Endoscope-assisted microsurgical resection of intrinsic brainstem epidermoid: technical note and review of the literature

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  • 1 Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland; and
  • 2 Department of Neurosurgery, Boston Children’s Hospital, Harvard Medical School, Boston, Massachusetts
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Intrinsic epidermoid tumors of the brainstem are rare, histologically benign lesions associated with high surgical morbidity and mortality due to their eloquent location. The authors report a child with progressive severe neurological deterioration from a large midline intrinsic brainstem epidermoid at the pontomedullary junction. The mass was removed through a posterior fossa craniotomy and midline endoscope-assisted microsurgical corridor through the floor of the fourth ventricle, using neurophysiological monitoring. Postoperatively, there was dramatic improvement in the patient’s symptoms. Early recurrence of the mass necessitated reoperation with more aggressive resection of the cyst capsule, which led to complete radiographic reconstitution of the brainstem. The patient remains well with a durable recovery 7 years after presentation. The authors review the literature on brainstem epidermoids and discuss the differential diagnosis and management strategies for approaching these lesions, advocating for conservative surgery with resection of as much of the tumor capsule as is safely possible.

ABBREVIATIONS CISS = constructive interference in steady state; CPA = cerebellopontine angle; DWI = diffusion-weighted imaging; OR = operating room.

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Contributor Notes

Correspondence Alan R. Cohen: The Johns Hopkins University School of Medicine, Baltimore, MD. alan.cohen@jhmi.edu.

INCLUDE WHEN CITING Published online October 2, 2020; DOI: 10.3171/2020.6.PEDS20482.

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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