Slit ventricle syndrome

Last revised by Yahya Baba on 19 May 2023

Slit ventricle syndrome refers to cerebrospinal fluid shunt-related symptoms in the setting of small ventricles demonstrated on radiologic studies.

The presence of characteristic symptoms is required to diagnose slit-ventricle syndrome and must be distinguished from slit-like ventricles, a radiologic finding.

Patients present with chronic intermittent headache, with or without neurologic signs or symptoms such as nausea/vomiting and altered mental status. Ophthalmic symptoms such as visual acuity loss, field defects, palsy and nystagmus have been reported in children 5. The shunt reservoir refills slowly on palpation.

Mild forms are seen in adults and more severe forms are seen in children.

The exact etiology behind slit ventricle syndrome is debatable but likely is related to intermittent intracranial hypertension and hypotension. Various hypotheses have been proposed 3,4:

  • intermittent ventricular isolation, in which one over drained ventricle collapses and occludes the shunt catheter resulting in under drainage of the contralateral ventricle

  • noncompliant ventricles due to periventricular gliosis ("stiff ventricle"), which is unable to adapt to shunt malfunction (underdrainage), leading to intraventricular hypertension

  • overdrainage leading to venous congestion, which decreases cerebral compliance and causes susceptibility to small changes in intracranial pressure

  • capillary absorption laziness, in which the capillary-venous system in the setting of chronic overdrainage is less able to absorb variations in ventricular fluid pressures

  • overdrainage in children leading to acquired craniocerebral disproportion (container-content mismatch) due to early suture ossification, resulting in intracranial hypertension

Small ventricles with a characteristic slit-like appearance.

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Cases and figures

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