Journal of Pediatric Ophthalmology and Strabismus

Short Subjects 

Acute-Onset Esotropia: Should We Look Inside?

Jennifer Schreuders, MD; G. W. S. Thoe Schwartzenberg, MD; Edmee Bos, BSc; Florens G. A. Versteegh, MD, PhD

Abstract

The authors report a case of acute onset of comitant esotropia with diplopia in a 5-year-old boy with a diffuse pontine glioma. On first presentation, the angle of esodeviation was 30 prism diopters at distance fixation and 25 prism diopters at near fixation. Neurological symptoms appeared 10 weeks after the first visit.

From the Departments of Pediatrics (JS, FGAV) and Ophthalmology (GWSTS, EB), Groene Hart Ziekenhuis, Gouda, the Netherlands.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Florens G. A. Versteegh, MD, PhD, Groene Hart Ziekenhuis, P.O. Box 1098, 2800BB Gouda, the Netherlands. E-mail: florens.versteegh@ghz.nl

Received: February 14, 2012
Accepted: August 24, 2012
Posted Online: December 04, 2012

Abstract

The authors report a case of acute onset of comitant esotropia with diplopia in a 5-year-old boy with a diffuse pontine glioma. On first presentation, the angle of esodeviation was 30 prism diopters at distance fixation and 25 prism diopters at near fixation. Neurological symptoms appeared 10 weeks after the first visit.

From the Departments of Pediatrics (JS, FGAV) and Ophthalmology (GWSTS, EB), Groene Hart Ziekenhuis, Gouda, the Netherlands.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Florens G. A. Versteegh, MD, PhD, Groene Hart Ziekenhuis, P.O. Box 1098, 2800BB Gouda, the Netherlands. E-mail: florens.versteegh@ghz.nl

Received: February 14, 2012
Accepted: August 24, 2012
Posted Online: December 04, 2012

Introduction

Several case reports1–4 have described children who presented with acute onset of comitant esotropia and were found to have intracranial tumors. A cerebellar tumor was identified as the underlying pathology in most of these cases. We describe a 5-year-old boy with acute onset of comitant esotropia in the absence of neurological findings. He developed neurological symptoms 2½ months after first presentation and computed tomography of the cerebrum was performed, which revealed an arachnoid cyst in the left hemisphere and a diffuse pontine glioma.

This rare presentation of severe intracranial pathology raises the question of whether it is efficient to perform further radiologic examination of all children with acute onset of comitant esotropia without neurological symptoms or whether the costs outweigh the benefits for this commonly seen problem in childhood.

Case Report

On first presentation, a 5-year-old boy had acute comitant esotropia with diplopia for 2 weeks with no other neurologic findings. Except for a post-partum Group B streptococcal infection and Pfeiffer’s disease, his medical history was unremarkable. He had no antecedent head trauma, systemic illness, or psychogenic stress.

Visual acuity measured with a picture chart was 6/6 in both eyes (uncorrected). On the alternating prism cover test, the angle of esodeviation was 30 prism diopters (PD) at distance fixation and 25 PD at near fixation. The ocular rotations were full and there was no nystagmus. Fundus examination was normal.

One month after the first symptoms, the patient underwent strabismus surgery. A 5-mm recession of the internal rectus muscle of both eyes was performed. Three weeks after surgery, the angle of esodeviation had increased to 55 PD at distance fixation and 65 PD at near fixation and the first sign of a bilateral abduction deficit was noticed, becoming more obvious in time.

Further neurological problems appeared 2½ months after the first visit. According to the patient’s mother, he started to have difficulties walking and his fine motor skills had regressed. Because of these symptoms, the boy was referred to a pediatrician. Neurological examination revealed a bilateral nerve palsy of the abducens nerve. His mouth was hanging a bit open, with his tongue pointing out to the right. The pharyngeal arch was asymmetrical, with the left side being higher than the right. The uvula was pointing to the right. Muscular strength was symmetrical and normal. There was a bilateral Babinsky reflex.

