Minimal invasive vertical muscle transposition for the treatment of large angle exotropia due to congenital medial rectus hypoplasia: Case Report and Literature Review.

Reports of isolated anomalies of the medial rectus (MR) muscle in literature are sparse. It has been identified as a subtype of congenital cranial dysinnervation disorder that affects the normal development of brainstem motor neurons. Herein, we report a 37-year-old male presented with large-angle exotropia since the birth of right eye with palpebral fissure widening. On examination of ocular movements, there was -6 limitation of adduction. There was no limitation in other ocular movements. In the preoperative CT scan, all extraocular muscles were present. He underwent surgery in right eye. Intraoperatively in the site of medial rectus, we found an empty sheath without muscle fibers indicating medial rectus hypoplasia. The width of muscle insertion was normal. Surgery consisted of lateral rectus muscle recession 10 mm in hang-back method and vertical muscle transposition procedure, by a modification of Nishida technique, in which the vector of superior and inferior recti was transposed medially by inserting non-absorbable sutures at nasal margins of muscles secured to sclera 8 mm posterior to medial rectus site without tenotomy or splitting. The deviation was decreased to less than 10 PD exotropia in primary position. The adduction was improved from -6 to -4. The palpebral fissure asymmetry was also corrected. Here, we also reviewed clinical features of all cases of medial rectus hypoplasia/aplasia in the literature and discussed surgical approaches. For vertical rectus transposition and horizontal muscle weakening, this technique has the advantages of being simpler and less traumatic to ocular tissues and unlike the traditional transposition procedures, there is no need for tenotomy and splitting.