Dr. Nasser Ibrahim Al-Rashid Orbital Vision Research Center. Bascom Palmer Eye Institute, Oculofacial Plastic and Reconstructive Surgery Service, Department of Ophthalmology, University of Miami Miller School of Medicine, Miami, Florida, USA.
Orbit. 2021 Oct;40(5):435-443. doi: 10.1080/01676830.2020.1866023. Epub 2021 Mar 16.
A 34-year-old Caucasian male who underwent a ventricular shunt at age 21 presented with bilateral enophthalmos, poor eyelid-globe apposition and exposure keratopathy characteristic of silent brain syndrome. Progressive enophthalmos and corneal decompensation were documented in serial photographs and radiographic studies over 36 years. Over this period, no sequelae of shunt over-drainage were observed. A lumbar puncture at the last follow-up measured CSF opening pressure to be within the normal range. Additional systemic findings included pneumosinus dilatans, loss of adipose tissue in the temporalis fossa and atrophy of the dorsal interossei of the hand. Surgical interventions to preserve ocular function included insertion of orbital floor wedge and sheet implants, sheet orbital roof implants, and retroplacement of canthal tendons. This report chronicles the long-term clinical course of a patient with silent brain syndrome. The systemic changes suggest factors beyond low intracranial pressure may contribute to the pathogenesis of the condition in our patient.
一位 34 岁的白人男性，21 岁时接受了脑室分流术，出现双侧眼球内陷、眼睑-眼球贴合不良和暴露性角膜炎，这些都是无症状性脑综合征的特征。在 36 年的时间里，通过系列照片和影像学研究记录了进行性眼球内陷和角膜失代偿。在此期间，未观察到分流过度引流的后遗症。最后一次随访时的腰椎穿刺测量 CSF 开放压力在正常范围内。其他全身表现包括鼻窦扩张、颞肌窝脂肪组织丧失和手部背侧骨间肌萎缩。为了保护眼部功能而进行的手术干预包括眶底楔形和片状植入物、眶顶片状植入物以及内眦腱的逆行复位。本报告记录了一位无症状性脑综合征患者的长期临床病程。全身性变化表明，除了颅内压低之外，还有其他因素可能导致患者的病情发生。
