This chapter was reviewed for currency by Joshua Schliesser, MD on November 13, Establishing a diagnosis Definition. Infantile esotropia is esotropia with an onset before the age of six months, with a constant, large angle of strabismus (> 30 PD), no or mild amblyopia, small to moderate hyperopia, latent nystagmus, dissociated vertical deviation, limitation of abduction (although the. Sep 03, · Well-Child Eye Exams Eye exams through TRICARE’s Well-child Care benefit are routine eye exams that may be given every two years between the ages of three and six that also include screening for amblyopia (lazy eye) and strabismus (crossed eyes). These eye exams are provided by an optometrist or ophthalmologist.
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Establishing a diagnosis
The birth prevalence of infantile esotropia has been studied and is reported to range from 1 in live births to as high as 1 in 50 of all newborn children. Except in rare circumstances, large angle, constant esotropia that persists after months of age does not resolve spontaneously. Patients with infantile esotropia are typically neurologically normal, have hyperopia less than 3. Equal visual acuity is normally observed, with amblyopia occurring in less than half of patients.
Nystagmus is also frequently observed and is usually latent type. There appears to be a multifactorial genetic basis for congenital esotropia; however, no specific genes have been identified.
Parks has noted that primary monofixation syndrome occurs 11 times more frequently among first-degree relatives of patients with infantile esotropia than the general population. Accommodative esotropia or refractive esotropia is often acquired after six months of age, intermittent in nature, and patients typically have hyperopia greater than 3.
Treatment is spectacles for the full cycloplegic refraction. Partially accommodative esotropia is similar to accommodative esotropia; patients will have esotropia in which the angle of deviation is less with full cycloplegic refraction.
In these patients, surgery is indicated to correct the remaining deviation. Patients with cranial nerve VI palsy will have an abduction deficit and esotropia of the affected eye. Patients with esotropic Duane syndrome have limited abduction with small-angle esotropia in primary gaze, abnormal face turn, and incomitance. They also have palpebral fissure narrowing of the affected eye on adduction. Patients with restrictive esotropia will frequently have incomitance and an anomalous head posture.
Forced duction testing is helpful in differentiating a restrictive component. Patients with congenital fibrosis of the extraocular muscles will frequently have poor ductions. Most frequently this is an upgaze deficit. Ptosis is also frequently observed. A history of trauma can be useful in differentiating medial wall fracture as a cause of esotropia. Similar to restrictive esotropia, patients will frequently have an anomalous head posture and incomitance.
Forced duction testing can be helpful in confirming the diagnosis in this situation. Sensory esotropia can be seen in patients with poor vision. Infantile esotropic patients will frequently have normal vision in each eye, so any patient with suspected infantile esotropia and poor vision should undergo a complete and thorough eye exam to rule out cause for poor vision.
Patients with nystagmus blockage syndrome will have moderate to large amounts of manifest nystagmus and be orthotropic when inattentive, but will have variable esotropia and mild nystagmus when attentive. Observing the patient for pupillary constriction during the esotropic phase may be helpful in making this diagnosis. The cause of infantile esotropia has been a topic of great debate for the greater part of the last century.
Claude Worth believed that infantile esotropia was a congenital defect in fusion faculty, suggesting that despite correction, patients with infantile esotropia could never achieve good binocular vision. This led to the idea that early surgery for infantile esotropia could potentially result in good binocular vision. Research done by Eileen Birch and David Stager showed that stereopsis emerges abruptly in the first few months of life but degenerates pathologically within a few months in uncorrected esotropes.
Primate studies done by Lawrence Tychsen showed that in monkeys with the constellation deficits that serve as markers of infantile esotropia in humans, there are structural deficits in both superficial and deep layers of the striate cortex. The treatment of infantile esotropia is extraocular muscle surgery. Corrective lenses are often prescribed if the hyperopia is greater than 2. Amblyopia treatment is often reserved for those patients with a strong fixation preference.
Frequently, bilateral medial rectus recession is the initial surgery for infantile esotropia. Alternatively, recession and resection of horizontal eye muscles of the same eye may be preferred if there is amblyopia or an anatomic defect of one eye. The timing of strabismus surgery for infantile esotropia has been greatly debated and studied.
