ISSN:
1432-1076
Keywords:
Upper airway obstruction
;
Pulmonary hypertension
;
Right-sided heart failure
;
Pychodysostosis
;
Childdren
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract A male patient with pycnodysostosis suffered from chronic respiratory insufficiency and pulmonary hypertension. This was caused by concomitant upper airway obstruction, resulting from a low implanted uvula and a long soft palate, in combination with glossoptosis and retrognathia due to the flattened mandibular angles. An inter-current respiratory infection gave rise to an acute deterioration, with right-sided heart failure, severe liver damage and coma. Surgical shortening of the soft palate was performed, after which the blood gas values returned to normal.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00572773
