Idiopathic Bilateral Profound Hypotony in an Unknown Progressive Neurodegenerative Disorder

Lorraine M Provencher; Pamela C Carter; Wallace L M Alward

doi:10.1097/IJG.0000000000000639

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Idiopathic Bilateral Profound Hypotony in an Unknown Progressive Neurodegenerative Disorder

Journal article

Peer reviewed

Idiopathic Bilateral Profound Hypotony in an Unknown Progressive Neurodegenerative Disorder

Lorraine M Provencher, Pamela C Carter and Wallace L M Alward

Journal of glaucoma, Vol.26(5), pp.e168-e170

05/2017

DOI: 10.1097/IJG.0000000000000639

PMID: 28221333

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Abstract

To present a unique case of idiopathic bilateral hypotony in a patient with progressive, undiagnosed neurological decline, possibly due to mitochondrial disease, and to explore mechanisms of disease and potential treatment options. This is a case report. A 17-year-old boy with a history of chronic progressive bilateral vision loss and hypotony in the setting of progressive gait abnormalities, lower extremity spasticity, nystagmus, and urinary retention starting around age 8. Despite extensive biochemical and genetic evaluation, no systemic etiology has been identified. He had no history of ocular trauma or surgery. Examination confirmed the above history as well as decreased vision, significant bilateral astigmatism (7 D), short axial-eye-lengths, and disc edema with chorioretinal folds in the left eye. There was no inflammation or ciliary body detachment. We propose the etiology is similar to hypotony in myotonic dystrophy, in which low intraocular pressure may result from aqueous egress across the ciliary body face. The best treatment remains unclear, but surgical closure of the iridocorneal angle is under careful consideration. This may halt nonconventional (suprachoroidal) outflow. Management of ocular hypotony is typically directed at the underlying etiology. Idiopathic hypotony poses a unique treatment challenge. If excess aqueous flow across the ciliary body face is responsible, intentional closure of the iridocorneal angle may preserve vision.

Myotonic Dystrophy - diagnosis

Humans

Intraocular Pressure - physiology

Male

Disease Progression

Mitochondrial Diseases - complications

Ocular Hypotension - etiology

Myotonic Dystrophy - complications

Adolescent

Ocular Hypotension - physiopathology

Gait Disorders, Neurologic - etiology

Mitochondrial Diseases - diagnosis

Vision Disorders - etiology

Details

Title: Subtitle: Idiopathic Bilateral Profound Hypotony in an Unknown Progressive Neurodegenerative Disorder
Creators: Lorraine M Provencher - Department of Ophthalmology and Visual Sciences, The University of Iowa Hospitals and Clinics, Iowa City, IA
Pamela C Carter
Wallace L M Alward
Resource Type: Journal article
Publication Details: Journal of glaucoma, Vol.26(5), pp.e168-e170
DOI: 10.1097/IJG.0000000000000639
PMID: 28221333
ISSN: 1057-0829
eISSN: 1536-481X
Language: English
Date published: 05/2017
Academic Unit: Internal Medicine; Ophthalmology and Visual Sciences
Record Identifier: 9984101605402771

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2 Times Cited - Web of Science