Sleep Apnea Syndrome after Posterior Fossa Surgery: A Case of Acquired Ondine's Curse

Document Type : Case Report

Authors

1 Department of Neurosurgery, Mashhad University of Medical Sciences, Mashhad, Iran.

2 Department of Anesthesiology, Mashhad University of Medical Sciences, Mashhad, Iran.

Abstract

Introduction:
Ondine’s Curse is a catastrophic but rare condition in adults. It is referred to as a congenital or acquired condition, in which the patient cannot breathe automatically while asleep. Acquired causes of this disease can be any cause affecting the ventrolateral part of the medulla, which is considered to be the breathing center in humans. 
 
Case Report:  
A 51-year-old woman, with ataxia and the symptoms and signs of rising Intra-Cranial Pressure, who underwent ventriculoperitoneal shunting and removal of tumour, developed episodic apnea during sleep after surgery and hypercapnia when awake. In her post-operative CT scan, some fine spots of hypodensity in the left lateral part of the medulla were observed. She was managed pharmacologically and underwent tracheotomy. After 50 days, she was discharged from the hospital when she was able to breathe normally.
 
Conclusion: 
Having experience with this condition after resection of a fourth ventricle tumor, it was found that Ondine’s Curse can be considered as one of the complications of  posterior fossa surgery and is curable by proper management.

Keywords

Main Subjects


1. Chin, Terry. Congenital Central Hypoventilation Syndrome. EMedicine 27 Nov. 2006. 17 Sept. 2008.Available from: URL: http://www.emedicine. Com/ ped/topic1645.htm
2. Takeda S, Fujii Y, Matsuda H, Kawahara h, Nakahara K. Central alveolar hypoventilation syndrome with gastroesophageal reflux. Chest 1996; 110(3):850-2.
3. Juan G, Ramon M, Ciscar MA, Garcia B, Lloret T, Cervello MA, et al. Acute respiratory insufficiency as initial manifestation of brainstem lesion. Arch Bronconeum 1999; 35(11):560-3.
4. Bullemer F, Heindl S, Karg O. Ondines curse in adults. Pneumologie 1999;53:s91-92
5. Sadler M, Wiles CM, stoodley N, Linnane S J, Smith A P. Ondine's curse in a woman with leber's hereditary optic neuropathy. J Neurol neurosurg psychiatry 2002; 73(3):347-8.
6.  Nannapaneni R, Behari S, Todd NV, Mendelow AD. Retracing "Undine's curse". Neurosurgery 2005; 57(2): 354–63
7. Hui-Tzu H, peterus T, Ching-Chi L. Ondines Curse in a patient With unilateral medullary and bilateral erebellar infarctions. j chin med assoc 2005: 68(11): 531-4.
8. Trang H, Dehan M, Beaufils F, Zaccaria I, Amiel J, Gaultier C. The French Congenital Central Hypoventilation Syndrome Registry: general data, phenotype, and genotype. Chest 2005;127 (1):72-9.
9. Schestatsky P, Nelson Teixeira Fernandes L. Aquired ondine's curse. Arq Neuro-psiquiatr 2004; 62(2b):523-7.
10. Flageole H, Davis M. Diaphragmatic pacing in children. In: Parikh D, Crabbe D, Auldist A, Pothenberg S. (editors). Pediatric thorasic surgery. London:Springer London; 2009: 523-33.
11. Hong-Nan S, Chi-tzong H, Hsing-Yu W. Medullary infarction with centeral hypoventilation:A case report. acta neural Taiwan 2003;12:201-4.
12. Pedroso J, Baiense R, Scalzaretto A, Braga Neto P, Teixeira de Gois A, Ferraz M. Ondine's curse after brainstem infarction. Arq Neuropsiquiatr 2009; 57(2):206-7.
13.  Kapnadak S, Mikolaenko I,   Enfield K, Gress D, R. Nathan B. Ondine’s Curse with Accompanying Trigeminal and Glossopharyngeal Neuralgia Secondary to Medullary Telangiectasia. Neurocritical Care 2010; 12(3): 395-9.
14.  Stankiewicz JA, Pazevic JP. Acquired Ondine’s curse. Otolaryngol Head Neck Surg 1989; 101(5): 611-3.
15. Butin M, Labbé G, Vrielynck S, Franco P, Massenavette B, Bellon G. Late onset Ondine syndrome: literature review on a case report. Arch Pediatr. 2012; 19(11):1205-7.
16. Lee P, Su YN, Yu CJ, Yang PC, Wu HD. PHOX2B mutation-confirmed congenital central hypoventilation syndrome in a Chinese family: presentation from newborn to adulthood. Chest 2009; 135(2):537-44.