The Cobb syndrome
Robert D. Mercer, M.D.
Department of Pediatrics and Adolescent Medicine
A. David Rothner, M.D.
Department of Neurology, Department of Pediatrics and Adolescent Medicine
Sebastian A. Cook, M.D.
Department of Nuclear Medicine
Ralph J. Alfidi, M.D.
Department of Diagnostic Radiology
The Cobb syndrome is defined as a spinal cord arteriovenous malformation (AVM) associated with a cutaneous hemangioma (flame nevus) of the same metamere. The flame nevus is of significance in that it is suggestive of the presence of spinal cord AVM and indicates its location. The hereditary nature of this condition has been newly recognized.Case report
A girl, 6 years and 5 months of age, was examined at the Cleveland Clinic in August 1973 because of a birthmark involving the left buttock. Birth and development had been normal, and she had been otherwise healthy and well. The left buttock was reported to have recently increased in size and the child’s chief concern was related to her friends’ comments about her asymmetry from the rear view. She also reported discomfort whenever she happened to fall in a sitting position.
Results of the physical examination were generally normal except for the region of the buttocks. The left buttock was larger than the right. A flame angioma covered most of the surface of the left buttock (Fig. 1). Deep cavernous elements were not identified in this angioma, but the increased size and warmth of the buttock were suggestive of a more extensive vascular anomaly than was apparent on the surface. Additional small flame angiomas were noted on both hands and wrists, left anterior thigh, and left foot and ankle. A prominent pulsation was felt and a loud bruit was heard in the region of the coccyx. The rectal examination was negative. The . . .