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 Table of Contents  
Year : 2018  |  Volume : 56  |  Issue : 4  |  Page : 254-256

Congenital rubella syndrome

1 Department of Ophthalmology, Government Medical College, Omandurar Government Estate, Chennai, Tamil Nadu, India
2 Department of ENT, Government Kasturba Gandhi Hospital, Chennai, Tamil Nadu, India

Date of Web Publication19-Feb-2019

Correspondence Address:
Dr. K Kavitha
Department of Ophthalmology, Government Medical College, Omandurar Government Estate, Chennai, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_89_18

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This is a report of a very rare case of congenital rubella syndrome with congenital sensorineural hearing loss, rubella retinopathy, and congenital heart disease with patent ductus arteriosus. After the introduction of the rubella vaccine in 1969, the number of babies born with congenital rubella is much less and rare. Not all the babies born with congenital rubella have the classical triad. Hence, our patient who has all the features of the classical triad is an extremely rare one. The baby underwent surgery for patent ductus arteriosus and cochlear implant procedure for sensorineural hearing loss. She is now under follow-up with us for pigmentary retinopathy.

Keywords: Classical triad, cochlear implant, congenital heart disease, congenital rubella syndrome, pigmentary retinopathy, salt-and-pepper fundus, sensorineural deafness

How to cite this article:
Pramila B, Mohan M B, Kavitha K. Congenital rubella syndrome. TNOA J Ophthalmic Sci Res 2018;56:254-6

How to cite this URL:
Pramila B, Mohan M B, Kavitha K. Congenital rubella syndrome. TNOA J Ophthalmic Sci Res [serial online] 2018 [cited 2022 Dec 7];56:254-6. Available from: https://www.tnoajosr.com/text.asp?2018/56/4/254/252498

  Introduction Top

Congenital rubella syndrome is one of the most devastating congenital viral infections caused by rubella virus. The first-trimester infection of the mother leads to the most common presentation of sensorineural hearing loss, heart defect, and ocular involvement. Ocular manifestations are microphthalmos, congenital cataract, salt-and-pepper appearance of fundus, and glaucoma. Ocular finding of pigmentary retinopathy is present in 50% of cases.

  Case Report Top

Our patient is a 5-year-old girl child admitted in the ENT department of our hospital with profound hearing loss and speech delay. She has been referred to our department for ophthalmologic evaluation [Figure 1]. The parent gave a history of surgery done for patent ductus arteriosus at 3 years of age at another institute. ENT otoacoustic emission results were suggestive of outer hair cell dysfunction. Pure tone audiometry [Figure 2] and Brainstem-evoked response audiometry results revealed severe to profound sensorineural hearing loss.
Figure 1: Profile picture of the girl with congenital rubella syndrome

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Figure 2: Audiogram picture showing severe to profound hearing loss

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On ocular examination, the girl was able to fix and follow the light. The patient was not cooperative for vision testing with Snellen's chart. The retinoscopic examination was found to be normal. Slit-lamp examination in both the eyes showed normal anterior segment. Fundus examination revealed clear media with salt-and-pepper appearance [Figure 3] of the posterior pole (more in right eye). Disc, vessels, and macula were normal. Thus she has the classical triad of congenital rubella with ocular, auditory and cardiac involvement.
Figure 3: Fundus picture showing salt-and-pepper appearance

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Psychologist opinion was obtained. The psychological evaluation revealed mild mental retardation. The patient underwent a cochlear implant procedure [Figure 4] in our hospital on October 5, 2017. Postoperatively, she was given auditory training in combination with visual gestures. She is doing well with auditory evaluation now. The patient is undergoing regular ophthal assessment of intraocular pressure measurement and fundus examination once in a year to look for the progression of retinopathy and development of subretinal neovascular membrane.
Figure 4: Postoperative picture of the patient with cochlear implant

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  Discussion Top

Rubella virus belongs to Togaviridae family. Rubella virus spreads by droplet infection and by vertical transmission.[1] Rubella infection of the mother during the period of organogenesis in the first trimester may lead to congenital anomalies of the baby. Severity depends on the period of gestation during which the mother has contracted the infection. Earlier the trimester more will be the congenital anomalies.[2]

Embryologically, the human eye and inner ear develop from neuroectoderm just adjacent to each other very early in life. Hence, prenatal insult and toxins causing congenital anomalies can affect both of them.

