Neurocutaneous vascular hamartomas mimicking Cobb syndrome - Case report

The authors report the rare case of a patient with neurocutaneous vascular hamartomas mimicking Cobb syndrome. An 8-year-old boy was admitted to the a uthors' hospital with progressive urinary disturbance and upper back pain. Multiple skin nevi had been noted at the child's birth. Radiological examin ation revealed multiple cavernous angiomas in the spinal cord in the same m etamere in which the skin nevi had been observed and also in the left cereb ral hemisphere. His symptoms gradually improved without surgical interventi on. Four years later he was readmitted because of a cerebral hemorrhage inv olving the left cerebral peduncle. Nonsurgical treatment was chosen because his symptoms promptly improved. To the best of the authors' knowledge, this is the first case of multiple c avernous angiomas in the brain and spinal cord associated with skin nevi. T he authors discuss this clinical entity and the significance of the disease .