直性眼眶发育异常。蝶骨翼发育异常是一种复杂的畸形，需要多学科护理和治疗计划。如我们的病例系列所示，通过精心规划和手术，可以取得良好的效果。
