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Signa Vitae  2011 

Platypnea-orthodeoxia-like syndrome in a septic newborn with late appearance of right congenital diaphragmatic hernia

Keywords: cyanosis , right-to-left shunt , infant , sepsis , congenital diaphragmatic hernia

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Abstract:

Introduction. Cyanosis and dyspnoea with interatrial shunting of blood occurring without change in body position are termed platypnea-orthodeoxia-like syndrome.Case presentation. We describe a female newborn with sepsis, who developed cyanosis. Two weeks after the initial appearance of cyanosis she presented with a right-sided diaphragmatic hernia, which was not seen on the initial X-rays. The hernia was surgically repaired. Her postoperative course was uneventful. Conclusions. In our case, interatrial shunting of blood was presumably caused by disturbed intrathoracic pressures. Positive pressure ventilation and sepsis may also contribute to the development of such a syndrome.

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