Symptomatic large or giant capillary telangiectasias: management and outcome in 5 cases

Abstract

B rain capillary telangiectasia (BCT) is a rare vascular malformation with a prevalence of 0.4%-0.7% based on autopsy and MRI diagnosis. 12 Most BCTs are small in size and clinically silent with a predilection of pons and basal ganglia. Clinical data of large and symptomatic BCTs are rare. Only 10 symptomatic cases with 3 diffuse BCTs have been reported in the English-language literature of more than 200 BCTs. 12,20,24,27,29 Large BCTs have a much higher risk of causing uncontrolled bleeding and severe neurological deficits and even death if left untreated. 2,12,13,21,22 Therefore, large BCTs should be managed with special caution. Due to their rarity, the diagnosis of large BCTs has been difficult. 29,30 Large BCTs have different radiological characteristics, histopathological characteristics, and clinical courses from small BCTs. 8,12,25 Standard management is undefined for large or giant BCTs. 7,12,20,24,27,29 To our knowledge, the current study presents the largest case series of large BCTs ever reported. We analyzed the radiological findings, histopathological characteristics, clinical presentation, and management in 5 symptomatic, unusually large BCTs (mean diameter 5.06 cm, range 1.8-8 cm). Coexisting cavernous malformations (CMs) were confirmed in 3 cases. Resections were performed in all patients to control seizures and other recurrent neurological events.abbreviatioNs BCT = brain capillary telangiectasia; CM = cavernous malformation; DSA = digital subtraction angiography; ECoG = electrocorticography; EEG = electroencephalography. Brain capillary telangiectasias (BCTs) are usually small and benign with a predilection in the pons and basal ganglion. Reports of large and symptomatic BCTs are rare. Large BCTs have a much higher risk of causing uncontrolled bleeding and severe neurological defects, and they can be fatal if left untreated. Therefore, large BCTs should be managed with special caution. Because of the lack of reports, diagnosis of large BCTs has been difficult. Strategies of management are undefined for large or giant BCTs. The current study presents 5 cases of giant and large BCTs. To the authors' knowledge, this is the largest series of this disease ever reported. Radiological findings, histopathological characteristics, clinical presentations, and surgical management were analyzed in 5 symptomatic, unusually large BCTs (mean diameter 5.06 cm, range 1.8-8 cm). Four patients presented with focal or generalized seizures, and 1 patient presented with transient vision loss attributed to the lesions. Gross-total resection of the lesion was achieved in all patients. After surgery, the 4 patients with seizures were symptom free for follow-up periods varying from more than 1 to 5 years with no additional neurological deficits. The unique location, radiological characteristics, and clinical course suggest that giant BCTs could be a different entity from small BCTs. Surgery might be a good option for treatment of patients with intractable neurological symptoms, especially in those with surgically acce...