Bilginer Burcak, Narin Firat, Hanalioglu Sahin, Oguz Kader Karlı, Soylemezoglu Figen, Akalan Nejat
Department of Neurosurgery, Hacettepe University School of Medicine, Sıhhıye, 06100, Ankara, Turkey,
Childs Nerv Syst. 2014 Aug;30(8):1355-66. doi: 10.1007/s00381-014-2442-3. Epub 2014 May 31.
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.
中枢神经系统（CNS）海绵状血管畸形（CMs）是血管造影隐匿性血管病变，约影响0.5%的普通人群，且所有CMs中有四分之一发生于儿童。
我们回顾性分析了来自单一机构的36例儿童CMs患者的人口统计学、临床、放射学、治疗及随访数据。
患儿首次就诊及手术时的平均年龄分别为8.7岁和9.6岁。然而，发现年龄分布呈双峰型，高峰在4岁以下和12岁以上。癫痫是最常见的单一首发症状（38.9%），61.1%的患者在入院前至少有一次癫痫发作。局灶性神经功能缺损（410.7%）、颅内高压（27.8%）和头痛（2.8%）是其他表现。63.9%的患者在就诊时可见急性/亚急性出血。发现6岁以下患者在诊断时比12岁以上患者有更多的巨大海绵状血管瘤（69%对20%；p = 0.011）和更明显的出血（81%对47%；p = 0.065）。31例患者（32个CMs）接受了手术，其中26例全切，6例部分切除。平均随访时间为6.9±4.1年。所有患者中，63.8%临床结局极佳，30.5%良好，并且在最后一次随访时90.9%的癫痫患者无癫痫发作（Engel I级）。
年幼儿童比年长儿童更容易患较大的CMs且更易出现出血。对于有症状且可手术的CMs，显微手术切除应是首选治疗方法。
