Infantile esotropia

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Infantile esotropia
Specialty Ophthalmology

Infantile esotropia is an ocular condition of early onset in which one or either eye turns inward. It is a specific sub-type of esotropia and has been a subject of much debate amongst ophthalmologists with regard to its naming, diagnostic features, and treatment.

Contents

Presentation

Historically the term 'congenital strabismus' was used to describe constant esotropias with onset between birth and six months of age. However, this term was felt to be an inadequate classification as it covered a variety of esotropias with different causes, features and prognoses.

In 1988, American ophthalmologist Gunter K. Von Noorden discussed what he described as 'Essential Infantile Esotropia'. [1] He described the condition as: "early acquired, not... congenital ..., although congenital factors may favor its development between the ages of 3 and 6 months".

He identified this squint sub-type as having the following features:

  1. Onset between birth and six months of age.
  2. Large size (greater than 30 dioptres)
  3. Stable size
  4. Not associated with abnormalities of the central nervous system.
  5. Often associated with defective abduction (outward movement) and excessive adduction (inward movement) of the eyes.
  6. Also associated with oblique muscle dysfunction and Dissociated Vertical Deviation.
  7. Initial alternation of the squint present with crossed fixation, i.e. the affected individual uses the left eye to look right and the right eye to look left.
  8. Limited potential for binocular vision.

The same condition had also previously been described by other ophthalmologists, notably Cianca (1962) who named it Cianca's Syndrome and noted the presence of manifest latent nystagmus, and Lang (1968) who called it Congenital Esotropia Syndrome and noted the presence of abnormal head postures. In both cases, however, the essential characteristics were the same, but with emphasis placed on different elements of the condition.

Helveston [2] (1993) further clarified and expanded upon von Noorden's work, and incorporated the work of both Lang and Cianca into his summary of the characteristics of the condition:

  1. Esotropia between 10 and 90 dioptres in size
  2. Either alternation or fixation preference may be present (if the latter then amblyopia may result).
  3. Neurologically normal.
  4. Hyperopic correction does not eliminate or significantly reduce the squint size.
  5. Frequent nystagmus (latent or manifest latent).
  6. The patient may or may not have any or all of the following associated conditions: Oblique muscle dysfunction, vertical incomitance, dissociated vertical deviation, asymmetric optokinetic nystagmus, torticollis.
  7. Presence will be 'confirmed' by six months.
  8. Best treatment results in subnormal binocular vision.

The expressions congenital esotropia, infantile esoptropia, idiopathic infantile esotropia and essential infantile esotropia are often used interchangeably.

Cross-fixation

Cross-fixation congenital esotropia, also called Cianci's syndrome is a particular type of large-angle infantile esotropia associated with tight medius rectus muscles. With the tight muscles, which hinder adduction, there is a constant inward eye turn. The patient cross-fixates, that is, to fixate objects on the left, the patient looks across the nose with the right eye, and vice versa. The patient tends to adopt a head turn, turning the head to the right to better see objects in the left visual field and turning the head to the left to see those in the right visual field. Binasal occlusion can be used to discourage cross-fixation. [3] However, the management of cross-fixation congenital esotropia usually involves surgery. [4]

Cause

This remains undetermined at the present time. A recent study by Major et al. [5] reports that:

Prematurity, family history or secondary ocular history, perinatal or gestational complications, systemic disorders, use of supplemental oxygen as a neonate, use of systemic medications, and male sex were found to be significant risk factors for infantile esotropia.

