Hydrocephalus in Congenital Rubella Syndrome A Case Report

Abstract

The Congenital Rubella Syndrome is a multisystemic disease and CNS involvement occurs in the form of microcephaly, mental/ motor disabilities, leptomeningitis, encephalitis, vascular damage and retardation of myelination. We report a case of gross non-communicating hydrocephalus in a neonate of Congenital Rubella Syndrome which is a rare presentation.

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Abstract
e Congenital Rubella Syndrome is a multisystemic disease and CNS involvement occurs in the form of microcephaly, mental/
motor disabilities, leptomeningitis, encephalitis, vascular damage and retardation of myelination. We report a case of gross non-
communicating hydrocephalus in a neonate of Congenital Rubella Syndrome which is a rare presentation.
Introduction
Hydrocephalus in Congenital Rubella Syndrome: A Case Report
Tiwari C, Sandlas G, Jayaswal S and Shah H*
Department of Paediatric Surgery, TNMC & BYL Nair Hospital, Mumbai, Maharashtra, India
*Corresponding author: Shah H, Department of Paediatric Surgery, TNMC & BYL Nair Hospital, Mumbai,
Maharashtra, India, E-mail: hemanshisshah@gmail.com
Citation: Tiwari C, Sandlas G, Jayaswal S, Shah H (2014) Hydrocephalus in Congenital Rubella Syndrome:
A Case Report. J Immunol Infect Dis 1(1): 108
Case Report Open Access
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Congenital Rubella Syndrome is one of the most important and common causes of birth defects. e major defects due to CRS
occur in the form of sensorineural, eye defects, cardiovascular defects and brain damage.
Key Words: Congenital Rubella Syndrome; Hydrocephalus Leptomeningitis; Greg’s Triad; Expanded Congenital Rubella Syndrome
Congenital Rubella Syndrome occurs due to in-utero infection of the fetus early in pregnancy by rubella virus. e virus has
teratogenic properties and crosses the placenta and infects the fetus where it stops cells from developing or destroys them by direct
involvement of viral replication in cell clones during rst trimester of pregnancy. When infection occurs within rst two months
of pregnancy, there are 90% chances of fetal transmission leading to death or Congenital Rubella Syndrome which reduce to 80%
in 8 to 12 weeks and 50% in 12 to 16 weeks. Deafness is most common in the latter period of gestation. Congenital anomalies with
growth impairment are rare if infection occurs aer 16 weeks of gestation. Hydrocephalus in a child suering from CRS is rare and
scarcely reported in the Literature. We report a case of hydrocephalus in a neonate of Congenital Rubella Syndrome (Figure 1).
Figure 1: Clinical Photos of the patient.
Case Summary
A 20 days old, female child with birth weight 2.5kg presented with increasing head size and bilateral corneal opacities since birth.
On examination, she had pallor, macrocephaly (Head Circumference-40cm, i.e., more than two standard deviations), sutural
separation, bilateral corneal opacities and nystagmus, bat-like and low set ears, clinodactyly of the right hand and bilateral at foot;
systemic examination was essentially normal. ere were no classical blue-berry mun rashes or microphthalmia. No history of
convulsions.
Routine blood investigations revealed microcytic hypochromic anaemia. IgG for Rubella was positive in high titres (>200AU/ml)
in both the mother and the baby.

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Ultrasound (USG) of the skull demonstrated a gross non-communicating hydrocephalus (V: H Ratio-0.9) with thinned out
neuroparenchyma and hypoplastic cerebellum. USG Spine revealed a 10cm bony defect at cauda equina though the child had no
neurosensory symptoms or any external stigmata of spina bida occulta. USG abdomen was suggestive of mild hepatosplenomegaly.
Computed Tomography (CT scan) of the Brain revealed grossly dilated lateral ventricles with severe thinning of neuroparenchyma
with intraventricular haemorrhage in right lateral ventricle and a small posterior fossa with hypoplastic cerebellum and brainstem
(Figure 2). No evidence of cerebral calcication. Cerebro-spinal Fluid examination was normal (Figure 3). 2-D Echocardiography
revealed Patent Ductus Arteriosus (PDA) and Ventricular Septal Defect (VSD). Ophthalmic examination showed right corneal
opacity and le corneal haziness. Fundus examination could not be done because of corneal pathology bilaterally.
Figure 2: CT Brain showing Hydrocephalus
e child’s mother was registered and immunized for Tetanus Toxoid but not for Rubella Vaccine. She had no symptoms during
her pregnancy (i.e., fever with rashes). e child was rst birth order and was delivered normally. No history was suggestive of any
perinatal infection.
Ventriculo-Peritoneal Shunt insertion has been done and the baby is on regular follow-up (Figure 4).
Figure 3: Cerebro-spinal Fluid of the patient.
Figure 4: Chamber of Ventriculo-peritoneal Shunt.

