Diseases causing vision loss eg, cataract, retinoblastoma, ptosis Miscellaneous disorders eg, albinism, nystagmus Other strabismus conditions Innervational eg, 3rd nerve palsy, Duane syndrome Mechanical eg, Brown syndrome, strabismus fixus, orbital tumor Systemic Disorders Neurologic disease eg, cerebral palsy, hydrocephalus Craniofacial syndrome Genetic syndrome eg, Prader-Willi syndrome From Table , Kraft SP. Selected Exotropia Entities and Principles of Management. Philadelphia, WB Saunders. Reprinted with permission. Incidence Because of its rarity, there are few population-based studies to identify its true incidence. A study by Archer et al identified that only 2 in 6 per hundred thousand neonates develop true primary infantile exotropia,6 although other researchers estimated this at 1 in 30, births in the general population.

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Infantile esotropia is esotropia that occurs during the first 6 months of life in an otherwise neurologically normal child. The term "congenital esotropia" has been used synonymously but the condition is rarely present at birth.

Transient misalignment of the eyes is common up to the age of 3 months and this should not be confused with infantile esotropia. Etiology The etiology of infantile esotropia is unknown. Many theories have been postulated regarding the pathogenesis of the disease. On the other hand, Chavasse [2] suggested a primary motor dysfunction, where the associated poor fusion and lack of high-grade stereopsis is probably a sensory adaptation to abnormal visual stimulation during early binocular development caused by the motor misalignment.

Thus, surgical correction should be performed early during infancy. This second view was largely accepted afterwards by Costenbader and Parks. There can be a hereditary component with other family members having history of strabismus.

Diagnosis History Detailed history taking regarding birth weight, complications of birth, the health of the child and developmental milestones may help in the management of the case. History of first presentation aided by photographs of the child in the first few months of life can assist in documenting the onset, detecting the stability of the condition and confirming the diagnosis. Physical examination Appropriate diagnosis and management requires full ophthalmologic evaluation with cycloplegia and dilation to examine for other causes of esotropia in young children, including media opacities, refractive error, cranial nerve palsy etc.

Detailed examination of motility and alignment is needed and can be challenging in the young child. The exam should be quick and performed in a fun atmosphere.

This can be done by engaging the patient through the use of colorful toys, whistling and making noises. Corneal light reflex tests Hirshberg and Krismky can be helpful estimating angle of deviation, but cover testing should be employed when possible. Signs Size of the devation As mentioned previously, the angle of deviation is usually larger than 30 PD, and it is constant.

Many infants may freely alternate their fixation. Others may cross fixate using alternate eyes in the opposite field of gaze, i. Accommodative esotropia manifests as eye inturning secondary to the increased work of focusing through significant hypermetropia.

Ocular rotation Some infants may show some limitation of abduction upon initial examination of eye movements as a result of cross fixation. DVD is elevation of the non-fixing eye when covered or with visual inattention.

This is seen as over-elevation of the eye in supra-adduction. It is a predominantly horizontal jerk nystagmus elicited by occluding either eye. The slow phase is toward the side of the occluded eye.

On average, by 2 months of age, the optic nerve completes myelinization. Additional tests may include prism adaptation and diagnostic occlusion. Some signs may give a clue about the sensory development and the prognosis of the condition. Optokinetic response to detect smooth pursuit and saccade eye movements will aid in the diagnosis of amblyopia. Differential diagnosis.


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