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 Table of Contents  
ORIGINAL ARTICLE
Year : 2019  |  Volume : 31  |  Issue : 1  |  Page : 33-38

Acute acquired comitant esotropia in children: A benign entity or an ominous sign?


1 Senior Consultant and Head, Strabismus, Peadiatric Ophthalmology and Neuro-Ophthalmology Services, Giridhar Eye Institute, Kochi, Kerala, India
2 Medical Director, Giridhar Eye Institute, Kochi, Kerala, India

Date of Web Publication15-Apr-2019

Correspondence Address:
R Neena
Strabismus, Paediatric Ophthalmology and Neuro-Ophthalmology Services, Giridhar Eye Institute, Kochi - 682 020, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kjo.kjo_11_19

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  Abstract 

Purpose: The purpose is to study the clinical features of acute acquired comitant esotropia (AACE) in children and analyze the course and outcome. Materials and Methods: A retrospective, clinical study of all patients under the age of 18 years with acute onset, nonaccommodative comitant esotropia in a tertiary eye care center from September 2013 to December 2016. Parameters studied were age, sex, visual acuity, eye involved, age of onset, precipitating event, amount of deviation, presence or absence of amblyopia, cycloplegic refraction, systemic involvement, surgical or nonsurgical intervention, course, and outcome. All patients underwent magnetic resonance imaging of brain and orbits, and systemic evaluation was done in suspicious cases. Minimum follow-up period was 6 months. Results: Out of 12 patients, 8 were male. Average age of the patients was 7.9 years. The mean esodeviation was 33.75 PD, and mean age of onset was 6.14 years (range: 2–15 years). There was left eye preponderance. Three patients (25%) had a history of a precipitating event. Cycloplegic refraction ranged from +0.50 DS to −6.75 DS, with majority having <+2.00 DS. Amblyopia was noted in 8 (66.6%) patients. Systemic involvement was seen in three patients in the form of ocular myasthenia, central nervous system glioma, and viral fever, respectively. Esotropia disappeared in one patient with overcorrected, high myopia following atropinization, and change of glass. Eight patients (66.6%) underwent strabismus surgery with good postoperative alignment, and the rest were managed conservatively. Conclusion: AACE is an unusual ocular alignment disorder characterized by a nonaccommodative esodeviation which can occur in older children, adults, and even the elderly. Although most children with this form of esotropia are otherwise healthy, systemic diseases must be considered and ruled out before planning surgery. Prompt amblyopia therapy and timely surgery can result in a satisfactory outcome in those without systemic involvement.

Keywords: Acute acquired comitant esotropia, amblyopia, esotropia


How to cite this article:
Neena R, Giridhar A. Acute acquired comitant esotropia in children: A benign entity or an ominous sign?. Kerala J Ophthalmol 2019;31:33-8

How to cite this URL:
Neena R, Giridhar A. Acute acquired comitant esotropia in children: A benign entity or an ominous sign?. Kerala J Ophthalmol [serial online] 2019 [cited 2019 Apr 24];31:33-8. Available from: http://www.kjophthal.com/text.asp?2019/31/1/33/256257


  Introduction Top


Acquired nonaccommodative esotropia (ANAET) is a relatively rare, distinct subtype of esotropia characterized by a nonaccommodative esodeviation which can occur in older children, adults, and even the elderly.[1],[2] Onset can be acute and associated with diplopia when it is called acute acquired comitant esotropia (AACE) or it can result from deterioration of existing, previously controlled, esotropia.[3],[4] AACE is thought to contribute to about 0.3% of childhood strabismus. AACE is classically divided into five different subtypes.[5],[6],[7],[8],[9],[10],[11] The Swan type (Type I) occurs after a period of interrupted binocularity.[6] Type II AACE, known as Burian-Franceschetti, has minimal hypermetropia and diplopia that are often associated with physical or psychological stress.[5] The Bielschowsky type (Type III) is associated with patients with myopia, convergence spasm, and divergence paralysis.[7],[8],[9] Type IV/refractive-accommodative type is characterized by high hypermetropia that can be adequately controlled with the refractive correction alone.[10] Type V, a lesser common entity, is associated with intracranial pathology, most commonly a posterior fossa lesion.[11],[12],[13],[14],[15] The aim of our study was to understand the clinical characteristics of AACE in children in our hospital over a period of 40 months and analyze the course and outcome.


