Soeda Akio, Sakai Nobuyuki, Iihara Koji, Nagata Izumi
Department of Neurosurgery, National Cardiovascular Center, Osaka, Japan.
Neurosurgery. 2003 Mar;52(3):711-5; discussion 714-5. doi: 10.1227/01.neu.0000048483.21777.b7.
Cobb syndrome is a rare clinical entity characterized by the combination of a vascular skin nevus and an angioma in the spinal canal at the same metamere. We present a case report of an infant with Cobb syndrome.
A 5-month-old girl presented with cutaneous hemangioma of the thoracolumbar region (T5-T12) and paraparesis. The infant was examined by magnetic resonance imaging and aortography and was diagnosed with Cobb syndrome.
The patient received orally administered prednisolone therapy and underwent endovascular embolization of paravertebral and spinal angiomas with the use of n-butyl-2-cyanoacrylate. Her symptoms improved by combined treatment with liquid embolization and corticosteroid therapy.
Although Cobb syndrome has been reported in older children, it is extremely rare in infants. To our knowledge, this is the first report of an infant with Cobb syndrome treated with endovascular embolization and corticosteroid therapy. The combined treatment with corticosteroid therapy and endovascular embolization of cutaneomeningospinal angioma in Cobb syndrome seems effective in alleviating symptoms and minimizing morbidity.
科布综合征是一种罕见的临床病症,其特征为在同一脊髓节段出现皮肤血管痣和椎管内血管瘤。我们报告一例患有科布综合征的婴儿病例。
一名5个月大的女孩出现胸腰段(T5 - T12)皮肤血管瘤及双下肢轻瘫。对该婴儿进行了磁共振成像和主动脉造影检查,诊断为科布综合征。
患者接受口服泼尼松龙治疗,并使用正丁基 - 2 - 氰基丙烯酸酯对椎旁和脊髓血管瘤进行血管内栓塞。通过液体栓塞和皮质类固醇治疗联合应用,她的症状得到改善。
尽管科布综合征在大龄儿童中已有报道,但在婴儿中极为罕见。据我们所知,这是首例采用血管内栓塞和皮质类固醇治疗的患有科布综合征的婴儿病例。在科布综合征中,皮质类固醇治疗与皮肤脊髓血管畸形的血管内栓塞联合治疗似乎对缓解症状和降低发病率有效。