Congenital diaphragmatic hernia with intrathoracic kidney: case report, review of the literature, and strategy for treatment in neonates and infants.

Abstract

Background: Congenital diaphragmatic hernia (CDH) is a rare developmental defect in the diaphragm, occurring in 2 in 10,000 births. Herniation of intraperitoneal organs through the diaphragmatic opening is always present, however few cases mention the herniation of retroperitoneal organs, such as a kidney. Due to the rarity of this condition, the optimal treatment strategy remains unclear.

Methods: A PubMed search was conducted, gathering all published reports of CDH with intrathoracic herniation of the kidney. Cases of isolated intrathoracic kidney without CDH and cases of traumatic hernia were excluded. Patients who underwent surgical repair before the age of 5 years were included for further analysis.

Results: Thirty-seven cases were found from 1970 to 2022. The approach used for surgical repair was not mentioned in 55.6% of cases. 52.9% of the remaining patients were treated through laparotomy, whereas in 23.5% a thoracoscopy was performed. A primary repair of the hernia was performed in 88.6%. A hernia sac was noted in 70%. Most patients had a normal origin of the renal vessels and reduction of the intrathoracic kidney was achieved in 78.8%. Moreover, we report a case of CDH with intrathoracic kidney treated through thoracoscopic repair.

Conclusion: A thoracoscopic approach is effective for the treatment of CDH with an intrathoracic kidney case with an associated intrathoracic kidney. A therapeutic strategy for CDH with intrathoracic kidney is suggested based on data from published cases.