Get your full text copy in PDF
Ülfet Vatansever, Ridvan Duran, Nermin Tunçbilek, Şemsi Altaner, Betül Acunaş
CaseRepClinPractRev 2005; 6:258-260
Background:Our aim is to present a case with asphyxiating thoracic dysplasia (ATD).
Case Report:A male newborn infant was born with congenital anomalies and severe respiratory insufficiency. In his physical examination we found that he had a constricted thorax, short limbs, in his radiographic studies we saw a constricted thorax, short limbs, a trident appearance of the acetabulum, coneshaped phalangeal epiphysis. Despite of being treated with conventional ventilatory support he died at 36 hours after birth. Post mortem examination of the infant revieled that he had hepatic fibrosis and polycyctic renal disease. With all this findings we diagnosed he had ATD.
Conclusions:ATD has a wide clinical spectrum. Lethal forms lead to death in infancy because of a severely constricted thoracic cage and respiratory insufficiency. We wonder if high-frequency oscillatory ventilation and inhaled nitric oxide could save his life and lead to a more favourable outcome or not.
Keywords:asphyxiating thoracic dysplasia • newborn • ventilatory support