Surgical management of hiatal hernia in a 3-year-old child with asplenia syndrome, congenital heart disease, thoracic scoliosis, and intestinal malrotation: A case report.

INTRODUCTION AND IMPORTANCE

Asplenia syndrome, along with its associated congenital abnormalities such as diaphragmatic hernia and scoliosis, presents significant diagnostic and therapeutic challenges. This case highlights the complexity of surgical management of a hiatal hernia in a pediatric patient with congenital scoliosis and asplenia syndrome, which was associated with multiple congenital anomalies, including congenital heart disease and gastrointestinal malformations. The case underscores the necessity of a multidisciplinary and coordinated surgical approach to optimize patient outcomes.

CASE PRESENTATION

A 3-year-old female with a history of transposition of the great arteries, atrioventricular septal defect, secondary atrial septal defect, and pulmonary stenosis presented with congenital scoliosis, postprandial dyspnea, and choreiform movements. Radiological evaluation revealed scoliosis due to hemivertebrae and a large hiatal diaphragmatic hernia, with herniation of the stomach and pancreas into the thoracic cavity within the hernia sac.

CLINICAL DISCUSSION

In cases like this, surgical intervention primarily aims to relieve respiratory distress and enhance gastrointestinal function. The strategy employed in this instance involved reducing the herniated stomach, reestablishing the position of the pylorus without performing fundoplication, and repairing the esophageal hiatus by approximating the diaphragmatic crura.

CONCLUSION

This case emphasizes the critical role of early surgical intervention in patients with complex congenital defects to alleviate symptoms and prevent complications. A timely, well-coordinated surgical approach resulted in favorable postoperative outcomes, with resolution of gastrointestinal symptoms and improved quality of life.