Congenital defects of the diaphragm are most commonly found at the pleuroperitoneal hiatus, while defects at the sternocostal hiatus are extremely rare. This discussion focuses on herniation of abdominal organs through the pleuroperitoneal hiatus defect. First reported by Bochdalek in 1848, this condition is more prevalent in infants and young children, with a higher incidence in males than females. It occurs in approximately 1 in 4,000 live births, with 90% of cases occurring on the left side. The right side is less affected, likely due to protection from the liver or earlier closure of the right diaphragm during embryonic development compared to the left.

bubble_chart Pathogenesis

The weak or absent muscle layer in the lumbocostal triangle is the anatomical basis for the occurrence of this disease.

bubble_chart Clinical Manifestations

The disease primarily presents with respiratory and gastrointestinal symptoms. Newborns exhibit acute respiratory distress and cyanosis, while children and adults show grade I chronic respiratory and gastrointestinal symptoms. Barrel chest or flat abdomen may be observed, with absent breath sounds or borborygmi heard on the affected side. The presence of vomiting indicates concurrent incomplete intestinal volvulus.

bubble_chart Auxiliary Examination

The X-ray shows a rightward shift of the heart and mediastinum, multiple air-filled intestinal loops in the left chest, and a decrease in air-filled intestinal loops in the abdominal cavity. A "liverless sign" is visible on the right side of the defect. Barium meal examination may worsen intestinal obstruction and should be used as sparingly as possible.

bubble_chart Treatment Measures

Early surgery is recommended as soon as the diagnosis is confirmed, with transabdominal repair of the defect being the preferred approach. Since the child often has other associated malformations, pulmonary hypoplasia is recognized as the leading cause of death, as hernia repair cannot eliminate pathological changes such as pulmonary hypoplasia, increased pulmonary vascular resistance, and pulmonary hypertension.