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Bilateral ocular hypertension with rapidly progressive optic neuropathy in a teen.
Optom Vis Sci 2009; 86(9):E1117-26OV

Abstract

PURPOSE

We report a rare case of bilateral juvenile open-angle glaucoma (JOAG), with discussion of current understanding of its pathogenesis, differential diagnosis, genetics, and management. The importance of tonometry and dilated fundus examination as essential parts of a complete ocular examination, regardless of patient age, is emphasized.

CASE REPORT

A Hispanic female teenager presented for an updated eye examination as a requirement before joining military boot camp. Chief concern was blurry vision at distance, with no other reported ocular or systemic problems. She manifested simple myopia in both eyes and was correctable to 20/20 in each eye with glasses. However, intraocular pressure (IOP) in each eye was measured above 40 mm Hg. Subsequent automated perimetry showed significant visual field defects, and laser polarimetry analysis of the optic nerve fibers corresponded with the visual field loss pattern. Maximal medical therapy was administered to lower the IOP, with minimal success. Subsequent incisional trabeculectomy with topical antimetabolite were performed in both eyes to achieve adequate control of her IOPs.

CONCLUSIONS

Juvenile-onset Open Angle Glaucoma (JOAG) has been proposed to be a small subset of Primary Open Angle Glaucoma (POAG) and on a continual spectrum of Primary Open Angle Glaucoma. Because most patients with JOAG are asymptomatic, tonometry and optic nerve analysis are crucial in early detection and, thus, must be performed on all patients, young and old. JOAG has a variable onset, with rapidly progressive neuropathy that does not respond well to medical therapy alone, and surgical intervention is often the eventual treatment of choice. Mutations in the myocillin gene have been strongly linked to the disease. JOAG follows an autosomal dominant inheritance with relatively high penetrance. As such, close monitoring, genetics screening, and/or early medical management to prevent irreversible optic neuropathy and blindness should be considered as well for presymptomatic family members.

Authors+Show Affiliations

Pacific University College of Optometry, Forest Grove, Oregon 97116, USA. lenvhua@pacificu.eduNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

19661835

Citation

Hua, Len V., et al. "Bilateral Ocular Hypertension With Rapidly Progressive Optic Neuropathy in a Teen." Optometry and Vision Science : Official Publication of the American Academy of Optometry, vol. 86, no. 9, 2009, pp. E1117-26.
Hua LV, Byrne E, Yudcovitch L. Bilateral ocular hypertension with rapidly progressive optic neuropathy in a teen. Optom Vis Sci. 2009;86(9):E1117-26.
Hua, L. V., Byrne, E., & Yudcovitch, L. (2009). Bilateral ocular hypertension with rapidly progressive optic neuropathy in a teen. Optometry and Vision Science : Official Publication of the American Academy of Optometry, 86(9), pp. E1117-26. doi:10.1097/OPX.0b013e3181b620d3.
Hua LV, Byrne E, Yudcovitch L. Bilateral Ocular Hypertension With Rapidly Progressive Optic Neuropathy in a Teen. Optom Vis Sci. 2009;86(9):E1117-26. PubMed PMID: 19661835.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Bilateral ocular hypertension with rapidly progressive optic neuropathy in a teen. AU - Hua,Len V, AU - Byrne,Erin, AU - Yudcovitch,Lorne, PY - 2009/8/8/entrez PY - 2009/8/8/pubmed PY - 2009/12/16/medline SP - E1117 EP - 26 JF - Optometry and vision science : official publication of the American Academy of Optometry JO - Optom Vis Sci VL - 86 IS - 9 N2 - PURPOSE: We report a rare case of bilateral juvenile open-angle glaucoma (JOAG), with discussion of current understanding of its pathogenesis, differential diagnosis, genetics, and management. The importance of tonometry and dilated fundus examination as essential parts of a complete ocular examination, regardless of patient age, is emphasized. CASE REPORT: A Hispanic female teenager presented for an updated eye examination as a requirement before joining military boot camp. Chief concern was blurry vision at distance, with no other reported ocular or systemic problems. She manifested simple myopia in both eyes and was correctable to 20/20 in each eye with glasses. However, intraocular pressure (IOP) in each eye was measured above 40 mm Hg. Subsequent automated perimetry showed significant visual field defects, and laser polarimetry analysis of the optic nerve fibers corresponded with the visual field loss pattern. Maximal medical therapy was administered to lower the IOP, with minimal success. Subsequent incisional trabeculectomy with topical antimetabolite were performed in both eyes to achieve adequate control of her IOPs. CONCLUSIONS: Juvenile-onset Open Angle Glaucoma (JOAG) has been proposed to be a small subset of Primary Open Angle Glaucoma (POAG) and on a continual spectrum of Primary Open Angle Glaucoma. Because most patients with JOAG are asymptomatic, tonometry and optic nerve analysis are crucial in early detection and, thus, must be performed on all patients, young and old. JOAG has a variable onset, with rapidly progressive neuropathy that does not respond well to medical therapy alone, and surgical intervention is often the eventual treatment of choice. Mutations in the myocillin gene have been strongly linked to the disease. JOAG follows an autosomal dominant inheritance with relatively high penetrance. As such, close monitoring, genetics screening, and/or early medical management to prevent irreversible optic neuropathy and blindness should be considered as well for presymptomatic family members. SN - 1538-9235 UR - https://www.unboundmedicine.com/medline/citation/19661835/Bilateral_ocular_hypertension_with_rapidly_progressive_optic_neuropathy_in_a_teen_ L2 - http://Insights.ovid.com/pubmed?pmid=19661835 DB - PRIME DP - Unbound Medicine ER -