| Abstract | Otorhinolaryngological manifestations of 13 patients with von Recklinghausen's disease appearing at Lagos University Teaching Hospital over a five-year period have been studied. Among patients with ENT manifestations of the disease, the most common general features exhibited were cutaneous neurofibromas (100 per cent), headache (69.23 per cent) and pruritus (46.15 per cent). But the head and neck findings included external meatal canal stenosis (30.77 per cent), conductive deafness (30.77 per cent), nasal discharge (30.77 per cent), cranial nerve involvement (30.77 per cent) and disfigurement of the soft tissues of the face (30.77 per cent). Involvement of pinna (23.31 per cent), rhinolalia aperta (15.38 per cent), mental retardation (15.38 per cent) and pharyngeal swelling (7.69 per cent) also featured. Clinically detectable bilateral acoustic neurofibromas in adults or astrocytomas in children were not found in this series. Involvement of the frontoparietal bone (7.65 per cent) presented with skull bossing which had to be differentiated from that due to sickle cell disease in the African. There was also a singular case of phrenic nerve involvement. However, malignancy occurred in one (7.69 per cent) of these patients. Thus, it is important always to follow-up these patients closely so as to detect malignant transformation in time. |