Further recent evidence indicates that a cause for infantile strabismus may lie with the input that is provided to the visual cortex. [6] In particular, neonates who suffer injuries that, directly or indirectly, perturb binocular inputs into the primary visual cortex (V1) have a far higher risk of developing strabismus than other infants. [7]

Diagnosis

Differential diagnosis

Clinically Infantile esotropia must be distinguished from:

  1. VIth Cranial nerve or abducens palsy
  2. Nystagmus Blockage Syndrome
  3. Esotropia arising secondary to central nervous system abnormalities (in cerebral palsy for example)
  4. Primary Constant esotropia
  5. Duane's Syndrome

Treatment

According to a Cochrane review of 2023, controversies remain regarding type of surgery, non-surgical intervention and age of intervention and that more good studies are needed to guide clinical practice. [8]

The aims of treatment are as follows:

Initially

It is essential that a child with strabismus is presented to the ophthalmologist as early as possible for diagnosis and treatment in order to allow best possible monocular and binocular vision to develop. Initially, the patient will have a full eye examination to identify any associated pathology, and any glasses required to optimise acuity will be prescribed – although infantile esotropia is not typically associated with refractive error. Studies have found that approximately 15% of infantile esotropia patients have accommodative esotropia. For these patients, antiaccommodative therapy (with spectacles) is indicated before any surgery as antiaccommodative therapy fully corrects their esotropia in many cases and significantly decreases their deviation angle in others. [9] [10]

Amblyopia will be treated via occlusion treatment (using patching or atropine drops) of the non-squinting eye with the aim of achieving full alternation of fixation. Management thereafter will be surgical. As alternative to surgery, also botulinum toxin therapy has been used in children with infantile esotropia. [11] [12] [13] Furthermore, as accompaniment to ophthalmologic treatment, craniosacral therapy may be performed in order to relieve tension [14] [15] (see also: Management of strabismus).

Surgery

Controversy has arisen regarding the selection and planning of surgical procedures, the timing of surgery and about what constitutes a favourable outcome.

1. Selection and planning

Some ophthalmologists, notably Ing [16] and Helveston, [17] favour a prescribed approach often involving multiple surgical episodes whereas others prefer to aim for full alignment of the eyes in one procedure and let the number of muscles operated upon during this procedure be determined by the size of the squint.

2. Timing and outcome

This debate relates to the technical anatomical difficulties of operating on the very young versus the possibility of an increased potential for binocularity associated with early surgery. Infants are often operated upon at the age of six to nine months of age [18] and in some cases even earlier at three or four months of age. [19] Some emphasize the importance of intervening early such as to keep the duration of the patient's abnormal visual experience to a minimum. [20] Advocates of early surgery believe that those who have their surgery before the age of one are more likely to be able to use both eyes together post-operatively.

A Dutch study (ELISSS) [21] compared early with late surgery in a prospective, controlled, non-randomized, multicenter trial and reported that:

Children operated early had better gross stereopsis at age six as compared to children operated late. They had been operated more frequently, however, and a substantial number of children in both [originally-recruited] groups had not been operated at all.

Other studies also report better results with early surgery, notably Birch and Stager [22] and Murray et al. [23] but do not comment on the number of operations undertaken. A recent study on 38 children concluded that surgery for infantile esotropia is most likely to result in measureable stereopsis if patient age at alignment is not more than 16 months. [24] Another study found that for children with infantile esotropia early surgery decreases the risk of dissociated vertical deviation developing after surgery. [25]

Aside the strabismus itself, there are other aspects or conditions that appear to improve after surgery or botulinum toxin eye alignment. Study outcomes have indicated that after surgery the child catches up in development of fine-motor skills (such as grasping a toy and handling a bottle) and of large-muscle skills (such as sitting, standing, and walking) in case a developmental delay was present before. [26] Evidence also indicates that as of the age of six, strabismic children become less accepted by their peers, leaving them potentially exposed to social exclusion starting at this age unless their eye positioning is corrected by this time [27] (see also: Psychosocial effects of strabismus).

