Post-operative anterior diaphragmatic hernias in children with Trisomy 21 after cardiac surgery

Abstract

Purpose: Morgagni hernias account for less than 5% of congenital diaphragmatic hernias. They are characteristically retrosternal and bilateral, with right-sided predominance. An association between Trisomy 21 and diaphragmatic hernias resembling Morgagni hernia has been reported, but the effect of cardiac surgery on its formation has not been investigated. The purpose of this study was to determine whether there is a higher incidence of anterior diaphragmatic hernias in children with Trisomy 21 after cardiac surgery.Methods: We compared the prevalence of anterior diaphragmatic hernias in 92 patients with Trisomy 21 who underwent cardiac surgery with its prevalence in two control groups -children without Trisomy 21 who underwent cardiac surgery, and children with Trisomy 21 who did not undergo cardiac surgery, 100 patients in each. All available CXRs of all children underwent revision for the presence of an anterior diaphragmatic hernia by a pediatric radiologist.Results: Within the study group, four cases of an anterior diaphragmatic hernia were detected, all upon presentation to the emergency room due to breathing di culties. No cases of an anterior diaphragmatic hernia were found in the two control groups (P = 0.0094) Conclusions: A high index of suspicion for an anterior diaphragmatic hernia should be maintained in children with Trisomy 21 who have undergone cardiac surgery and present with breathing di culty. If CXR ndings are uncertain, UGI series and\or CT should be performed. In light of our ndings, the surgical technique has been modi ed in patients with DS in our medical center. What Is KnownSeveral studies reported an association between Trisomy 21 and diaphragmatic hernia resembling Morgagni hernia, but the effect of cardiac surgery on its formation has not been investigated. What Is NewThere is a higher incidence of anterior diaphragmatic hernia resembling a Morgagni hernia in children with Trisomy 21 after cardiac surgery.A high index of suspicion for an anterior diaphragmatic hernia should be maintained in children with Trisomy 21 who have undergone cardiac surgery and present with breathing di culty.If CXR ndings are uncertain, UGI series and\or CT should be performed.