VON RECKLINGHAUSEN'S NEUROFIBROMATOSIS WITH BONE MANIFESTATIONS

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Abstract
The authors present 2 cases of von Recklinghausen''s neurofibromatosis with osseous changes, the one exhibiting marked cystic changes in the mandible, the other gigantism and bowing of the tibia associated with elephantiasis of the lower extremity and osteoporosis, decalcification and atrophy and deformity of the cervical vertebrae in the region of a rapidly growing cervical neurofibroma. This disease is of highly dynamic nature. No adequate method of treatment is available; surgery and radiation are both unsatisfactory. Diagnosis is generally possible without biopsy; in fact, biopsy may actually be dangerous because of the possibility of inducing sarcomatous metaplasia. Because of the frequency and seriousness of the bone changes apt to occur in association with von Recklinghausen''s neurofibromatosis general roentgen studies are recommended to detect latent osseous changes. Such radiological studies should include the skull, entire vertebral column and all the bones of the upper and lower extremities.