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目的总结儿童颅内多发性海绵状血管瘤(CA)的临床特点与治疗策略。方法选取2003年1月-2011年5月临床诊断为儿童颅内CA患儿15例。对其临床表现、头颅影像学特征及预后进行回顾性分析。结果 15例患儿颅内CA中脑内型14例,脑外型1例。14例脑内型患儿临床表现为头痛8例,呕吐5例,癫发作4例,意识障碍3例,头晕、偏瘫各2例,肢体感觉障碍1例;1例脑外型患儿临床表现为单侧眼睑下垂,伴头痛、呕吐、复视。14例脑内型患儿CT检查发现30个病灶;MRI检查发现35个病灶,病灶周围一圈低信号30个,呈环状或半环状;颅脑血管磁共振成像检查结果阴性;2例数字减影脑血管造影检查阴性。14例患儿行手术治疗,其中3例因急性头痛、呕吐伴意识障碍于病程10 d内手术;11例病情呈间歇性发作,于病程0.5～12.0 a择期手术。结论儿童颅内CA以脑内型多见,临床表现缺乏特异性,主要表现为头痛、呕吐等颅高压症状,其次为癫发作等症状。头颅MRI检查是目前临床术前确诊颅内CA的最佳方法。外科手术是治疗颅内CA的主要方法,无症状或症状轻微的患儿可临床随访观察,如出现难治性局灶性癫、严重头痛、局部神经功能障碍应及时手术治疗。
Objective To summarize the clinical features and treatment strategies of intracranial multiple cavernous hemangiomas (CA) in children. Methods From January 2003 to May 2011, 15 children with intracranial CA were diagnosed clinically. The clinical manifestations, craniographic features and prognosis were retrospectively analyzed. Results Fifteen children with intracranial CA had 14 cases of intracerebral type and 1 case of extracerebral brain type. The clinical manifestations of 14 intracerebral children were headache in 8 cases, vomiting in 5 cases, epilepsy in 4 cases, disturbance of consciousness in 3 cases, dizziness and hemiplegia in 2 cases, limb sensory disturbance in 1 case, 1 case of cerebral manifestations in children with clinical manifestations For unilateral eyelid ptosis, with headache, vomiting, diplopia. Thirteen lesions were detected by CT in 14 children with intracranial type. Thirty-five lesions were found by MRI. Thirty low-signal signals were observed in one circle around the lesion, showing a ring or semi-ring shape. Negative results were found in cranial and cerebrovascular magnetic resonance imaging. Digital subtraction cerebral angiography negative. Fourteen children underwent surgical treatment. Three of them underwent surgery within 10 days due to acute headache and vomiting associated with disturbance of consciousness. Eleven patients had intermittent seizures and had elective surgery during the course of 0.5-12.0 a. Conclusion Intracranial CA is more common in intracerebral CA and lack of specific clinical manifestations. The main symptoms are headache, vomiting and other symptoms of intracranial hypertension followed by epileptic seizures. Cranial MRI is the best method for clinical diagnosis of intracranial CA before surgery. Surgery is the main method of treatment of intracranial CA, asymptomatic or mild symptoms of clinical follow-up in children, such as refractory focal epilepsy, severe headache, local neurological dysfunction should be timely surgical treatment.