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Bilateral dissociated vertical deviation in a case of congenital hereditary endothelial dystrophy

Bhola, Rahul MS; Saxena, Rohit MD; Sethi, Harinder Singh MD; Sharma, Pradeep MD; Panda, Anita MD; Sen, Seema MD

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Indian Journal of Ophthalmology: Jan–Mar 2006 - Volume 54 - Issue 1 - p 41-42
doi: 10.4103/0301-4738.21614
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Abstract

Dissociated vertical deviation (DVD) is an intermittent anomaly of the non-fixing eye consisting of upward excursion, excyclotorsion, and lateral deviation, which may occur either spontaneously, or owing to disrupted binocularity by monocular occlusion.1 Although association of DVD with sensory visual deprivation owing to congenital or acquired opacities of the ocular media has been reported,2 its association with congenital hereditary endothelial dystrophy (CHED) has not been reported hitherto. We report a case having a bilateral asymmetric DVD in a know case of bilateral CHED.

Case Report

A 5-year-old girl presented with a history of poor vision and hazy cornea since birth. There was no complaint of glare, photophobia, or watering, and there was no systemic abnormality. There was no family history of any corneal or ocular motor disorder, nor any medical or surgical intervention in the child. The best corrected visual acuity was 7/200 with accurate projection of rays in all quadrants in either eye. The corneas of both eyes had diffuse haze with stromal edema and Descemet's folds. The intraocular pressure and corneal diameters were normal. The rest of the anterior segment and adnexal tissue were normal. Fundus details could not be elicited owing to corneal haze. The patient had an alternate divergent strabismus of 20 prism diopter (pd) base in with manifest latent nystagmus and asymmetric DVD (right > left) in both the eyes. With the left eye fixing, there was a right hypertropia of 10 pd along with a 20 pd exotropia. A left hypertropia of 6 pd along with an exotropia of 20 pd was recorded with right eye fixing (Figure 1). The horizontal deviation was the same in all gazes, with no A or V phenomenon. There was no inferior or superior oblique overaction in either eye. There was no inhibitional palsy of the contralateral superior rectus when the patient fixed with the adducted and elevated eye. The Bielschowsky and the Red glass test could not be done owing to the poor vision in either eye. The child was admitted for penetrating keratoplasty in the right eye. Histopathology of the corneal button showed diffusely thickened Descemet's membrane with atrophy of the endothelium, there by confirming the diagnosis of CHED (Figure 2).

Figure 1
Figure 1:
Figure showing position of eyes in primary gaze (A), with left eye fixing (B) showing a right hypertropia of 10 pd along with a 20 pd exotropia and left hypertropia of 6 pd along with an exotropia of 20 pd with right eye fixing (C).
Figure 2
Figure 2:
Light microscopy demonstrates thickened Descemet's membrane and occasional endothelial cells (HandE ×400).

The electroretinogram and visual evoked potential were within normal range and the posterior segment appeared to be normal on ultrasonography.

Discussion

DVD is an enigmatic disorder characterized by a slow ascent of one eye followed, after a variable interval, by a slow descent of the higher eye back to the neutral position.13 DVD manifests at times of fatigue, inattention, or when binocular visual input is mechanically, optically, or sensorially pre-empted.4 Although several theories exist, recently it has been proposed that DVD is unmasking of a dorsal light reflex. Asymmetrical visual input to the two eyes evokes intermittent and alternate excitation of both subcortical centers that govern vertical divergence movement of the eyes.5 The fixating eye keeps its position unchanged because voluntary innervation of the depressors neutralizes the innervation to the elevators. This primitive visuovestibular reflex functions as a righting response to restore vertical orientation in lower lateral-eyed animals (fish) by equalizing binocular visual input. The dorsal light reflex is suppressed in humans as the development of fusion impedes the development of the vertical vergence centers, but can manifest as DVD when there is an early disruption of normal binocular development. Although the DVD is mostly seen in association with infantile esotropia, it is also seen with other types of strabismus and even in cases in which no other form of strabismus exists, such as after occlusion6 and postcataract surgery.7 Latent nystagmus exists in most of the cases with DVD. To the best of our knowledge, the presence of DVD in a case of CHED is being reported for the first time (PubMed Medline search). The association of DVD with manifest latent nystagmus is most likely owing to the early onset of CHED, causing lack of stability of fusion. According to Helveston,1 the earlier in life the fusion anomaly occurs, the more likely DVD is to occur and persist. The earlier the disruption of binocular cooperation, the less likely the patient will be able to re-establish it. Thus, improper fusion development could be an important factor in the association of DVD with sensory visual deprivations. According to Bielschowsky,3 the only means of reducing the onset of these deviations is by improving the fusional innervation—the stronger the fusional mechanism, the easier it is to keep dissociated vertical movements latent. This implies that early visual rehabilitation in such cases of sensory visual deprivation could prevent unmasking of primitive reflexes such as dorsal light reflex and, in turn, the DVDs.

1. Kutluk S, Avilla CW, von Noorden GK. The prevalence of dissociated vertical deviation in patients with sensory heterotropia Am J Ophthalmol. 1995;119:744–7
2. Helveston EM. Dissociated vertical deviation-a clinical and laboratory study Trans Am Ophthalmol Soc. 1980;76:734–79
3. Bielschowsky A. Disturbances of the vertical motor muscles of the eyes Arch Ophthalmol. 1938;20:190–6
4. Olsen RJ, Scott WE. Dissociated phenomena in congenital monocular elevation deficiency J Am Assoc Pediatr Ophthalmol Strabismus. 1998;2:72–8
5. Brodsky MC. Dissociated vertical deviation-a righting reflex gone wrong Arch Ophthalmol. 1999;117:1216–22
6. Bielschowsky A. Lecture on motor anomalies: II The theory of heterophoria Am J Ophthalmol. 1938;21:1129–36
7. White JW. Hyperphoria-diagnosis and treatment Arch Ophthalmol. 1933;7:739–47
Keywords:

Congenital hereditary endothelial dystrophy; corneal opacity; dissociated vertical deviation; nystagmus

© 2006 Indian Journal of Ophthalmology | Published by Wolters Kluwer – Medknow