eMedicine Specialties > Ophthalmology > Extraocular Muscles

Esotropia, Infantile

Vicente Victor D Ocampo, MD, Consulting Staff, Department of Ophthalmology, Asian Hospital and Medical Center, Philippines
C Stephen Foster, MD, FACS, FACR, FAAO, Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution

Updated: Feb 27, 2007

Introduction

Background

Strabismus is one of the most relevant health problems of the world, and infantile esotropia is perhaps the most visually significant yet is the least understood. Infantile esotropia is the inward deviation of the eyes noted before the patient reaches age 6 months. To date, its exact cause has yet to be identified, and an effective treatment strategy is yet to be formulated.

Pathophysiology

The exact cause of infantile esotropia remains unknown. While some opine that esotropia is due to excessive tonic convergence, few agree on what accounts for such conditions. Worth strongly believed that esotropia is an inborn and irreversible defect of fusion. As such, it is a primary dysfunction in the normal development of binocular sensitivity. This was countered by Chavasse who asserted that the neural components necessary for normal binocular vision are present in strabismic individuals at birth, but the development of fusion is eventually impeded by abnormalities of optical input (eg, monocular cataracts) or muscular output (eg, cranial nerve palsies).

The origins of infantile esotropia are just as undefined. A few authors have implicated practically everything from and between the extraocular muscles to the visual cortex in the causation of infantile esotropia.

Although understanding the mechanisms behind infantile esotropia has come a long way, there is still a lot of ground to cover to unearth and clearly understand such an elusive condition.

Frequency

United States

Strabismus is one of the most prevalent ocular problems among children, affecting 5 in every 100 US citizens, or some 12 million people in a population of 245 million. Of this, infantile esotropia is thought to affect about 1% of full-term, healthy newborns and a much higher percentage of newborns with perinatal complications due to prematurity or hypoxic/ischemic encephalopathy.

In an attempt to determine whether esotropia is present at birth or develops later in infancy, Nixon et al observed 1,219 alert infants in a normal newborn nursery at a city hospital and noted that only 40 babies (3.2%) had esotropia (intermittent esotropia in 17 patients, with 14 patients varying between esotropia and exotropia, and 9 patients with variable esotropia). In addition, no infant displayed characteristic features of infantile esotropia. As such, infantile esotropia is not believed to be connatal but rather develops in the first few weeks or months after birth.

Greenberg et al reported an annual age- and gender-adjusted childhood esotropia incidence of 111 per 100,000 patients younger than 19 years. This rate corresponds to a cumulative prevalence of approximately 2% of all children younger than 6 years, with a significant decrease in older ages. The incidence of childhood esotropia from this population-based study is comparable to the prevalence rates among Western populations. Esotropia is most common during the first decade of life, with the accommodative and acquired nonaccommodative forms occurring most frequently.

Mortality/Morbidity

Exotropia in infancy is believed to be associated with an increased prevalence of coexisting neurologic, ocular, and craniofacial abnormalities. To a lesser degree, infantile esotropia also has been associated with a high prevalence of systemic disorders, including prematurity, neurologic, and genetic disorders. Reports of coexisting brain lesions (eg, periventricular leukomalacia, enlargement of the lateral ventricles with hypoplasia of the corpus callosum, myelination delay at the anterior horn adjacent of the lateral ventricles) have been published.

Age

By definition, infantile esotropia is seen in infants before age 6 months.

Clinical

History

Infantile esotropia typically is not present at birth but rather develops in the early months of infancy. Often, the child manifests with chronic inward deviation (esodeviation) of the visual axes at age 2-4 months. This may be preceded by several weeks of transient episodes of misalignment, accounting for the often mentioned history of eyes crossing at birth.

Classic infantile esotropia is constant and involves a large angle of deviation exceeding 20 prism diopters (PD) on corneal light reflex measurement. As a rule, children with constant esotropia of greater than or equal to 40 PD in the first 2-4 months of life rarely resolve spontaneously to orthophoria. In addition, reduction of the angle of deviation below 40 PD is uncommon in these patients.

Children with initially smaller angles of deviation (<40 PD) or variable angle esotropia have a slightly better chance of resolution to orthophoria. However, 3 cases with infantile esotropia whose angle of deviation decreased spontaneously to less than 10 PD over a minimum follow-up period of 37 months eventually were observed to develop late complications of infantile esotropia (eg, bilateral inferior oblique muscle overaction, latent nystagmus, dissociated vertical deviation).

