Introduction: Strabismus affects as many as 60% to 70% of patients with cra
niofacial dysostosis. V-pattern strabismus with severe oblique muscle dysfu
nction 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. Methods: We retrospectively reviewed the surgical manage
ment 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 perf
ormed included medial rectus muscle infraplacement (n = 2), inferior obliqu
e (IO) recession (n = 3), IO myectomy (n = 3), IO anterior transposition (n
= 3), and IO denervation/extirpation (n = 5). Results:All patients had sig
nificant residual ocular motility dysfunction postoperatively, No beneficia
l effect was noted after IO anterior transposition or after medial rectus m
uscle infraplacement. Modest improvement of the V-pattern and oblique muscl
e dysfunction was noted after denervation/extirpation and myectomy of the I
O muscle, Bilateral absent or anomalous superior oblique tendons were noted
in 8 of 9 patients in whom the superior oblique tendon was examined at sur
gery, Conclusions: Strabismus in craniofacial dysostosis is complex and dif
ficult to cure with surgery. Denervation/extirpation and myectomy of the IO
muscle offered modest benefits, though neither procedure resulted in norma
lization of ocular motility. Agenesis of the superior oblique tendon may be
causally related in a large proportion of affected patients.