Although congenital rubella syndrome consists of classical triad of sensorineural deafness, congenital heart defect,[3] ocular manifestations such as pigmentary retinopathy, cataract,[2],[4],[5] glaucoma,[4],[5] and microphthalmos,[2],[5] it also leads to intrauterine growth retardation, myocarditis, hepatosplenomegaly, and late complications such as diabetes mellitus, thyroid disorder, and subacute panencephalitis. The hearing abnormality[4],[6],[7] in congenital rubella occurs in 58% of cases. Bilateral eye involvement is seen in 43% of cases. Only 50% of congenital rubella patients present with congenital heart disease. Less than 5000 cases of congenital rubella are notified per year in India. Hence, it is very rare to see a patient like this girl having the classical triad along with mild mental retardation.

In rubella, there occurs diffuse mottling of the retinal pigment epithelium with focal areas of decreased and increased pigmentation giving the characteristic salt-and-pepper appearance of the fundus.[8] The neural retina and choroid are unaffected. It usually involves the posterior pole.

The vision and electroretinogram response almost remain normal.[8] The choroidal neovascular membrane can develop as a very rare complication due to the progressive atrophy of retinal pigment epithelium.[9],[10] Later on, the development of diabetes mellitus and thyroidopathy is more in these group of patients. Hence, during the follow-up period, we have to look for diabetic retinopathy[11] also in adults. We have advised the parents to bring the girl for follow-up every year to do visual assessment, field testing, and to look for the development of choroidal neovascular membrane.

She underwent cochlear implant surgery on October 5, 2017 at our hospital. Now, the child has good hearing detection, speech perception, sound perception, and good communication skills.

A cochlear implant is an electronic medical device that works to perform the function of damaged inner ear cochlea to provide sound signals in the brain.

  Conclusion Top

Due to the successful immunization program was initiated in 1969, the congenital rubella syndrome rarely present today. The best way to prevent this is to vaccinate women who are planning to get pregnant at least 28 days beforehand[12] at least 28 days beforehand. This case was presented for its extreme rarity with the classical triad which has been managed well.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Dewan P, Gupta P. Burden of congenital rubella syndrome (CRS) in India: A systematic review. Indian Pediatr 2012;49:377-99.  Back to cited text no. 1
Vijayalakshmi P, Kakkar G, Samprathi A, Banushree R. Ocular manifestations of congenital rubella syndrome in a developing country. Indian J Ophthalmol 2002;50:307-11.  Back to cited text no. 2
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Stuckey D. Congenital heart defects following maternal rubella during pregnancy. Br Heart J 1956;18:519-22.  Back to cited text no. 3
Jivraj I, Rudnisky CJ, Tambe E, Tipple G, Tennant MT. Identification of ocular and auditory manifestations of congenital rubella syndrome in Mbingo. Int J Telemed Appl 2014;2014:981312.  Back to cited text no. 4
Khandekar R, Al Awaidy S, Ganesh A, Bawikar S. An epidemiological and clinical study of ocular manifestations of congenital rubella syndrome in Omani children. Arch Ophthalmol 2004;122:541-5.  Back to cited text no. 5
Gray RF. Causes of deafness in schools for the deaf in Madras. Int J Pediatr Otorhinolaryngol 1989;18:97-106.  Back to cited text no. 6
Rahman MM, Khan AM, Hafiz MM, Ronny FM, Ara S, Chowdhury SK, et al. Congenital hearing impairment associated with rubella: Lessons from Bangladesh. Southeast Asian J Trop Med Public Health 2002;33:811-7.  Back to cited text no. 7
Rubella Clinical Education Basic and Clinical Science Course – Excerpt – AAO. Available from: https://www.aao.org/ bcscsnippetdetail.aspx? id=9f181fb5-23c8-4586-9e6a-21961d6bf784. [Last accessed 2018 Sep 15].  Back to cited text no. 8
Menne K. Congenital rubella retinopathy – A progressive disease. Klin Monbl Augenheilkd 1986;189:326-9.  Back to cited text no. 9
Slusher MM, Tyler ME. Rubella retinopathy and subretinal neovascularization. Ann Ophthalmol 1982;14:292-4.  Back to cited text no. 10
Givens KT, Lee DA, Jones T, Ilstrup DM. Congenital rubella syndrome: Ophthalmic manifestations and associated systemic disorders. Br J Ophthalmol 1993;77:358-63.  Back to cited text no. 11
Robertson SE, Cutts FT, Samuel R, Diaz-Ortega JL. Control of rubella and congenital rubella syndrome (CRS) in developing countries, part 2: Vaccination against rubella. Bull World Health Organ 1997;75:69-80.  Back to cited text no. 12


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


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