Anesthetic care for the child with congenital central alveolar hypoventilation syndrome (Ondine's curse)

Idiopathic congenital central alveolar hypoventilation syndrome, otherwise known as Ondine's curse, is a rare neuropathologic syndrome characterized b y an inadequate respiratory drive with hypoventilation and periods of prolo nged apnea resulting in hypercarbia and hypoxemia. Although no definite pat hologic abnormality has been identified to account for the disorder, it is thought to represent a primary defect related to altered function of centra l chemoreceptors resulting in defective control of minute ventilation. Asso ciated problems related to neural crest cell migration, including neuroblas toma formation and Hirschsprung's disease, suggest that the primary defect is defective neural crest cell migration and function. Problems that may im pact on perioperative care include the defective central control of ventila tion and defective control of upper res;respiratory musculature, which may lead to upper. airway obstruction. Although many;patients will have previou sly undergone tracheostomy anti chronic mechanical ventilation, problems in other organ systems can impact on perioperative care. Cardiovascular issue s include the possible presence of cor pulmonale and autonomic nervous syst em dysfunction. Central nervous system issues include the frequent occurren ce of seizures and mental retardation. The preoperative work-up, premedicat ion, and the intraoperative/postoperative care and monitoring of these pati ents is reviewed. (C) 1999 by Elsevier Science Inc.