An unusual case of Heimann–Bielschowsky phenomenon associated with mature cataract

Shray Dogra; Navneet Kaur; Pawan Prasher

doi:10.4103/IJO.IJO_2929_22

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CASE REPORT

Year : 2023 | Volume : 3 | Issue : 2 | Page : 327-329

An unusual case of Heimann–Bielschowsky phenomenon associated with mature cataract

Shray Dogra¹, Navneet Kaur¹, Pawan Prasher²
¹ Department of Ophthalmology, Maharishi Markandeshwar Institute of Medical Sciences and Research, Mullana, Ambala, Haryana, India
² Department of Ophthalmology, Sri Guru Ram Das Institute of Medical Sciences and Research, Amritsar, Punjab, India

Date of Submission	03-Nov-2022
Date of Acceptance	05-Jan-2023
Date of Web Publication	28-Apr-2023

Correspondence Address:
Pawan Prasher
Department of Ophthalmology, Sri Guru Ram Das Institute of Medical Sciences and Research, Vallah, Amritsar, Punjab
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJO.IJO_2929_22

Abstract

Heimann–Bielschowsky phenomenon (HBP) is an unusual condition with a low-frequency, coarse, vertical nystagmoid movement that develops after long-standing profound vision loss in an eye. We report a case of HBP in an eye with a mature senile cataract that resolved spontaneously after cataract surgery. A 50-year-old male presented with a mature senile cataract in his right eye, without any previous history of abnormal movements of either eye. The affected eye was only perceiving hand motions at 2 ft due to the mature cataract, along with an intermittent, slow, vertical nystagmoid movement. The evaluation was consistent with the diagnosis of HBP, and it was decided to proceed with cataract surgery and reassess postoperatively. The patient underwent uneventful cataract surgery with good visual outcome as well as spontaneous resolution of the HBP. To the best of our knowledge, this is the first case of HBP associated with senile cataract, which resolved after cataract surgery was performed.

Keywords: Cataract, fusion, Heimann–Bielschowsky phenomenon, nystagmus

How to cite this article:
Dogra S, Kaur N, Prasher P. An unusual case of Heimann–Bielschowsky phenomenon associated with mature cataract. Indian J Ophthalmol Case Rep 2023;3:327-9

How to cite this URL:
Dogra S, Kaur N, Prasher P. An unusual case of Heimann–Bielschowsky phenomenon associated with mature cataract. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 2];3:327-9. Available from: https://www.ijoreports.in/text.asp?2023/3/2/327/375002

Senile cataracts are a significant cause of preventable blindness worldwide.^[1] Any delay in treatment can lead to the development of sequelae, such as lens-induced glaucomas, strabismus, and uveitis, which can irreversibly compromise the patient's quality of vision.^[2],[3]

Patients with long-standing severe vision loss in one eye can develop an unusual vertical nystagmus in the affected eye, known as the Heimann–Bielschowsky phenomenon (HBP).^[4] It is characterized by a coarse, intermittent, vertical nystagmoid movement with a low frequency, developing many years after profound mono-ocular vision loss.^[5] Its etiology and management are not clearly documented in literature, and it can be easily mistaken for a neurological disorder. It is important to recognize this entity to avoid unnecessary investigations and treatments.

Herein, we present a unique case of senile cataract with HBP in the right eye of a middle-aged male patient with no previous history of abnormal movements of his eyes, which spontaneously resolved after he underwent cataract surgery.

Case Report

A 50-year-old male presented with decreased vision in his right eye for the past 8 months, along with intermittent involuntary movements of the right eye for the past 2 months. The decrease in vision was gradual in onset and progressive, but there was no diplopia or oscillopsia. There was no history of associated pain, redness, watering, or discharge in the right eye.

1Previous history was not significant, and the patient was not using any medications for any ocular or systemic illness. There was no history of oscillation or deviation of eyes in any family member. The patient had a best corrected visual acuity (BCVA) of perceiving hand motions at 2 ft in the right eye. No relative afferent pupillary defect or restriction of ocular movements was observed in either eye. The right eye demonstrated a slow, intermittent, pendular movement, with a frequency of approximately 0.2 Hz, in both upward and downward directions, along with a constant exodeviation [Video 1][Additional file 1]. The maximum amplitude of the vertical movement was estimated to be 40 prism diopters (PD) and the exodeviation was estimated to be 30 PD, based on the Hirschberg test (HT) and Cover test (CT) [Figure 1]. Slit-lamp examination revealed a mature nuclear cataract in the right eye, with a relatively shallow anterior chamber (Van Herick grade 2). The rest of the anterior segment examination revealed no significant findings. Fundus evaluation of the right eye could not be performed due to the dense cataract; left eye fundus evaluation showed normal findings. B-scan findings of the posterior segment in the right eye were unremarkable.{Figure }

