Congenital Diaphragmatic Hernia
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Clinical History: New born in acute respiratory distress.
Findings: The left hemithorax is opacified with a midline shift to the right. The opacification of the left hemithorax believed to represent loops of bowel secondary to a Bochdalek type congenital diaphragmatic hernia.
An endotracheal tube is seen in satisfactory position. The nasogastric tube has its tip in the expected location of the herniated stomach, at the T6 level. An umbilical arterial catheter is seen with its tip overlying the right T9 pedicle, presumably in the descending aorta. An umbilical venous catheter is noted with its tip overlying the left posterior 9th rib, presumably within the herniated liver. A bladder catheter is identified with its tip overlying the L-5/S-1 interspace.
Diagnosis: Congenital Diaphragmatic Hernia.
Discussion: Normally at eight weeks gestation, just before the return of the bowel to the abdominal cavity, the communication between pleural and peritoneal cavity is closed by the development of the diaphragm. If the bowel returns to the abdomen prematurely or if the diaphragm develops late or incompletely, a diaphragmatic hernia occurs. These children have malrotation of the bowel because there is interruption of the normal rotation and fixation of the bowel as it returns to the abdomen. However, there is a surprisingly low risk of a mid-gut volvulus.
The neonatal radiographic appearance may be that of a large intrathoracic mass of soft tissue density. The more characteristic pattern of many intrathoracic air-filled loops of bowel is found after several hours. An absence of the normal gas-containing bowel in the abdomen is noted and the abdomen is scaphoid on physical exam.
Congenital diaphragmatic hernia occurs 6 to9 times more often on the left. This may be due to the fact that the pleuroperitoneal canal closes earlier on the right. Approximately 3% of children with congenital diaphragmatic hernias will have them bilaterally.
The prognosis in neonates with congenital diaphragmatic hernia correlates to the degree of underlying lung hypoplasia. Status post-surgical correction, survivors tend to have severe gastroesphageal reflux.
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Josie Alpers, M.D.
Stuart C. Morrison, M.D.
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