Trapped fourth ventricle

Case contributed by Dr Mohammad A. ElBeialy

Presentation

Worsening headache. The patient attended for assessment of the VP shunt tube function.

Patient Data

Age: 11 years old.
Gender: Female
MRI

MRI scan shows active hydrocephalus with periventricular T2/FLAIR hyperintensity (retrograde transependymal CSF permeation) caused by a large posterior fossa cyst, distorting and occluding the fourth ventricle outlet; and associated with significant mass effect on the brainstem. The 4th ventricle intraventricular cystic lesion shows near CSF signal intensity with inhomogeneous suppression on FLAIR due to turbulent flow and no restricted diffusibility. A right sided VP shunt tube is seen within the body of the right lateral ventricle.

Case Discussion

This patient clearly has shunt dysfunction accounting for the presentation. The differential for the posterior fossa cyst includes: 

A trapped fourth ventricle is by far the most likely aetiology in this case given that the patient has had prior shunt placement which implies A) absence of a large 4th ventricular cystic mass at that stage, and B) prior episode(s) of meningitis / haemorrhage, both common antecedents to ventricular isolation. 

Arachnoid cysts are frequent anomalies of the CNS. They are benign lesions within the arachnoid membrane and have been reported to occur in virtually all locations where arachnoid is present. An intraventricular location, however, is rare and occurrence within the fourth ventricle is particularly uncommon. The first report was published in 1979 in a paediatric patient. Since then, only a few further examples have been reported. Most of these patients presented with hydrocephalus. Shunting procedures were performed, but did not afford long-term improvement of symptoms. Definitive treatment consisted of open resection of the cyst-wall.

The Blake's Pouch Cyst is a cystic malformation of the posterior fossa. It represents persistence and expansion of the normally transient Blake pouch which normally regresses during the fifth to eighth gestational weeks. It is described as posterior ballooning of the inferior medullary velum into the cisterna magna, and is communicating with open fourth ventricle below and posterior to the vermis. In this case the vermis appears intact behind the cyst and is therefore thought most unlikely. 

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Case information

rID: 21602
Case created: 3rd Feb 2013
Last edited: 7th Feb 2016
Inclusion in quiz mode: Included

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