Pediatric Multiple Endocrine Neoplasia Type 2B: Clinicopathological Correlation of Perilimbal Mucosal Neuromas and Treatment of Secondary Open-Angle Glaucoma

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Abstract

Ocular findings of multiple endocrine neoplasia type 2B (MEN 2B) include prominent corneal nerves, mucosal neuromas of the conjunctiva, glaucoma, and dry eyes. A 15-year-old girl with MEN 2B presents with advanced secondary open-angle glaucoma and bilateral perilimbal masses. High-resolution optical coherence tomography (HR-OCT) of the perilimbal lesions showed normal epithelial thickness and subepithelial lobular areas of mixed reflectivity, which correlates well with histopathologic findings of benign mucosal neuromas. The patient underwent bilateral gonioscopy-assisted transluminal trabeculotomy (GATT) in both eyes, which achieved adequate intraocular pressure control at short-term follow-up. To the authors' knowledge, this is the first report correlating HR-OCT and histopathologic findings in benign mucosal neuroma associated with MEN 2B, and the first description of secondary open-angle glaucoma associated with MEN 2B treated with GATT.

Original languageEnglish (US)
Pages (from-to)196-198
Number of pages3
JournalOcular Oncology and Pathology
Volume4
Issue number3
DOIs
StatePublished - Apr 1 2018

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Multiple Endocrine Neoplasia Type 2b
Neuroma
Open Angle Glaucoma
Gonioscopy
Pediatrics
Trabeculectomy
Optical Coherence Tomography
Conjunctiva
Therapeutics
Intraocular Pressure
Glaucoma

Keywords

  • Anterior segment optical coherence tomography
  • Gonioscopy-assisted transluminal trabeculotomy
  • Juvenile open-angle glaucoma
  • Mucosal neuromas
  • Multiple endocrine neoplasia type 2B

ASJC Scopus subject areas

  • Nursing(all)

Cite this

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title = "Pediatric Multiple Endocrine Neoplasia Type 2B: Clinicopathological Correlation of Perilimbal Mucosal Neuromas and Treatment of Secondary Open-Angle Glaucoma",
abstract = "Ocular findings of multiple endocrine neoplasia type 2B (MEN 2B) include prominent corneal nerves, mucosal neuromas of the conjunctiva, glaucoma, and dry eyes. A 15-year-old girl with MEN 2B presents with advanced secondary open-angle glaucoma and bilateral perilimbal masses. High-resolution optical coherence tomography (HR-OCT) of the perilimbal lesions showed normal epithelial thickness and subepithelial lobular areas of mixed reflectivity, which correlates well with histopathologic findings of benign mucosal neuromas. The patient underwent bilateral gonioscopy-assisted transluminal trabeculotomy (GATT) in both eyes, which achieved adequate intraocular pressure control at short-term follow-up. To the authors' knowledge, this is the first report correlating HR-OCT and histopathologic findings in benign mucosal neuroma associated with MEN 2B, and the first description of secondary open-angle glaucoma associated with MEN 2B treated with GATT.",
keywords = "Anterior segment optical coherence tomography, Gonioscopy-assisted transluminal trabeculotomy, Juvenile open-angle glaucoma, Mucosal neuromas, Multiple endocrine neoplasia type 2B",
author = "Ta Chang and Okafor, {Kingsley C.} and Cavuoto, {Kara M} and Sander Dubovy and Carol Karp",
year = "2018",
month = "4",
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doi = "10.1159/000484053",
language = "English (US)",
volume = "4",
pages = "196--198",
journal = "Ocular Oncology and Pathology",
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T2 - Clinicopathological Correlation of Perilimbal Mucosal Neuromas and Treatment of Secondary Open-Angle Glaucoma

AU - Chang, Ta

AU - Okafor, Kingsley C.

AU - Cavuoto, Kara M

AU - Dubovy, Sander

AU - Karp, Carol

PY - 2018/4/1

Y1 - 2018/4/1

N2 - Ocular findings of multiple endocrine neoplasia type 2B (MEN 2B) include prominent corneal nerves, mucosal neuromas of the conjunctiva, glaucoma, and dry eyes. A 15-year-old girl with MEN 2B presents with advanced secondary open-angle glaucoma and bilateral perilimbal masses. High-resolution optical coherence tomography (HR-OCT) of the perilimbal lesions showed normal epithelial thickness and subepithelial lobular areas of mixed reflectivity, which correlates well with histopathologic findings of benign mucosal neuromas. The patient underwent bilateral gonioscopy-assisted transluminal trabeculotomy (GATT) in both eyes, which achieved adequate intraocular pressure control at short-term follow-up. To the authors' knowledge, this is the first report correlating HR-OCT and histopathologic findings in benign mucosal neuroma associated with MEN 2B, and the first description of secondary open-angle glaucoma associated with MEN 2B treated with GATT.

AB - Ocular findings of multiple endocrine neoplasia type 2B (MEN 2B) include prominent corneal nerves, mucosal neuromas of the conjunctiva, glaucoma, and dry eyes. A 15-year-old girl with MEN 2B presents with advanced secondary open-angle glaucoma and bilateral perilimbal masses. High-resolution optical coherence tomography (HR-OCT) of the perilimbal lesions showed normal epithelial thickness and subepithelial lobular areas of mixed reflectivity, which correlates well with histopathologic findings of benign mucosal neuromas. The patient underwent bilateral gonioscopy-assisted transluminal trabeculotomy (GATT) in both eyes, which achieved adequate intraocular pressure control at short-term follow-up. To the authors' knowledge, this is the first report correlating HR-OCT and histopathologic findings in benign mucosal neuroma associated with MEN 2B, and the first description of secondary open-angle glaucoma associated with MEN 2B treated with GATT.

KW - Anterior segment optical coherence tomography

KW - Gonioscopy-assisted transluminal trabeculotomy

KW - Juvenile open-angle glaucoma

KW - Mucosal neuromas

KW - Multiple endocrine neoplasia type 2B

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