SESSION TITLE: Airway Student/Resident Case Report Posters
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Ondine’s curse is a condition which involves autonomic respiratory failure during sleep that is fatal if untreated. This disorder can be inherited and present during infancy as congenital central hypoventilation or it can present during adulthood when it is called primary alveolar hypoventilation. Uncommonly it presents as a brain or high cervical spinal cord injury. Worldwide approximately 200 cases have been reported with very few presenting as adults. We present a case of this rare disease following pontomedullary hemorrhage in an adult woman.
CASE PRESENTATION: A 77 year-old African American woman with history of hypertension and peripheral vascular disease was hospitalized for dizziness secondary to carbon monoxide exposure. On hospital day four she developed new onset slurred speech, right sided facial droop and confusion requiring intubation and mechanical ventilation. Head computer tomography (CT) showed hemorrhage involving the right posterior medulla. She could not be liberated from the ventilator due to frequent, prolonged apneas while asleep consistent with Ondine's curse. These episodes continued despite minimizing sedating medications and trialing various modes of mechanical ventilation. In an effort to increase alveolar ventilation acetazolamide was started. It appeared to be effective as evidenced by decreased apneic spells. Despite less frequent apneas the patient ultimately required a tracheostomy and remains on mechanical ventilation when sleeping.
DISCUSSION: Hypoventilation syndromes are common following cerebrovascular accidents. However, hypoventilation that occurs exclusively during sleep, the dreaded “curse” of Ondine, is quite rare. This case reviews treatment considerations including acetazolamide and early tracheostomy. The tracheostomy simplifies ventilation while asleep whereas the acetazolamide increases ventilatory drive. While our patient remained “cursed” during her inpatient stay, the literature suggests that she may improve over the coming months, regaining her autonomic drive.
CONCLUSIONS: Ondine's Curse is a rare syndrome that affects autonomic ventilatory drive during sleep. This case reviews treatment considerations including acetazolamide and early tracheostomy that may improve overall outcomes.
Reference #1: Panossian L, Daley J. Sleep-disordered breathing. Continuum (Minneap Minn). 2013 Feb;19(1 Sleep Disorders):86-103. doi: 10.1212/01.CON.0000427211.98305.c6. PubMed PMID: 23385696.
Reference #2: Lovell BL, Bullock RE, Anderson KN. An unusual presentation of congenital central hypoventilation syndrome (Ondine's Curse). Emerg Med J. 2010 Mar;27(3):237-8. doi: 10.1136/emj.2009.072215. PubMed PMID: 20304901.
DISCLOSURE: The following authors have nothing to disclose: Hassan Sajjad, Keriann Van Nostrand, W. Graham Carlos
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