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

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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 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.

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Authors and Affiliations

  1. Department of Neurosurgery, Hacettepe University School of Medicine, Sıhhıye, 06100, Ankara, Turkey

    Burcak Bilginer, Sahin Hanalioglu & Nejat Akalan

  2. Hacettepe University Institute of Neurological Sciences and Psychiatry, Ankara, Turkey

    Firat Narin

  3. Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey

    Kader Karlı Oguz

  4. Department of Pathology, Hacettepe University School of Medicine, Ankara, Turkey

    Figen Soylemezoglu

Authors
  1. Burcak Bilginer
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  2. Firat Narin
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  3. Sahin Hanalioglu
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  4. Kader Karlı Oguz
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  6. Nejat Akalan
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Correspondence to Burcak Bilginer.

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Bilginer, B., Narin, F., Hanalioglu, S. et al. Cavernous malformations of the central nervous system (CNS) in children: clinico-radiological features and management outcomes of 36 cases. Childs Nerv Syst 30, 1355–1366 (2014). https://doi.org/10.1007/s00381-014-2442-3

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