Surgical management of V-pattern strabismus and oblique dysfunction in craniofacial dysostosis

Strabismus affects as many as 60% to 70% of patients with craniofacial dysostosis. V-pattern strabismus with severe oblique muscle dysfunction is the most common ocular motility problem seen and can be difficult to manage. Few studies have reported on the results of strabismus surgery in this condition.

We retrospectively reviewed the surgical management and outcomes of 14 patients with craniofacial dysostosis who underwent 16 operations to determine the optimal surgical procedure and to report on extraocular muscle anomalies noted at the time of surgery. Operations performed included medial rectus muscle infraplacement (n = 2), inferior oblique (IO) recession (n = 3), IO myectomy (n = 3), IO anterior transposition (n = 3), and IO denervation/extirpation (n = 5).

All patients had significant residual ocular motility dysfunction postoperatively. No beneficial effect was noted after IO anterior transposition or after medial rectus muscle infraplacement. Modest improvement of the V-pattern and oblique muscle dysfunction was noted after denervation/extirpation and myectomy of the IO muscle. Bilateral absent or anomalous superior oblique tendons were noted in 8 of 9 patients in whom the superior oblique tendon was examined at surgery.

Strabismus in craniofacial dysostosis is complex and difficult to cure with surgery. Denervation/extirpation and myectomy of the IO muscle offered modest benefits, though neither procedure resulted in normalization of ocular motility. Agenesis of the superior oblique tendon may be causally related in a large proportion of affected patients.