Bronchial atresia in a neonate with congenital cytomegalovirus infection
A 9-day-old boy presented with a history of increased work of breathing and cyanosis. He was born at 40 weeks gestation via vacuum extraction for fetal distress. His birth weight was 3.0 kg with normal Apgars and did not require any resuscitation. Meconium-stained liquor was noted at delivery. He was discharged home at 48 h of age. Antenatal ultrasound at 22 weeks of gestation demonstrated hyper-echoic changes in the thorax and abdomen which did not progress throughout pregnancy. At presentation to the hospital, he was in moderate respiratory distress and was commenced initially on continuous positive airway pressure, but he required mechanical ventilation for respiratory deterioration in the 2nd week of life. He had a normal white cell count and C-reactive protein. Blood cultures were negative. CMV was detected by polymerase chain reaction (PCR) in urine and blood, and in endotracheal aspirate samples. In addition, stored blood samples (from newborn screen) taken on day 2 of life were also positive for CMV by PCR. CMV immunoglobulin M antibody was also positive. A head ultrasound demonstrated diffuse peri-ventricular calcification. A chest computed tomography (CT) scan was performed [Figure –]. Flexible bronchoscopy and echocardiography were normal.\nDue to an ongoing requirement for mechanical ventilation associated with left mediastinal shift and lung compression from an overinflated right middle lobe (RML), the child had a RML lobectomy [].\nPathology revealed a bronchocele with an 11-mm mucus plug [] in a sub-segmental bronchus of the RML. Although bronchi were seen to arise from the cyst and communicate with the distal lung causing marked over-inflation, no direct continuity was identified between the bronchocele and proximal bronchi. Cytomegalovirus inclusions with minimal surrounding inflammation were noted on microscopy of the peripheral lung.\nAfter excision of the RML, the patient was extubated and gradually weaned off oxygen. However, following anesthesia for a central line placement at 1 month of age for a 6-week course of ganciclovir for cCMV infection, he developed further respiratory distress. A repeat CT scan demonstrated persistent hyperinflation of the residual right lung, especially the right lower lobe. Instead of further lobectomy with permanent loss of lung mass, right lung volume reduction surgery was performed. There was subsequent marked clinical improvement and he was discharged home aged 3½ months and continues to thrive with no respiratory distress.