Horizontal deviations in congenital brown syndrome

Purpose: To report the incidence of horizontal deviations requiring surgical correction in patients with congenital Brown syndrome. Methods: In a retrospective study, the medical records of all children who underwent a surgical correction of congenital Brown syndrome at Schneider Children's Medical Center of Israel from 1998 to 2016 were reviewed, analyzing the presence of preoperative primary position horizontal misalignment. Results: Overall, 19 eyes (8 right and 11 left eyes) of 16 patients (7 males, 9 females; mean age: 4.2 ± 2.6 years) were included in this study. Fourteen patients (88%) had surgery for correction of a compensatory head position, including 8 patients (50%) with a head tilt and 6 patients (38%) with a chin-up position, and 2 patients had surgery for primary position hypotropia. All of them underwent a weakening procedure of the superior oblique tendon, by either Z-tenectomy (81%, n = 13) or suture elongation of the superior oblique tendon (19%, n = 3). Fifty-six percent of patients (n = 9) had primary position horizontal deviation before surgery, including 50% (n = 8) exodeviations, ranging from exophoria of 4 prism diopters (PD) to exotropia of 30 PD, and one esotropia of 14 PD. Fifty percent of patients (n = 8) had surgery to correct the horizontal deviation by a recession of either one (31%, n = 5) or two (19%, n = 3) muscles. Mean preoperative horizontal deviation (9.3 ± 3.4 PD) decreased significantly following surgery (1.7 ± 1 PD, P = .001) (paired t test). Conclusions: Significant horizontal misalignment is often present in patients with congenital Brown syndrome and its correction should be considered at the time of surgery.