Multiple cavernomas of the brain: simultaneous hemorrhage of two lesions in a non-familial form

Abstract

BackgrOund: Cavernomas of the brain are congenital lesions clinically divided into hereditary and a sporadic forms. Multiple lesions are usually observed in the familial form, whereas the sporadic variant generally shows a single cavernoma. In this case we describe the simultaneous bleeding of two lesions in a patient with a non-familial cavernomatosis.case descrIptIOn: A 46-year-old woman presented with slight hyposthenia involving the left arm and cervical stiffness. Examinations revealed two cavernomas with recent signs of bleeding. The lesions were surgically removed achieving a total excision. The patient was discharged after 10 days with slight left hyposthenia.dIscussIOn: Simultaneous hemorrhage of multiple cavernomas is rare. To the best of our knowledge, this is the first case showing a sporadic form presenting with two hemorrhagic lesions. We suggest an aggressive approach in order to avoid the risk of rebleeding, which is particularly high in case of hemorrhagic cavernomatosis.keywOrds: Hemangioma, Cavernous, Central nervous system, Hemorrhage, Familial cerebral cavernous malformation ÖZ aMaÇ: Beyin kavernomları klinik olarak herediter ve sporadik forma bölünen konjenital lezyonlardır. Multipl lezyonlar genellikle ailesel formda görülürken, sporadik varyantta genelde tek kavernom görülür. Bu olguda ailesel olmayan kavernomatosisi olan bir hastada iki lezyonda eş zamanlı kanama tanımlıyoruz.OLgu sunuMu: 46 yaşında bir kadın sol kolda hafif hiposteni ve servikal sertlikle geldi. Muayeneler yakın zamanlı kanama bulgularıyla iki kavernom gösterdi. Lezyonlar cerrahi olarak çıkarılıp tam eksizyon elde edildi. Hasta 10 gün sonra hafif bir sol hiposteniyle taburcu edildi. tartIşMa: Multipl kavernomların eş zamanlı kanaması nadirdir. Bildiğimiz kadarıyla bu hasta iki hemorajik lezyonla gelen ilk sporadik form olgusudur. Özellikle hemorajik kavernomatoz olgularında yüksek olan tekrar kanama riskinden kaçınmak için agresif bir yaklaşım öneriyoruz.anaHtar sÖZcÜkLer: Hemanjiyom, Kavernöz, Merkez sinir sistemi, Kanama, Ailesel serebral kavernöz malformasyon

Simultaneous and sequential hemorrhage of multiple cerebral cavernous malformations: a case report

“…Cerebral cavernomas are estimated to occur in 1 out of every 200 individuals in the general population [ 3 , 6 , 7 ]. They are hypothesized to develop due to failure of vascular stabilization in angiogenesis of cerebral blood vessels [ 5 ].…”

“…In a large series studying the natural history of cavernomas, 18.7 to 20 % of patients had multiple lesions [ 9 , 12 , 13 ]. Multiple lesions are mostly seen in familial CCM (FCCM) forms [ 7 ]. FCCM has been associated with mutations of CCM1 , CCM2 and CCM3 genes.…”

“…CCMs have a hemorrhage rate of 0.7 to 1.1 % per lesion per year [ 4 , 7 ]. Most cases of hemorrhages reported in the literature tend to involve one lesion, even in patients with multiple CCMs.…”

“…Sporadic and inherited forms of CCM have been described. The sporadic form often results in single isolated lesions while the inherited form is associated with multiple lesions and mutations of the endothelial genes CCM1 , CCM2 or CCM3 [ 5 , 7 ].…”

“…CCM2 and CCM3 mutations have also been linked to higher hemorrhage rates [ 1 , 8 ]. Simultaneous hemorrhages of multiple CCM lesions are anecdotally common but few have been reported [ 7 , 9 , 10 ]. Causative physiologic parameters preceding hemorrhagic CCM events are often not described even in case reports.…”