Skeletal lesions in von Recklinghausen's disease of the head and neck in Nigerian Africans.
A study of skeletal lesions in the head and neck region of 20 adult patients with von Recklinghausen's disease, seen in the Surgical Out Patient clinic of Lagos University Teaching Hospital from 1979 to 1985, has been carried out. Among the 20 patients, 13 (65 per cent) had symptoms related to the head and neck while 7 (37 per cent) did not; however, on radiological screening, all of them were observed to have skeletal lesions. The commonest feature was enlarged intervertebral nerve root exit foramina from mesodermal dysplasia, which occurred in 16 (80 per cent) of our patients. Other major findings were erosion of the mandible, 8 (40 per cent); erosion of the wings of the sphenoid, 4 (20 per cent); poor mastoid bone pneumatisation, 3 (15 per cent); erosion of the parietal bone, 3 (15 per cent) and heterotopic bone formation, 1 (5 per cent). Congenital calvarial defects were not detected. In view of the fact that all our patients showed radiologically detectable skeletal lesions of the head and neck, despite absence of symptoms in 35 per cent, it is advisable that all patients with von Recklinghausen's disease should routinely have radiological screening of this region, to detect early lesions.
D002828:Choristoma / Q000000981:diagnostic imaging; D005147:Facial Bones / Q000000981:diagnostic imaging; D006801:Humans; D009333:Neck / Q000000981:diagnostic imaging; D010002:Osteitis Fibrosa Cystica / Q000000981:diagnostic imaging*; D011859:Radiography; D012886:Skull / Q000000981:diagnostic imaging*; D013100:Sphenoid Bone / Q000000981:diagnostic imaging