Glioependymal cyst regression in watchful waiting follow-up

Abstract

Background: Intracranial glioependymal cysts are rare developmental benign lesions usually derived from the third and lateral ventricles. They can show no symptoms throughout life and be an incidental finding or can cause mild-to-severe symptoms which need surgical treatment.

Case description: We present the case of a giant glioependymal cyst of the left lateral ventricle in a newborn baby diagnosed at birth. Hydrocephalus was suspected in the prenatal ultrasound check-up at 37 weeks, leading to careful imaging exploration with brain computed tomography and magnetic resonance imaging (MRI), which suggested the diagnosis of a neuroepithelial cyst. Apgar score at birth was normal and there was no sign of hydrocephalus. Watchful waiting follow-ups every month in the 1^st year and every 6 months for the upcoming years with fundoscopic examinations and brain MRI showed a significant cyst shrinking. The last clinical evaluation showed normal activity at the age of eight.

Conclusion: In symptomatic neuroepithelial cysts with progressive growth, surgery is recommended. The natural history of untreated glioependymal cysts remains unknown. A conservative approach with periodical follow-ups can be considered in asymptomatic cases, because, rarely, spontaneous shrinking of the cyst can happen.

Keywords: Brain magnetic resonance imaging; Conservative approach; Glioependymal cyst; Intracranial neuroepithelial cyst; Lateral ventricle; Surgery.

Figure 1:

Brain MRI performed 1 month after birth showing a left intraventricular glioependymal cyst (white arrow) a) hypointense on axial Flair sequence b) no contrast enhancement on axial T1-gadolinium sequence c) hyperintense on coronal T2-weighted sequence d) no restriction on axial DWI sequence.

Figure 2:

Brain MRI performed one year later showing noted shrinking in size of the cyst (yellow arrow) a) axial Flair sequence b) T1-gadolinium sequence c) coronal T2 sequence d) sagittal T1 sequence.

Figure 3:

Last brain MRI 8 years later revealing further reduction in size of the cyst (blue arrow) a) axial Flair sequence b) T1-gadolinium sequence c) coronal T2 sequence d) axial DWI sequence.