A Case of Cobb Syndrome

ABSTRACT

Cobb syndrome is a rare neurocutaneous angiomatosis characterized by a vascular skin nevus associated with a spinal cord angioma of the same metamere. A 14-year-old girl had an asymptomatic large cutaneous hemangioma distributed from the TI dermatome downward to the L3 dermatome since birth and complained of a gait disturbance and urination difficulty for 1 year. A biopsy specimen in the skin lesion revealed the findings of capillary hemangioma. From C7 downward to L4 posterior epidural hemangioma composed of arteriovenous and cavernous components was diagnosed by radiological examination and surgical exploration. Because of very extensive cord hemangioma, only partial removal of the tumor at T11, T12 and L1 level was performed and postoperatively she was transferred to a special facility for rehabilitative therapy.