Case Study by C . Preston Pugh , MD 1 ; Jennifer A . Rumpel , MD 1 ; David N . Matlock , MD 1
Department of Pediatrics , University of Arkansas for Medical Sciences , Little Rock , Ark .
Progressive Respiratory Failure with Pulmonary Hypoplasia and Persistent Pulmonary Hypertension Associated with Congenital Cytomegalovirus Infection
Congenital cytomegalovirus is one of the most common intrauterine infections of human birth , and while most infected infants are asymptomatic , those infants that are symptomatic are often premature infants that may develop one or more deficits . In this case , we present an infant that developed progressive respiratory failure with pulmonary hypoplasia , which has rarely been associated with congenital cytomegalovirus infection and that ultimately led to demise . Evidence pertaining to prenatal and postnatal treatment options for this condition is limited , and more data is needed to guide the management of infants with overwhelming congenital cytomegalovirus infection .
delays , seizures , sepsis-like syndrome , or chorioretinitis . 1 , 3 However , lung complications have also been reported as a rare manifestation of symptomatic CMV infections in infants . 4
Lung complications have mostly been described in the literature as interstitial pneumonitis , bronchopulmonary dysplasia , cystic interstitial emphysema , and interstitial pneumonia ; but there have also been a few reports of pulmonary hypoplasia , with fetal ascites associated with congenital CMV infections . 4-8 The diagnosis of pulmonary hypoplasia has been associated with significant neonatal morbidity and up to 70 % mortality . 9 While there are only a few reports of lung disease , particularly
The growth restriction had prompted referral to maternal-fetal medicine specialists who sent prenatal testing , which identified elevated immunoglobulin titers and avidity for CMV . The infant delivered early and by caesarean section due to non-reassuring fetal heart tones detected during monitoring , which was performed due to the growth restriction . She required resuscitation at birth including bag-mask ventilation , but transitioned to nasal continuous positive airway pressure ( CPAP ) then weaned off respiratory support over the next few days . She did not receive surfactant .
An echocardiogram shortly after birth revealed a structurally normal heart with a
Figure 1 . Cranial sonography images revealing periventricular calcifications and a right posterior occipital cyst .
Congenital cytomegalovirus ( CMV ) is one of the most common intrauterine infections of human birth , with a reported prevalence ranging from 0.2 % to 2.2 %. 1 At birth , almost 90 % of infected infants are asymptomatic . The 10 % of infected infants who are symptomatic are often premature infants born to seropositive mothers . 2 , 3 Most infants with symptomatic congenital CMV infection develop one or more deficits including sensorineural hearing loss , mental disability , motor pulmonary hypoplasia in congenital CMV infections , symptomatic cases in immunocompromised or preterm infants have resulted in devastating outcomes . As such , we present the case of a six-week-old infant who died from progressive respiratory failure with pulmonary hypoplasia attributable to congenital cytomegalovirus infection .
A 1520-gram baby girl was born to a 20-year-old mother following a 35-week , four-day gestation , which was complicated by intrauterine growth restriction . flattened interventricular septum consistent with mild pulmonary hypertension . Cranial sonography revealed periventricular calcifications and a right occipital cyst ( Figure 1 ). Liver enzymes were elevated , thrombocytopenia was present , and CMV was detected via polymerase chain reaction in the blood , urine , and cerebrospinal fluid . Retinal examination did not reveal signs of retinitis . Bilateral hearing screens were failed . The infant was transferred to a level IV neonatal intensive care unit on day of life four for pediatric infectious disease consultation . After discussion of the risks and benefits of antiviral treatment , the
126 • The Journal of the Arkansas Medical Society www . ArkMed . org