Abstract

Background: We present an extraordinary case of Helveston syndrome exhibiting a trifecta of A-pattern exotropia, DVD, and superior oblique overaction, making its diagnosis and management particularly challenging.
Case Presentation: A 13-yr-old female presented with Alternate outward deviation of eyes since birth. Ocular examination revealed A-pattern exotropia with manifest DVD in right eye, along with superior oblique overaction both eyes and right eye amblyopia.
Management: Conservative management, including occlusion therapy, was attempted initially. followed by surgical intervention involving bilateral superior oblique tenectomy, bilateral lateral rectus resection and right superior rectus recession was performed. Postoperatively, the patient had significantly improved ocular alignment.
Conclusion: Comprehensive evaluation and through tailored surgical strategies can yield favorable outcomes, improving both ocular alignment and quality of life in patients with atypical strabismus.