Giant intracranial teratoma with epignathus in a neonate

Case report and review of the literature

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✓Epignathus, a congenital oropharyngeal teratoma, is a rare clinical entity with variable clinical outcomes described in the literature. Even fewer cases of epignathus with intracranial extension have been reported, all with poor outcomes. In this manuscript, the authors present a case of epignathus with intracranial extension, emphasizing clinical presentation, imaging findings, a staged surgical approach, multidisciplinary management, and outcome.

Abbreviations used in this paper:CSF = cerebrospinal fluid; MR = magnetic resonance.

Article Information

Address reprint requests to: James M. Johnston, M.D., Department of Neurosurgery, Washington University School of Medicine, 660 Euclid Avenue, Campus Box 8057, St. Louis, Missouri 63110. email: johnstonj@nsurg.wustl.edu.

© AANS, except where prohibited by US copyright law.

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Figures

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    Photographs. A: Preoperative view of the soft tissue mass protruding from the child's oropharynx. B: Anterior view after first-stage resection of the oropharyngeal component, demonstrating obvious cleft palate deformity without evidence of CSF fistula. C: The child's appearance after primary repair of the cleft palate and lip deformity.

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    Preoperative T1-weighted contrast-enhanced MR images depicting oropharyngeal (A) and intracranial (B) components of a heterogeneously enhancing, multicystic mass.

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    Contrast-enhanced T1-weighted MR images. A and B: Images obtained prior to the second operation showing interval enlargement of the right frontotemporal cyst and brainstem compression. C and D: Postoperative images demonstrating gross-total resection.

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    Intraoperative photograph obtained after right fron-totemporal craniotomy and resection of the intracranial teratoma. A1 = anterior cerebral artery; CN II = right optic nerve; CN III = right oculomotor nerve; CN IV = right trochlear nerve; M1 = middle cerebral artery; PCA = posterior cerebral artery.

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    Photomicrographs showing mature teratoma cells with squamous epithelium and keratinous debris (A), cartilaginous tissue (B), adipose tissue with duct-like and glandular elements (C), and enteric-type epithelium with goblet cells (D). H & E, original magnification × 40 (A and B) and × 10 (C and D).

References

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