Systemic examination by the physician and the neurologist showed no associated neurological abnormality, and no special neurological investigations were advised at this time. Based on the slit-lamp findings and ocular motility evaluation, a diagnosis of mature senile cataract with HBP was made. After discussion with the neurologist, a decision was made to proceed with cataract surgery in the right eye and reassess the HBP postoperatively. In view of the cataract grade, the patient successfully underwent manual small-incision cataract surgery with implantation of a rigid polymethyl methacrylate intraocular lens (IOL) under local anesthesia. Slit-lamp examination on the first postoperative day revealed mild conjunctival injection, clear cornea, and a well-centered IOL. It was observed that the intermittent vertical movement and exodeviation of the right eye had improved and the eyes were well aligned on HT and CT [Figure 2]. No fundus abnormality was found in the right eye, and the patient was prescribed topical moxifloxacin 0.5% eye drops four times and prednisolone 1% eye drops six times a day. Topical prednisolone was gradually tapered over the next 4 weeks, and all medications were continued for a total of 6 weeks. No abnormal ocular movement was seen at any postoperative visit, and the ocular alignment was maintained [Video 2][Additional file 2]. The final BCVA was recorded as 20/20 in the right eye of the patient after 6 weeks, with a refractive correction of + 0.50

Figure 2: (a) HT showing well-aligned eyes after right eye cataract surgery. (b) CT showing a well-fixated right eye. (c) CT showing a well-fixated left eye. CT = Cover test, HT = Hirschberg test

Click here to view

D / + 0.50 D × 20°.

Discussion

HBP is an underdiagnosed and a poorly understood condition, with little documentation in literature. It is a coarse, intermittent, vertical nystagmoid movement of an eye which has profound long-standing visual loss.^[4] Its exact mechanism is unknown, but it is believed to be caused due to disruption of binocular fusional mechanisms.^[4] Our patient presented with HBP, exhibiting a slow, intermittent drifting movement of the right eye, along with a dense senile cataract. We speculate that it was likely due to disruption of sensory fusion due to the cataract. Our hypothesis is strengthened by the resolution of ocular movements and thus re-establishment of fusion following cataract extraction. The movement had stopped on the first follow-up visit, and the alignment was maintained on all subsequent visits, thus alleviating the need for any corrective procedure for the movement itself.

Reports of HBP associated with cataract are rare, and we could not find any report of resolution of HBP after removal of cataract. Jeong et al.^[6] have reported a case of emergence of HBP after cataract surgery for traumatic cataract. Choi and Chun reported persistence of HBP after cataract extraction and strabismus surgery for long-standing traumatic cataract with sensory esotropia.^[7] Our patient had no history of trauma and had relatively recent onset of visual impairment, suggesting that the mechanism of HBP may be different from cases of long-standing visual deprivation; early cataract surgery may be helpful in such cases.

In previous reports, symptomatic and asymptomatic HBP has been managed to varying degrees of success with various medical and surgical treatments such as recession of the superior rectus, inferior rectus, and inferior oblique muscles; medial rectus recession and lateral rectus resection; and oral gabapentin up to a dose of 2400 mg/d.^[8],[9],[10] We also considered the possibility of the movement representing an entity other than HBP with a separate neurological etiology; however, the workup done by the neurologist in our patient was unremarkable. The clinical evidence suggests that HBP in this case likely manifested as a result of sensory deprivation due to cataract, leading to disruption of fusion.

Conclusion

In conclusion, intermittent vertical movement in the form of HBP can be seen in eyes with long-standing senile cataracts and should be differentiated from other causes of nystagmoid ocular movements. There is a probability that the movement will correct once fusion is restored, and any surgical procedure for the movement should be considered after sufficient time has been allowed after cataract extraction. Ophthalmologists should be aware of this entity and should evaluate coexisting neurological signs before planning any intervention.

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

1.	Lee CM, Afshari NA. The global state of cataract blindness. Curr Opin Ophthalmol 2017;28:98-103.
2.	Azhany Y, Hemalatha C, Nani D, Rosediani M, Liza-Sharmini A. Sequelae of neglected senile cataract. Malays Fam Physician 2013;8:33-37.
3.	Chaudhuri Z, Pandey PK. Sensory deviations subsequent to senile cataract. J Pediatr Ophthalmol Strabismus 2000;37:159-62.
4.	Nguyen A, Borruat FX. The Heimann-Bielschowsky phenomenon: A retrospective case series and literature review. Klin Monbl Augenheilkd 2019;236:438-41.
5.	Smith JL, Flynn JT, Spiro HJ. Monocular vertical oscillations of amblyopia. The Heimann-Bielschowsky phenomenon. J Clin Neuroophthalmol 1982;2:85-91.
6.	Jeong SH, Oh YM, Hwang JM, Kim JS. Emergence of diplopia and oscillopsia due to Heimann-Bielschowsky phenomenon after cataract surgery. Br J Ophthalmol 2008;92:1402. doi: 10.1136/bjo. 2007.135624.
7.	Choi HJ, Chun BY. Heimann-Bielschowsky phenomenon and hypotropic DVD in case of monocular vision loss: A case report. BMC Ophthalmol 2020;20:254.
8.	ElKamshoushy A, Sprunger DT. Recession of three muscles to reduce ocular oscillations in patients with Heimann-Bielschowsky phenomenon. J AAPOS 2011;15:67-8.
9.	Choi SR, Baek SH, Samuel Kim U. The effect of horizontal extraocular muscle surgery on the Heimann-Bielschowsky phenomenon. Neuroophthalmology 2013;37:41-2.
10.	Rahman W, Proudlock F, Gottlob I. Oral gabapentin treatment for symptomatic Heimann-Bielschowsky phenomenon. Am J Ophthalmol 2006;141:221-2.