19th annual Frank Costenbader Lecture--the origins of congenital esotropia
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1Department of Ophthalmology, Indiana University School of Medicine, Indianapolis.
Insights
Congenital esotropia, a condition present from birth, is best treated with surgery around 4 months of age. Early alignment by 18 months improves outcomes for infantile esotropia patients.
Area of Science:
- Ophthalmology
- Pediatric ophthalmology
- Strabismus research
Background:
- Congenital esotropia is a primary infantile strabismus developing within the first four months of life.
- It is characterized by inward turning of the eyes, unrelated to hyperopia correction, in neurologically normal infants.
- The condition stems from an inborn deficit in the motor fusion mechanism, potentially aggravated by other factors.
Purpose of the Study:
- To outline the diagnostic and therapeutic timeline for congenital esotropia.
- To discuss surgical outcomes and associated motor defects.
- To explore the underlying etiology and future treatment strategies for infantile esotropia.
Main Methods:
- Review of clinical presentation and diagnostic criteria for congenital esotropia.
- Analysis of surgical timing and outcomes, specifically bimedial rectus recession.
- Examination of associated motor anomalies and their relationship to congenital esotropia.
Main Results:
- Surgery is recommended as early as 4 months of age for optimal outcomes.
- Bimedial rectus recession achieves satisfactory alignment in 80-85% of infants in a single procedure.
- Associated motor defects like latent nystagmus and DVD can manifest post-alignment.
Conclusions:
- Early surgical alignment by 18 months is crucial for achieving subnormal binocular vision in congenital esotropia.
- Understanding the etiology is key to developing innovative nonsurgical treatments.
- Congenital esotropia is distinct from secondary strabismus, emphasizing its unique developmental origin.
Abstract:
Congenital esotropia develops in the first 4 months of life in an infant who lacks the inborn mechanism for motor fusion. It manifests as an esotropia which is not eliminated by correction of hyperopia and occurs in an otherwise neurologically normal infant. The earliest practical time for surgery is 4 months of age. The eye is anatomically suited for surgery at this age and also, this is the earliest age that congenital esotropia can be diagnosed with confidence. The best attainable result of treatment of congenital esotropia is subnormal binocular vision. This result is more likely to be attained if infants are aligned by 18 months of age. Satisfactory alignment is produced in 80% to 85% of infants in one procedure with an appropriate bimedial rectus recession. An array of motor defects including DVD, latent nystagmus, oblique dysfunction, and A- and V-pattern appear at varying times after successful alignment. These associated findings are commonly found with, but are not unique to, congenital esotropia. The onset and clinical picture of congenital esotropia is satisfactorily explained by a theory first suggested by Worth that the strabismus is caused by an inborn defect in the motor fusion mechanism and aggravated by esotropital factors as suggested by Chavasse. In contrast to congenital esotropia, all other strabismus can be thought of as occurring on a secondary basis in a person with the inborn capacity for motor fusion, but who failed to maintain it because of conatal insurmountable strabismus (congenital third nerve palsy), who lost it because of acquired (postnatal) strabismus, who uses a strategy such as head posture alteration to retain fusion under favorable circumstances (Duane syndrome), who has intermittent strabismus with part-time suppression (X(T)), or who is maintaining alignment with nonsurgical means (refractive esotropia). For the future, I believe that advances in the management of congenital esotropia will depend on a better understanding of etiology leading to design and use of innovative nonsurgical techniques to discourage convergence and stimulate bifoveal fusion.