Surgical Treatment of V-pattern Exotropia in Crouzon Syndrome.

PURPOSE: Strabismus affects as many as 60% to 70% of patients with Crouzon syndrome. V-pattern strabismus is common and is thought to be secondary to severe oblique muscle dysfunction. The complex motility disorders produced by abnormal extraocular muscles in this syndrome can be difficult to manage. Few studies have reported findings regarding the extraocular muscles or how to plan and adapt the required surgery for this condition.

METHODS: The surgical management and outcomes of four patients with Crouzon syndrome who had undergone craniofacial reconstruction surgery were retrospectively reviewed.

RESULTS: All patients showed significant improvement in the primary eye position, but had residual ocular motility dysfunction postoperatively, especially relating to the up gaze position. During surgery it was noted that the lateral rectus muscle or superior oblique tendons were absent or anomalous in one of the four patients. In two of the patients, there was an absence of both inferior rectus muscles. The extraocular muscles were examined pathologically and revealed evidence of tendon capsule and mild fiber degeneration.

CONCLUSIONS: Strabismus in Crouzon syndrome is complex and the absence of certain extraocular muscles is unpredictable. It is not possible to perform strabismus surgery according to a defined surgical plan; one needs to adapt according to the patient's individual anatomy. Also, the strabismus is difficult to completely correct. However, improving ocular motility in the most functional fields of gaze will benefit the quality of life for both children and their families.