Rubella and Congenital Rubella Syndrome

A rubella epidemic in USA in 1964–1965 resulted in 12.5 million cases of rubella infection and about 20,000 newborns with CRS.

The number of reported cases of rubella in USA remains low, with a median of 11 cases annually in 2005–2011 because of vaccination.

The overall burden of CRS is still high in developing countries. There were 66 cases reported in Bangladesh, 26 in Romania, 16 in Nepal, 10 in Zambia, 9 in Japan, and 4 in Sri Lanka in 2014.

Incidence of cardiac defects in CRS with eye involvement could be as high as 95%. Most common cardiac anomaly in CRS is PDA.

CRS is a constellation of multisystem abnormalities

Such pts may require cardiac surgery like congenital cardiac septal defect correction and/or other noncardiac surgery like cleft lip/cleft palate repair and a variety of eye procedures under anesthesia. Cataract extraction is an urgent vision saving procedure, so complete optimization (correction of cardiac defects, adequate weight gain) of neonate may not be possible.

Unexpected difficult intubation in various upper airway anomalies like subglottic stenosis, shortened trachea, and short glottis carinal length associated with many congenital syndromes.

Hypothermia.

Hypoglycemia: Exogenous sodium, water and glucose should be provided periop, as they have low GFR and are more prone for hypoglycemia.

Balance of PVR and SVR.

Drug metabolism may be deranged because of associated liver abnormalities and hypothyroidism.

IE prophylaxis is essential, as the turbulent flow produced by the high velocity systolic jet in pulm artery stenosis increases the potential for development of endocarditis.

Rubella is a viral illness characterized by a mild, maculopapular rash. The rubella rash occurs in 50–80% of rubella-infected persons and is sometimes misdiagnosed as measles or scarlet fever.

Rubella is contagious disease which spreads in droplets. The respiratory secretion, cataractous lens is one of the most infectious materials hence warrants universal precaution.

CRS is an illness resulting from rubella virus infection during pregnancy. When rubella infection occurs during early pregnancy, serious consequences—such as miscarriages, stillbirths, and a constellation of severe birth defects in infants—can result. The risk of congenital infection and defects is highest during the first 12 wk of gestation and decreases after the 12th week of gestation, with defects rare after the 20th wk of gestation.

Common congenital defects of CRS include cataracts; congenital heart disease, including PDA, coarctation of aorta, VSD, ASD, and pulm artery stenosis; hearing impairment and developmental delay; brain damage (microcephaly, mental retardation, meningoencephalitis); hepatosplenomegaly; thrombocytopenia; and neonatal jaundice. Other manifestations are type I diabetes mellitus, growth retardation, transient hemolytic anemia, metaphyseal “celery stalking” changes in long bones, transient pneumonitis, transient generalized lymphadenopathy, cryptorchidism, inguinal hernia, and dermal erythropoiesis (“blueberry muffin syndrome”). Infants with CRS usually present with more than one sign or symptom consistent with congenital rubella infection. However, infants may present with a single defect. Hearing impairment is the most common single defect.

Rubella is a viral illness caused by a togavirus of the genus Rubivirus , which is most closely related to group A arboviruses, such as eastern and western equine encephalitis viruses.

It is an enveloped RNA virus with a single antigenic type that does not cross-react with other members of the togavirus group.

Rubella virus is relatively unstable and is inactivated by lipid solvents, trypsin, formalin, ultraviolet light, low pH, heat, and amantadine.

Rubella is transmitted through direct or droplet contact from nasopharyngeal secretions and has an average incubation period of 17 d (range: 12–23 d). Persons with rubella are most infectious when rash is erupting, but they can shed the virus from 7 d before to 7 d after rash onset. CRS develops in an infant as a result of maternal infection in first trimester and subsequent fetal infection with rubella virus (German measles). Because infants can shed the virus for prolonged periods (up to 1 y of age or longer), infants with CRS should be considered infectious until they are at least 1 y old or until two cultures of clinical specimens obtained 1 mo apart after the infant is >3 mo of age are negative for rubella virus.