Related Research Articles

<span class="mw-page-title-main">Esotropia</span> Form of strabismus

Esotropia is a form of strabismus in which one or both eyes turns inward. The condition can be constantly present, or occur intermittently, and can give the affected individual a "cross-eyed" appearance. It is the opposite of exotropia and usually involves more severe axis deviation than esophoria. Esotropia is sometimes erroneously called "lazy eye", which describes the condition of amblyopia; a reduction in vision of one or both eyes that is not the result of any pathology of the eye and cannot be resolved by the use of corrective lenses. Amblyopia can, however, arise as a result of esotropia occurring in childhood: In order to relieve symptoms of diplopia or double vision, the child's brain will ignore or "suppress" the image from the esotropic eye, which when allowed to continue untreated will lead to the development of amblyopia. Treatment options for esotropia include glasses to correct refractive errors, the use of prisms and/or orthoptic exercises and/or eye muscle surgery. The term is from Greek eso meaning "inward" and trope meaning "a turning".

Orthoptics is a profession allied to the eye care profession. Orthoptists are the experts in diagnosing and treating defects in eye movements and problems with how the eyes work together, called binocular vision. These can be caused by issues with the muscles around the eyes or defects in the nerves enabling the brain to communicate with the eyes. Orthoptists are responsible for the diagnosis and non-surgical management of strabismus (cross-eyed), amblyopia and eye movement disorders. The word orthoptics comes from the Greek words ὀρθός orthos, "straight" and ὀπτικός optikοs, "relating to sight" and much of the practice of orthoptists concerns disorders of binocular vision and defects of eye movement. Orthoptists are trained professionals who specialize in orthoptic treatment, such as eye patches, eye exercises, prisms or glasses. They commonly work with paediatric patients and also adult patients with neurological conditions such as stroke, brain tumours or multiple sclerosis. With specific training, in some countries orthoptists may be involved in monitoring of some forms of eye disease, such as glaucoma, cataract screening and diabetic retinopathy

<span class="mw-page-title-main">Strabismus</span> Eyes not aligning when looking at something

Strabismus is a vision disorder in which the eyes do not properly align with each other when looking at an object. The eye that is focused on an object can alternate. The condition may be present occasionally or constantly. If present during a large part of childhood, it may result in amblyopia, or lazy eyes, and loss of depth perception. If onset is during adulthood, it is more likely to result in double vision.

Anisometropia refers to a condition when two eyes have unequal refractive power. Generally, a difference in power of one diopter (1D) or more is the accepted threshold to label the condition anisometropia. Patients can tolerate 3 D of anisometropia before it becomes clinically symptomatic with headaches, asthenopia, double vision and photophobia.

Pediatric ophthalmology is a sub-speciality of ophthalmology concerned with eye diseases, visual development, and vision care in children.

<span class="mw-page-title-main">Duane syndrome</span> Rare congenital disease characterized by external gaze palsy

Duane syndrome is a congenital rare type of strabismus most commonly characterized by the inability of the eye to move outward. The syndrome was first described by ophthalmologists Jakob Stilling (1887) and Siegmund Türk (1896), and subsequently named after Alexander Duane, who discussed the disorder in more detail in 1905.

<span class="mw-page-title-main">Exotropia</span> Visual disorder where eyes work independently

Exotropia is a form of strabismus where the eyes are deviated outward. It is the opposite of esotropia and usually involves more severe axis deviation than exophoria. People with exotropia often experience crossed diplopia. Intermittent exotropia is a fairly common condition. "Sensory exotropia" occurs in the presence of poor vision in one eye. Infantile exotropia is seen during the first year of life, and is less common than "essential exotropia" which usually becomes apparent several years later.

<span class="mw-page-title-main">Sixth nerve palsy</span> Medical condition

Sixth nerve palsy, or abducens nerve palsy, is a disorder associated with dysfunction of cranial nerve VI, which is responsible for causing contraction of the lateral rectus muscle to abduct the eye. The inability of an eye to turn outward, results in a convergent strabismus or esotropia of which the primary symptom is diplopia in which the two images appear side-by-side. Thus, the diplopia is horizontal and worse in the distance. Diplopia is also increased on looking to the affected side and is partly caused by overaction of the medial rectus on the unaffected side as it tries to provide the extra innervation to the affected lateral rectus. These two muscles are synergists or "yoke muscles" as both attempt to move the eye over to the left or right. The condition is commonly unilateral but can also occur bilaterally.

