Chen Tiffany A, Weinert Marguerite C, See Alfred P, Kielian Agnieszka, Lehman Laura L, Remenschneider Aaron K, Robson Caroline D, Heidary Gena, Dagi Linda R, Gise Ryan
Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
Department of Neurosurgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
Eye (Lond). 2025 Mar;39(4):741-747. doi: 10.1038/s41433-024-03455-w. Epub 2024 Nov 19.
Otitic hydrocephalus is increased intracranial pressure without ventricular dilation secondary to mastoiditis and cerebral venous sinus thrombosis (CVST). It is associated with significant visual morbidity, though more detailed data on visual outcomes is lacking. We sought to better characterize the management of increased intracranial pressure and visual outcomes in this population.
Retrospective chart review at a quaternary Children's Hospital of patients <18 years of age who were diagnosed with otitic hydrocephalus from January 2009 to July 2023. Data were collected on patient demographics, clinical course, imaging, and treatment outcomes.
Fifteen patients were identified with mastoiditis complicated by otitic hydrocephalus. The average age was 5.7 years (range 2-15). Eight patients were male (53%). Six patients (40%) had cranial nerve VI palsy and 14 (93%) developed papilloedema. Eleven patients (73%) developed progressively worsening papilloedema despite improving infection, clot burden, and acetazolamide; of these, three required ventriculostomy catheters. Eight were started on corticosteroids. Six had resolution of papilloedema without the need for shunt placement. Two patients had evidence of optic atrophy from increased ICP and visual loss prior to corticosteroid initiation. Both had stabilization of their condition without further visual loss.
This study highlights the importance of systemic corticosteroids as part of the treatment paradigm for otitic hydrocephalus to prevent vision loss in patients otherwise unresponsive to medical management. It also identifies the risk of papilloedema progression and visual morbidity even after the initiation of appropriate medical therapy. Visual outcomes were significantly improved and invasive neurosurgical procedures were avoided with use of corticosteroids.
耳源性脑积水是继发于乳突炎和脑静脉窦血栓形成(CVST)的无脑室扩张的颅内压升高。它与显著的视觉损害相关,尽管缺乏关于视觉结局的更详细数据。我们试图更好地描述该人群颅内压升高的管理和视觉结局。
在一家四级儿童医院对2009年1月至2023年7月期间诊断为耳源性脑积水的18岁以下患者进行回顾性病历审查。收集了患者的人口统计学、临床病程、影像学和治疗结局数据。
确定了15例乳突炎合并耳源性脑积水的患者。平均年龄为5.7岁(范围2 - 15岁)。8例为男性(53%)。6例(40%)有第六颅神经麻痹,14例(93%)出现视乳头水肿。尽管感染、血栓负荷改善且使用了乙酰唑胺,但11例患者(73%)的视乳头水肿仍逐渐加重;其中3例需要脑室造瘘管。8例开始使用皮质类固醇。6例视乳头水肿消退,无需放置分流管。2例患者在开始使用皮质类固醇之前有因颅内压升高和视力丧失导致的视神经萎缩证据。两者病情均稳定,未进一步视力丧失。
本研究强调了全身性皮质类固醇作为耳源性脑积水治疗模式一部分的重要性,以防止对药物治疗无反应的患者视力丧失。它还确定了即使在开始适当的药物治疗后视乳头水肿进展和视觉损害的风险。使用皮质类固醇可显著改善视觉结局并避免侵入性神经外科手术。