A Study of Patients with Neurofibromatosis Hospitalized in Firoozgar and Shafayahyaian Hospitals, Tehran

Neurofibromatosis is a congenital disorder with different kinds of disabling manifestations. There are several types of operations for these manifestations with relative improvement. This study set out to determine age, sex, clinical manifestations and evaluate methods of treatment of these patients. This descriptive, cross-sectional study was conducted through studying the hospital records of patients who had previously undergone surgery. 45 patients with neurofibromatosis who were hospitalized and operated in surgical ward of Firoozgar and orthopedic ward of Shafa hospital from 1995 to 1998 were studied in terms of clinical manifestations and treatment methods. Patients with central nervous system involvement were excluded from the study. 55% of the subjects were male and the rest of them were female. Most of the patients were in the second decade of their life. Skeletal abnormalities were seen in 35 patients (77.77%) and 16 patients (35.55%) were suffering from scoliosis which was the most common complication of this disease. With respect to the progression of the disease, brace was used in 2 patients, which led to their improvement. In 13 patients Horington Rod implantation was used, three cases of which developed scoliosis, who underwent surgical vertebral fusion. Pseudoarthrosis was seen in 8 patients (17/77%). Bone graft and plate were used in their operation. In five cases with pseudoarthrosis in lower limbs no: :union:: occurred. Two patients died with malignant shwanoma. scoliosis is the most common manifestation of neurofibromatosis which can improve in early stages with surgical treatment but pseudoarthrosis in lower limbs has not improved with bone graft and plate. Operation with pediculated fibula resulted in the improvement of this complication. Surgical intervention would be more effective in the early stages of the disease. 2 of thses 5 cases were reoperated with pediculated fibula graft and 3 others were amputated.