Cutaneomeningospinal angiomatosis (Cobb syndrome) with tethered cord

A newborn presented with a skin-covered lumbar mass with a subcutaneous hem angioma and on a magnetic resonance image (MRI) revealed a tethered spinal cord with a local mass. The mass had signal characteristics compatible with a lipoma, An initial diagnosis of a lipomeningocele with tethered cord was made, and the patient underwent surgical exploration and subtotal resectio n of the mass. A follow-up MRI revealed that the cord was still tethered, b ut an additional mass was present. The initial mass with signal characteris tics of lipomatous tissue was accompanied by a low-signal mass in the lumbo sacral canal, ventral to the cord, and bilateral enlargement of the foramin a at the lumbosacral level. Because of a concern for an intraspinal tumor, a second operative intervention was performed. Multiple biopsies of the mas s inside the spinal cord, the nerve roots and at the level of the foramina revealed angiomas that had similiar pathology in a II the specimens. A part ial resection of the masses and a release of the tethered cord was performe d by sectioning the thickened filum terminale, The diagnosis of Cobb's synd rome was made. The unique association of a tethered cord and the Cobb syndr ome is reported here.