Following neurological examination, computed tomography of the cerebrum was done. Two intracranial mass lesions were detected (Figure 1). One was located in the left hemisphere, which was diagnosed as an arachnoid cyst, and another in the brain stem, which was diagnosed as a pontine glioma.

Computed tomography of the cerebrum shows an arachnoid cyst in the left hemisphere and a pontine glioma.

Figure 1. Computed tomography of the cerebrum shows an arachnoid cyst in the left hemisphere and a pontine glioma.

Discussion

Primary malignant tumors of the central nervous system are the second most common childhood malignancies, after hematologic malignancies, and are the most common pediatric solid organ tumors.5 Of all pediatric central nervous system tumors, 10% to 15% are located in the brain stem. Most of these tumors are diffuse intrinsic pontine gliomas.6

Children with pontine gliomas often present with classic brain stem symptoms such as cranial nerve palsies, pyramidal signs, ataxia, hydrocephalus with elevated intracranial pressure, and personality changes instead of sensory and motor deficits and convulsions, which are often seen in children with supratentorial tumors. For cerebellar tumors, defects in coordination and nystagmus could be distinctive for the location. Acute comitant esotropia as the first sign of a pontine glioma as in the case presented here is rare, but there are several cases described with a similar presentation to our patient. In the majority of these case reports,1–4 a cerebellar tumor was identified as the underlying cause. Williams and Hoyt3 described six patients with acute comitant esotropia in children with brain tumors. Five of these children were diagnosed as having a cerebellar tumor after neuroimaging. In the other case, a pontine glioma was the underlying pathology.

There are both similarities and differences between the patient described by Williams and Hoyt and our patient. In both cases, acute comitant esotropia and diplopia were the first signs of the pontine glioma. Neither of the patients initially had neurological symptoms. Both patients were hyperopic; our patient had refractive values of +2.0 in the right eye and +2.25 in the left eye. Our patient showed an increase in the angle of esodeviation after strabismus surgery in both eyes. The patient described by Williams and Hoyt died before strabismus surgery could be performed.

Other authors have described patients with acute comitant esotropia as the first sign of a cerebellar tumor.1,2,4 Case characteristics of these reports and of our patient are summarized in Table 1.

Summary of Case Characteristics

Table 1: Summary of Case Characteristics

Esotropia is a commonly seen problem in infancy and childhood and is mostly caused by an ophthalmological problem. In accommodative esotropia, most patients undergo a phase of intermittent strabismus. Symptoms of intermittent diplopia commonly occur during the development of this strabismus.7 Acute comitant strabismus can occur after artificial interruption of binocular vision, but also without any obvious exogenous cause.7

Acute comitant esotropia as the first sign of intracranial pathology is rare, but should always be considered. Because of the increasing numbers of cases reporting this rare presentation of brain tumor, it is necessary to evaluate whether further radiologic examination of all children with acute comitant esotropia could be worthwhile. Lyons et al.8 believe that a high index of clinical suspicion should be maintained and further neuroimaging should be considered in the absence of expected findings associated with acute comitant esotropia such as hypermetropia, fusion potential, atypical features, or neurological signs. Brown and Iacuone9 suggest that the absence of sensory or motor fusion may be sensitive for intracranial disease, but their presence is not specific for the absence of intracranial disease. Cruysberg et al.10 believe that in cases of acute-onset comitant esotropia, the same risk factors for serious neurological disease should be considered as in cases of paralytic strabismus. The acronym DON’T PANIC, described by Cruysberg11 summarizes these risk factors: Diplopia, Ophthalmic symptoms, Neurological and general symptoms, Trauma, Papilledema, Anisocoria, Nystagmus, Incomplete visual fields, and Corneal hypoesthesia. Our patient’s symptoms at first presentation were acute-onset comitant esotropia and diplopia. If the DON’T PANIC risk factors were applied in our particular case, neuroradiologic examination probably could have been performed earlier. However, in our experience, we more often see children with a slight difference in esotropia, some slightly more at distance, without any other accompanying symptoms. We do not perform central nervous system imaging as a standard procedure in these cases. To date, our case has been the only one at our department with an intracranial tumor presenting with esotropia and normal eye movements.