Consequently, children who undergo surgical alignment at age 6 months have a higher prevalence of coarse stereopsis than those who are corrected surgically at age 7-15 months.

• Associated clinical findings
  • Infantile esotropia may be associated with a spectrum of clinical presentations, including amblyopia, impaired binocularity, central scotomas, and incomitance.
  • Amblyopia is relatively common in patients with infantile esotropia. Weakley et al stated that amblyopia should be suspected strongly in patients with esotropia and asymmetric inferior oblique activity, specifically in the eye with more inferior oblique overaction.
  • Most clinical evidence suggests that sensory and motor functions are nearly normal if alignment (within 10 PD of orthophoria) is attained within the first 2 years of life. Virtually all patients with infantile esotropia fail to develop normal binocular vision and stereopsis.
    • Mohindra et al reported that children with infantile esotropia corrected with prisms equal in size to the deviation showed some degree of binocularity up to at least 2.5 years, as measured by a Polaroid bar stereogram procedure with 1800 seconds of arc disparity; however, all older patients (>6 y) with a history of infantile esotropia failed the test.
    • On the other hand, studies have shown that compared to an age-matched population, monocular preferential-looking acuity in infantile esotropes was not significantly different during months 3-14 for the preferred eye and during months 3-8 in the nonpreferred eye. Stereopsis and monocular acuity were noted to be significantly lower in the older esotropic patients. These studies showed that deficits in preferential-looking acuity and subsequent amblyopia occur after the onset of fixation preference and that stereoscopic pathways are present and functional in at least some esotropic infants.
  • Central scotomas almost always are identifiable, even in patients with optimal motor alignment and with the highest levels of binocular vision. On the other hand, it had been stated that the inferonasal quadrant of the visual filed is constricted in patients with infantile esotropia as a result of dissociated vertical deviation. Haefliger et al subsequently refuted this statement.
  • Incomitance also may be observed. The most common type is the V-pattern infantile esotropia, wherein esodeviation is greater in downgaze than in upgaze. V-pattern infantile esotropia is attributed largely to overaction of the inferior obliques. The reverse of this type, the A-pattern, also may be noted in infantile esotropes.

Physical

According to Tychsen, infantile esotropes manifest with a constellation of ocular motor signs, as follows:

• Esotropia, with or without strabismic amblyopia
• Pursuit asymmetry
• Latent fixation nystagmus
• Motion visual-evoked potential (VEP) asymmetry and motion perception abnormalities
• A face turn and abduction deficit
• Dissociated vertical deviation

Causes

Both the exact cause and the associated risk factors of infantile esotropia have yet to be identified distinctively. While it strongly is believed that a genetic component exists, a solid basis for linkages among family members still needs to be established. However, several studies have made significant inroads toward establishing a genetic causation for infantile esotropia. Tychsen and Lisberger reported in 1986 that the strabismic patient who had the most severe pursuit/motion processing asymmetry had 2 siblings with infantile esotropia. Furthermore, large-scale investigations have shown that 20-30% of children born to a strabismic parent eventually will develop strabismus themselves.

The added risks of perinatal complications (eg, prematurity, birth injury, low birth weight) to infantile esotropia have been investigated, yielding equivocal, if not contrary, results.

Differential Diagnoses

Esotropia and Exotropia, A-patterns
Esotropia and Exotropia, V-patterns
Esotropia, Accommodative
Esotropia, Acquired
Esotropia, Pseudo
Esotropia, with High AC/A Ratio

Workup

Procedures

• Perform the alternate prism cover test to accurately measure the angle of strabismus. This procedure gauges the full magnitude of any combined esotropia and esophoria. Carefully perform a prism cover testing for both distance and near fixation in primary position, with the patient wearing any prescribed spectacle correction. Measurement of the strabismus also is carried out in side and vertical gazes to rule out restriction, palsy, and significant oblique muscle overaction or underaction.

Treatment

Medical Care

Smaller angles of deviation may be addressed with prism lenses with or without occlusion therapy, depending on the existence of amblyopia. Perform a good refraction with full cycloplegia on all esotropic infants. A common cycloplegic combination is 2.5% phenylephrine and 1% cyclopentolate. It is necessary to occlude one eye at a time during retinoscopy to make sure that the examiner maintains accurate alignment with the visual axis.

