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:: Volume 22, Issue 3 (Iranian Journal of Ophthalmology 2010) ::
2010, 22(3): 53-56 Back to browse issues page
Familial Presentation of Monocular Elevation Deficiency Syndrome due to Hypoplasia and Absence of Superior Rectus
Mohammad Reza Talebnejad Dr. *, Mansure Jamshidian Dr., Mahnaz Mosallaei Dr.
, talebmor@sums.ac.ir
Abstract:   (11024 Views)

Purpose : To report a new familial presentation of hypoplasia and absence of superior rectus in the form of unilateral monocular elevation deficiency

Case report : A 7-year-old boy was referred to our center (Poostchi eye clinic) with a chief complaint of ocular misalignment in his right eye since birth. One of his siblings was a known case of unilateral monocular elevation deficiency and was operated previously. Other family members were normal. Ocular motility examinations revealed more than 60 prism diopters hypotropia of right eye in primary position associated with limitation of elevation in both abduction and adduction. No pattern (A, V, etc.) was detected. There was no sign of craniosynostosis by neurologic exams or by imagings. Unilateral monocular elevation deficiency was diagnosed. Under operation congenital absence of superior rectus was found while in her sister’s chart review, only congenital hypoplasia of superior rectus was reported.

Conclusion : This is the first report of familial presentation of monocular elevation deficiency resulted from both hypoplasia and absence of superior rectus. This finding may suggest the possible role of a genetic mechanism to be responsible for this disorder.

 

Iranian Journal of Ophthalmology 201022(3):53-56 © 2010 by the Iranian Society of Ophthalmology
Keywords: Familial, Congenital, Hypoplasia, Absence, Superior Rectus
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Type of Study: Case report | Subject: General
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Talebnejad M R, Jamshidian M, Mosallaei M. Familial Presentation of Monocular Elevation Deficiency Syndrome due to Hypoplasia and Absence of Superior Rectus. Iranian Journal of Ophthalmology. 2010; 22 (3) :53-56
URL: http://irjo.org/article-1-379-en.html


Volume 22, Issue 3 (Iranian Journal of Ophthalmology 2010) Back to browse issues page
مجله چشم پزشکی ایران Iranian Journal of Ophthalmology
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