Cavernous malformations of the central nervous

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May 31, 2014 - in children: clinico-radiological features and management outcomes of 36 cases ..... Supratentorial+infratentorial. 4. Supratentorial+spinal. 1.
Childs Nerv Syst (2014) 30:1355–1366 DOI 10.1007/s00381-014-2442-3

ORIGINAL PAPER

Cavernous malformations of the central nervous system (CNS) in children: clinico-radiological features and management outcomes of 36 cases Burcak Bilginer & Firat Narin & Sahin Hanalioglu & Kader Karlı Oguz & Figen Soylemezoglu & Nejat Akalan

Received: 10 April 2014 / Accepted: 14 May 2014 / Published online: 31 May 2014 # Springer-Verlag Berlin Heidelberg 2014

Abstract Background Cavernous malformations (CMs) of the central nervous system (CNS) are angiographically occult vascular lesions that affect approximately 0.5 % of the general population, and one quarter of all CMs occurs in children. Methods We retrospectively analyzed demographic, clinical, radiological, management, and follow-up data of 36 pediatric patients with CMs from a single institution. Results The mean age of the children at first presentation and at operation was 8.7 and 9.6 years, respectively. However, a bimodal age distribution was found with peak under 4 years and above 12 years. Seizure was the most common single presenting symptom (38.9 %), and 61.1 % of patients had at least one seizure before the admission. Focal neurological deficits (410.7 %), intracranial hypertension (27.8 %), and headache (2.8 %) were the other manifestations. Acute/ subacute hemorrhage was evident at presentation in 63.9 %. The patients under 6 years of age were found to have significantly more giant cavernomas (69 vs 20 %; p=0.011), and more overt hemorrhages (81 vs 47 %; p=0.065) at diagnosis than those patients above 12 years. Surgery was performed in 31 patients (32 CMs), with 26 total and 6 incomplete B. Bilginer (*) : S. Hanalioglu : N. Akalan Department of Neurosurgery, Hacettepe University School of Medicine, Sıhhıye, 06100 Ankara, Turkey e-mail: [email protected] F. Narin Hacettepe University Institute of Neurological Sciences and Psychiatry, Ankara, Turkey

resections. Mean follow-up duration was 6.9±4.1 years. Of all patients, 63.8 % had excellent and 30.5 % had good clinical outcomes, and also 90.9 % of the epileptic patients were seizure-free (Engel Class I) at the last follow-up. Conclusions Younger children tend to harbor larger CMs and present with hemorrhage more frequently than older ones. Microsurgical resection should be the treatment of choice in symptomatic and accessible CMs. Keywords Cavernous malformation . Cavernoma . Central nervous system (CNS) . Children . Epilepsy . Hemorrhage

Introduction Cavernous malformations (CMs), also referred to as c a v er n o m a s , c a v er n o u s a ng i o m a s o r c a v e r no u s hemangiomas, of the central nervous system (CNS) are characterized by multiple dilated sinusoidal blood vessels lined by a single layer of endothelium that lack the muscular and adventitial layers and intervening neural tissue [5, 15]. Since CMs are low-flow vascular malformations, they are occult to angiography, hence diagnosed by magnetic resonance imaging (MRI) [15, 27, 32]. They affect about 0.5 % of the general population [5, 33] and one fourth of all cases occur during childhood [1, 2]. The main clinical presentations include seizures, hemorrhage, neurological deficits, and headache [5, 17, 33]. Pediatric cases may have different presentation and natural history than adults [1–3, 20, 26, 35].

K. K. Oguz Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey

Material and methods

F. Soylemezoglu Department of Pathology, Hacettepe University School of Medicine, Ankara, Turkey

Using hospital database and patient charts, we retrospectively investigated 36 consecutive symptomatic pediatric patients (age