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:

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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    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).

  • View in gallery

    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.

  • View in gallery

    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.

  • View in gallery

    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.


  • 1

    Anderson RKieffer SAWolfson JJLong DPeterson HO: Thickening of the skull in surgically treated hydrocephalus. Am J Roentgenol Radium Ther Nucl Med 110:961011970

  • 2

    Arriada NSotelo J: Continuous-flow shunt for treatment of hydrocephalus due to lesions of the posterior fossa. J Neurosurg 101:7627662004

  • 3

    Aschoff AKremer PHashemi BKunze S: The scientific history of hydrocephalus and its treatment. Neurosurg Rev 22:67951999

  • 4

    Bernardini FPRose GECruz AAPriolo E: Gross enophthalmos after cerebrospinal fluid shunting for childhood hydrocephalus: the “silent brain syndrome. Ophthal Plast Reconstr Surg 25:4344362009

  • 5

    Browd SRGottfried ONRagel BTKestle JR: Failure of cerebrospinal fluid shunts: part II: overdrainage, loculation, and abdominal complications. Pediatr Neurol 34:1711762006

  • 6

    Chan WMadge SNSenaratne TSenanayake SEdussuriya KSelva D: Exophthalmometric values and their biometric correlates: the Kandy Eye Study. Clin Experiment Ophthalmol 37:4965022009

  • 7

    Cruz AAMesquita IMde Oliveira RS: Progressive bilateral enophthalmos associated with cerebrospinal shunting. Ophthal Plast Reconstr Surg 24:1521542008

  • 8

    Davidson JKSoparkar CNWilliams JBPatrinely JR: Negative sinus pressure and normal predisease imaging in silent sinus syndrome. Arch Ophthalmol 117:165316541999

  • 9

    Di Rocco CMarchese EVelardi F: A survey of the first complication of newly implanted CSF shunt devices for the treatment of nontumoral hydrocephalus. Cooperative survey of the 1991–1992 Education Committee of the ISPN. Childs Nerv Syst 10:3213271994

  • 10

    Drake JMKestle JRTuli S: CSF shunts 50 years on—past, present and future. Childs Nerv Syst 16:8008042000

  • 11

    Durig JBorruat FXJaques B: [Silent sinus syndrome: an unusual cause of vertical diplopia.]. Klin Monatsbl Augenheilkd 212:3973991998. (Fr)

  • 12

    Fishman RA: Cerebrospinal Fluid in Diseases of the Nervous System ed 2PhiladelphiaWB Saunders1992

  • 13

    Fishman RADillon WP: Intracranial hypotension. J Neurosurg 86:1651997. (Letter)

  • 14

    Ganchi PAMovassaghi KYaremchuk MJ: Treatment of bilateral symptomatic enophthalmos associated with weight loss. J Craniofac Surg 15:84872004

  • 15

    Griscom NTOh KS: The contracting skull. Inward growth of the inner table as a physiologic response to diminution of intracranial content in children. Am J Roentgenol Radium Ther Nucl Med 110:1061101970

  • 16

    Horton JCFishman RA: Neurovisual findings in the syndrome of spontaneous intracranial hypotension from dural cerebrospinal fluid leak. Ophthalmology 101:2442511994

  • 17

    Hwang KKim JHBaik SH: Thickness map of parietal bone in Korean adults. J Craniofac Surg 8:2082121997

  • 18

    Hwang TNRofagha SMcDermott MWHoyt WFHorton JCMcCulley TJ: Sunken eyes, sagging brain syndrome: bilateral enophthalmos from chronic intracranial hypotension. Ophthalmology 118:228622952011

  • 19

    Kaufman BSandstrom PHYoung HF: Alteration in size and configuration of the sella turcica as the result of prolonged cerebrospinal fluid shunting. Radiology 97:5375421970

  • 20

    Kestle JR: Pediatric hydrocephalus: current management. Neurol Clin 21:8838952003

  • 21

    Kestle JRWalker ML: A multicenter prospective cohort study of the Strata valve for the management of hydrocephalus in pediatric patients. J Neurosurg 102:2 Suppl1411452005

  • 22

    Magnaes B: Body position and cerebrospinal fluid pressure. Part 2: clinical studies on orthostatic pressure and the hydrostatic indifferent point. J Neurosurg 44:6987051976

  • 23

    Meyer DRNerad JANewman NJLin JC: Bilateral enophthalmos associated with hydrocephalus and ventriculoperitoneal shunting. Arch Ophthalmol 114:120612091996

  • 24

    Moseley JERabinowitz JGDziadiw R: Hyperostosis cranii ex vacuo. Radiology 87:110511071966

  • 25

    Naradzay JFBrowne BJRolnick MADoherty RJ: Cerebral ventricular shunts. J Emerg Med 17:3113221999

  • 26

    Nulsen FSpitz E: Treatment of hydrocephalus by direct shunt from ventricle to jugular vein. Surg Forum 2:3994031952

  • 27

    Numa WADesai UGold DRHeher KLAnnino DJ: Silent sinus syndrome: a case presentation and comprehensive review of all 84 reported cases. Ann Otol Rhinol Laryngol 114:6886942005

  • 28

    Pannullo SCReich JBKrol GDeck MDPosner JB: MRI changes in intracranial hypotension. Neurology 43:9199261993

  • 29

    Poca MASahuquillo JTopczewski TLastra RFont MLCorral E: Posture-induced changes in intracranial pressure: a comparative study in patients with and without a cerebrospinal fluid block at the craniovertebral junction. Neurosurgery 58:8999062006

  • 30

    Robinson SKaufman BAPark TS: Outcome analysis of initial neonatal shunts: does the valve make a difference?. Pediatr Neurosurg 37:2872942002

  • 31

    Sivasubramaniam RSacks RThornton M: Silent sinus syndrome: dynamic changes in the position of the orbital floor after restoration of normal sinus pressure. J Laryngol Otol 125:123912432011

  • 32

    Weinzweig JBartlett SPChen JCLosee JSutton LDuhaime AC: Cranial vault expansion in the management of postshunt craniosynostosis and slit ventricle syndrome. Plast Reconstr Surg 122:117111802008

  • 33

    Yoon MKEconomides JRHorton JC: Extraocular muscle dynamics in diplopia from enophthalmos. Strabismus 19:1421462011




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