<span class="mw-page-title-main">Congenital fourth nerve palsy</span> Medical condition

Congenital fourth nerve palsy is a condition present at birth characterized by a vertical misalignment of the eyes due to a weakness or paralysis of the superior oblique muscle.

<span class="mw-page-title-main">Hypertropia</span> Condition of misalignment of the eyes

Hypertropia is a condition of misalignment of the eyes (strabismus), whereby the visual axis of one eye is higher than the fellow fixating eye. Hypotropia is the similar condition, focus being on the eye with the visual axis lower than the fellow fixating eye. Dissociated vertical deviation is a special type of hypertropia leading to slow upward drift of one or rarely both eyes, usually when the patient is inattentive.

<span class="mw-page-title-main">Strabismus surgery</span> Surgery to correct strabismus

Strabismus surgery is surgery on the extraocular muscles to correct strabismus, the misalignment of the eyes. Strabismus surgery is a one-day procedure that is usually performed under general anesthesia most commonly by either a neuro- or pediatric ophthalmologist. The patient spends only a few hours in the hospital with minimal preoperative preparation. After surgery, the patient should expect soreness and redness but is generally free to return home.

Dissociated vertical deviation (DVD) is an eye condition which occurs in association with a squint, typically infantile esotropia. The exact cause is unknown, although it is logical to assume it is from faulty innervation of eye muscles.

<span class="mw-page-title-main">Nystagmus</span> Dysfunction of eye movement

Nystagmus is a condition of involuntary eye movement, sometimes informally called "dancing eyes". People can be born with it but more commonly acquire it in infancy or later in life. In many cases it may result in reduced or limited vision.

Monofixation syndrome (MFS) is an eye condition defined by less-than-perfect binocular vision. It is defined by a small angle deviation with suppression of the deviated eye and the presence of binocular peripheral fusion. That is, MFS implies peripheral fusion without central fusion.

<span class="mw-page-title-main">Stereopsis recovery</span>

Stereopsis recovery, also recovery from stereoblindness, is the phenomenon of a stereoblind person gaining partial or full ability of stereo vision (stereopsis).

Botulinum toxin therapy of strabismus is a medical technique used sometimes in the management of strabismus, in which botulinum toxin is injected into selected extraocular muscles in order to reduce the misalignment of the eyes. The injection of the toxin to treat strabismus, reported upon in 1981, is considered to be the first ever use of botulinum toxin for therapeutic purposes. Today, the injection of botulinum toxin into the muscles that surround the eyes is one of the available options in the management of strabismus. Other options for strabismus management are vision therapy and occlusion therapy, corrective glasses and prism glasses, and strabismus surgery.

In ophthalmology, horror fusionis is a condition in which the eyes have an unsteady deviation, with the extraocular muscles performing spasm-like movements that continuously shift the eyes away from the position in which they would be directed to the same point in space, giving rise to diplopia. Even when the double vision images are made to nearly overlap using optical means such as prisms, the irregular movements prevent binocular fusion. The name horror fusionis arises from the notion that the brain is, or at least appears to be, actively preventing binocular fusion.

The management of strabismus may include the use of drugs or surgery to correct the strabismus. Agents used include paralytic agents such as botox used on extraocular muscles, topical autonomic nervous system agents to alter the refractive index in the eyes, and agents that act in the central nervous system to correct amblyopia.

Daniel Mojon is a Swiss ophthalmologist and ophthalmic surgeon who is considered to be the inventor of minimally invasive strabismus surgery (MISS), a method of surgically correcting squinting that uses only very small incisions of two to three millimeters and is supposed to lead to quicker rehabilitation and wound healing. Daniel Mojon is president of the program committee of the Swiss Academy of Ophthalmology (SAoO).

