Infantile (Congenital) Esotropia
Infantile esotropia is defined as an esotropia that is present by 6 months of age. Some ophthalmologists refer to this disorder as congenital esotropia, although the deviation is usually not manifest at birth.
Variable, transient, intermittent strabismus is commonly noted in the first 2–3 months of life. Also, it is common to see both intermittent esotropia and exotropia in the same infant (termed ocular instability of infancy). This condition should resolve by 3 months of age but sometimes persists, especially in premature infants. If an esotropia is present after age 2 months, is constant, and measures 30 prism diopters (Δ) or more, it is unlikely to resolve and will probably require surgical intervention.
Patients with infantile esotropia often have a family history of esotropia or other strabismus, but well-defined genetic patterns are unusual. Infantile esotropia occurs more frequently in children born prematurely and in up to 30% of children with neurologic and developmental problems, including cerebral palsy and hydrocephalus. Infantile esotropia has been associated with an increased risk of development of mental illness by early adulthood (2.6 times higher in patients with infantile esotropia than in controls).
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Olson JH, Louwagie CR, Diehl NN, Mohney BG. Congenital esotropia and the risk of mental illness by early adulthood. Ophthalmology. 2012;119(1):145–149.
Pathogenesis
The cause of infantile esotropia remains unknown. The debate regarding its etiology has focused on the implications of 2 conflicting theories. The Worth “sensory” concept proposes that infantile esotropia results from a congenital deficit in a “fusion center” in the brain. According to this theory, the goal of restoring binocularity is futile. In contrast, the Chavasse theory proposes that the primary problem in infantile esotropia is one of motor development, which is potentially curable if ocular alignment is achieved in infancy. Several authors have reported favorable sensory results in infants operated on between 6 months and 2 years of age, and these encouraging results have become the basis for the practice of early surgery for infantile esotropia.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.