RT Journal Article
SR Electronic
T1 Hunter's syndrome and associated sleep apnoea cured by CPAP and surgery
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1195
OP 1197
VO 13
IS 5
A1 Orliaguet, O
A1 Pepin, JL
A1 Veale, D
A1 Kelkel, E
A1 Pinel, N
A1 Levy, P
YR 1999
UL http://erj.ersjournals.com/content/13/5/1195.abstract
AB A 42-yr-old male with Hunter's syndrome presented with severe obstructive sleep apnoea syndrome (OSAS) and daytime respiratory failure. Continuous positive airway pressure (CPAP) therapy was initially ineffective and produced acute respiratory distress. Extensive Hunter's disease infiltration of the upper airway with a myxoma was confirmed. Following surgery to remove the myxoma at the level of the vocal cords, CPAP therapy was highly effective and well tolerated. This report demonstrates the necessity of evaluating fully the upper airway in patients with unusual variants of OSAS, particularly where the disease is not adequately controlled by CPAP.