Safety and efficacy of Endoscopic Third Ventriculostomy in Diffuse Intrinsic Pontine Glioma related hydrocephalus: a Systematic Review

Objective Diffuse intrinsic pontine glioma (DIPG) related hydrocephalus occurs as the result of tumor growth and sylvian aqueduct obstruction. There is no consensus about the best surgical option; thus, a review has been performed to clarify the rate of success, complications, and possible issues of endoscopic third ventriculostomy (ETV) in comparison to other available techniques. Methods This systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement, and was registered with the International Prospective Register of Systematic Reviews (PROSPERO) (registration number CRD42018089001). MEDLINE, Web of Knowledge, and EMBASE were searched for published series in which ETV was performed to treat hydrocephalus in DIPG patients. Results Six studies were included. Two further cases from our experience were added for a total amount of 55 patients treated through either ETV, ventriculoperitoneal shunt (VPS), or ventriculocisternal shunt according to Torkildsen. Eighty-six percent of patients who underwent ETV experienced clinical improvement after surgery ( P value 0.03). Torkildsen shunt placement was associated with a 50% failure rate. Two patients implanted with VPS developed symptoms of shunt malfunction and increased ventricular sizes (10%). The Fisher exact test was applied to compare efficacy of VPS and ETV with no statistical difference between the 2 groups ( P value 0.17). Patients who underwent ETV did not experience major complications, and no procedural termination was observed. Conclusions ETV is an effective and safe treatment option, associated with a low complication rate and a high success rate. Evidences from this review suggest considering ETV as the first-line treatment of hydrocephalus in DIPG patients.