Acute presentation of congenital diaphragmatic hernia past the neonatal period: a life threatening emergency.

PURPOSE Major gastric distension in the left hemithorax can threaten life in patients with congenital diaphragmatic hernia (CDH) presenting after the neonatal period. After presentation of two pediatric cases, guidelines for the optimal care of these patients are given. CLINICAL FEATURES Both children had respiratory and cardio-circulatory compromise on arrival. The diagnosis of late presenting CDH was made and the severity of symptoms was related to a voluminous distension of an intrathoracic stomach. Successful placement of an naso-gastric tube in the first patient, lead to a rapid clinical improvement, allowing surgical repair. In the second patient, oro- or naso-gastric decompression was not possible, and while the lungs were mechanically ventilated and the patient was prepared for surgery, a sudden cardiocirculatory arrest was managed by external chest compressions and rescuscitation drugs. Transthoracic percutaneous decompression of the stomach was the sole treatment allowing spontaneous cardiac activity to reappear, and haemodynamic condition to normalize. However, the child died from brain death after this episode. CONCLUSION Gastric decompression is the key for the treatment of patients with CDH who present respiratory and/or cardiocirculatory distress due to the intrathoracic distension of the stomach. If an oro- or naso-gastric decompression is not possible, then radiologically directed percutaneous decompression under local anesthesia is required. After decompression, the patient is prepared for surgery, with particular emphasis on fluids infusion, in order to correct the frequently associated hypovolaemia.