|Year : 2021 | Volume
| Issue : 3 | Page : 566-567
Bilateral congenital rubella retinopathy in monochorionic monoamniotic twins: A case report
Suklengmung Buragohain1, Dipankar Das2, Nilutparna Deori3, Hemlata Deka4, Damaris Magdalene3, Anubhi Paharia1
1 Department of Ophthalmology, Uveitis and Neuro-Ophthalmology Services, Guwahati, Assam, India
2 Ocular Pathology, Uveitis and Neuro-Ophthalmology Services, Guwahati, Assam, India
3 Paediatric Ophthalmology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
4 Medical Retina, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
|Date of Submission||27-Oct-2020|
|Date of Acceptance||25-Jan-2021|
|Date of Web Publication||02-Jul-2021|
Dr. Suklengmung Buragohain
Sri Sankaradeva Nethralaya, 96 Basistha Road, Guwahati - 781 028, Assam
Source of Support: None, Conflict of Interest: None
Congenital rubella syndrome in twins is rare, and when present, there is a variable effect on the fetuses depending on unknown factors. The available literature on CRS in twins is very limited with only a few reported cases. We report a rare case of a pair of monochorionic monoamniotic twins who were diagnosed as having bilateral congenital rubella retinopathy in both eyes.
Keywords: Congenital rubella retinopathy, congenital rubella syndrome, monochorionic monoamniotic twins, pigmentary retinopathy
|How to cite this article:|
Buragohain S, Das D, Deori N, Deka H, Magdalene D, Paharia A. Bilateral congenital rubella retinopathy in monochorionic monoamniotic twins: A case report. Indian J Ophthalmol Case Rep 2021;1:566-7
|How to cite this URL:|
Buragohain S, Das D, Deori N, Deka H, Magdalene D, Paharia A. Bilateral congenital rubella retinopathy in monochorionic monoamniotic twins: A case report. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 26];1:566-7. Available from: https://www.ijoreports.in/text.asp?2021/1/3/566/320073
Congenital rubella syndrome (CRS) is a condition that is capable of inducing numerous multisystem abnormalities. Cardiac, ocular and hearing defects are the common involvements, although any organ can be affected in the fetus. The virus can have a variable effect on twins depending on unknown factors and literature on the subject is limited. We report possibly the first case of bilateral congenital rubella retinopathy in a pair of monochorionic monoamniotic twins in India.
| Case Report|| |
Two 8-month-old twins who were born to a 24-year-old mother, presented to our hospital with complaints of on and off rubbing of eyes. The pregnancy was uneventful with a full-term delivery. The mother underwent an anomaly scan at 21 weeks of pregnancy which showed monochorionic monoamniotic twins with no obvious fetal defects. The birth weight of twin A was 2.5 kilograms (kg) and that of twin B was 1.9 kg and developmental milestones were normal.
A thorough systemic examination (including imaging investigations) of the twins revealed no abnormality. On ocular examination, twin A and twin B both had central unsteady and maintained (C/US/M) fixation with nystagmus in both eyes (OU). In twin A bilateral esotropia was present as well. On examination under anesthesia (EUA) twin A had normal anterior segment findings with a hyperopic refractive error of +6.5 dioptres spherical (DS) in OU with an axial length (AL) of 18.83 mm and 18.43 mm in the right (OD) and left (OS) eyes, respectively. Posterior segment findings showed a fine pigmentary bony spicule like deposit at the macula of OU [Figure 1]a and [Figure 1]b with loss of the foveal reflex. The retina surrounding the vascular arcades appeared pale. Twin B similarly had normal anterior segment findings with a hyperopic refractive error of +6.5 DS in OU with an AL of 18.66 mm and 18.58 mm in OD and OS, respectively and the posterior segment showed a hyperpigmented clump surrounded by an area of hypopigmentation in the macula [Figure 1]c and [Figure 1]d.
|Figure 1: Colour fundus photograph (3nethra Neo (Forus Health)) showing a fine pigmentary deposit in the macula surrounded by an area of hypopigmentation in the eyes of twin A right eye (a) and left eye (b) and twin B right eye (c) and left eye (d)|
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Laboratory tests showed that serum IgG and IgM for Rubella were positive with low avidity in both the twins (Twin A: Serum IgG = 101.47 IU/mL, serum IgM = 7.31 IU/mL and avidity = 7%; Twin B: Serum IgG = 107.37 IU/mL, serum IgM = 5.8 IU/mL and avidity = 12%).
Taking into account the ocular features and the laboratory findings, both the twins were diagnosed as bilateral congenital rubella retinopathy and were kept on regular follow-up.
| Discussion|| |
Literature regarding CRS in twins is scarce. Although there have been a few reported cases,, the underlying pathogenesis of rubella infection in twin pregnancies is largely unknown. What is known however is that the effects on the fetus by the virus are dependent on the timing of the maternal infection. The approximate risk for malformations is about 90% if the infection occurs between weeks 2–10, 34% for weeks 11–18, and infection after 18 weeks usually results in no malformation.,
In our case, the twins were monochorionic monoamniotic, which means that the two fetuses shared a common placenta as well as an amniotic sac. The presence of a single placenta is of significance as infection and replication of the rubella virus in the placenta may increase the chances of infection to both the fetuses. As both the twins had ocular features of rubella retinopathy but were without any systemic abnormality, the mother was likely infected post 18 weeks of gestation.
Systemic abnormalities in CRS is a wide spectrum, nevertheless, a classic presentation is of cardiac, ocular, and hearing defects. Other abnormalities may include deafness, mental retardation, cardiovascular defects, and ocular defects. None of these features were present in the twins.
As with any other organ in the body, the eye is also commonly affected in CRS. The most common ocular feature in CRS is cataract, although it was absent in the twins. In our case pigmentary retinopathy was present. Pigmentary retinopathy is a known ocular feature of CRS and it occurs due to focal atrophy and pigment alterations of the retinal pigment epithelium. In a study by Vijayalakshmi et al., Rubella retinopathy was seen in 36% of cases of CRS in India. Even with pigmentary retinopathy, vision is known to remain stable, although the child needs to remain under regular follow-up to detect any subretinal neovascularization. Other common ocular features in CRS include microphthalmia, iris hypoplasia, nystagmus, concomitant strabismus, primary optic atrophy, congenital glaucoma, and dacryostenosis.
Many mothers are unaware of the infection during pregnancy and several cases go undetected without proper screening. This is of particular significance in a developing country like India where nearly 45% of females are susceptible to Rubella infection during pregnancy. It is also reported that Rubella is responsible for at least 25% of cases of congenital cataract in India, thereby adding to the burden of childhood blindness. CRS is a preventable condition and can be prevented with proper screening and vaccination. The occurrence of CRS is a reflection on the inadequacies in the provision of vaccination during pregnancy.
| Conclusion|| |
Our case is possibly the first reported case of bilateral congenital rubella retinopathy without systemic abnormalities in monochorionic monoamniotic twins in the Indian scenario. Routine vaccination during pregnancy in India especially in the rural setting is still a challenge. There is also a possible lacuna in the literature on CRS in twins and more studies on the subject would be beneficial.
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
Conflicts of interest
There are no conflicts of interest.
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