Central hypoventilation and diaphragmatic eventration: diagnosis and management.

Congenital central alveolar hypoventilation syndrome (CCAHS) is a disorder of ventilatory control that occurs without neuromuscular blockade or pulmonary disease. It is characterized by a lack of response to habitual respiratory stimulants, especially hypercapnia. In this article, the management of this syndrome by diaphragmatic pacing is discussed. Paralysis of the phrenic nerve in small children usually results from injury during birth or during a cardiothoracic operation and results in eventration of the hemidiaphragm. Alternatively, eventration of the diaphragm may be a congenital condition. In both cases it may lead to respiratory distress in the newborn. In this article, we review the diagnosis of these conditions and their management, focusing on the surgical indications. We also discuss outcome.