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Congenital Rubella Syndrome is an ensemble of anomalies in an infant as a result of primary infection due to Rubella virus in
a non-immunized pregnant woman and subsequent fetal infection. Its incidence has greatly decreased aer the introduction of
Rubella vaccine [1]. It has been estimated that 100,000 infants are aected every year globally [2], more so in the third world
countries where immunization with rubella vaccine is not routinely done. e incidence in India is 3 to 5 per 1000 live births [3].
Congenital Rubella Syndrome is probably underdiagnosed [2], especially among the aected children who have sensorineural
hearing loss as the only defect.
Discussion
Increased susceptibility to infection might be inherited as there is some indication that HLA-A1 or factors surrounding A1 on
extended haplotypes are involved in virus infection or non-resolution of the disease [4].
Rubella, also known as German measles was rst described by the German around mid-18th century. Rubella embryopathy was
documented early in 1941 by an Australian Ophthalmologist-Sir Norman Gregg from Sydney. Chess et al expanded the classic
triad of Congenital Rubella Syndrome to include all other various defects (Expanded Congenital Rubella Syndrome) [5,10].
e classic triad of Congenital Rubella Syndrome (also called the Greg’s Triad) [5] involves-Sensorineural deafness (~58%),
Eye abnormalities like microphthalmia, cataract and retinopathy (~43%) and Congenital Heart Diseases like PDA, VSD, Atrial
Septal Defects (ASD), Coarctation of aorta (~50%). Other manifestations are spleen, liver and bone marrow involvement,
mental retardation, microcephaly, Low Birth Weight, hepatomegaly, micrognathia and thrombocytopenic purpura leading
to the characteristic Blueberry Mun Rash. e child is also predisposed to developmental delay, growth retardation, autism,
schizophrenia, learning disabilities, diabetes and glaucoma [5,11].
Congenital Rubella Syndrome mostly involves the Central Nervous System, which occurs in the form of persistence of rubella
virus in the CNS and neurosensory structures like eyes and ears leading to leptomeningitis, encephalitis, vascular damage and
retardation of myelinization. It also aects cell growth. Lethargy, bulging fontanelle, irritability, motor disturbances and dysphasia
are common. ere may be moderate increases in spinal uid proteins and cells [6].
Hydrocephalus is a very rare presentation in Congenital Rubella Syndrome with very few cases been reported in literature [7]. e
development of hydrocephalus in this child may be attributed to be the sequalae of some form of leptomeningitis and also to the
Intraventricular haemorrhage seen in CT Brain. ere may be some form of Arnold Chari Malformation in this child in view of
the USG Spine nding.
e diagnosis of Congenital Rubella Syndrome is made by increased levels of rubella IgG or seroconversion with IgM antibodies;
low avidity of IgG; IgM in fetal blood (aer 22 weeks); gene amplication of viral genome in amniotic uid; or the presence of
specic IgM antibodies in the newborn [8,12].
Prognosis depends on the extent of damage. e mortality rate in severely aected neonates is 10% to 20%. Serious permanent
damage occurs in ~50%. About 5% have some form of thyroid disease in teenage years and 20% have diabetes by 35 years. Long-
term follow-up must continue at least through the rst decade of life to detect late-appearing decits.
Conclusion
ere is no denitive cure of this congenital condition and the management consists of treating the specic anomalies. Prevention
is the best cure which can be achieved by aggressive vaccination of all females as per the National Immunization Programme.
Females can be vaccinated upto 28 days before pregnancy but being a live vaccine, it is contraindicated during pregnancy [13].
Infants with CRS should be considered infectious until they are at least 1 year old (as they can shed the virus for prolonged periods
even up to 1 year of age or longer) or until two cultures of clinical specimens obtained one month apart aer the infant is older
than three months of age are negative for rubella virus. Infants with CRS should be placed in contact isolation during any hospital
admission before age one year or until the infant is no longer considered infectious [9].
References
1. O’Donnell N (1996) History of the congenital rubella syndrome. J Vocat Rehabil 6: 149–57.
4. George B. Olson, Peter B. Dent, William E. Rawls, Mary Ann South, JR. Montgomery, et al. (1968) Abnormalities of in vitro lymphocyte responses during rubella
virus infections. J Exp Med 128: 47–68.
3. Robertson SE, Cutts FT, Samuel R, Diaz- Ortega JL (1997) Control of rubella and congenital rubella syndrome (CRS) in developing countries, part 2: vaccination
against rubella. Bull World Health Organ 75: 69–80.
2. World Health Organization (2009) Surveillance Guidelines for Measles, Rubella and Congenital Rubella Syndrome in the WHO European Region. Geneva,
Switzerland 1-48.
5. Burgess MA (1991) Gregg’s rubella legacy 1941–1991. Med J Aust 155: 355–7.
6. Brown AS (2006) Prenatal Infection as a Risk Factor for Schizophrenia. Schizophr Bull 32: 200-2.

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10. Robert-Gnansia E (2004) Congenital rubella syndrome. Orphanet Encyclopedia1-2.
9. Greaves WL, Orenstein WA, Stetler HC, Preblud SR, Hinman AR, et al. (1982) Prevention of rubella transmission in medical facilities. JAMA 248: 861-4.
11. Gale EA (2008) Congenital rubella: citation virus or viral cause of type 1 diabetes? Diabetologia 51: 1559–66.
8. Peckham CS (1972) Clinical and laboratory study of children exposed in utero to maternal rubella. Arch Dis Child 47: 571-77.
7. Sriparna Basu, Aditya Kumar Gupta, Ayan Kumar, Poonam Singh, Ashok Kumar (2009) Communicating hydrocephalus in a case of Congenital Rubella
Syndrome. J Pediat Neurol 7: 423-6.
13. World Health Organization (2002) Department of Vaccines and Biologicals. WHO vaccine preventable diseases monitoring system: 2002 global summary.
Geneva, Switzerland.
12. Grin DE (2007) Alphaviruses Fields Virology (5th edition) Lippincott, Williams & Wilkins Publishers, Philadelphia, USA 1023–68.