Neurfibromatosis is known as a congenital & familial disease presenting abnormalities of the skin, nervous system, bones, and soft tissue.
In addition, the clinical symptoms were represented by fibroma molluscum, cafe-au-lait spots, and neurofibromas of peripheral nerves.
In the last few decades, the variety of skeletal defects associated with neurofibromatosis has received increasing attention, and the bony abnormalities are known as severe scoliosis, defects of the walls of the orbits, erosive defects caused by adjacent neurogenic tymors, bowing and pseudoarthrosis of the lower leg, and disorders of growth of bone associated with elephantoid hypertrophy of soft tissue, over all 20 patients, those who are admitted to our Department from 1979 to 1987, were evaluated in clinical aspects.
The bone change was seen in 8 patients and other clinical results are shown as follows.
1. The neurofibromatosis is mainly developed in childhood.
2. The familial tendency is 35% and short status is characteristic in height.
3. The malignant changes are 20% which are all malignant schwannoma in pathologic study.
4. Bone change was 40% and the most common type is scoliosis of the spine and the bone change is frequently seen in malignant schwannoma than benign neurofibromatosis.
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