  Materials and Methods Top


This was a retrospective, clinical study of all patients under the age of 18 years, who presented to the Pediatric Ophthalmology and Strabismus Services of a tertiary eye care center, from September 2013 to December 2016 with acute-onset, nonaccommodative comitant esotropia.

The diagnosis of AACE was made on the following criteria:

  1. Acute onset of esotropia within hours/days/weeks, with photographic evidence of previously aligned eyes
  2. Age of onset after 1 year of age
  3. Comitant esodeviation with normal ocular movements
  4. No accommodative component as confirmed by a trial of full cycloplegic correction.


Parameters studied were as follows:

  1. Age
  2. Sex
  3. Eye involved
  4. Visual acuity
  5. Age of onset
  6. Precipitating event
  7. Amount of deviation
  8. Presence or absence of amblyopia
  9. Cycloplegic refraction with atropine
  10. Systemic involvement
  11. Surgical or nonsurgical intervention
  12. Course and outcome.


A careful history and a meticulous ocular evaluation were done. All patients were examined by a strabismologist. A trial of full plus got on cycloplegic refraction was given to all patients to rule out accommodative esotropia. Occlusion therapy was started in all children detected to have amblyopia or at risk of amblyopia. All patients underwent magnetic resonance imaging (MRI) of the brain and orbits to rule out intracranial pathology, and systemic evaluation was done in suspicious cases. Those children without any systemic involvement underwent strabismus surgery under general anesthesia. The minimum follow-up period was 6 months.


  Results Top


There were a total of 12 patients, of which, 8 were male. Average age of the patients was 7.9 years (range: 4–17 years). Visual acuity was good in all patients with none having a vision <20/30 in the worse eye. Mean esodeviation was 33.75 PD (range: 20–5 PD), and mean age of onset was 6.14 years (range: 2–15 years) [Figure 1]a. Average duration of strabismus till presentation was 21.11 months [Figure 1]b. There was left eye preponderance in six children, right eye involvement in five children, and alternating esotropia in one child. Three patients (25%) had a history of a precipitating event in the form of fall, fever, and use of overminus lenses, respectively. Cycloplegic refraction ranged from +0.50 DS to −6.75 DS, with majority having <+2.00 DS. Amblyopia was noted in 8 (66.6%) patients. The systemic involvement was seen in three patients in the form of ocular myasthenia, central nervous system (CNS) glioma, and viral fever, respectively. Esotropia disappeared in one patient with overcorrected, high myopia following atropinization, and change of glass. One patient with AACE and diplopia for 2 years was detected to have ocular myasthenia and his esotropia and diplopia disappeared with oral pyridostigmine therapy [Figure 2]a and [Figure 2]b. One patient who had AACE and diplopia following fever spontaneously recovered by 3 months. One patient with AACE, who was referred for surgery, had significant distance–near disparity in the measured esodeviation and suspicious temporal pallor. He was detected to have neurofibromatosis type-1 and diffuse central nervous system (CNS) glioma on magnetic resonance imaging (MRI) and magnetic resonance (MR) spectroscopy of the brain and was referred to oncologist for further management [Figure 3]a,[Figure 3]b,[Figure 3]c,[Figure 3]d. Eight patients with no systemic complications and normal neuroimaging (66.6%) underwent strabismus surgery with good postoperative alignment (<8 pd esotropia); [Figure 4].
Figure 1: (a) Age of onset of esotropia. (b) Duration of esotropia in the study population

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Figure 2: (a) Acute acquired comitant esotropia patient before oral pyridostigmine therapy. (b) Acute acquired comitant esotropia patient after oral pyridostigmine therapy

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Figure 3: (a) Serial pictures showing previously normal alignment and onset of esotropia at 5 years of age. (b) Esotropia in primary gaze with normal ocular movements. (c) Magnetic resonance imaging of the brain and orbits showing predominantly white matter lesions involving bilateral optic tracts, extending from tectum of midbrain up to pons on the left side, mild, with subtle contrast enhancement and no diffusion restriction. (d) Magnetic resonance spectroscopy showing increased choline with reduction in N-acetyl aspartate suggestive of central nervous system glioma

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Figure 4: Acute acquired comitant esotropia patients before and after surgery

