Outcome of strabismus surgery in patients of type 3 gaucher’s disease (original) (raw)

Journal of the Egyptian Ophthalmological Society, 2018

Abstract

Purpose Surgical correction of esotropia in patients of neuronopathic Gaucher’s disease (GD) type 3 aiming to improve the cosmetic appearance with the evaluation of the success rate, presence of residual angle, or recurrence during the follow-up period. Study design A retrospective, descriptive study. Patients and methods Ten patients with type 3 neuronopathic GD were referred from the Pediatric Gastroenterology and Neurology Units of Mansoura Children’s Hospital. All patients suffered from systemic, neurological, and ophthalmological manifestations such as hepatosplenomegaly, horizontal gaze palsy and esotropia, respectively. They received enzyme replacement therapy, and showed a stationary course for at least 1 year before strabismus surgery. Patients were admitted and operated for cosmetic correction of esotropia. They were followed up regularly every month for 1 year. Postoperative data were recorded at the end of 1, 3, 6, and 12 months. Results In all, 80% of cases showed improvement of the angle. Orthotropia (within 10 pd) was obtained at the end of 6 months and maintained till the end of 1 year postoperatively; 20% of the cases showed residual esotropia. Conclusion Surgical correction of strabismus in type 3 GD can achieve good cosmetic results with low risk of residual angle or recurrence.

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