Fourth ventricle tumors in children: complications and influence of surgical approach

Sebastian M. Toescu MBChB(Hons), MRCS 1 , 2 , Gargi Samarth MBBS 1 , Hugo Layard Horsfall MBBS 1 , 3 , Richard Issitt DClinP 4 , Ben Margetts PhD 4 , Kim P. Phipps BSc(Hons) 1 , Noor-ul-Owase Jeelani MBA, MPhil (Medical Law), FRCS(SN) 1 , Dominic N. P. Thompson MBBS, FRCS(SN) 1 , and Kristian Aquilina MD, FRCS(SN) 1
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  • 1 Department of Neurosurgery, Great Ormond Street Hospital for Children, London;
  • 2 Developmental Imaging and Biophysics Section, UCL GOS Institute of Child Health, London;
  • 3 Department of Neurosurgery, Addenbrooke’s Hospital, Cambridge; and
  • 4 Digital Research Environment, Great Ormond Street Hospital for Children, DRIVE Office, London, United Kingdom
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OBJECTIVES

The goal of this study was to characterize the complications and morbidity related to the surgical management of pediatric fourth ventricle tumors.

METHODS

All patients referred to the authors’ institution with posterior fossa tumors from 2002 to 2018 inclusive were screened to include only true fourth ventricle tumors. Preoperative imaging and clinical notes were reviewed to extract data on presenting symptoms; surgical episodes, techniques, and adjuncts; tumor histology; and postoperative complications.

RESULTS

Three hundred fifty-four children with posterior fossa tumors were treated during the study period; of these, 185 tumors were in the fourth ventricle, and 167 fourth ventricle tumors with full data sets were included in this analysis. One hundred patients were male (mean age ± SD, 5.98 ± 4.12 years). The most common presenting symptom was vomiting (63.5%). The most common tumor types, in order, were medulloblastoma (94 cases) > pilocytic astrocytoma (30 cases) > ependymoma (30 cases) > choroid plexus neoplasms (5 cases) > atypical teratoid/rhabdoid tumor (4 cases), with 4 miscellaneous lesions. Of the 67.1% of patients who presented with hydrocephalus, 45.5% had an external ventricular drain inserted (66.7% of these prior to tumor surgery, 56.9% frontal); these patients were more likely to undergo ventriculoperitoneal shunt (VPS) placement at a later date (p = 0.00673). Twenty-two had an endoscopic third ventriculostomy, of whom 8 later underwent VPS placement. Overall, 19.7% of patients had a VPS sited during treatment.

Across the whole series, the transvermian approach was more frequent than the telovelar approach (64.1% vs 33.0%); however, the telovelar approach was significantly more common in the latter half of the series (p < 0.001). Gross-total resection was achieved in 70.7%. The most common postoperative deficit was cerebellar mutism syndrome (CMS; 28.7%), followed by new weakness (24.0%), cranial neuropathy (18.0%), and new gait abnormality/ataxia (12.6%). Use of intraoperative ultrasonography significantly reduced the incidence of CMS (p = 0.0365). There was no significant difference in the rate of CMS between telovelar or transvermian approaches (p = 0.745), and multivariate logistic regression modeling did not reveal any statistically significant relationships between CMS and surgical approach.

CONCLUSIONS

Surgical management of pediatric fourth ventricle tumors continues to evolve, and resection is increasingly performed through the telovelar route. CMS is enduringly the major postoperative complication in this patient population.

ABBREVIATIONS CMS = cerebellar mutism syndrome; CPA = cerebellopontine angle; CUSA = Cavitron ultrasonic surgical aspirator; EOR = extent of resection; ETV = endoscopic third ventriculostomy; EVD = external ventricular drain; GTR = gross-total resection; STR = subtotal resection; VPS = ventriculoperitoneal shunt.

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Contributor Notes

Correspondence Kristian Aquilina: Great Ormond Street Hospital for Children, London, United Kingdom. kristian.aquilina@gosh.nhs.uk.

INCLUDE WHEN CITING Published online October 23, 2020; DOI: 10.3171/2020.6.PEDS2089.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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