Back ground:Congenital rubella syndrome (CRS) is a triad of congenital malformations (Greg’s triad) which includes hearing impairment, congenital cataract, heart disease. Apart from Greg’s triad, CRS may present with congenital glaucoma, pigmentary retinopathy, purpura, hepatosplenomegaly, jaundice, microcephaly, developmental delay, meningoencephalitis or radiolucent bone disease.

Infants and children with CRS need anaesthesia for surgeries (ophthalmic, cardiac and cochlear implant) as well as for imaging studies. Even after improvement of anesthetic techniques, these cases are still an anesthetic challenge due to scarce literature on perioperative management of such children. We performed a retrospective review of the perioperative course of children with CRS, who underwent ophthalmic surgery in our institute.

Material and Methods: Institutional ethical committee approval was obtained for this retrospective study. Medical records of children with CRS who have had ophthalmic surgeries in our institute were collected and reviewed. Children with CRS ,less than 10 years of age who underwent ophthalmic surgery or examination under anesthesia (EUA) were included for analysis.
Results:Total 46 cases were eligible for analysis; demographics and other preoperative variables were analyzed . Out of 46 children, 28 were infants and 18 were more than 1 year old. Among 46 children, 33 had congenital cataract  (71.74%) and congenital glaucoma (4) were next common condition. Congenital cardiac anomaly was recorded in 42 children.Patent Ductus Arteriosus was found in 16 children.  Pulmonary stenosis (PS) with PDA was present in five children.

History of limited activity, cyanosis, cyanotic spells, forehead sweating, suck–rest-suck cycle & failure to thrive were elicited from all patients and 12 (26.09%) parents gave a positive history for one or more.  Total of 13 children (28.26%) were on cardiac drugs like beta-blockers, diuretics & digoxin. Extracardiac manifestations like high arched palate, low set ears, or delayed milestones were present in 43.48% children. Mental retardation was present in 15 of 46 children.

Conclusion:
To conclude CRS is a multiorgan dysfunction with varied cardiac anomalies, knowledge about varied manifestations and apt clinical suspicion is vital. Extreme care should be exerted in children with CRS and symptomatic heart disease to improve perioperative outcomes

Anesthetic management of pediatric patients with Congenital Rubella syndrome: our experience and a review of the literature