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

OBJECT: Diffuse Intrinsic Pontine Gliomas (DIPG) related hydrocephalus occurs as the result of tumor growth and 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 the other available techniques.

METHODS: This systematic review followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement and it was registered with the PROSPERO International Prospective Register of Systematic Reviews(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 coming from our experience were added, for a total amount of 55 patients treated through either ETV, VPS or Ventriculocisternal shunt according to Torkildsen. 86% of patients who underwent ETV experienced clinical improvement after surgery (p-value 0.03). Torkildsen shunt placement was associated to 50% failure rate. Two patients implanted with VPS developed symptoms of shunt malfunction and increased ventricular sizes (10%). Fisher's exact test was applied to compare efficacy of VPS and ETV with no statistical difference between the two group (p-value 0,17). Patients who underwent ETV did not experienced major complications and no procedural abortion was observed.

CONCLUSIONS: ETV is an effective and safe treatment option, associated to a low complications rate and a high success rate. Evidences from this review suggest to consider ETV as the first line treatment of hydrocephalus in DIPG patients.