Alan Brown Scott was an American ophthalmologist specializing in eye muscles and their disorders, such as strabismus. He is best known for his work in developing and manufacturing the drug that became known as Botox, research described as "groundbreaking" by the ASCRS.

References

  1. ↑ von Noorden G.k.; A Reassessment of Infantile esotropia. XLIV Edward jackson Memorial lecture; American Journal of Ophthalmology, 1988; 105:1-10
  2. ↑ Helveston EM (1993). "19th annual Frank Costenbader Lecture--the origins of congenital esotropia". Journal of Pediatric Ophthalmology and Strabismus. 30 (4): 215–32. doi:10.3928/0191-3913-19930701-03. PMID   8410573.
  3. ↑ Marc B. Taub; Mary Bartuccio; Dominick Maino (26 September 2012). Visual Diagnosis and Care of the Patient with Special Needs. Lippincott Williams & Wilkins. p. 303. ISBN   978-1-4511-7834-0.
  4. ↑ Kenneth W. Wright; Yi Ning J. Strube (19 November 2014). Color Atlas Of Strabismus Surgery: Strategies and Techniques. Springer. pp. 16–17. ISBN   978-1-4939-1480-7.
  5. ↑ Major, Archima; Maples, W.C.; Toomey, Shannon; DeRosier, Wesley; Gahn, David (2007). "Variables associated with the incidence of infantile esotropia". Optometry - Journal of the American Optometric Association. 78 (10): 534–541. doi:10.1016/j.optm.2006.11.017. ISSN   1529-1839. PMID   17904494.
  6. ↑ Lawrence Tychsen (2012). "The Cause of Infantile Strabismus Lies Upstairs in the Cerebral Cortex, Not Downstairs in the Brainstem". Archives of Ophthalmology. 130 (8): 1060–1061. doi:10.1001/archophthalmol.2012.1481. PMID   22893080.
  7. ↑ Tychsen L (2007). "Causing and curing infantile esotropia in primates: the role of decorrelated binocular input (an American Ophthalmological Society thesis)". Transactions of the American Ophthalmological Society. 105: 564–93. PMC   2258131 . PMID   18427630.
  8. ↑ Mehner, Lauren; Ng, Sueko M.; Singh, Jasleen (2023-01-16). "Interventions for infantile esotropia". The Cochrane Database of Systematic Reviews. 1 (1): CD004917. doi:10.1002/14651858.CD004917.pub4. ISSN   1469-493X. PMC   9841886 . PMID   36645238.
  9. ↑ S.A. Havertape; C.R. Whitfill; O.A. Cruz (1999). "Early-onset accommodative esotropia". Journal of Pediatric Ophthalmology and Strabismus. 36 (2): 69–73. doi:10.3928/0191-3913-19990301-05. PMID   10204132.
  10. ↑ F. Koç; H. Ozal; E. Firat (August 2003). "Is it possible to differentiate early-onset accommodative esotropia from early-onset essential esotropia?". Eye (Lond). 17 (6): 707–710. doi:10.1038/sj.eye.6700483. PMID   12928681. S2CID   8045841.
  11. ↑ Thouvenin D, Lesage-Beaudon C, Arné JL (January 2008). "(translated from French) Botulinum injection in infantile strabismus. Results and incidence on secondary surgery in a long-term survey of 74 cases treated before 36 months of age". J Fr Ophtalmol. 31 (1): 42–50. doi:10.1016/S0181-5512(08)70329-2. PMID   18401298 . Retrieved January 3, 2019.
  12. ↑ de Alba Campomanes AG, Binenbaum G, Campomanes Eguiarte G (April 2010). "Comparison of botulinum toxin with surgery as primary treatment for infantile esotropia". J AAPOS. 14 (2): 111–116. doi:10.1016/j.jaapos.2009.12.162. PMID   20451851.
