Research: Delayed presentation of a right congenital diaphragmatic hernia following left congenital diaphragmatic hernia repair in infancy

Int J Surg Case Rep. 2023 Mar 21;105:108020. doi: 10.1016/j.ijscr.2023.108020. Online ahead of print.

Kaddie Kwok Chen 1Mary Jude Edwards 2

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Free PMC article


Introduction and importance: Congenital diaphragmatic hernias (CDH) affect variable portions of diaphragm, resulting in herniation of abdominal contents into the chest. CDH typically is diagnosed prenatally or presents soon after birth with respiratory distress and abnormal chest X-ray (CXR) findings. Presentation after infancy is rare but well described. We present a case of delayed presentation of a right CDH following left CDH repair.

Case presentation: An 18-month-old boy with a history of a left CDH repaired in the newborn period presented with a newly elevated right hemidiaphragm presumed to be an eventration. He re-presented 9 months later with abdominal pain and respiratory distress. Xray was concerning for gastric volvulus within the right chest. Surgical exploration revealed a small right sided diaphragmatic hernia with an incarcerated liver and stomach. This was repaired without event.

Clinical discussion: Normal appearing CXR following L CDH repair led to delayed recognition of this right CDH. Delayed presentation was likely due to the liver covering the small diaphragmatic defect, preventing early migration of the intra-abdominal viscera into the chest. Recurrence of the CDH is the most common cause of respiratory and gastrointestinal symptoms following prior repair, but an unrecognized contralateral defect is a rare possibility.

Conclusion: In a patient with a prior history of a left CDH who presents with respiratory and gastrointestinal symptoms, along with a recurrence of the left sided CDH, the presence of a right sided defect should be considered if the right hemidiaphragm is newly elevated.

Keywords: Bochdalek hernia; Case report; Congenital diaphragmatic hernia; Delayed presentation.

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