Abstract

PurposeTo evaluate and compare different surgical approaches for the treatment of Helveston syndrome and provide further information for preoperative planning.MethodsFrom 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.ResultsNine 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.ConclusionsThe 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.

Highlights

  • Helveston syndrome or triad exotropia was first described by Helveston in 1969; it is characterized by a triad of A-pattern exotropia, dissociated vertical deviation (DVD), and superior oblique overaction (SOOA) [1].The decision of surgical management of this triad is quite challenging; the treatment plan aimed at achieving postoperative ocular movement coordination should consider the possible complications and unpredictable outcomes of the surgery

  • 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

  • We report the surgical outcomes of different surgical methods based on the extent of patients’ manifestations; the degree of A-pattern, SOOA, and DVD

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Summary

Introduction

Helveston syndrome or triad exotropia was first described by Helveston in 1969; it is characterized by a triad of A-pattern exotropia, dissociated vertical deviation (DVD), and superior oblique overaction (SOOA) [1]. The decision of surgical management of this triad is quite challenging; the treatment plan aimed at achieving postoperative ocular movement coordination should consider the possible complications and unpredictable outcomes of the surgery. Existing reports suggest different surgical approaches and variable outcomes of each surgery [2,3,4,5,6,7]. Different surgical approaches were used in managing 52 cases of Helveston syndrome that were reviewed retrospectively. We report our experience of exploring the surgical effects and provide further information for preoperative planning

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