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A 2-year-old boy with multiple congenital anomalies, including bilateral club feet, arthrogryposis, and multilevel aortic coarctation and aneurysms, presented with acute abdominal pain, vomiting, abdominal distention, and lethargy. An unusual intraluminal air collection projecting over the left heart was seen on the chest radiograph. Echocardiography showed a structurally normal heart with a small concentric pericardial effusion and an echogenic mass posterior to the left ventricle (Fig. 1). There were echo-free spaces and cavitations evident within the mass. In addition, the left atrium was compressed, and there was respiratory variability of mitral valve inflow seen on spectral Doppler echocardiography, which suggested borderline tamponade. Urgent computed tomography of the chest and abdomen confirmed the presence of dilated loops of bowel within the pericardial space (Fig. 2). On computed tomography, there was a diaphragmatic defect posterior to the left lobe of the liver, rather than anterior, as would be expected with a Morgagni hernia.
The patient was subsequently taken to the operating room, where he was noted to have a dilated transverse colon and several loops of small intestine herniating through a diaphragmatic defect that was anterior and just to the left of the esophageal hiatus. Inspection of the bowel revealed no areas of frank ischemia or necrosis. The bowel was easily reduced through the diaphragmatic defect, and the defect was closed primarily with pledgeted sutures.
In this patient, the diagnosis of distal aortic coarctation with multiple regions of aortic narrowing in combination with aortic aneurysms was suspected antenatally. At several days of age, he underwent diagnostic cardiac catheterization, which confirmed severe coarctation of the upper abdominal aorta just below the level of the diaphragm, with multiple aneurysmal dilations distal to the coarctation. At 3 weeks of age, he underwent an uncomplicated interventional cardiac catheterization, during which 5 overlapping intravascular stents were placed in the abdominal aorta. The most proximal stent was in the distal thoracic aorta at the level of the diaphragm.
Most cases of herniation into the pericardial space are related to traumatic pericardiophrenic rupture.1 In our patient, it is difficult to exclude the possibility that the defect was related to the deployment of the intravascular stents. However, this seems unlikely, given that the intravascular stents were distant from the defect in the pericardium.
Other complications can occur with diaphragmatic defects, such as herniation of the liver into the pericardium as a result of a congenital defect in the central tendon of the diaphragm.2 In addition, a deficiency of the diaphragmatic pericardium is associated with pentalogy of Cantrell, which includes a variety of midline defects.3 It is likely that our patient had a rare, isolated congenital defect in the inferior pericardial space at the level of the diaphragm that allowed herniation of the abdominal contents into the pericardium. This anatomic defect appears to be unrelated to his other congenital malformations.
Address for reprints: Jack C. Salerno, MD, University of Washington School of Medicine, Children's Hospital and Regional Medical Center, Pediatric Cardiology W-4841, 4800 Sand Pointe Way NE, Seattle, WA 98105 E-mail: email@example.com