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.
Abbreviations used in this paper:GPNF = giant plexiform neurofibroma; NF1 = neurofibromatosis Type 1; OD = occipital dysplasia; PNF = plexiform neurofibroma; SNF = segmental neurofibromatosis.
Address correspondence to: Ravi Dadlani, D.N.B., Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, EPIP area, Whitefield, Bangalore 560 066, India. email: firstname.lastname@example.org.
Please include this information when citing this paper: published online September 13, 2013; DOI: 10.3171/2013.8.PEDS12624.