Anomalous Extraocular Muscles with Strabismus

Abstract

SUMMARY: An 8-month-old boy with Gorlin syndrome presented with a large right-face turn and constant exotropia of the left eye. Eight-millimeter recession of the left lateral rectus muscle was performed at 23 months of age without complete postoperative improvement. Orbital imaging revealed bilateral anomalous extraocular muscles inferolateral to the optic nerves. Surgical resection of the tissue confirmed the accessory musculature with postoperative correction of the strabismus. To our knowledge, this appears to be the first reported case in the radiologic literature.

Accessory Lateral Rectus in a Patient With Normal Ocular Motor Control

“…It has been reported in one case of congenital third nerve palsy (2) and in a patient with orbital malformation syndrome (6). As in other patients evaluated with CT (2,6,7) and MRI (3,8,9), the accessory lateral rectus in our patient appeared to run parallel and just medial to the normally sized lateral rectus but was much smaller in size (6,8,9).…”

“…It may have a muscle belly or exist simply as a fibrous band. There is 1 reported case of bilateral, asymmetric accessory lateral rectus muscles in a patient with Gorlin syndrome, a condition attributed to Ptch 1 mutation and disruption of sonic hedgehog signaling (6). The majority of cases of accessory lateral rectus are unilateral (8).…”

“…Supernumerary extraocular muscles should be considered in cases of unusual strabismus patterns (5,9) or when strabismus surgery does not achieve the expected result, because release of accessory muscle is sometimes necessary as part of the surgical procedure (2,6,7,11,12). Supernumerary extraocular muscles should be considered in cases of unusual strabismus patterns (5,9) or when strabismus surgery does not achieve the expected result, because release of accessory muscle is sometimes necessary as part of the surgical procedure (2,6,7,11,12).…”

Accessory eye muscle in a young boy with external ophthalmoplegia

“…In this study, we described a unilateral atavistic retractor bulbi muscle in a young patient. Accessory eye muscles are very rare in human beings and are described only in few publications and published case reports (e.g., von Lüdinghausen et al,1999; Lueder,2002; Dobbs et al,2010). These reports mostly describe the clinical symptoms of the patients.…”

“…Moreover, attendant syndromic conditions are reported in some patients. Dobbs et al (2010) presented an eight‐month‐old boy with a history of Gorlin syndrome also called basal cell nevus syndrome. This syndrome is an autosomal dominant inherited disease, which can cause unusual facial appearances and predispose the patient to basal cell carcinoma.…”