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成人颈胸段皮肤脑脊膜脊髓血管瘤病(科布综合征):急性四肢瘫病例报告

Cervicothoracic cutaneomeningospinal angiomatosis in adults (Cobb's syndrome): A case report of acute quadriparesis.

作者信息

Papalini Pablo E, Papalini Francisco R

机构信息

Department of Neurosurgery, Hospital Córdoba, Córdoba, Argentina.

出版信息

Surg Neurol Int. 2017 Jul 18;8:147. doi: 10.4103/sni.sni_363_16. eCollection 2017.

Abstract

BACKGROUND

Cutaneomeningospinal angiomatosis or Cobb syndrome is a rare, not well understood phacomatosis that features metameric cutaneous and spinal arteriovenous malformations (AVMs). The first case was described in Boston in 1915, and since then, few more cases have been reported in the English literature. No case was found to be from Argentina.

CASE DESCRIPTION

The authors present a 16-year-old boy with acute quadriparesis and respiratory failure who was diagnosed as Cobb syndrome and treated with microsurgery alone with very good results.

CONCLUSION

Authors believe that microsurgery, alone or combined with embolization, should be the mainstay of treatment. They also acknowledge Harvey Cushing's contribution to the description of the syndrome and propose the syndrome to be renamed as Cobb-Cushing syndrome.

摘要

背景

皮肤脑脊膜血管瘤病或科布综合征是一种罕见的、了解尚不充分的错构瘤病,其特征为节段性皮肤和脊髓动静脉畸形(AVM)。1915年在波士顿报道了首例病例,自那时起,英文文献中报道的病例较少。未发现来自阿根廷的病例。

病例描述

作者报告了一名16岁急性四肢瘫和呼吸衰竭男孩,被诊断为科布综合征,仅接受显微手术治疗,效果良好。

结论

作者认为,显微手术单独或联合栓塞应作为主要治疗方法。他们还认可哈维·库欣对该综合征描述的贡献,并提议将该综合征重新命名为科布 - 库欣综合征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e49/5525462/b4ff0df50c5d/SNI-8-147-g001.jpg

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