A Natural History of Silent Brain Syndrome over 36 Years: A case report

ABSTRACT

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.

KEYWORDS:

• Enophthalmos
• hydrocephalus
• pneumosinus dilatans
• silent brain syndrome
• ventricular shunt

Disclosure of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the article.

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Additional information

Funding

This work was supported in part by the NIH Center Core Grant P30EY014801; Research to Prevent Blindness Unrestricted Grant GR004596., Inc, New York, New York; NIH NEI Grant R01, NIH NIA Grant R56 AG053369, and the Dr. Nasser Ibrahim Al-Rashid Orbital Vision Research Fund. The sponsor or funding organizations had no role in the design or conduct of this research.