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  Discussion Top


AACE was described as an infrequent form of strabismus by Burian and Miller (1958).[5] Five different subtypes[5],[6],[7],[8],[9],[10],[11] have been identified. The Swan type (Type I) occurs after a period of interrupted binocularity.[6] Type II AACE, known as Burian-Franceschetti, has minimal hypermetropia and diplopia that are often associated with physical or psychological stress.[5] The Bielschowsky type (Type III) is associated with patients with myopia, convergence spasm, and divergence paralysis.[7],[8],[9] Type IV/refractive-accommodative type is characterized by high hypermetropia that can be adequately controlled with the refractive correction alone.[10] Type V, a lesser common entity, is associated with intracranial pathology, most commonly a posterior fossa lesion.[11],[12],[13],[14],[15]

We did not have any patient with a history of monocular occlusion preceding the onset of esotropia in our study. We excluded accommodative esotropia (Type IV) by doing an atropine refraction in all patients and giving a trial of glasses. There was one child with overcorrected high myopia, who developed esotropia and features of Bielschowsky type (Type III) AACE. One patient with AACE had an intracranial pathology (Type V AACE) in the form of CNS glioma. Apart from these, the sytemic involvement in the form of ocular myasthenia and viral fever was seen in one patient each. The rest of our patients (66.66%) seem to fit into the subgroup that resembles Type II (Burian-Franceschetti type) AACE [Figure 5]a.
Figure 5: (a) Types of acute acquired comitant esotropia in the study population. (b) Postoperative motor alignment. (c) Binocular status postsurgery. (d) Comparison with other studies

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In Burian's paper,[5] the age group was 11–72 years, patients had low hypermetropic errors, they complained of diplopia, angle of deviation ranged between 20 and 60 PD, and had excellent functional outcome after the strabismus surgery. We had included only children in our study (range: 4–17 years), and only four children complained of diplopia. This could be due to the rapidity of suppression in very young children or inability on the part of very young children to complain of diplopia. All patients (66.6%) who underwent strabismus surgery in our study had good postoperative alignment (<8 pd esotropia). The mechanism proposed by von Noorden[10] for the pathogenesis of the Burian-Franceschetti syndrome is the loss of borderline fusion (poor fusional divergence) and the precipitation of the nonaccommodative comitant esodeviation.

AACE of childhood has a small but significant association with intracranial disease as was seen in the case from our study. Many brain tumors such as cerebellar astrocytomas, medulloblastomas, pontine gliomas, astrocytoma of the corpus callosum (with hydrocephalus), and Arnold– Chiari malformation More Details are associated with AACE in childhood without any neurological deficit, and sometimes, acute-onset nonaccommodative esotropia may be the only presenting sign.[11],[12],[13],[14],[15] The exact mechanism responsible for acute comitant esotropia in patients with brain tumors is not clear. Comitant strabismus might result from involvement of supranuclear mesencephalic structures, which control vergence eye movements. Some have ascribed acquired comitant esotropia to infranuclear insults, such as varying degrees of bilateral sixth nerve paresis. Spread of comitance is another suggested mechanism.[12]

Although most children with this form of esotropia are otherwise healthy, CNS lesions must be considered and ruled out before planning surgery. If you miss a brain tumor in a child, it may prove costly. Hence, we feel that neuroimaging should be done in any patient presenting with acquired comitant nonaccommodative esotropia, especially when there is a high index of suspicion.

The duration between the onset of strabismus and realignment is important in getting optimal postoperative results. A favorable motor alignment (ET <8PD) was got in all patients who underwent surgery in our study [Figure 5]b, but only 3 (37.5%) patients achieved good binocular vision in the form of fusion [Figure 5]c. This could be due to the long duration of strabismus (average duration: 21.11 months) before presentation and the young age of the patients. The incidence of unilateral amblyopia (66.66%) was also found to be high in this study.

In a study of 48 children with AACE over a 13-year period by Buch and Vinding,[16] the mean age of onset was 4.7 years, and intracranial disease was present in 6%. As compared to this, average age of onset was 6.14 years and intracranial disease was present in 8.33% in our study [Figure 5]d. In a study of 47 patients (adults and children) with AACE over a 4-year period, Chen et al.[17] found coexisting or underlying neurological diseases to be infrequent. In his study on clinical characteristics of spontaneous, late-onset comitant acute, nonaccommodative esotropia (ANAET) in children, Kothari,[18] found ANAET to be more common than previously reported, with a variable time of onset, high incidence of amblyopia, and good results with strabismus surgery. We also got comparable results.


