Congenital Cytomegalovirus Infection: The Elephant in Our Living Room.

Congenital cytomegalovirus (cCMV) canbe considered anelephant in our living room. Since Weller1 published his landmark article in 1971 reporting the ubiquity of the problem of cCMV infection in theUnited States and stressing itsmajor public health importance, cCMVhasbeen recognizedas a common congenital infection that can cause liver disease, thrombocytopenia,hearingandvision loss,microcephaly, and neurodevelopmental disorders. Despite how long we have known how common and destructive CMV can be, little has been done to stop this virus from affecting our newborns. A logical next step toward getting our sights on the elephant in our living room is to screen newborns for cCMV infection at birth.2 Two approaches have been proposed: targeted CMV testing (or targeted CMV screening) of newborns with failedhearing screens anduniversal CMVscreeningof all newborns. Both approaches are evaluated by Gantt and colleagues3 in this issueof JAMAPediatrics. Because cCMV infection isacommoncauseofcongenitalhearing loss,manyhospitals and 2 states (Utah and Connecticut) have already adopted targeted CMV testing (or targeted CMV screening) for newbornswho fail their newbornhearing screens.4 Universal newborn CMV screening, however, remains elusive. If adopted, universal newborn CMV screening will appropriately robCMVof its invisible elephant in the living roomstatus and force us to see the pachyderm on our couch. The newborns identified throughscreeningwillbenefit fromsensoryandneurodevelopmental follow-up, speech and language programs, and educational accommodations to optimize their potential. Severely affected infants may require antiviral treatment, surgical procedures, and hearing devices to help them successfully navigate their childhood and adolescence. Through the programs that identify these newborns, clinicians, audiologists, laboratories, policy makers, and public health officials also will be forced to face the tens of thousands of newborns born each year with cCMV infection and to develop practical and effective management and prevention strategies.5 Does cCMV hit themark? Does it satisfy accepted criteria fornewborn screening? Is itworthyofuniversal screening and for acceptance to the Recommended Uniform Screening Panel?6,7 For a newborn condition to be considered a worthy candidate for universal newborn screening, the condition should represent a significantpublichealthproblem.Congenital CMV hits the bull’s eye here! It is a common congenital infection present in 0.4% to 1% of newborns that for decades has been underappreciated. It is a common cause of progressive sensorineural hearing loss as well as ocular and visual disorders, microcephaly, developmental andmotordisabilities, liverdisease, and growth failure.4 An accurate, reliable, validated, and low-cost method of detection shouldalsobeavailable andpreferably tested inprospective population-based studies. Another near-the-center target hit! Detection of cCMV can be easily performed using a variety of tests, including viral culture and shell vial culture assay on urine or saliva samples and CMV DNA polymerase chain reactionor other nucleic acid test detectionmethodson urine, saliva, or dried blood spot (Guthrie cards) samples already obtained for newborn screening of genetic and metabolic disorders. These detection methods have been validated in large-scale population studies.8,9 Congenital CMV infection has a diagnostic window of opportunity in the first 21 days of life, however, after which the diagnosis is confoundedbyperinatal transmission frommaternal sources, such as cervical vaginal secretions during delivery and breastmilk feedings, andfrompostnatal sources frombloodproduct transfusions and person-to-person transmission. Therefore, CMV testing in the newborn period is key. Furthermore, screening programs for all newborns would eliminate “the diagnostic odyssey”2(p293) experiencedbymanychildrenwith cCMVwho present after the newborn period. A condition under consideration for universal newborn screening also should have treatments or interventions with provenefficacy.Another clean shot!Randomizedclinical trials have shownearlyneonatal treatment, initiatedwithin the first month of life, with intravenous ganciclovir or oral valganciclovir hydrochloride provides benefit for newborns diagnosedwith cCMVdisease by improving head size growth and developmental milestones and reducing the risk for hearing loss and its progression into childhood.10 In addition, early identification and treatment of hearing loss,whether congenitalor lateronset, results in improvedspeechand languageoutcomes as well as improved behavior and school performance duringchildhood.11For these interventions toprovide themost benefit, health care systems and public health and education programsshouldbecapablenotonlyof conducting thescreening programs, but also be braced and adequately prepared to provide theneeded follow-up and indicated interventions for the tensof thousandsof newbornswhowill be identified each year as having cCMV infection. If cCMV hits the mark and satisfies the criteria for a condition worthy of universal newborn screening, what are the Related article Opinion