European Journal of Pediatrics

, Volume 141, Issue 4, pp 251–253

Cor pulmonale and acute liver necrosis, due to upper airway obstruction as part of pycnodysostosis


  • D. C. Aronson
    • Department of PediatricsUniversity of Amsterdam, Academisch Medisch Centrum
  • H. S. A. Heymans
    • Department of PediatricsUniversity of Amsterdam, Academisch Medisch Centrum
  • R. P. G. M. Bijlmer
    • Pediatric Intensive Care UnitUniversity of Amsterdam Academisch Medisch Centrum
Case Reports

DOI: 10.1007/BF00572773

Cite this article as:
Aronson, D.C., Heymans, H.S.A. & Bijlmer, R.P.G.M. Eur J Pediatr (1984) 141: 251. doi:10.1007/BF00572773


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.

Key words

Upper airway obstructionPulmonary hypertensionRight-sided heart failurePychodysostosisChilddren

Copyright information

© Springer-Verlag 1984