2019 Research Forum

Presenter: Mark Rhoades RA Principal Investigator & Faculty Sponsor: Adria Ottoboni MD

Acquired Pediatric Right-Sided Diaphragmatic Hernia and Prolonged QT Syndrome

Adria Ottoboni MD, Larissa Morsky MD R3, Laura Celine Castro MS RA, Mark Rhoades RA

INTRODUCTION Right-sided diaphragmatic hernias are difficult to diagnose in children, and diagnostic delay highly contributes to morbidity and mortality.

PURPOSE A 1-year-old male with a past medical history of prolonged QT syndrome and past surgical history of a sternotomy with an implantable cardioverter-defibrillator (ICD) and pacemaker placements presented to the emergency department with severe respiratory distress, increased crying and illness for the past two weeks. The father reported that the patient had been irritable, coughing, vomiting, and diaphoretic. Chest radiograph demonstrated infiltrate vs. atelectasis right lung base with a large 5 cm right-sided diaphragmatic hernia impairing gas exchange. Consolidation is seen in the right lower lobe with infiltration of the large colon in the right hemi-thorax with apparent bowel gas in the chest cavity. The patient was medicated with amoxicillin, albuterol, and ceftriaxone. Later radiograph showed migration and erosion of the automatic implantable cardiovascular defibrillator (AICD) from the original peritoneal pocket in the right upper quadrant into the peritoneal cavity, and a left pelvic mass. The large anterior congenital diaphragmatic hernia, likely caused from the path of the AICD wires, was repaired through laparotomy. DISCUSSION Right-sided diaphragmatic hernias are often more difficult to diagnose in children, and diagnostic delay highly contributes to morbidity and mortality. The resulting infiltrate vs. atelectasis in the right lung base with right-sided diaphragmatic hernia could be secondary to the initial surgical placement of the ICD with subsequent expansion. As seen on previous chest radiographs, it increased in size and was likely the cause of respiratory embarrassment and mismatch. CONCLUSION Since right traumatic diaphragmatic hernia is rare in children and does not contain specific clinical signs, any child presenting with respiratory distress should be suspected of possible right diaphragmatic hernia. The child should be radiographically examined quickly upon presentation to avoid the morbidity and mortality associated with delayed diagnosis

28