Surgical management of Helveston syndrome (Triad exotropia)

To evaluate and compare different surgical approaches for the treatment of Helveston syndrome and provide further information for preoperative planning.

From February 2008 to December 2018, data of 52 patients with Helveston syndrome were retrospectively reviewed. Different surgical approaches were selected based on the extent of A-pattern exotropia, dissociated vertical deviation (DVD), and both superior oblique muscle overaction (SOOA) with fundus photograph intorsion. Eye position, A-pattern, DVD, superior oblique muscle function, and binocular vision function were evaluated pre- and postoperatively. The average follow-up duration was 20.5 months.

Nine cases underwent simultaneous horizontal deviation correction with bilateral superior rectus recession, 24 underwent simultaneous horizontal deviation correction with bilateral superior oblique muscle lengthening, and 19 underwent two stages of horizontal deviation correction with superior oblique muscle lengthening, and later bilateral superior rectus recession. A-pattern, DVD, SOOA, and fundus intorsion were all collapsed in all patients postoperatively. Forty-five patients had an orthophoric eye position with considerably aligned ocular movements postoperatively. The total success rate was 86.5%. Postoperatively, eight of the 10 patients with diplopia experienced a recovery of binocular single vision and three had a recovery of rudimentary stereopsis (Titmus 3000-400 s of arc). The compensatory head posture of patients improved significantly postoperatively.

The surgical planning of Helveston syndrome should be designed based on the degree of the A-pattern, SOOA, DVD, and the intorsion in fundus photographs, and the appropriate approach should be selected to improve patient satisfaction.