Corrective lenses generally are prescribed with hyperopia greater than +2.50 diopter (D) and/or when anisometropia exceeds 1.50 D. In addition, any cylinder greater than or equal to +0.50 D should be given spectacles. On the other hand, myopia above -4.00 D warrants corrective lenses. Correcting moderate-to-severe hyperopia is performed to eliminate a significant refractive esotropia superimposing upon a preexistent infantile esotropia. Correction of myopia is performed for 2 reasons. First, it is to clear the images seen by the infant so that it increases the chances of accurate fixation and consequently generates more accurate strabismus measurements. Second, minus lenses may alter the accommodative demand and the infant's strabismus angle, particularly when fixating near targets.

If amblyopia is discovered, appropriate occlusion therapy is instituted at once. The rule of thumb observed is 1-2 weeks of high percentage (eg, 90% of waking hours) occlusion of the nonamblyopic eye per year of life, especially if a strong fixation preference for one eye is detected. The infant is reexamined after a few weeks to determine response to therapy and to ensure that occlusion-induced amblyopia has not developed in the occluded dominant eye. If close, frequent follow-up visits will not be possible, then lower percentage occlusion therapy can be initiated. The endpoint of occlusion therapy is to achieve a pattern of freely alternating equal vision.

Botulinum toxin (BOTOX®) injection into the medial rectus has recently been explored as an alternative therapy to surgery. Several studies have investigated the merits of such a procedure with contrasting results. In the work using concurrent bilateral medial rectus BOTOX® injections, McNeer et al noted a decrease in the esotropic angle in 27 patients with infantile esotropia younger than age 12 months, from 43 to 1±2 PD, and in patients younger than age 24 months, from 31±12 to 2±3 delta. Long-term studies up to 95 months postinjection follow-up were conducted by the same authors showing not only a significant reduction in esotropic angle but also successful binocular alignment (±10 PD) in 89% of the patients.

In a separate study by Scott et al, it was noted that 65% of the study patients with infantile esotropia achieved correction of 10 PD or less following BOTOX® injection, with smaller deviations (10-20 PD) more frequently corrected than larger deviations (20-110 PD). No globe perforation, amblyopia, or visual loss was reported as a result of the injections. Tejedor and Rodriguez claimed that BOTOX® injection was a rapid and less invasive alternative to reoperation in children who had been treated unsuccessfully with surgery to correct infantile esotropia.

However, not all studies were convincing of the efficacy of BOTOX® injections. Ing contended that alignment by BOTOX® injections appeared to be less effective in establishing evidence for binocularity than incisional surgery in the treatment of congenital esotropia. On the other hand, while Biglan et al agreed that BOTOX® injections may be useful in the management of patients with recent surgical overcorrections, it was not as successful as traditional strabismus surgery for the treatment of infantile esotropia.

Surgical Care

Infantile esotropia is characterized by large angles of deviation (>40 PD) and customarily is corrected surgically. Tychsen stressed that when the surgeon has documented that the infant has a constant esotropia exceeding 12 PD, surgical realignment should be performed. The surgeon must obtain 2 reasonably high-quality reproducible strabismic measurements, which agree to a range of 5-10 PD, before proceeding with the operation. Over the years, a number of surgical techniques have been developed, but most of them involve bilateral medial rectus recession, wherein the insertion of the muscles into the globe is transferred posteriorly. Variations in technique depend on the total number of muscles initially involved (eg, 2, 3, or 4, wherein lateral rectus resection or shortening also is performed) and the amount of medial rectus recession carried out.

Adjustment of the amount of correction is just as important. Chang et al described a one-stage intraoperative adjustment of strabismus surgery with adjustable sutures to be a simple, well-tolerated, and effective procedure.

A controversial issue is the timing of surgery. While the beneficial effect of accurate alignment by age 2 years has been well established and widely practiced, the earliest age at which surgery should be performed has yet to be defined convincingly. Whether or not to undertake alignment procedures before age 1 year has triggered much debate and vigorous investigations.

Zak and Morin claimed that corrective surgery from age 5-24 months produced successful alignment of the eyes to within 10 PD of orthophoria with a higher prevalence of fusion and stereopsis and a lower prevalence of dissociated vertical deviation (DVD). Furthermore, inferior oblique overaction and amblyopia were less frequent when the initial operation had been performed before age 12 months.

Shirabe et al concurred with such findings but added that it was necessary to confirm a stable angle of deviation with accurate preoperative evaluation and to maintain good postoperative eye alignment throughout the follow-up period to achieve and maintain the binocular visual function resulting from early corrective surgery. Birch et al explained that better stereopsis is achieved with early surgical alignment because the duration of misalignment is shortened and not because alignment is achieved during an early critical period of visual maturation.

