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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 317-319

Rubella eye disease: A clinicopathological case report


1 Department of Ocular Pathology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
2 Department of Ophthalmology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India

Date of Submission12-Aug-2020
Date of Acceptance24-Sep-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Dipankar Das
Department of Ocular Pathology, Uveitis and Neuro-Ophthalmology Services, Sri Sankaradeva Nethralaya, 96 Basistha Road, Beltola, Guwahati, Assam
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_2621_20

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  Abstract 


Rubella is a viral disease that affects human particularly pregnant females and causes transplacental spread to affect the fetus in utero. Congenital rubella in a child can manifest from mild to severe involvement. We report a case of a young boy with bilateral blindness and sensorineural deafness. Enucleated eye revealed the evidence of rubella eye disease. TORCH titer showed increase of IgG antibody in the serum. An extensive systemic examination and investigations by physician did not show any other signs of extended rubella syndrome.

Keywords: Deafness, enucleation, inflammation, rubella, sensorineural, titer


How to cite this article:
Das D, Das JK, Agarwal B, Bhattacharjee H, Bharali G, Deka D, Mahato DK, Deshmukh S, Deka A. Rubella eye disease: A clinicopathological case report. Indian J Ophthalmol Case Rep 2021;1:317-9

How to cite this URL:
Das D, Das JK, Agarwal B, Bhattacharjee H, Bharali G, Deka D, Mahato DK, Deshmukh S, Deka A. Rubella eye disease: A clinicopathological case report. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Aug 3];1:317-9. Available from: https://www.ijoreports.in/text.asp?2021/1/2/317/312388



Rubella is a viral disease transmitted by respiratory droplets.[1],[2],[3] When pregnant lady gets infected for the first time, virus is transmitted transplacentally and it can lead to miscarriage, stillbirth or give birth to a child with multiple systemic abnormalities.[1],[2],[3],[4],[5] The range of defects differs according to gestational age of the child and earlier the gestational age; the worse is the damage.[1],[2],[3],[4],[5] Infection in the first 12 weeks leads to ocular defects and from 12 to 28 weeks results in sensorineural deafness and pulmonary artery stenosis.[1],[2],[3],[4],[5] The results of rubella infection in utero are collectively known as congenital rubella syndrome.[2],[4],[6],[7] We report a young boy with bilateral blindness since birth and deafness which had enucleation done in one eye with evidence of rubella eye disease in that eye. Other features of extended rubella syndrome were absent in the case.


  Case Report Top


A 6-year-old boy presented to a tertiary institute of North-East India with redness, watering, discharge, and inability to open both eyes [Figure 1]a for the last 2 weeks. The boy was blind since birth. Immunization history revealed that measles-mumps-rubella and Varicella (MMRV) vaccine was given in 9 month. Other standard vaccinations protocol was followed in the child. Vision could not be recorded in the child. On examination, there was whitish opacity on the cornea in both eyes (OU) and parents gave a history of delayed developmental milestones in the child. The mother gave history of viral fever with skin rash in the first trimester of pregnancy and was treated with antipyretic medication. The physician examined the child and found to be normal systemically except for mild sensorineural deafness. Examination under anesthesia was carried out in the boy and found his left eye (OS) was phthisical and right eye (OD) had band-shaped keratopathy with complicated cataract (OU). Ultrasound B-scan of OD showed disfigured globe suggestive of phthisical change [Figure 1]b and OS showed high reflective membrane echoes with open funnel-shaped configuration of old retinal detachment associated with calcification ensuing phthisical change. The visual evoked potential was flat in OD and moderately subnormal in OS. On investigation, X-ray PA view chest was normal; bleeding time, clotting time, total and differential leukocyte count, and hemoglobin were normal; erythrocyte sedimentation rate was elevated; creatinine and blood glucose were normal; and infectious panel-like HIV 1 and 2 and HBsAg were negative. ELISA for TORCH titer was done and showed elevated rubella titer (IgG-61.6 IU/ml-high, IgM-0.1 IU/ml); after proper parental consent, enucleation of OD was carried out.
Figure 1: (a) Clinical photograph of the child showing discharge, watering, and inability to open both eyes. (b) Ultrasound B-scan of the right eye showing disfigured globe suggestive of phthisical change. High spikes in vector A-Scan depicted the intraocular calcification which was correlated with histopathology later

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The enucleated eyeball measured anteroposteriorly 13.97 mm, horizontally 15.09 mm, and vertically 13.32 mm; cornea measured horizontally 11.96 mm and vertically 11.1 mm; and optic nerve was cut flush to the surface. The transillumination defect was not seen. The eyeball was sectioned vertically. Lens was partially calcified. Internal structures were disorganized with scleral thickening.

