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Clinical Pediatrics
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What's this?

Congenital Central Alveolar Hypoventilation Syndrome (Ondine's Curse) With Survival Into Adulthood

Benjamin D. Liess, MD

Department of Otolaryngology-Head and Neck Surgery University of Missouri-Columbia School of Medicine

Jamie S. Dost, BS

University of Missouri-Columbia School of Medicine

Jerry W. Templer, MD

Department of Otolaryngology-Head and Neck Surgery University of Missouri-Columbia School of Medicine

Joseph D. Tobias, MD

Departments of Anesthesiology and Pediatrics University of Missouri-Columbia, Columbia, Missouri, Tobiasj{at}health.missouri.edu

Ondine's curse or central alveolar hypoventilation (CCAH) syndrome is a disorder of the autonomic nervous system resulting in respiratory dysregulation. The clinical outcome is typically poor, with few individuals living into adulthood and even fewer surviving to adulthood with normal neurological function. Our patient initially presented following an uncomplicated delivery with hypotonia, poor respiratory effort, and hypoxemia that required ventilatory support. Laboratory workup, radiographic evaluation, and ancillary testing ruled out brain stem lesions, neuromuscular diseases, cardiovascular and pulmonary disease, and metabolic disorders, resulting in a diagnosis of CCAH syndrome. The patient underwent tracheotomy and chronic ventilatory support. Close long-term management and appropriate treatment modifications have provided for an excellent outcome and good quality of life. The patient is currently 22 years old and is earning her teaching degree for K-12 art education. A combination of early recognition and a multidisciplinary approach may lead to a successful outcome in patients with CCAH syndrome.

Key Words: congenital central hypoventilation syndrome • Ondine's curse • hypoventilation syndrome • central alveolar hypoventilation

This version was published on November 1, 2008

Clinical Pediatrics, Vol. 47, No. 9, 941-946 (2008)
DOI: 10.1177/0009922808320701
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