Dadlani Ravi, Sadanand Venkatraman, Ghosal Nandita, Hegde Alangar S
Departments of Neurosurgery and.
J Neurosurg Pediatr. 2013 Nov;12(5):458-64. doi: 10.3171/2013.8.PEDS12624. Epub 2013 Sep 13.
Giant plexiform neurofibroma (GPNF) of the scalp is an extremely rare lesion reported in association with neurofibromatosis. Occipital location of GPNF is even more infrequent, especially in association with occipital dysplasia (OD). The authors report 2 pediatric cases of GPNF associated with OD. The first case had an associated meningoencephalocele, and the second had large vascular channels within the lesion and the dominant ipsilateral transverse sinus lying in the center of the calvarial defect. The authors present these 2 unusual cases with a review of literature and discuss the radiological findings, theories of etiopathogenesis of the OD, and management dilemmas.
头皮巨大丛状神经纤维瘤（GPNF）是一种与神经纤维瘤病相关的极为罕见的病变。GPNF位于枕部的情况更为少见，尤其是与枕部发育异常（OD）相关时。作者报告了2例与OD相关的儿童GPNF病例。第一例伴有脑膜脑膨出，第二例病变内有大的血管通道，且优势侧同侧横窦位于颅骨缺损中心。作者展示这2例不寻常病例并回顾文献，讨论影像学表现、OD的病因发病机制理论及治疗困境。
