Hemangioma cavernoso intramedular: relato de caso

Abstract

1RESUMO -O hemangioma cavernoso intramedular é anomalia vascular de baixo fluxo, curável através de ressecção cirúrgica. Entretanto, se não forem instituídos o diagnóstico precoce e a terapêutica adequada, pode levar à graves sequelas neurológicas. É extremamente raro a ocorrência de um angioma cavernoso intramedular. É relatado o caso de uma paciente de 33 anos, com um angioma cavernoso intramedular no nível de T6 -T7, que apresentava quadro clínico de compressão medular lenta e progressiva, com piora recente importante. A ressonância magnética da coluna torácica demonstrou, entre a sexta e a sétima vértebras dorsais, lesão expansiva intramedular que se apresentava hipointensa em T1 e discretamente hiperintensa em T2, com pequeno halo hipointenso à sua volta nas duas sequências. A paciente foi submetida a tratamento cirúrgico tardio e não apresentou recuperação neurológica. Os achados clínicos, de imagem e a importância de instituir precocemente o tratamento adequado são analisados e discutidos.PALAVRAS-CHAVE: angioma cavernoso, cavernoma, intramedular, malformação vascular, hemangioma cavernoso.Spinal cord intramedullary cavernous haemangioma: case report Spinal cord intramedullary cavernous haemangioma: case report Spinal cord intramedullary cavernous haemangioma: case report Spinal cord intramedullary cavernous haemangioma: case report Spinal cord intramedullary cavernous haemangioma: case report ABSTRACT -The intramedullary cavernous haemangioma is a low flow vascular anomaly curable through surgical resection, howewer if the precocious diagnosis and the appropriate therapeutics are not done it can cause serious neurological sequels.It is extremely rare the occurrence of intramedullary cavernous haemangioma. We report the case of a 33 year-old woman patient with an intramedullary cavernous angioma at T6-T7, that presented a clinical picture of slow and progressive spinal cord compression , with an important recent worsening.The magnetic resonance of the thoracic column demonstrated a T6 -T7 a intramedullary expansive lesion wich presented hypointense on T1 and discretely hyperintense on T2 with a small hypointense area in the two sequences.The patient was submitted to late surgical treatment and did not presented neurological recovery. The clinical and imagelogy pictures, and the importance of instituing precociously the appropriate treatment of this entity is reviewed, analyzed and discussed.KEY WORDS: cavernous angioma, cavernoma, intramedullary vascular malformation, cavernous haemangioma.Serviço de Neurocirurgia do Hospital Santa Casa Misericórdia de Pelotas, RS, Brasil (UCPE). Hemangiomas cavernosos são malformações vasculares que ocorrem no tecido nervoso 1,2 . E quando intramedulares são classicamente descritas como raras 3 . Malformações cavernosas espinhais ocorrem usualmente nas vértebras e podem se estender para dentro do canal medular extradural 4 . Cavernoma extradural e intradural já foram relatados. No presente relato, descrevemos um caso de angioma cavernoso, revisamos a literatura relaciona...

Symptomatic Intramedullary Cavernous Angioma During Pregnancy. Case report

“…Regarding the familial pattern, the lesions tend to be multiple, being inherited in an autosomal dominant way, with high clinical penetrance [1][2][3] . Up to the present moment, three genes whose mutations could predispose to cavernous angioma have been identified: 1) CCM1 (Cerebral Cavernous Malformation 1), located on the long arm of chromosome 7, position 21.2; 2) CCM2, located on the short arm of chromosome 7, position 13; and 3) CCM3, located on the long arm of chromosome 3, position 26.1.…”

“…These lesions are typically intra-axial, with 78% of the cases located in the supratentorial region and 22% in the infratentorial 1,3 . Also, it corresponds to 3 to 15% of vascular malformations of the spinal cord 3,5 . The frequency of occurrence of association between spinal and intracranial cavernomas is only 8% 4 .…”

“…The total surgical resection with microsurgical techniques is the treatment of choice 1,3,4,6,[11][12][13][14] . Total surgical resection may result in improvement (57%), stabilization (33%) or worsening (10%) of symptoms 14 .…”

“…Hemosiderin is less abundant in extradural and vertebral body cavernomas, probably due to the more abundant vascularization at this site, as well as the absence of a blood-brain barrier in this topography, conditions that facilitate its removal. In juxtamedullary and intramedullary lesions, hemosiderin is almost always present in greater abundance 1,3 .…”

Angioma cavernoso intramedular: relato de caso

“…Pacientes sintomáticos geralmente estão entre a terceira e a sexta década, sendo relatada predominância de 2:1 para o sexo feminino 1 . Hemorragia subaracnóidea e hematomielia são descritas, mas são manifestações raras 11 .…”