Recurrent divergence-insufficiency esotropia in Machado-Joseph disease (spinocerebellar ataxia type 3)

Purpose: To describe a case of incomitant divergence insufficiency esotropia in the setting of Machado-Joseph disease (spinocerebellar ataxia type 3) that recurred completely within one week after augmented bilateral medial rectus recession. Observations: A 53-year-old female with a history of Macha...

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Main Authors: Jeannette Y. Stallworth, Nailyn Rasool, Maanasa Indaram
Format: Article
Language:English
Published: Elsevier 2022-12-01
Series:American Journal of Ophthalmology Case Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S245199362200500X
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author Jeannette Y. Stallworth
Nailyn Rasool
Maanasa Indaram
author_facet Jeannette Y. Stallworth
Nailyn Rasool
Maanasa Indaram
author_sort Jeannette Y. Stallworth
collection DOAJ
description Purpose: To describe a case of incomitant divergence insufficiency esotropia in the setting of Machado-Joseph disease (spinocerebellar ataxia type 3) that recurred completely within one week after augmented bilateral medial rectus recession. Observations: A 53-year-old female with a history of Machado-Joseph disease presented with horizontal diplopia primarily at distance consistent with divergence insufficiency esotropia. Augmented bilateral medial rectus recessions were performed which initially produced orthotropia, but recurrence of the esodeviation to the full preoperative amount occurred by post-operative week one. The patient subsequently underwent bilateral lateral rectus resections with excellent result. Conclusions and importance: Divergence insufficiency is common in the spinocerebellar ataxia variants and is thought to be secondary to atrophy of brainstem structures involved in the control of ocular vergence. Strabismus surgery in these patients may be complicated by limited response or even rapid regression despite augmented surgery as suggested for divergence insufficiency in the setting of neurologic disease. Patients should be counseled on these risks as well as the potential for multiple procedures in order to achieve surgical success.
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spelling doaj.art-c01455fc07494ca0961b739fcfc067b02022-12-22T04:21:50ZengElsevierAmerican Journal of Ophthalmology Case Reports2451-99362022-12-0128101754Recurrent divergence-insufficiency esotropia in Machado-Joseph disease (spinocerebellar ataxia type 3)Jeannette Y. Stallworth0Nailyn Rasool1Maanasa Indaram2Department of Ophthalmology, University of California, San Francisco, United States; Corresponding author. University of California, Department of Ophthalmology, 490 Illinois Street, Floor 5, Room 5X8, San Francisco, CA, 94158, United States.Department of Ophthalmology, University of California, San Francisco, United States; Department of Neurology, University of California, San Francisco, United StatesDepartment of Ophthalmology, University of California, San Francisco, United StatesPurpose: To describe a case of incomitant divergence insufficiency esotropia in the setting of Machado-Joseph disease (spinocerebellar ataxia type 3) that recurred completely within one week after augmented bilateral medial rectus recession. Observations: A 53-year-old female with a history of Machado-Joseph disease presented with horizontal diplopia primarily at distance consistent with divergence insufficiency esotropia. Augmented bilateral medial rectus recessions were performed which initially produced orthotropia, but recurrence of the esodeviation to the full preoperative amount occurred by post-operative week one. The patient subsequently underwent bilateral lateral rectus resections with excellent result. Conclusions and importance: Divergence insufficiency is common in the spinocerebellar ataxia variants and is thought to be secondary to atrophy of brainstem structures involved in the control of ocular vergence. Strabismus surgery in these patients may be complicated by limited response or even rapid regression despite augmented surgery as suggested for divergence insufficiency in the setting of neurologic disease. Patients should be counseled on these risks as well as the potential for multiple procedures in order to achieve surgical success.http://www.sciencedirect.com/science/article/pii/S245199362200500XDivergence-insufficiency esotropiaMachado-Joseph diseaseSpinocerebellar ataxia
spellingShingle Jeannette Y. Stallworth
Nailyn Rasool
Maanasa Indaram
Recurrent divergence-insufficiency esotropia in Machado-Joseph disease (spinocerebellar ataxia type 3)
American Journal of Ophthalmology Case Reports
Divergence-insufficiency esotropia
Machado-Joseph disease
Spinocerebellar ataxia
title Recurrent divergence-insufficiency esotropia in Machado-Joseph disease (spinocerebellar ataxia type 3)
title_full Recurrent divergence-insufficiency esotropia in Machado-Joseph disease (spinocerebellar ataxia type 3)
title_fullStr Recurrent divergence-insufficiency esotropia in Machado-Joseph disease (spinocerebellar ataxia type 3)
title_full_unstemmed Recurrent divergence-insufficiency esotropia in Machado-Joseph disease (spinocerebellar ataxia type 3)
title_short Recurrent divergence-insufficiency esotropia in Machado-Joseph disease (spinocerebellar ataxia type 3)
title_sort recurrent divergence insufficiency esotropia in machado joseph disease spinocerebellar ataxia type 3
topic Divergence-insufficiency esotropia
Machado-Joseph disease
Spinocerebellar ataxia
url http://www.sciencedirect.com/science/article/pii/S245199362200500X
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