The intrathoracic kidney: should we fix it?

Background Intrathoracic kidney is a rare congenital anomaly, with only 13 cases reported in the pediatric age group over the past 25 years. The relevant literature is limited to individual case reports or small case series with no follow-up data. Both operative and nonoperative management has been advocated. We report our experience in the management of children with an intrathoracic kidney as well as the efficacy of nonoperative management in select patients. Methods Five cases of intrathoracic kidney were collected prospectively since 1992 and carefully followed up long term. Results Two children presented with acute respiratory distress and underwent right diaphragmatic hernia repair and nephropexy. Incidental diagnosis of a left intrathoracic kidney was made in 3 children. Long-term follow-up has demonstrated normal function and development of these kidneys in all 5 children with no late bowel herniation in the nonoperative group. Conclusion Intrathoracic kidney associated with bowel in the chest should undergo standard repair and nephropexy. An isolated intrathoracic kidney without evidence of bowel herniation can safely be observed. This is the largest pediatric series of intrathoracic kidney as well as the first to document the efficacy of nonoperative management with long-term follow-up.