Elsevier

Ophthalmology

Volume 118, Issue 11, November 2011, Pages 2286-2295
Ophthalmology

Original article
Sunken Eyes, Sagging Brain Syndrome: Bilateral Enophthalmos from Chronic Intracranial Hypotension

https://doi.org/10.1016/j.ophtha.2011.04.031Get rights and content

Purpose

To explain the mechanism for acquired enophthalmos after ventriculoperitoneal shunting (VPS).

Design

Case series and a case-control study.

Participants and Controls

Four study patients with bilateral enophthalmos after VPS and 10 control subjects.

Methods

Case description of 4 study patients. Calculated orbital volumes for 2 study patients were compared with controls using the Wilcoxon rank-sum test.

Main Outcome Measures

Exophthalmometry measurements and total orbital and fat volumes.

Results

Patient 1 is a 25-year-old man who presented with progressive enophthalmos 3 years after VPS for traumatic intracranial bleeding. Imaging demonstrated upward expansion of the orbital roof and evidence of intracranial hypotension. The intracranial pressure (ICP) was 20 mm H2O. The enophthalmos improved after shunt revision. Patient 2 is a 19-year-old man who presented with progressive enophthalmos 18 months after VPS for traumatic intracranial hemorrhage. Patient 3 is a 38-year-old woman who presented with bilateral enophthalmos 15 years after VPS after a ruptured aneurysm. Imaging showed orbital expansion. Patient 4 is a 16-year-old man who presented with severe enophthalmos 5 years after a VPS for aneurysm-related hemorrhage. Imaging demonstrated orbital enlargement and findings of intracranial hypotension. Intracranial pressure ranged between −200 and 0 mm H2O. Shunt revision improved the enophthalmos. Total orbital volumes were significantly greater in the study patients than in the controls. Control subjects (5 male, 5 female, ages 23–45 years) had an average right orbital volume of 24.6±3.3 cm3 (n = 10). In comparison, the right orbital volumes of patients 1 and 3 were 32.6 and 32.1 cm3. Similar results were found for the left orbits (23.9±2.7 cm3 [control average] vs. 35.9 and 32.6 cm3). In patient 1, the post-shunt volumes increased 14% (right) and 23% (left) from pre-shunt volumes. In contrast, orbital fat volume was not statistically significantly different between the control group and enophthalmic patients (right orbit control mean 7.94±3.1 cm3 [n = 10] vs. 7.9 and 9.8 cm3; left orbit control mean 7.88+3.1 cm3 vs. 9.2 and 10.0 cm3).

Conclusions

Enophthalmos after VPS results primarily from chronic intracranial hypotension. Low ICP causes expansion of orbital volume with no fat atrophy. In such patients, shunt revision with a pressure-regulating valve to correct intracranial hypotension should be considered.

Financial Disclosure(s)

The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Section snippets

Materials and Methods

Institutional review board/ethics committee approval was obtained, and the research adhered to the tenets of the Declaration of Helsinki. From 1991 to 2010, 4 patients were identified at the University of California San Francisco with progressive enophthalmos after VPS. They are described in detail, with focus on neuro-ophthalmologic, orbital, and radiographic evaluations.

Orbital volumetric analysis was performed on 2 enophthalmic patients (patients 1 and 3) who had adequate imaging studies.

Case Descriptions

Patient 1 is a 25-year-old man who was referred for evaluation of disconjugate gaze in September 2004. He was involved in a motor vehicle accident in November 2000 that resulted in extensive hemorrhagic brain contusions, frontotemporal epidural hematoma, and intraventricular hemorrhage. There were several skull fractures identified on CT, but none involved the orbits. In January 2001, the patient had a sudden decrease in mental status. A CT scan revealed new ventriculomegaly from presumed

Discussion

Shunting of CSF is a well-established surgical approach for treatment of increased ICP. Over-shunting is a known complication of this procedure. In pediatric patients when the skull is still developing, over-shunting inhibits normal expansion of the cranial vault and can lead to premature suture fusion and secondary craniosynostoses.35, 36 In contrast, our data support the hypothesis that over-shunting and intracranial hypotension in adult patients can present with a different type of bony

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    Manuscript no. 2010-354.

    Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

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