Successful management of symptomatic hydrocephalus using a temporary external ventricular drain with or without endoscopic third ventriculostomy in pediatric patients with germinoma

Rebecca Ronsley MD, FRCPC1, Eric Bouffet MD2, Peter Dirks MD, PhD3, James Drake MBBCh3, Abhaya Kulkarni MD, PhD, FRCSC3, and Ute Bartels MD2
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  • 1 Division of Hematology, Oncology & Bone Marrow Transplant, Department of Pediatrics, British Columbia Children’s Hospital, University of British Columbia, Vancouver, British Columbia;
  • | 2 Division of Hematology, Oncology & Bone Marrow Transplant, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Ontario; and
  • | 3 Pediatric Neurosurgery, Department of Surgery, The Hospital for Sick Children, University of Toronto, Ontario, Canada
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OBJECTIVE

The objective of this study was to describe the management of hydrocephalus in a cohort of pediatric patients with germinoma.

METHODS

The authors conducted a retrospective chart review of patients with germinoma and symptomatic hydrocephalus treated at the Hospital for Sick Children between 2002 and 2020. Descriptive data included tumor location, CSF diversion procedure (external ventricular drain [EVD], endoscopic third ventriculostomy [ETV], ventriculoperitoneal [VP] shunt) and outcomes. The frontooccipital horn ratio (FOR) method was used to determine the presence of ventriculomegaly.

RESULTS

Of 39 patients with germinoma, 22 (73% male) had symptomatic hydrocephalus at diagnosis (11 pineal, 4 suprasellar, and 7 bifocal). Management of hydrocephalus included EVD (n = 5, 22.7%), ETV (n = 5, 22.7%), and combination ETV and EVD (n = 7, 31.8%), whereas 5 patients (22.7%) did not undergo surgical intervention. The median FOR at diagnosis was 0.42 (range 0.38–0.58), which correlated with moderate to severe ventriculomegaly. Carboplatin and etoposide–based chemotherapy induced fast tumor shrinkage, avoiding CSF diversion (n = 5) and resolving hydrocephalus with a transient EVD (n = 5). The median duration until EVD removal was 7 days (range 2–10 days). Two of 12 patients with EVD ultimately required a VP shunt. Kaplan-Meier overall survival was 100% and progression-free survival was 96.4% at a median follow-up of 10.4 years.

CONCLUSIONS

Timely initiation of chemotherapy is imperative to rapidly reduce tumor bulk in children with germinoma and limits the need for VP shunt insertions. In children in whom CSF diversion is required, hydrocephalus may be successfully managed with a temporary EVD ± ETV.

ABBREVIATIONS

AFP = alpha-fetoprotein; ETV = endoscopic third ventriculostomy; EVD = external ventricular drain; FOR = frontooccipital horn ratio; GCT = germ cell tumor; hCGβ = human chorionic gonadotropin-beta; OS = overall survival; PFS = progression-free survival; VP = ventriculoperitoneal.

Schematics of transseptal interforniceal resection of a superiorly recessed colloid cyst. ©Mark Souweidane, published with permission. See the article by Tosi et al. (pp 813–819).

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