The long-term outcome of early surgical correction of infantile esotropes (mean postoperative follow-up period is 14.7±3.7 y) showed that an eventual loss of binocular alignment occurred in some patients but at a much later age, with reduced chances of developing oblique muscle overactions.

Moreover, in a separate study of infantile esotropes who underwent surgical alignment before age 1 year, 3 distinct groups were defined, as follows: (1) those who remained stable following their initial early alignment, (2) those who were well aligned and remained stable for prolonged periods of time and then decompensated, and (3) those who were unstable throughout the observation period.

These findings illustrate the instability of the conditions of patients with infantile esotropia. While apparent benefits with regard to improved binocularity and visual acuity had been demonstrated with early surgical correction of infantile esotropia, a need exists for repeated thorough observations in the first decade of the patient's life.

• Over the years, new surgical modalities have been proposed to address childhood esotropia and concomitant ocular problems in children.
  • Lueder and Norman performed strabismus surgery as an alternative to bifocal glasses in treating accommodative esotropia with favorable results. They concluded that strabismus surgery may eliminate the need for bifocal glasses in patients with accommodative esotropia with a high accommodative convergence/accommodation (AC/A) ratio.
  • Ticho et al noted that simultaneous extraocular muscle and lens surgery is an option for patients with strabismus and lens abnormalities. The authors recommended using standard strabismus surgical amounts. Very few postoperative complications were reported.
  • Godts et al reported that preoperative intermittent or manifest strabismus was not a contraindication for refractive surgery, provided some specific recommendations are taken into account, such as an adequate preoperative orthoptic examination and a goal of emmetropia for both eyes.

Consultations

Consultation with a pediatric ophthalmologist may be indicated.

Activity

Upon fully recovering from general anesthesia, the child is allowed to roam and play freely. Parents can bathe and wash the patient's face without undue concern, especially if a fornix approach was used for the incision. Occlusion therapy is discontinued during the first postoperative week. However, spectacles should be worn during this time.

Medication

Very few medications are used in the treatment of infantile esotropia. Combination antibiotic-steroid ointments are prescribed for the first postoperative week. BOTOX® injection has been used as an alternative to initial or repeat surgical ocular alignment.

Combination antibiotic-steroid ointments

Used in first postoperative week to control any inflammation and to prevent any infection resulting from surgery, particularly in the conjunctiva.

Tobramycin and dexamethasone (TobraDex)

Consist of 0.3% tobramycin and 0.1% dexamethasone. Tobramycin has been found to be active against numerous gram-positive (eg, Staphylococcus aureus, Staphylococcus epidermidis, Streptococcus pneumoniae) and gram-negative organisms (eg, Pseudomonas aeruginosa). Dexamethasone is a potent corticoid.

Dosing

Adult

Apply small portion topically into conjunctival sac 3-4 times/d

Pediatric

Not established

Interactions

Effects of tobramycin decreased when used concurrently with gentamicin

Contraindications

Documented hypersensitivity; epithelial herpes simplex keratitis (dendritic keratitis), vaccinia, varicella, and many other viral diseases of the cornea and conjunctiva; mycobacterial infection of the eye; fungal diseases of ocular structures

Precautions

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Prolonged use of steroid may result in glaucoma and its accompanying complications; development of secondary infection has occurred after use of combinations containing steroids and antimicrobials, particularly fungal infection

Neurotoxins

Botulinum toxin type A (BOTOX®) is most commonly used. Inhibits transmission of nerve impulses in neuromuscular tissue.

Botulinum toxin type A (BOTOX®)

Blocks neuromuscular conduction by binding to receptor sites on motor nerve terminals, entering nerve terminals, and inhibiting the release of acetylcholine. Intended for injection into extraocular muscles. Initial doses administered in 1.25-2.5 U. Use lower doses for smaller deviations and larger doses for larger deviations.

