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青春期前小儿特发性颅内高压:特征、治疗及预后

Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment, and outcome.

作者信息

Cinciripini G S, Donahue S, Borchert M S

机构信息

Division of Ophthalmology, Childrens Hospital Los Angeles, USC School of Medicine, California, USA.

出版信息

Am J Ophthalmol. 1999 Feb;127(2):178-82. doi: 10.1016/s0002-9394(98)00386-9.

DOI:10.1016/s0002-9394(98)00386-9
PMID:10030560
Abstract

PURPOSE

To report the features of idiopathic intracranial hypertension in prepubertal children, with emphasis on presentation, treatment, and outcome.

METHODS

We retrospectively reviewed the charts of all patients 11 years and younger diagnosed with idiopathic intracranial hypertension at two university-affiliated medical centers. Inclusion critera included papilledema, normal brain computed tomography or magnetic resonance imaging, cerebrospinal fluid pressure greater than 200 mm H2O, normal cerebrospinal fluid content, and a nonfocal neurologic examination except for sixth nerve palsy. Patients with concomitant systemic illness were excluded.

RESULTS

Of the 10 patients, four were girls and six were boys. Only one patient was obese. The most common presenting symptoms were stiff neck (four patients) and diplopia (four patients), and the most common presenting sign was strabismus (eight patients). Six of eight patients with strabismus had abducens nerve palsy (four bilateral), one patient had a sensory exotropia, and one had a comitant esotropia. Visual field abnormalities were present in 11 of 13 eyes (85%), and severe visual loss resulting in no light perception vision occurred in one eye of one patient. Nine patients were treated medically, four with a combination of prednisone or dexamethasone and acetazolamide and five with acetazolamide alone. One patient in this group also required a lumboperitoneal shunt. One patient was treated with lumbar puncture only. Resolution of papilledema occurred rapidly in all patients, with a mean of 4.7 +/- 2.6 months. Resolution of sixth nerve palsy also occurred rapidly in four of six patients in a mean of 1.6 +/- 1.2 months. One patient required strabismus surgery for persistent esotropia and one was lost to follow-up.

CONCLUSIONS

Idiopathic intracranial hypertension in prepubertal children is rare and is different than the disease in adults. In our series, there appeared to be no sex predilection, and obesity was uncommon. Children are likely to present with strabismus and stiff neck rather than headache or pulsatile tinnitus. Both papilledema and sixth nerve palsy resolved rapidly with treatment. However, children can sustain loss of visual field and visual acuity despite treatment.

摘要

目的

报告青春期前儿童特发性颅内高压的特征,重点关注其临床表现、治疗及预后。

方法

我们回顾性分析了两所大学附属医院诊断为特发性颅内高压的所有11岁及以下患者的病历。纳入标准包括视乳头水肿、脑部计算机断层扫描或磁共振成像正常、脑脊液压力大于200mmH₂O、脑脊液成分正常,以及除第六神经麻痹外无局灶性神经系统检查异常。伴有全身性疾病的患者被排除。

结果

10例患者中,4例为女孩,6例为男孩。仅1例患者肥胖。最常见的临床表现为颈部僵硬(4例患者)和复视(4例患者),最常见的体征为斜视(8例患者)。8例斜视患者中,6例有外展神经麻痹(4例双侧),1例为感觉性外斜视,1例为共同性内斜视。13只眼中有11只(85%)存在视野异常,1例患者的1只眼出现严重视力丧失,导致无光感视力。9例患者接受药物治疗,4例联合使用泼尼松或地塞米松及乙酰唑胺,5例仅使用乙酰唑胺。该组中有1例患者还需要行腰大池腹腔分流术。1例患者仅接受腰椎穿刺治疗。所有患者视乳头水肿均迅速消退,平均消退时间为4.7±2.6个月。6例第六神经麻痹患者中有4例也迅速恢复,平均恢复时间为1.

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