Multiple studies have shown that surgery done before two years of age results in better binocular vision and stereopsis. Patients with infantile esotropia frequently have large deviations and are more likely to have residual esotropia following maximum bilateral medial rectus recessions.
There is some debate about whether three-muscle surgery should be performed at the time of initial operation for large angle esotropia.
Most reoperations for infantile esotropia are for undercorrection. The incidence of overcorrection is low following surgery for infantile esotropia. Sight-threatening complications from strabismus surgery are uncommon. A large proportion of patients with infantile esotropia will exhibit cross-fixation, and in these patients, amblyopia, if present, is frequently only mild.
Typically, occlusion therapy is reserved for patients with a fixation preference. Also known as microtropia, monofixation syndrome is normally seen in patients following strabismus surgery for infantile esotropia. Patients have central suppression with decreased binocular vision. There is presence of binocular peripheral fusion. Latent nystagmus is frequently seen in patients with infantile esotropia. The nystagmus is conjugate and binocular.
Fast phase is directed temporally of the fixating eye and switches with alternation of occlusion. No other forms of nystagmus follow this pattern. Dissociated deviations are seen in patients with decreased binocular vision. The nonfixating eye will drift up, drift out, or extort but will recover with binocular refixation. Dissociated horizontal deviation is treated with lateral rectus recession. Addressing any oblique overaction that exists will help treat the pattern. If there is no oblique dysfunction, shifting the medial rectus muscles at time of recession towards the apex of the pattern will weaken the effective pull of the medial rectus at the apex, decreasing the amount of V or A pattern.
Patients with infantile esotropia frequently have good vision despite reduced binocularity. Earlier surgery is associated with improved binocularity. Treatment of amblyopia, along with multiple surgeries, may be required to achieve the best visual and binocular outcomes.
About Foundation Museum of the Eye. JAN 28, Strabismus: Infantile Esotropia. Cranial nerve VI palsy Patients with cranial nerve VI palsy will have an abduction deficit and esotropia of the affected eye. Duane Syndrome Patients with esotropic Duane syndrome have limited abduction with small-angle esotropia in primary gaze, abnormal face turn, and incomitance.
Restrictive esotropia Patients with restrictive esotropia will frequently have incomitance and an anomalous head posture. Congenital fibrosis of the extraocular muscles Patients with congenital fibrosis of the extraocular muscles will frequently have poor ductions.
Medial wall fracture A history of trauma can be useful in differentiating medial wall fracture as a cause of esotropia. Sensory esotropia Sensory esotropia can be seen in patients with poor vision.
Nystagmus blockage syndrome Patients with nystagmus blockage syndrome will have moderate to large amounts of manifest nystagmus and be orthotropic when inattentive, but will have variable esotropia and mild nystagmus when attentive. Amblyopia treatment Amblyopia treatment is often reserved for those patients with a strong fixation preference.
Surgical therapy options Frequently, bilateral medial rectus recession is the initial surgery for infantile esotropia. Overcorrection The incidence of overcorrection is low following surgery for infantile esotropia. Surgical complications Sight-threatening complications from strabismus surgery are uncommon. Monofixation syndrome Also known as microtropia, monofixation syndrome is normally seen in patients following strabismus surgery for infantile esotropia.
Latent nystagmus Latent nystagmus is frequently seen in patients with infantile esotropia. Prognosis Patients with infantile esotropia frequently have good vision despite reduced binocularity. References Costenbader FD. Infantile esotropia. Trans Am Ophthalmol Soc. Is the incidence of infantile esotropia declining? Arch Ophthalmol. Graham PA. Epidemiology of strabismus. Br J Ophthalmol. The natural history of infantile esotropia during the first six months of life.
Pediatric Eye Disease Investigator Group. Characteristics of infantile esotropia following early bimedial rectus recession. Ing MR. Early surgical alignment for congenital esotropia. Binocular Vision and Ocular Motility. Results of early alignment of congenital esotropia. Plager DA, ed. Ophthalmology Monographs Oxford: Oxford University Press.
J Pediatr Ophthalmol Strabismus. Effect of gestational age and birth weight on the risk of strabismus among premature infants. JAMA Pediatr. Tychsen L.