A 6-month-old girl presented to the pediatric emergency department with four days of intermittent fever, tachypnea, and persistent cough. According to the patient's mother, she had not experienced previous nausea, vomiting, dyspnea, or peripheral edema; but she did exhibit poor oral intake and general failure to thrive. Her mother reported no history of trauma, birth complications, or evidence of other congenital malformations and a thorough review of systems was otherwise negative. Her mother was a 23-year-old woman who denied any personal or family history of chronic disease, sick contacts (including anyone infected with tuberculosis), and reported a negative HIV status. She was seen by a physician prenatally but did not receive routine ultrasound.
Physical examination revealed a small female infant with low body weight (4.8 kg, <5 percentile weight for age), breathing at a rate of 52 breaths/min, with a heart rate of 145 beats/min, and blood pressure of 126/111 mmHg. She was afebrile. Abdominal evaluation was soft and nontender. Chest auscultation was notable for diffuse bilateral rhonchi and rales, though cardiac sounds were normal. She was not cyanotic. Preoperative labs demonstrated anemia (HgB: 10.2 g/dL) but no leukocytosis (WBC: 9.4 x 103/muL). Echocardiography was reported as normal. An initial chest radiograph (Fig. 1) showed dilated loops of bowel located centrally in the thoracic cavity, obscuring the cardiac silhouette, with opacification of the lower lobe of the left lung. These findings were suspicious for CDH, which was confirmed by a thoracic CT scan (Fig. 2). Plans for corrective surgery were made with the preoperative diagnosis of a likely Morgagni-type CDH.
The patient was taken to the operating room and underwent a left thoracotomy. It was readily apparent that there was no hernia sac or loops of bowel within either pleural cavities. However, the pericardium was grossly enlarged and ballotable. We carefully incised the pericardium, from which drained roughly 20 ml of serous fluid. Within the pericardium we found the hernia sac, which was also incised, revealing loops of small bowel that were incarcerated, but not strangulated (Fig. 3). The heart itself was visualized directly without overlying pericardium. On two occasions during manipulation of the hernia contents the heart became asystolic. Each time systole was quickly restored with direct cardiac massage. We proceeded to reduce 10 cm of small bowel, and corrected the 2 cm defect in the cardiac portion of the central tendon of the diaphragm. The remainder of the diaphragm was intact, the abdominal wall was intact, the great vessels entering and leaving the heart were grossly normal, and we found no evidence of any other congenital malformations within the thorax. We closed the pericardium, leaving a small defect to prevent future pericardial effusion. We subsequently closed the thoracic cavity in layers, leaving a 28 French chest tube draining the pleural cavity by gravity under water seal.
Postoperative management included treatment with furosemide for acute pulmonary edema on post-operative day one. On the second post-operative day, the patient developed a new leukocytosis and fever. A chest radiograph demonstrated bilateral opacification of the lung fields. She was given penicillin, acetaminophen, and salbutamol and a follow-up chest radiograph demonstrated clear lung fields and no evidence of hernia. The patient was discharged seven days after her operation, breastfeeding normally clinically stable. Despite attempts to contact the mother, the patient was lost to follow up and not seen in our surgical clinic.