Clinical features and surgical treatment of congenital ocular muscle palsy characterized with double elevator dysfunction

Abstract

To investigate the clinical features of congenital ocular elevator muscle palsy and to evaluate the efficacy of surgical treatment. Pre- and post-operative eye position, superior rectus muscle and inferior oblique muscle function, clinical features were examined and analyzed in 11 patients with congenital double elevator palsy of ocular muscles. When fixated with the nonparetic eye, ten of 11 patients showed hypotropia and pseudoblepharoptosis in the primary position; One of 11 patients fixated with the paretic eye and showed hypotropia in the primary position. All cases were a monocular elevation deficiency in abduction and adduction, and showed pseudoblepharoptosis. In ten patients fixated with the nonparetic eye, two of 10 were undergone inferior rectus recession on paretic eyes; six of 10 were undergone inferior rectus recession and superior oblique tenectomy on paretic eyes; two of 10 were applied inferior rectus recession and superior oblique tenectomy on paretic eyes, superior rectus recession was performed on healthy eyes in six months postoperation. The patient fixated with the paretic -eye were undergone superior rectus recession and inferior oblique tenectomy on healthy eyes. Seven patients with exotropia had lateral rectus muscle surgery performed at the time of their vertical operation. Strabismus were complete corrected in 81.8% (9/11) of patients in our study and 18.2% (2/11) of patients were partial corrected. The functions of superior rectus muscle and inferior oblique muscle were not corrected. Nine patients showed complete correction of the psuedoptosis, one patient showed partial correction of the psuedoptosis, one patient did not showed any improvement of the pseudoptosis. We conclude that superior rectus muscle and inferior oblique muscle insufficiency are the primary factors in the etiology of congenital double ocular elevator palsy. Vertical rectus recession and superior oblique tenectomy on paretic eyes is an effective way of surgical treatment the disease.