Congenital central hypoventilation syndrome: Failure from birth of central nervous system control over breathing while asleep. There are usually no breathing problems while awake. The involuntary (autonomic) control of respiration is impaired, but the voluntary control of ventilation which operates during waking hours is generally intact.
The neurologic problem can extend to the control over the motility of the esophagus and the colon. Reduced esophageal motility causes food to move too slowly down to the stomach. Reduced motility of the colon produces a condition called Hirschsprung disease (megacolon) with severe constipation and even obstruction. The heart rate may be abnormally slow.
Some patients have down slanting eyes, a small nose, a triangular-shape mouth, and ears that are low-set and backwards rotated.
The names for this disease include congenital central hypoventilation syndrome with Hirschsprung disease, congenital failure of autonomic control, idiopathic central alveolar hypoventilation, central sleep apnea with severe hypoventilation, and Ondine's curse.
Ondine (Undine) was a female water sprite who loved a knight but was condemned to stay awake in order to breathe.
The appropriate nomenclature for the disorder known as Ondine curse is congenital central hypoventilation syndrome (CCHS).
Disease characteristics. Classic congenital central hypoventilation syndrome (CCHS) is characterized by adequate ventilation while the affected individual is awake and by ...
Welcome to the CCHS Family Network! CCHS - Congenital Central Hypoventilation Syndrome. CCHS is a multisystem disorder of the central nervous system where, most ...
Congenital central hypoventilation syndrome (CCHS) is a disorder that affects breathing. People with this disorder take shallow breaths (hypoventilate ...
Important It is possible that the main title of the report Central Hypoventilation Syndrome, Congenital is not the name you expected. Please check the synonyms ...