Ibrahim Dalia, Mashhour Shady
Department of Radiology, Kasr Al Ainy Hospital, Cairo, Egypt.
BJR Case Rep. 2020 Oct 23;7(1):20200145. doi: 10.1259/bjrcr.20200145. eCollection 2021 Feb 1.
Cobb syndrome is a rare vascular disorder characterized by vascular skin lesions distributed in a dermatomal pattern, with corresponding muscular, osseous, paraspinal, and/or spinal vascular lesions occurring at the same body somite (metamere). We present a case of a 25-year-old man who presented with a history of right upper limb paresthesia followed by bilateral progressive upper and lower limb weakness and heaviness. Physical examination showed large cutaneous port wine stains on the right side of the chest, the nape, and along the whole right upper limb in a dermatomal distribution, with no corresponding limb hypertrophy or asymmetry. MRI and CT scan of the cervical spine showed aggressive vertebral hemangiomas involving the right side of C1 down to C4 vertebrae associated with extraosseous epidural lesion causing cervical cord compression, in addition to right paraspinal muscular low flow vascular malformations. Digital subtraction angiography of the neck vessels showed corresponding vascular blush and delayed contrast pooling in the affected regions. Cobb syndrome was diagnosed based on the dermatomal distribution of the cutaneous vascular lesions and the corresponding vertebral, epidural, and paraspinal vascular lesions occurring at the same metamere. The patient underwent a decompressive laminectomy at C2-C6 levels with removal of the epidural lesion, after which his symptoms had improved.
科布综合征是一种罕见的血管疾病，其特征为血管性皮肤病变呈皮节分布，在同一身体体节（同质节）出现相应的肌肉、骨骼、椎旁和/或脊髓血管病变。我们报告一例25岁男性病例，该患者有右上肢感觉异常病史，随后出现双侧进行性上肢和下肢无力及沉重感。体格检查发现胸部右侧、颈后部及整个右上肢有大片皮肤葡萄酒色斑，呈皮节分布，无相应肢体肥大或不对称。颈椎MRI和CT扫描显示侵袭性椎体血管瘤累及C1右侧至C4椎体，并伴有椎体外硬膜外病变导致颈髓受压，此外还有右侧椎旁肌肉低流量血管畸形。颈部血管数字减影血管造影显示病变区域有相应的血管造影剂充盈和延迟造影剂聚集。根据皮肤血管病变的皮节分布以及在同一同质节出现的相应椎体、硬膜外和椎旁血管病变，诊断为科布综合征。患者接受了C2 - C6节段减压性椎板切除术并切除硬膜外病变，术后症状有所改善。
