J Korean Ophthalmol Soc.  2012 Dec;53(12):1904-1909. 10.3341/jkos.2012.53.12.1904.

A Case of Congenital Monocular Elevation Deficiency Associated with Inferior Rectus Anomaly

• Park YL
• Kang NY

• KMID: 2216410
• DOI: http://doi.org/10.3341/jkos.2012.53.12.1904

Abstract

PURPOSE
To report a congenital monocular elevation deficiency (MED) associated with inferior rectus anomaly.
CASE SUMMARY
A 3-year-old, otherwise healthy boy presented with left hypotropia. He showed chin-up and left head turn with left pseudoptosis. On examination, there was a left hypertropia of 60 prism diopters (PD) and esotropia in primary position. Upgaze -4 limitation in abduction and -3 from primary position, poor Bell's phenomenon, and normal vertical saccadic velocity until midline were observed. On orbital CT, thin and nasally displaced left inferior rectus showing enlargement at its posterior segments near the orbital apex was observed. Forced duction test at surgery revealed a strong positive restriction and thin, taut, fibrotic inferior rectus was inserted and displaced nasally.
CONCLUSIONS
Left inferior rectus recession of 5.5 mm with 2.0 mm of temporal transposition improved elevation deficiency, but consecutive exotropia and hypotropia remained. Knapp procedure combined with left lateral rectus recession of 5.5 mm and left medial rectus resection of 4.5 mm, and right superior rectus recession of 9.5 mm were additionally performed. Pseudoptosis and chin elevation resolved but left hypotropia 10PD, and exotropia 6PD remained. Elevation restriction in primary position and abduction were improved but still persisted. This case indicated that MED may accompany congenital inferior rectus anomaly. If severe inferior rectus restriction is observed during the forced duction test, a structural anomaly of the inferior rectus causing motility defects of MED.

Keyword

Inferior rectus anomaly; Monocular elevation deficiency

MeSH Terms

Chin
Esotropia
Exotropia
Head
Orbit
Preschool Child
Strabismus

Figure

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  Figure 1 Preoperative ocular versions showing limited elevation on abduction and primary position in the left eye. Near- normal left inferior oblique rotation can be seen. Note accompanying deep epicanthal fold and pseudoptosis of the left eyelids.

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  Figure 2 Coronal orbital CT showing thickening of the left inferior rectus muscle (arrow) compared to the right inferior rectus muscle near the orbital apex area.

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  Figure 3 Intraoperative findings showing nasally translating left inferior rectus muscle (white lines). The muscle hook is trying to pull the stiff and fibrotic inferior rectus muscle to the midline position.

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  Figure 4 Ocular versions 1week after inferior rectus muscle recession showing improvement of upgaze in the primary position and abduction. But exotropia in the primary position and limitation of infraduction developed.

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  Figure 5 Ocular versions after Knapp procedure and right superior rectus recession. Upgaze limitation on primary position and abduction is improved but left hypotropia and downgaze limitation of the left eye remained.

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