克-特-韦综合征与颈髓内海绵状血管瘤：一种极为罕见的关联。病例报告。
Klippel-Trenaunay-Weber syndrome and intramedullary cervical cavernoma: a very rare association. Case report.
作者信息
Pichierri Angelo, Piccirilli Manolo, Passacantilli Emiliano, Frati Alessandro, Santoro Antonio
机构信息
Department of Neurological Sciences--Neurosurgery, University of Rome "La Sapienza," Rome, Italy.
出版信息
Surg Neurol. 2006 Aug;66(2):203-6; discussion 206. doi: 10.1016/j.surneu.2005.11.062.
BACKGROUND
Klippel-Trenaunay-Weber syndrome is a rare mesodermal phakomatosis characterized by cutaneous hemangiomata, venous varicosities, and osseous-soft tissue hypertrophy of the affected limb. As the pathologic aspect of KTWS arises from the site in which malformations occur, the clinical picture varies widely from patients who complain for cosmetic reasons to patients with life-threatening lesions.
CASE DESCRIPTION
We describe a very rare case in which KTWS was associated with a cervical intramedullary cavernous angioma surgically treated.
CONCLUSION
This report confirms the wide range of expression of vascular abnormalities in neurocutaneous developmental diseases and the need of a careful multisystemic evaluation of these patients.
背景
克-特-韦综合征是一种罕见的中胚层错构瘤病，其特征为皮肤血管瘤、静脉静脉曲张以及患侧肢体的骨-软组织肥大。由于克-特-韦综合征的病理表现源于畸形发生的部位，临床表现差异很大，从因美容原因就诊的患者到有危及生命病变的患者都有。
病例描述
我们描述了一例非常罕见的病例，其中克-特-韦综合征与经手术治疗的颈髓内海绵状血管瘤相关。
结论
本报告证实了神经皮肤发育疾病中血管异常表现的广泛多样性，以及对这些患者进行仔细的多系统评估的必要性。
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