Infant Readmission With Severe Acute Respiratory Failure

A 60-day-old, former 34.5-week gestational boy presents to the pediatrician for new-onset fussiness. His birth history was notable for an 8-day neonatal intensive care unit stay with initial continuous positive airway pressure and supplemental oxygen use after delivery, which was quickly weaned off. A chest radiograph was obtained during his hospitalization and demonstrated no abnormalities (Figure 1), and prenatal ultrasonography was normal. After 8 days, he was discharged home on room air and tolerating full feeds.On day of life 60, he was brought to the pediatrician by his mother because of fussiness. She reported that he last ate 2 hours ago. Immediately after feeding, he became inconsolable, with rapid-onset labored breathing. She reported contact with sick siblings recently and denied recent trauma. On examination in his pediatrician’s office, he demonstrated grunting and severe retractions. He was tachycardic, with heart rates reaching 200 beats per minute, and tachypneic, with oxygen saturations in the 90s. Based on his vital signs, he was immediately taken to the emergency department at an outside facility. In the emergency department, he was noted to be hypothermic to 35.4 °C and still demonstrated increased work in breathing. He was intubated because of respiratory failure.The differential diagnosis for an infant with no known congenital abnormalities who presents with severe respiratory failure and inconsolability requiring intubation is broad. The most common suspected cause of respiratory failure in this age group is infection, including viral infections, bacterial pulmonary infections, bloodstream infections, or meningitis. Delayed presentation of congenital pathologies is also a consideration, including cardiac disease, congenital diaphragmatic hernias (CDHs), or congenital lung malformations. Additional etiologies include inborn errors of metabolism, electrolyte derangements leading to acidosis, or airway obstruction. Common causes of inconsolability in this age group similarly include infections like meningitis or encephalitis, abdominal pathologies such as intussusception or constipation, nonaccidental trauma, or hair tourniquets.On presentation to the emergency department, a chest radiograph showed opacification of the left chest (Figure 2), and echocardiogram demonstrated preserved cardiac function. A chest computed tomography scan performed 24 hours later revealed total atelectasis of the left lung with mediastinal shift to the right (Figure 3A), with the left chest containing an inhomogeneous multicystic appearance in the left hemithorax with differential including congenital pulmonary airway malformation, cystic adenomatoid malformation, extrapulmonary sequestration, or diaphragmatic hernia per the outside hospital radiology report. After intubation, ventilatory support continued to escalate, requiring FiO2 of 0.70. The hospital initiated a transfer to a higher level of care for consideration of extracorporeal membrane oxygenation. On review of the scan with radiology at our institution, there was evidence of thickened and edematous bowel with free fluid in the left chest, suggesting diaphragmatic hernia as the etiology of his presentation.CDHs are a rare anomaly, affecting fewer than 5 per 10 000 births. They are caused by a diaphragm defect that leads to the herniation of intraabdominal contents into the thoracic cavity, which can displace pulmonary and cardiac structures. The defect is generally located on the left side as a posterolateral defect.1 The size of the diaphragmatic defects varies. Larger defects often lead to greater degrees of pulmonary hypoplasia and abnormal arterioles; this can lead to the development of pulmonary hypertension. Accurate and early diagnosis of CDH is essential for counseling and preparation of treatment strategies. CDH is typically diagnosed prenatally with screening ultrasonography. If the diagnosis is missed prenatally, patients are typically diagnosed immediately after birth with symptoms including respiratory distress, displacement of heart sounds to the contralateral side, or bowel sounds in the chest. A lung-to-head ratio can be calculated to predict severity postnatally.2 After birth, management includes mechanical ventilation, serial blood gases, and echocardiography to determine efficiency of gas exchange and cardiac output. Extracorporeal membrane oxygenation may be a necessary adjunct if gas exchange or cardiac output are not adequate. Surgical repair is typically performed once the cardiopulmonary status is stable.CDHs are classified based on 2 different frameworks. Posterolateral hernias, also known as Bochdalek hernias, are the most common type, representing 70% to 75% of CDHs. Anterior defects, also known as Morgagni hernias, comprise 20% of CDHs, followed by rare central hernias comprising 2% to 5%.3 The second classification is based on the size of the defect. Type A defects are the smallest, involving less than 10% of the chest wall circumference. Type B involves less than 50% of the chest wall circumference, and type C are the largest defects with complete or near-complete absence of the diaphragm.4Rarely, a CDH can present after birth. Although the defect in this case was on the left side, delayed onset of CDH, particularly on the right side, is associated with early onset Group B streptococcal (GBS) sepsis.5,6 Alternative causes of diaphragmatic hernia in this age group include diaphragmatic rupture secondary to trauma. However, cases of delayed CDH presentation outside of the setting of neonatal GBS sepsis are rare.Because of concern for an alternative diagnosis to CDH on presentation, the patient underwent a child abuse workup for the evaluation of traumatic diaphragmatic rupture, and the results were negative. He underwent blood and urine cultures, both with negative results, including for GBS.He was taken to the operating room on the first day after transfer for an exploratory laparotomy, where he was found to have a large volume of ascites. Approximately 100 cm of grossly ischemic jejunum and proximal ileum in the chest were resected. A type A congenital hernia defect approximately 1 cm in size was found and was repaired with permanent suture. The defect appeared to be congenital and not traumatic in origin. He was left in bowel discontinuity with his abdomen open and returned to the operating room the following day for anastomosis and abdominal wall closure. The anastomosis was approximately 10 cm distal from the ligament of Treitz. He had approximately 150 cm of bowel remaining. His feeds were advanced, and he was extubated to room air by postoperative day 5. He was discharged on postoperative day 19 to home with nasogastric tube feeds. He was seen in the clinic several months after discharge and was doing well without any ongoing concerns. A chest radiograph was completed without evidence of recurrence, and he was tolerating full feeds orally without need for supplementation.Congenital diaphragmatic hernias should remain on the differential for postbirth respiratory failure even if not known prenatally with normal postbirth imaging.American Board Of Pediatrics Neonatal–Perinatal Content SpecificationKnow common disorders of the chest wall, diaphragm, and pleura.Understand indications, management, and complications of medical technology (eg, central lines, gastronomy, tracheostomy, ostomy, ventriculoperitoneal shunt, and extracorporeal membrane oxygenation).