Successful alignment following multiple surgeries in a child with severe esotropia and a congenital cranial dysinnervation disorder

Aim: To report the alignment, visual outcome and surgical strategy for severe bilateral congenital esotropia caused by a congenital cranial dysinnervation disorder (CCDD). This was successfully treated with a combination of interventions that included alternate occlusion, botulinum toxin, and conventional and augmented transposition procedures. Methods: A girl presented at 2 months of age with a marked esotropia and severe bilateral impairment of abduction of both eyes, and an inability to fix with either eye in the primary position. Her investigation and treatment are described. Results: The patient initially underwent alternate daily occlusion to prevent the development of amblyopia, followed by simultaneous bilateral medial rectus (MR) botulinum toxin and large bilateral MR recessions based on pre-operative forced duction testing (FDT). This operation was performed at 1 year of age with the aim of releasing the tight MR and reducing the esotropia. After initial improvement, the impairment of abduction gradually increased over the next month. She subsequently underwent sequential inferior oblique myectomy and augmented transposition procedures, which enabled her to fix in the primary position with either eye and with a small-angle esotropia. Conclusion: Substantial improvement in the alignment of the eyes in a child with a complex congenital esotropia secondary to a CCDD is achievable.