  13. ↑ Gursoy, Huseyin; Basmak, Hikmet; Sahin, Afsun; Yildirim, Nilgun; Aydin, Yasemin; Colak, Ertugrul (2012). "Long-term follow-up of bilateral botulinum toxin injections versus bilateral recessions of the medial rectus muscles for treatment of infantile esotropia". Journal of American Association for Pediatric Ophthalmology and Strabismus. 16 (3): 269–273. doi:10.1016/j.jaapos.2012.01.010. ISSN   1091-8531. PMID   22681945.
  14. ↑ Clinical Application of Neuromuscular Techniques: Practical Case Study Exercises. Elsevier Health Sciences. 2005. p. 48. ISBN   978-0-443-10000-0.
  15. ↑ Leon Chaitow (2005). Cranial Manipulation: Theory and Practice : Osseous and Soft Tissue Approaches. Elsevier Health Sciences. p. 9. ISBN   978-0-443-07449-3.
  16. ↑ Ing, M.R. (February 1983). "Early Surgical Alignment for Congenital Esotropia". Ophthalmology. 90 (2): 132–135. doi:10.1016/s0161-6420(83)34586-3. PMC   1312199 . PMID   6856250.
  17. ↑ Helveston, E.M.; Ellis, F.D.; Patterson, J.H.; Weber, J. (May 1978). "Augmented Recession of the Medial Recti". Ophthalmology. 85 (5): 507–511. doi:10.1016/s0161-6420(78)35647-5. PMID   673330.
  18. ↑ Esotropia (Crossed Eyes), Pediatric Ophthalmic Consultants
  19. ↑ Experts discuss infantile esotropia, airbag injuries and timing of surgery, Ocular Surgery News U.S. Edition, June 1, 2002 (downloaded 3 October 2013)
  20. ↑ Birch EE, Wang J (2009). "Stereoacuity outcomes after treatment of infantile and accommodative esotropia". Optometry and Vision Science (Review). 86 (6): 647–52. doi:10.1097/OPX.0b013e3181a6168d. PMC   2769257 . PMID   19390468.
  21. ↑ Simonsz HJ; Kolling GH; Unnebrink K. (December 2005). "Final report of the early vs. late infantile strabismus surgery study (ELISSS), a controlled, prospective, multicenter study". Strabismus. Vol. 4, no. 13. pp. 169–199. PMID   16361188.
  22. ↑ Birch EE, Stager DR Sr.Long-term motor and sensory outcomes after early surgery for infantile esotropia. J AAPOS. 2006 Oct;10(5):409-13
  23. ↑ Murray AD, Orpen J, Calcutt C. Changes in the functional binocular status of older children and adults with previously untreated infantile esotropia following late surgical realignment. J AAPOS. 2007 Apr;11(2):125-30. Epub 2007 Feb 15
  24. ↑ Eren Çerman; Muhsin Eraslan; Mehdi S. Öğüt (2014). "The relationship of age when motor alignment is achieved and the subsequent development of stereopsis in infantile esotropia". Journal of American Association for Pediatric Ophthalmology and Strabismus. 18 (3): 222–225. doi:10.1016/j.jaapos.2013.12.017. PMID   24924272.
  25. ↑ Arslan U, Atilla H, Erkam N (June 2010). "Dissociated vertical deviation and its relationship with time and type of surgery in infantile esotropia". The British Journal of Ophthalmology. 94 (6): 740–2. doi:10.1136/bjo.2008.157016. PMID   20508049. S2CID   9272386.
  26. ↑ Babies' Development 'Catches Up' After Surgery To Fix Crossed Eyes, sciencedaily.com, 21 April 2008 (downloaded 4 October 2013)
  27. ↑ Six-Year-Olds With Squint Less Likely to Be Invited to Birthday Parties, Study Suggests, sciencedaily.com, 19 August 2010 (downloaded 4 October 2013)
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