Spinal Cavernous Angioma Associated with Klippel-Trenaunay-Weber Syndrome: Case Report and Literature Review.

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Spinal Cavernous Angioma Associated with Klippel-Trenaunay-Weber Syndrome: Case Report and Literature Review.

World Neurosurg. 2018 Jan;109:333-337

Authors: Oda K, Morimoto D, Kim K, Yui K, Kitamura T, Morita A

Abstract
BACKGROUND: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital vascular disorder characterized by the classic triad of cutaneous nevi, venous varicosities, and osseous and soft tissue hypertrophy of the affected limb. Various vascular anomalies of the central nervous system have also been described in patients with KTWS. The English language literature to date contains 6 reports of associations between KTWS and spinal cord cavernous angioma (CA), but management of these patients has not been well described.
CASE DESCRIPTION: A 23-year-old woman was admitted to our institution with acute onset of leg weakness accompanied by upper back pain. Thoracic magnetic resonance imaging of the spinal cord showed a heterogeneous mass with a slit component at the T1-2 level. The patient underwent left hemilaminectomy followed by removal of the tumor, and her neurologic symptoms improved postoperatively. Pathologic examination showed the spinal lesion was characterized by hemosiderin deposition and thin-walled vascular channels surrounded by fibrous tissue.
CONCLUSIONS: This is the first report to provide a detailed pathologic description of the features of spinal CA in a patient with KTWS. Assessment of the clinical features and management of CA associated with KTWS are discussed. This syndrome is rare, and further experience in the treatment of these patients is needed. However, considering that the pathologic findings of spinal CA in patients with KTWS include the typical features of CA, the management of CA in patients with KTWS may be identical to management of isolated CA.

PMID: 29054771 [PubMed - indexed for MEDLINE]

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