Anesthesia in an infant with congenital lobar emphysema (CLE)

BODY: A 2-month old, 4.1-kg baby girl was scheduled for right thoracotomy and lobectomy for congenital lobar emphysema. On preoperative physical exam, she was noted to be awake and tachypneic but not cyanotic. Her vital signs showed a blood pressure of 88/50, pulse rate of 110, respiratory rate of 58 with an oxygen saturation of 95% on 1 liter per minute of oxygen via nasal cannula. There was air entry in both lung fields but was decreased on the right. There were no cardiac murmurs heard and no tracheal deviation noted. She had a right chest tube placed in the emergency room. Her laboratory results were unremarkable. The chest X-RAY showed marked hyperinflation of the right lung extending across the midline with marked left mediastinal shift possibly secondary to suspected congenital lobar emphysema. There was no pneumothorax. A chest CT with contrast revealed marked emphysematous hyperexpansion of the right middle lobe with anterior herniation across the midline. There was minimal visualization of the right lower and right upper lobes which were compressed by the emphysematous lung. There was right-to-left mediastinal shift with compression of the left lung. The trachea and left bronchial tree were patent. The patient was brought to the operating room and ASA standard monitors were placed. The chest tube was connected to suction. Her peripheral intravenous access was infiltrated. A mask induction was started using sevoflurane and 100% oxygen while keeping the patient spontaneously breathing. After a peripheral intravenous access was obtained, fentanyl (1 mcg/kg) was administered in preparation for intubation. Peak airway pressure was kept at a minimum. At this point, the patient’s oxygen saturation was 100% with a stable heart rate and blood pressure. The trachea was intubated easily using 3.5 endotracheal tube. However, there were no breath sounds heard over either lung field or gastric region. There was neither chest rise nor end-tidal carbon dioxide noted. The oxygen saturation started to decrease to the low 70’s. The endotracheal tube was removed and mask ventilation resumed. At this time it was noted that it was increasingly difficult to ventilate the patient even with an oral airway in place. No breath sounds were audible and the oxygen saturation remained in the 80’s. However, the systolic blood pressure dropped to the low 60’s and the surgeon was asked to emergently open the right chest. Immediately after part of the emphysematous lobe herniated out of the chest, mask ventilation became easier and oxygen saturation increased to 98100%. The systolic blood pressure stabilized. The patient was reintubated and surgery started. The remainder of the case was uneventful.