References

  1. Musazadeh M, Hartmann K, Simon F. Late onset esotropia as first symptom of a cerebellar tumor. Strabismus. 2004;12:119–123. doi:10.1080/09273970490517557 [CrossRef]
  2. Lee JM, Kim SH, Lee JI, Ryou JY, Kim SY. Acute comitant esotropia in a child with a cerebellar tumor. Korean J Ophthalmol. 2009;23:228–231. doi:10.3341/kjo.2009.23.3.228 [CrossRef]
  3. Williams AS, Hoyt CS. Acute comitant esotropia in children with brain tumors. Arch Ophthalmol. 1989;107:376–378. doi:10.1001/archopht.1989.01070010386029 [CrossRef]
  4. Dikici K, Cicik E, Akman C, Kendiroglu G, Tolun H. Cerebellar astrocytoma presenting with acute esotropia in a 5 year-old girl. Int Ophthalmol. 2001;23:167–170. doi:10.1023/A:1010676913684 [CrossRef]
  5. Linabery AM, Ross JA. Trends in childhood cancer incidence in the U.S. (1992–2004). Cancer. 2008;112:416–432. doi:10.1002/cncr.23169 [CrossRef]
  6. Fangusaro J. Pediatric high-grade gliomas and diffuse intrinsic pontine gliomas. J Child Neurol. 2009;24:1409–1417. doi:10.1177/0883073809338960 [CrossRef]
  7. Burian HM, von Noorden GK. Textbook of Binocular Vision and Ocular Motility (2nd ed). St. Louis, MO: C. V. Mosby; 1980:291, 396.
  8. Lyons CJ, Tiffin PA, Oystreck D. Acute acquired comitant esotropia: a prospective study. Eye (Lond). 1999;13:617–620. doi:10.1038/eye.1999.169 [CrossRef]
  9. Brown SM, Iacuone JJ. Intact sensory fusion in a child with divergence paresis caused by a pontine glioma. Am J Ophthalmol. 1999;128:528–530. doi:10.1016/S0002-9394(99)00198-1 [CrossRef]
  10. Cruysberg JRM, Draaijer RW, Sellar PW. When is acute onset of concomitant esotropia a sign of serious neurological disease?Br J Ophthalmol. 1996;80:380. doi:10.1136/bjo.80.4.380 [CrossRef]
  11. Cruysberg JRM. DON’T PANIC with ocular motor palsies. Lancet. 1992;340:1540. doi:10.1016/0140-6736(92)92787-G [CrossRef]

Summary of Case Characteristics

Case Sex Age (Y) Clinical Symptoms at First Presentation Angle of Esotropia: Far/Near (PD) Diagnosis Time Between First Presentation and Diagnosis (Wk)
Musazadeh et al.1 M 3 Comitant esotropia 50/70 Cerebellar astrocytoma 34
Lee et al.2 M 3 Comitant esotropia and mild bilateral papilledema 50/50 Cerebellar astrocytoma Not known
Fangusaro6 F 5 Comitant esotropia and moderate bilateral papilledema 60/60 Cerebellar astrocytoma Not known
Dikici et al.4 M 4 Comitant esotropia and upbeat nystagmus 25/25 Cerebellar astrocytoma 3
M 6 Comitant esotropia and torsional nystagmus 18/20 Cerebellar astrocytoma 4
M 10 Comitant esotropia 30/35 Cerebellar medulloblastoma 21
F 5 Comitant esotropia 15/20 Pontine glioma 6
M 3 Comitant esotropia 25/25 Cerebellar astrocytoma 71
F 7 Comitant esotropia and torsional nystagmus 35/35 Cerebellar astrocytoma 4
Current case M 5 Comitant esotropia and diplopia 30/25 Pontine glioma and arachnoid cyst in the left hemisphere 10

10.3928/01913913-20121127-05

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