  Conclusion Top


AACE is an unusual ocular alignment disorder characterized by a nonaccommodative esodeviation which can occur in older children, adults, and even the elderly. Although most children with this form of esotropia are otherwise healthy, systemic diseases must be considered and ruled out before planning surgery. Prompt amblyopia therapy and timely surgery can result in a satisfactory outcome in those without systemic involvement.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Legmann Simon A, Borchert M. Etiology and prognosis of acute, late-onset esotropia. Ophthalmology 1997;104:1348-52.  Back to cited text no. 1
    
2.
Clark AC, Nelson LB, Simon JW, Wagner R, Rubin SE. Acute Acquired Comitant Esotropia. Br J Ophthalmol 1989;73:636-8.  Back to cited text no. 2
    
3.
Lyons CJ, Tiffin PA, Oystreck D. Acute Acquired Comitant Esotropia: A prospective study. Eye (Lond) 1999;13 (Pt 5):617-20.  Back to cited text no. 3
    
4.
Sturm V, Menke MN, Töteberg M, Jaggi GP, Schoeffler C. Early onset of acquired comitant non-accommodative esotropia in childhood. Klin Monbl Augenheilkd 2012;229:357-61.  Back to cited text no. 4
    
5.
Burian HM, Miller JE. Comitant convergent strabismus with acute onset. Am J Ophthalmol 1958;45:55-64.  Back to cited text no. 5
    
6.
Swan KC. Esotropia following occlusion. Arch Ophthal 1947;37:444-51.  Back to cited text no. 6
    
7.
Franceschetti A. Acute concomitant strabismus. Ophthalmologica 1952;123:219-26.  Back to cited text no. 7
    
8.
Bielschowsky A. Das einwärtsschielen der myopen [Convergent strabismus of myopes]. Ber Dtsch Ophthalmol Ges 1922;43:245-59.  Back to cited text no. 8
    
9.
Campos EC. Why do the eyes cross? A review and discussion of the nature and origin of essential infantile esotropia, microstrabismus, accommodative esotropia, and acute comitant esotropia. J AAPOS 2008;12:326-31.  Back to cited text no. 9
    
10.
von Noorden GK. Esodeviations. In: von Noorden GK, Campos E, editors. Theory and Management of Strabismus in Binocular Vision and Ocular Motility. 5th ed.. USA: The C.V. Mosby Company; 1990. p. 309.  Back to cited text no. 10
    
11.
Astle WF, Miller SJ. Acute comitant esotropia: A sign of intracranial disease. Can J Ophthalmol 1994;29:151-4.  Back to cited text no. 11
    
12.
Hoyt CS, Good WV. Acute onset concomitant esotropia: When is it a sign of serious neurological disease? Br J Ophthalmol 1995;79:498-501.  Back to cited text no. 12
    
13.
Macpherson H, De Becker I, MacNeill JR. Beware: Armed and dangerous – Acquired non-accommodative esotropia. Am Orthopt J 1996;46:44-56.  Back to cited text no. 13
    
14.
Williams AS, Hoyt CS. Acute comitant esotropia in children with brain tumors. Arch Ophthalmol 1989;107:376-8.  Back to cited text no. 14
    
15.
Zweifach PH. Childhood esotropia with delayed appearance of cerebellar tumor. Neuroophthalmology 1981;1:291-3.  Back to cited text no. 15
    
16.
Buch H, Vinding T. Acute Acquired Comitant Esotropia of childhood: A classification based on 48 children. Acta Ophthalmol 2015;93:568-74.  Back to cited text no. 16
    
17.
Chen J, Deng D, Sun Y, Shen T, Cao G, Yan J, et al. Acute acquired concomitant esotropia: Clinical features, classification, and etiology. Medicine (Baltimore) 2015;94:e2273.  Back to cited text no. 17
    
18.
Kothari M. Clinical characteristics of spontaneous late-onset comitant acute nonaccommodative esotropia in children. Indian J Ophthalmol 2007;55:117-20.  Back to cited text no. 18
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