Craniofacial Neurofibromatosis: A Roentgen Profile

Abstract

Neurofibromatosis is an hereditary, hamartomatous disorder, probably of neural crest origin, involving not only the neuroectoderm and mesoderm but also the endoderm and has the potential to appear in any organ system of the body. Osseous craniofacial abnormalities are uncommon compared with those of the axial and peripheral skeleton. The former include calvarial defects, changes in the mandible, zygomatic arch, orbit, tympanic bone, mastoid and sella turcica. The present communication records clinical and roentgen observation in the skull and facial bones in twenty cases of neurofibromatosis. MATERIAL AND METHOD Twenty cases of neurofibromatosis with involvement of the head and face region form the study material for this presentation. They were selected I:IGURE 1. -Lateral roentgenogram of the skull showing a well corticated defect in the lambdoid suture. A plexiform neuromatous lesion is superimposed on the parietotemporal region.