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SCIENTIFIC LETTER TO THE EDITOR
Congenital Rubella Syndrome
Meenu Pandey & Ajay Dudeja & Vikram Datta &
Bhupesh Singla & Arvind Saili
Received: 26 December 2011 /Accepted: 10 May 2012# Dr. K C Chaudhuri Foundation 2012
Sir,Congenital Rubella Syndrome (CRS) is a transplacentallyacquired rubella infection which leads to multiple organdefects. We are presenting three babies with CRS diagnosedwithin a span of 2 mo.
The first patient was a premature (35 wk), baby girl bornwith an Apgar score (AS) of 7, 8. On day one, she hadbluish-purplish papulo-nodular, non blanchable and nontender rash over the cheeks, trunk and upper limbs(Fig. 1). The rash was identified as blueberry muffin rashand confirmed on skin biopsy. She was diagnosed withCRS. The echocardiogram showed mild valvular pulmonarystenosis (PS) with a patent foramen ovale. The baby wasdischarged on day 18. She has been followed till the age of2 y and is presently having global developmental delay. Shehas been referred to the ENT department for hearingimpairment.
The second patient was a preterm (34 wk) baby (AS 8, 9),who also had blueberry muffin rash on day 1. He also had asmall patent ductus arteriosus (PDA). He was diagnosed asCRS. The baby was discharged on day 16. The third patientwas a term baby boy, born vaginally (AS 7, 9). At birth,this baby had bilateral corneal opacities, diagnosed as
buphthalmos (Fig. 2). The echocardiogram revealed Tetral-ogy of Fallot (TOF), with PS, with PDA and a septumsecundum Atrial Septal Defect (ASD). The diagnosis of
Fig. 1 The blueberry muffin rash involving the face and neck in thechild
Fig. 2 Buphthalmos in the baby boy
A. Dudeja :V. Datta :A. SailiDivision of Neonatology, Department of Pediatrics,Lady Hardinge Medical College and associatedKalawati Saran Children’s Hospital,New Delhi, India
M. Pandey (*) : B. SinglaDepartment of Pediatrics, Lady Hardinge Medical Collegeand associated Kalawati Saran Children’s Hospital,New Delhi 110001, Indiae-mail: [email protected]
Indian J PediatrDOI 10.1007/s12098-012-0782-0
CRS was made. The skeletal survey showed a moth eatenappearance in the long bones (classic metaphyseal translu-cencies) (Fig. 3). The baby was discharged on day seven.However, he died at 1.5 mo due to heart disease.
The salient features of these three patients are shown inTable 1.
Rubella is a communicable exanthematous diseasecaused by Rubella virus. Any injury affecting the fetus inthe first trimester (the phase of organogenesis) results incongenital organ defects. The manifestations of CRS in-clude transient manifestations like hepatitis, thrombocyto-penia, and blueberry muffin rash. Permanent manifestationsinclude deafness, eye lesions, cardiac lesions, microcephaly,and mental retardation. The European Regional Committeeof World Health Organization had adopted the goals of“Elimination of CRS” by 2000AD in the Health for Allprograms [1], which still remains a distant dream. We fear
that cases of CRS may be increasing, although more data isrequired to conclusively prove this. Our work highlightspeculiar presenting features of a patient with CRS, likeblueberry muffin rash and buphthalmos, which led to thework up of these patients and established the diagnosis ofCRS in all three.
Reference
1. World Health Organization. Expanded pro-gram on immunization.European conference on immunization policies. Wkly Epid Rec.1985;60:165–72.
Table 1 Comparison of findings seen in the three cases
Case 1 Case 2 Case 3
IUGR + + +
Birth weight 1.145 kg 1.085 kg 1.64 kg
Antenatal history Uneventful Uneventful Uneventful
Blueberry muffin rash + + −
Microcephaly + + +
Thrombocytopenia + + +
Cardiac involvement + + +
Ophthalmologicalinvolvement
− − +
Bony involvement − − +
Hepatitis − − −
Deafness + NKa NKa
Mental retardation + NKa NKa
Serological confirmation + + +
a Not Known
Fig. 3 Radiograph showing metaphyseal translucencies in the longbones (femur)
Indian J Pediatr