Dosing

Adult

Vertical muscles and horizontal strabismus <20 PD: 1.25-2.5 U in any 1 muscle; subsequent doses for residual or recurrent strabismus
Maximum recommended dose as a single injection for any single muscle is 25 U; subsequent doses for patients experiencing incomplete paralysis of target muscle may be increased up to twice the previous dose; subsequent injections should not be administered until effects of the previous dose have dissipated as evidenced by substantial function in the injected and adjacent muscles
For horizontal strabismus of 20-50 PD: 2.5-5.0 U in any 1 muscle
For persistent VI nerve palsy of 1 mo or longer duration: 1.25-2.5 U in the medial rectus muscle

Pediatric

<12 years: Not established
>12 years: Administer as in adults

Interactions

Effect may be potentiated by aminoglycoside antibiotics or any other drug that interferes with neuromuscular transmission

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Recommended dosages and frequencies of administration should not be exceeded

Follow-up

Further Outpatient Care

• Surgical correction is the first step in the visual rehabilitation of children with infantile esotropia. Patients who are aligned successfully early in life still need careful postoperative monitoring for amblyopia, nystagmus, inferior oblique overactions, dissociated vertical divergence, and accommodative esotropia.
• A follow-up visit usually is scheduled 3-4 months after the initial postoperative consult. At this point, occlusion therapy can be restarted if amblyopia is present. In cases of significant overcorrection or undercorrection, while the patient may be seen earlier, reoperations seldom are performed before the third to fourth month postoperative period. If alignment is optimal (eg, within 8 PD of orthophoria) and acuity is equal in both eyes, subsequent follow-up visits are scheduled every 6 months until age 6 years. At this point, the risk of strabismic amblyopia is decreased, and yearly visits are sufficient. After age 10, consultations are performed on an as-needed basis.
• The infant typically is seen 3-10 days after the surgery. Visual acuity is checked, an afferent pupillary defect is ruled out, and a good red reflex is elicited from both fundi. Furthermore, conjunctival incisions are inspected with a penlight for dehiscence and infection. Most importantly, alignment is assessed, and eye movements are observed for gross underaction and a slipped muscle. If no excessive inflammation is noted, use of antibiotic-corticosteroid ointments may be stopped at this time.

Inpatient & Outpatient Medications

• Aside from the antibiotic-steroid ointment used in the immediate postoperative period, no other medications are needed.

Complications

• Complications of initial surgical correction of infantile esotropia include the following:
  • Marked overcorrection and undercorrection
  • Infection
  • Scleral perforation
  • Foreign body granuloma at the suture site
  • Allergic reaction to suture material
  • Conjunctival inclusion cyst
  • Conjunctival scarring
  • Anterior segment ischemia
  • Change in eyelid position
  • Lost muscle
  • Slipped muscle
  • Oculocardiac reflex

Prognosis

• It is accepted that better ocular alignment and visual prognosis can be achieved if surgical correction is performed before age 2 years. Long-term follow-up studies on esotropic infants who underwent surgical alignment by age 2 years have shown that close to 60% achieve a small angle (<20 PD) cosmetically acceptable strabismus. Although some binocular vision is achieved, it generally is subnormal, often involving peripheral fusion. Factors contributing to poor ocular alignment and visual prognosis include persistent preoperative amblyopia, latent manifest nystagmus, and myopia from -2.5 to 5.0 D.

Patient Education

• Parents and other caregivers must be educated on the various presentations of infantile esotropia to ensure early detection and management.

Miscellaneous

Medicolegal Pitfalls

• Proper diagnosis: Some ocular tumors, particularly intraocular retinoblastoma, may present initially as esodeviation. As such, a careful history and a thorough physical examination, with the pupils fully dilated, must be performed. Furthermore, careful refraction must be performed to determine a refractive component in the patient's esodeviation.
• Timing of surgery: The appropriate age in which to perform surgery remains to be determined.
• Complications of ocular alignment surgery: Complications may lead to further progression of the esotropia and may warrant additional surgery.
• Visual prognostication, especially when amblyopia is present on initial consult: The rehabilitation of the infantile esotrope is a long process, and, at best, the prognosis for adequate binocular vision remains guarded.

References

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Keywords

congenital esotropia, infantile esotropia, essential infantile esotropia, strabismus, inward deviation of eyes

Contributor Information and Disclosures

Author

Vicente Victor D Ocampo, MD, Consulting Staff, Department of Ophthalmology, Asian Hospital and Medical Center, Philippines
Vicente Victor D Ocampo, MD is a member of the following medical societies: American Academy of Ophthalmology and Philippine Ocular Inflammation Society
Disclosure: Nothing to disclose.

Coauthor(s)

C Stephen Foster, MD, FACS, FACR, FAAO, Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution
C Stephen Foster, MD, FACS, FACR, FAAO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Gerhard W Cibis, MD, Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas, Kansas City
Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

J James Rowsey, MD, Former Director of Corneal Services, St Luke's Cataract and Laser Institute, Florida
J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

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