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Congenital diaphragmatic hernia beyond infancy
T R Weber1, T Tracy, P V Bailey
1Department of Surgery, St. Louis University School of Medicine, Missouri.
Insights
Congenital diaphragmatic hernia (CDH) in older patients presents differently than in newborns, often with fewer respiratory issues. Postoperative gastric atony is a significant complication requiring specific surgical approaches.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Gastroenterology
Background:
- Congenital diaphragmatic hernia (CDH) is a frequent cause of neonatal respiratory distress.
- CDH presentation, management, and complications in older children and adults are less understood.
Purpose of the Study:
- To investigate the clinical presentation, surgical management, and outcomes of CDH in non-newborn patients.
- To identify common symptoms, diagnostic methods, and postoperative complications in this rare demographic.
Main Methods:
- Retrospective review of 13 patients (2 months to 26 years) with congenital diaphragmatic hernia.
- Analysis of patient symptoms, physical signs, diagnostic imaging (chest roentgenogram, GI contrast radiograph), surgical repair techniques, and postoperative complications.
Main Results:
- CDH in older patients often presents with chronic respiratory infections or gastrointestinal issues, rarely severe respiratory distress.
- Right-sided hernias were more common in this cohort (4/13), contrasting with neonatal CDH.
- 92% survival rate, but 58% of survivors experienced severe postoperative gastric atony, necessitating further surgery.
Conclusions:
- Congenital diaphragmatic hernia in older children and adults has distinct clinical features compared to neonates.
- Postoperative gastric atony is a major morbidity, supporting transabdominal repair with pyloroplasty or feeding jejunostomy as preferred surgical strategies.
Abstract:
Congenital diaphragmatic hernia (CDH) is a common cause of severe respiratory distress in the newborn. However, the presentation of CDH in older children and adults is rare, and, therefore, little is known concerning its symptoms, operative management, and postoperative complications. Thirteen patients (age range: 2 months to 26 years; 5 males, 8 females) presented with CDH. Four patients had right-sided hernias, eight left-sided hernias, and one bilateral hernias. Symptoms included chronic respiratory tract infections in 6 patients, vomiting in 5, weight loss in 1, severe failure to thrive in 2, and severe respiratory distress in 3; one patient was asymptomatic. Physical signs included the absence of breathing sounds or bowel sounds in the chest in eight patients, hyperresonance in one, and cachexia in two. The diagnosis was confirmed in each patient by chest roentgenogram or gastrointestinal contrast radiograph. All patients underwent immediate repair. After reduction of the viscera, 12 of 13 patients underwent primary diaphragm repair, whereas one patient required a prosthetic diaphragm patch. Twelve of 13 patients (92%) survived. Postoperatively, 7 of the 12 survivors (58%) developed severe gastric atony, and four required further operative therapy. In contrast to newborns, CDH in the older child and adult is frequently seen on the right side, rarely presents with severe respiratory distress, and is occasionally asymptomatic. Postoperative gastric atony is a major cause of morbidity, making transabdominal repair with simultaneous pyloroplasty and/or feeding jejunostomy the preferred operative approach.