Background/purpose: The aim of the study was to review the gastrointestinal abnormalities occurring in association with situs anomalies.
Methods: Patients with situs anomalies were identified from the medical records of pediatric patients of Seoul National University Children's Hospital from January 1980 to July 2004. Retrospective study was undertaken. Diagnosis was made on the basis of the information obtained from a combination of echocardiography, angiography, abdominal ultrasonography, liver scan, upper gastrointestinal study, or abdominal computed tomography.
Results: A total 67 patients diagnosed as having situs anomalies were identified. There were 40 males and 26 females (1.54:1). Of these 67 patients, 45 patients (67%) were diagnosed as having situs inversus, 16 patients (24%) as having right isomerism, and 6 patients (9%) as having left isomerism. Of 45 patients with situs inversus, there were 26 patients (58%) who had intraabdominal abnormalities. These were duodenal atresia, biliary atresia, gastroschisis with malrotation, congenital hepatic fibrosis, tracheoesophageal fistula (type C), Currarino's triad, and pheochromocytoma. Of 16 patients with right isomerism, there were 14 patients (88%) who had intraabdominal abnormalities. These were hiatal hernia and diaphragmatic hernia. Of 6 patients with left isomerism, there were 4 patients (67%) who had intraabdominal abnormalities. These were malrotation and biliary atresia.
Conclusion: When a patient is noted to have congenital heart disease as part of situs anomalies, or if an atypical position of organs is noted at imaging evaluation, we recommend that the patient undergo chest radiography, abdominal ultrasonography, upper gastrointestinal study, and abdominal computed tomography.