Microscopic examination revealed thickened cornea with basophilic degeneration of superficial cornea [Figure 2]a. Episclera showed few lymphocytic infiltration. Iris showed neovascularization with necrosis of iris epithelium [Figure 2]b. Necrotic materials with foreign body giant cells were seen between the cornea and iris. Lymphocytic infiltrations were noted in the ciliary body region [Figure 3]. There was disorganization of iris and ciliary processes also. Lens was cataractous with both cortical and nuclear change. Nuclear portion of the lens revealed retained cell nuclei concentrated in the embryonic zone of nucleus [Figure 4]a. There was disorganization of angle of anterior chamber including the trabecular meshwork [Figure 4]b. Osseous metaplasia and bone formation at the level of retinal pigment epithelium (RPE) and sub-RPE was seen. Retina showed dysplastic change. The sclera was thickened posteriorly. All these features were suggestive of phthisical eye with iris, ciliary body, trabecular meshwork change; chronic intraocular inflammation and bone formations were consistent with rubella infected eye. The patient had elevated rubella IgG titer in serum and was blind in OU since birth. Raised IgG titer in serum in enzyme-linked immunosorbent assay (ELISA) test signified the chronicity of the rubella infection.
Figure 2: (a) x 100, H and E; basophilic degeneration of superficial cornea in an enucleated eyeball. (b) x100, H and E; necrosis of iris epithelium

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Figure 3: x 100, H and E; lymphocytic infiltration of ciliary body region

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Figure 4: (a): x 400, H and E; embryonic portion of partially cataractous lens showing nucleated cells. (b): x 100, H and E; distortion of trabecular area near the angle of the anterior chamber of eye

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  Discussion Top


Rubella (German measles) is an acute childhood infection that presents mildly in most of the cases for a short duration.[1],[2] It is accompanied by low-grade fever, lymph node enlargement, skin rash, and infection in early pregnancy results in severe congenital defects in newborns.[1],[2],[3] This disease has worldwide distribution.[1],[2],[3],[4],[5],[6],[8],[9],[10] Congenital rubella is a chronic infection, while acquired rubella is an acute infection.[1],[2],[3],[4] The disease was named after Norman Gregg, and Rubella is also called Gregg syndrome.[1],[2],[3] Dr. Norman Gregg was an ophthalmologist who reported an epidemic of congenital cataracts associated with congenital defects in 1941.[1],[2],[3],[4] Rubella is caused by an RNA virus that belongs to toga virus family.[5],[6],[8],[9],[10] Congenital rubella syndrome refers to the infants born with congenital defects secondary to intrauterine infection of pregnant mothers.[1],[2],[3],[4],[5],[6],[8],[9],[10] Rubella infection inhibits cell division, and this is the reason for birth defects and low birth weight.[4],[7] Congenital defect includes deafness, cardiac malformations, cataract, glaucoma, retinopathy, microphthalmos, microcephaly, cerebral palsy, intrauterine growth retardation, hepato-splenomegaly, motor and mental retardation.[1],[2],[3],[4],[5],[6],[8],[9],[10] Vaccines against rubella have been developed and RA 27/3 vaccine was developed from human diploid fibroblast.[2],[3],[4],[7]

Our case presented with bilateral blindness and mild sensorineural deafness. He was seen by a physician who could not find any other signs of extended rubella syndrome. Pathological findings such as basophilic corneal degeneration and phthisical change were consistent with chronicity of the disease. There was necrosis noted on the anterior surface of iris with disorganization of angle structures including the trabecular meshwork area. Extensive lymphocytic infiltration was noted in the ciliary body with evidence of nucleated cells in the center of partially cataractous lens. Intraocular bone formation in rubella eye was never been reported earlier in the literature; however, any chronic infectious intraocular cause or other causes of phthisical eye can show intraocular ossification. ELISA for IgG for rubella was raised in the child, which confirmed the correlation of rubella eye disease in this bilateral blind child.


  Conclusion Top


We report histopathological features in an enucleated eye of a child with serological evidence of rubella.

Acknowledgements

Kanchi Sankara Health and Educational Foundation, Guwahati, Assam, India.

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.
Vijaylakshmi P, Rajasundari TA, Prasad NM, Prakash SK, Narendran K, Ravindran M, et al. Prevalence of eye signs in congenital rubella syndrome in South India: A role of population screening. Br J Ophthalmol 2007;91:1467-70.  Back to cited text no. 1
    
2.
Cooper LZ, Krugman S. Clinical manifestations of postnatal and congenital rubella. Arch Ophthalmol 1967;77:434-9.  Back to cited text no. 2
    
3.
Esterly JR, Oppenheimer EH. Pathological lesions due to congenital rubella. Arch Pathol 1969;87:380-8.  Back to cited text no. 3
    
4.
Boniuk M, Zimmerman LE. Ocular pathology in the rubella syndrome. Arch Ophthalmol 1967;77:455-73.  Back to cited text no. 4
    
5.
Zimmerman LE. Histopathologic basis for ocular manifestations of congenital rubella syndrome. Am J Ophthalmol 1968;65:837-62.  Back to cited text no. 5
    
6.
Goldberg N, Chou J, Moore A, Tsang S. Autofluorescence imaging in rubella retinopathy. Ocul Immunol Inflamm 2009;17:400-2.  Back to cited text no. 6
    
7.
Chen M, Zhu Z, Liu D, Huang G, Huang F, Wu J, et al. Rubella epidemic caused by genotype 1E rubella viruses in Beijing, China, in 2007-2011. Virol J 2013;10:122.  Back to cited text no. 7
    
8.
Weiss DI, Ziring PR, Cooper LZ. Surgery of the rubella cataract. Am J Ophthalmol 1972;73:326-32.  Back to cited text no. 8
    
9.
Nguyen TV, Pham VH, Abe K. Pathogenesis of congenital rubella virus infection in human fetuses: Viral infection in the ciliary body could play an important role in cataractogenesis. EBioMed 2014;2:59-63.  Back to cited text no. 9
    
10.
Kemmanu V, Giliyar SK, Shetty BK, Singh AK, Kumaramanickavel G, McCarty CA. Emerging trends in childhood blindness and ocular morbidity in India: The pavagada pediatric eye disease study 2. Eye 2018;32:1590-8.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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