Figure 2 . Chest x-ray ( A ) done at delivery revealing lower-than-expected lung volumes and Chest x-ray ( B ) done at six weeks of life with progressive pulmonary disease .
parents elected not to start ganciclovir or valganciclovir at that time .
Although the infant initially weaned off all respiratory support by nine days of life , she remained off support for only two days . Lung size was subtly smaller than expected on chest radiographs done shortly after birth ( Figure 2 ). There was a slow escalation in nasal cannula flow and oxygen requirements , and she was placed again on CPAP at a few weeks of life . She had increased work of breathing , oxygen requirements , and carbon dioxide retention , which slowly progressed and required further escalation to nasal intermittent positive pressure ventilation then non-invasive , neurally adjusted ventilatory assist ( NAVA ). She required intubation for mechanical ventilation at six weeks of life . The infant ’ s respiratory status continued to worsen on conventional ventilation over the next week . The infant developed severe pulmonary hypertension with hypoxemia on 100 % fraction of inspired oxygen . She was placed on inhaled nitric oxide . Chest radiographs done at this time showed poorly-expanded lung fields occupying less of the thorax than expected despite high ventilator pressures ( Figure 2 ).
CMV viral load was 410 337 IU / mL ( increased from 286 IU / mL on day of life one ), and ganciclovir was started at this time . Broad spectrum antibiotics were administered due to clinical decompensation with negative blood cultures and a tracheal aspirate that was positive for Serratia marcescens and Acinetobacter species .
High-frequency ventilation was attempted on multiple occasions ; however , the neonate did not tolerate this , requiring chest compressions for bradycardia twice during these trials . The infant was sustained on pressure-control ventilation with mean-airway pressures of 22 , 100 % fraction of inspired oxygen , and 20 parts per million of inhaled nitric oxide . The infant required dopamine and norepinephrine infusions to maintain an adequate blood pressure . After maintaining marginal oxygenation for one week on this support , the infant became progressively edematous and acidotic . Eventually , the infant was unable to achieve oxygen saturations above 60-70 % despite increasing ventilator support . After discussion with the parents , the infant was removed from the ventilator and died within a few minutes . Autopsy was declined .
This case illustrates the findings of pulmonary hypoplasia with progressive development of respiratory failure and pulmonary hypertension , which was ultimately fatal in an infant with congenital CMV infection . Evidence pertaining to prenatal and postnatal treatment options for this condition are limited and more data is needed to guide the management of infants with overwhelming congenital CMV infection .
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For additional references , email ams @ arkmed . org .
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