ABSTRACT

ABSTRACT: Objective: Surgical management of a newly identified syndrome consisting various forms of developmental anomalies of extraocular muscles innervated by the III. cranial nerve resembling CFEOM and oligodactyly is described. Materials and Methods: Strabismus surgery is performed in four of the six members of the family. Two of the patients had clinical findings of double elevator palsy. On one of these patients an inferior rectus recession combined with superior rectus resection is performed, while the other patient underwent an inferior rectus recession. Superior oblique tenotomy and inferior rectus recession is performed in the third patient with clinical characteristics of Brown syndrome. Moderately affected fourth patient had the clinical presentation of a pseudo-Brown syndrome and exotropia and underwent merely a lateral rectus recession. Results: All of the patients yielded satisfactory clinical results with reduction of the significant misalignment of their eyes. Conclusions: The choice of procedure in CFEOM patients must be individualized according the pre-and peroperative findings.