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

Case report

Michael K. Yoon M.D.1,2, Andrew T. Parsa M.D., Ph.D.3, and Jonathan C. Horton M.D., Ph.D.4,5,6
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  • 1 Department of Ophthalmology, Harvard Medical School;
  • | 2 Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; and
  • | 3 Departments of Neurosurgery,
  • | 4 Ophthalmology,
  • | 5 Neurology, and
  • | 6 Physiology, University of California, San Francisco, California
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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.

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