NEUROFIBROMA IN A PATIENT WITH A CLOSED D EVELOPMENTAL DEFECT OF THE SPINE - REPORT OF A CASE

A boy with a faun-tail nevus developed a soft subcutaneous mass in the lumbar area at the age of three years. The mass expanded slowly and b ecame painful about eight months before the patient was evaluated at n ine years of age. There were no plantar ulcers or neurologic abnormali ties. Hemisacralization of L5 with S1 spina bifida was seen on plain r adiographs, and a magnetic resonance imaging study showed a normal spi nal cord with a tumor independent from the spinal canal. The tumor was removed surgically. Histologic examination established the diagnosis of neurofibroma. Investigations failed to detect evidence of neurofibr omatosis. Closed developmental defects of the spine are birth defects of the vertebras and spinal canal with no break in the overlying skin. Skin lesions, neuromuscular and orthopedic abnormalities, and sphinct er dysfunction are common manifestations. This is the first reported c ase of closed developmental defect of the spine with a subcutaneous ne urofibroma.