title: Congenital giant plexiform neurofibroma with occipital calvarial dysplasia in association with meningoencephalocele in neurofibromatosis Type 1 and segmental neurofibromatosis: report of 2 cases.
abstract: 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.
authors.affiliation: Departments of Neurosurgery and.
authors.forename: Ravi
authors.initials: R
authors.lastname: Dadlani
authors.forename: Venkatraman
authors.initials: V
authors.lastname: Sadanand
authors.forename: Nandita
authors.initials: N
authors.lastname: Ghosal
authors.forename: Alangar S
authors.initials: AS
authors.lastname: Hegde
mesh_terms: D000293:Adolescent; D001848:Bone Diseases, Developmental / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D004677:Encephalocele / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008588:Meningocele / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D018318:Neurofibroma, Plexiform / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D017253:Neurofibromatoses / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D012535:Scalp / Q000473:pathology* / Q000601:surgery; D014057:Tomography, X-Ray Computed