Skull thickening, paranasal sinus expansion, and sella turcica shrinkage from chronic intracranial hypotension

Case report

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In children or young adults, the morphology of the skull can be altered by excessive drainage of CSF following placement of a ventriculoperitoneal (VP) shunt. In Sunken Eyes, Sagging Brain Syndrome, gradual enlargement of the orbital cavity occurs from low or negative intracranial pressure (ICP), leading to progressive bilateral enophthalmos. The authors report several heretofore unrecognized manifestations of this syndrome, which developed in a 29-year-old man with a history of VP shunt placement following a traumatic brain injury at the age of 9 years. Magnetic resonance imaging showed typical features of chronic intracranial hypotension, and lumbar puncture yielded an unrecordable subarachnoid opening pressure. The calvaria was twice its normal thickness, owing to contraction of the inner table. The paranasal sinuses were expanded, with aeration of the anterior clinoid processes, greater sphenoid wings, and temporal bones. The sella turcica showed a 50% reduction in cross-sectional area as compared with that in control subjects, resulting in partial extrusion of the pituitary gland. These new features broaden the spectrum of clinical findings associated with low ICP. Secondary installation of a valve to restore normal ICP is recommended to halt progression of these rare complications of VP shunt placement.

Abbreviations used in this paper:ICP = intracranial pressure; VP = ventriculoperitoneal.

Article Information

Address correspondence to: Jonathan C. Horton, M.D., Ph.D., University of California, San Francisco, 10 Koret Way, San Francisco, California 94143. email: hortonj@vision.ucsf.edu.

Please include this information when citing this paper: published online March 29, 2013; DOI: 10.3171/2013.2.PEDS12560.

© AANS, except where prohibited by US copyright law.

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Figures

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    Computed tomography scans obtained when the patient was 21 years old, 12 years after placement of a VP shunt with no valve. Left: Axial section showing an extraaxial air collection adjacent to the left frontal sinus. The calvaria is abnormally thick. Right: Lower axial section demonstrating enlargement of the ethmoid and sphenoid sinuses, along with abnormal proliferation of the mastoid air cells.

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    Computed tomography scans revealing thickening of the calvaria with expansion of the diploë. Left: Axial view showing enlarged frontal sinuses and a titanium mesh closing a defect in the left frontal bone. The sphenoid sinus has expanded into the greater wings of the sphenoid bone (black arrows). Mastoid air cells are present in the squamous portion of the temporal bone (white arrow). Right: Coronal view showing aeration of the anterior clinoid processes (black arrows), large sphenoid sinuses, and air pockets in the temporal bone (white arrow).

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    Lateral scout film of the skull showing the locations where measurements of skull thickness were made from CT scans. The image was flipped horizontally to depict sites on the right (A) and left (B) calvariae. Values are expressed in millimeters. Color denotes the number of standard deviations greater than the published mean normal values (red = 3, blue = 2, yellow = 1). Note the shunt tubing, with no valve to regulate flow of CSF.

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    Axial CT scans from 1991 (left) just after the traumatic injury and from 2011 (right) showing an increase in skull thickness over 20 years. The imaging planes are not identical because of changes in head shape over 2 decades, but a marked increase in skull thickness is apparent.

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    Magnetic resonance images with features of chronic intracranial hypotension. Coronal T1-weighted MR image (A) showing thickening of the skull from widening of the diploë. The optic chiasm (arrow) is flattened by the pituitary gland. The brain morphology is distorted with midline shift to the right from the old traumatic injury. Coronal image (B) obtained after Gd administration and fat suppression, showing excessive enhancement of the dura (arrows). Sagittal T1-weighted image (C) showing marked expansion of the diploë (opposing arrow pairs), which is 1-cm thick in some areas. The sphenoid sinus has enlarged into the clivus (single white arrow). Sagittal image (D) obtained after Gd and fat suppression, showing dural enhancement (black arrows). The sella turcica (white arrow) is small, extruding the pituitary gland upward into the optic chiasm.

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