[Problems in anesthesia in pulmonary cystic adenomatosis]

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare disease. It is about an abnormal proliferation of mesenchymal elements and failure of maturation of bronchiolar structures, characterized by the replacement of normal pulmonary tissue with "cysts" in variable size and number. These lesions communicate with the tracheobronchial tree. During fetal period hydrops and polyhydramnios can be associated with CCAM. A cystic adenomatoid malformation can be detected by antenatal ultrasound, and, at the birth, it is confirmed by chest radiography. From January 1990 to December 1998, 24 cases with CCAM came to our observation; 16 of these patients underwent surgery and 14 have come to a complete recovery. The newborns, with CCAM, can show early acute respiratory distress for rapid expansion of the cysts leading to compression of normal lung and mediastinal shift. Conventional mechanical ventilation may cause further expansion of the involved lobe with a ball-valve effect: this take a clinical deterioration. Perioperative ventilatory management with high frequency oscillation (HFO) is useful to stabilize and to improve arterial blood gases of this patients. At the moment, thanks to the early prenatal sonographic diagnosis, it is possible, and strongly advisable, after adequate serial checkings during the pregnancy, to refer CCAM cases to a tertiary centre that is properly equipped, where a poly-specialist team consisting of obstetrician, neonatologist, pediatric anesthetist and pediatric surgeon, will be able to plan and arrange in the best treatment necessary for the newborn.