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

Autor: Chang TC; Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USA., Okafor KC; Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USA., Cavuoto KM; Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USA., Dubovy SR; Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USA., Karp CL; Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USA.
Jazyk: angličtina
Zdroj: Ocular oncology and pathology [Ocul Oncol Pathol] 2018 Apr; Vol. 4 (3), pp. 196-198. Date of Electronic Publication: 2017 Nov 18.
DOI: 10.1159/000484053
Abstrakt: 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.
Databáze: MEDLINE