Perioperative respiratory complications caused by cystic lung malformation in Proteus syndrome.

A 7-year-old girl with Proteus syndrome, complicated with severe kyphoscoliosis and cystic lung malformation, developed respiratory complications during and after posterior spinal fusion under general anesthesia. We speculated that low oxygenation while in the prone position contributed to ventilation perfusion mismatch owing to the cystic lung malformation that existed predominantly in the right lung, and that postoperative respiratory failure was caused by initially increased bronchial mucous secretions and secondary persistent bacterial pneumonia. Cystic lung malformation rather than reduced respiratory function, although both can be a cause of mucous clearance impairment, should be considered as one of the predictive factors for perioperative respiratory failure in Proteus syndrome.