Small Case Series |

Congenital Bilateral Aplasia of Medial Recti in a Family

Anirudh Singh, MS; Anudeepa Sharma, MBBS; Pradeep Sharma, MD, FAMS
JAMA Ophthalmol. 2013;131(6):798-800. doi:10.1001/jamaophthalmol.2013.2889.
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A 50-year-old male patient from a nonconsanguineous marriage (case 1) was seen with outward deviation of both eyes since birth. He and his 2 sons (case 2, 24 years old, and case 3, 21 years old) had similar concerns of outward deviation of eyes. There was no history of any orthoptic treatment like occlusion, prism, or convergence exercises. There was no other medical or surgical ailment in either the father or the sons. Pedigree analysis revealed no similar ailment in any family member other than the father and his 2 sons; the only other issue was a stillbirth.

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Figure 1. Preoperative and postoperative photographs of case 1. A, Large-angle exodeviation and a prominent forehead (cases 2 and 3 had similar findings). B, Postoperative photograph after bilateral lateral rectus recession and partial vertical rectus transposition.

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Figure 2. Intraoperative views. A, Aplasia of the distal medial rectus muscle with an empty sheath and anterior ciliary vessels alone. B, Partial vertical rectus transposition (white arrows) and Foster suture augmentation (black arrows).

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Figure 3. Magnetic resonance imaging of case 1 showing thin medial rectus muscle (orbital fibers) on both sides (arrows).




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