Endodermal Cyst Arising from the Oculomotor Nerve: A Case Report Outlining the Novel Insertion of Cysto-subarachnoid Shunt for Refractory Disease

Abstract

Background: Endodermal cysts of the oculomotor nerve are rare presentations. Only case reports are available to help guide clinicians with managing this rare entity.

Case description: A 3-year-old boy presented with an acute on chronic left oculomotor nerve palsy due to a left interpeduncular cistern cyst found on magnetic resonance imaging. He underwent a left pterional craniotomy and fenestration of the histologically proven endodermal cyst and had initial improvement at the 2-month review. He subsequently developed clinical and radiologic evidence of recurrence and was treated surgically with a refenestration and insertion of a cysto-subarachnoid shunt through a trans-sylvian approach. At 6-month follow-up, there was complete resolution of the oculomotor nerve palsy with interval development of oculomotor synkinesis.

Conclusions: Magnetic resonance imaging is an essential modality in the follow-up of these patients postoperatively in the setting of unchanged or deteriorated neurology. Fenestration of the cyst is appropriate first-line surgical management; however, a cysto-subarachnoid shunt is a safe consideration in recurrent, symptomatic cysts and provides sustained symptom resolution.

Keywords: Case report; Cysto-subarachnoid shunt; Endodermal cyst; Neurenteric; Oculomotor nerve; Palsy.