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Cavernous malformations of the central nervous system (CNS) in children: clinico-radiological features and management outcomes of 36 cases


Cavernous malformations of the central nervous system (CNS) in children: clinico-radiological features and management outcomes of 36 cases



Child's Nervous System: Chns: Official Journal of the International Society for Pediatric Neurosurgery 30(8): 1355-1366



ISSN/ISBN: 0256-7040

PMID: 24879529

DOI: 10.1007/s00381-014-2442-3

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. We retrospectively analyzed demographic, clinical, radiological, management, and follow-up data of 36 pediatric patients with CMs from a single institution. 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 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. 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.

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Accession: 051976979

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