Case of the Month 2016-09

Submitted by Jordan Gutovich, MD, and Thomas J. Learch, MD.

Respiratory Distress

An 11-month-old boy presented to an urgent care clinic for respiratory distress, fussiness and lethargy. His vital signs were significant for a slight fever of 100.6 F. He was treated with nebulizer treatments of albuterol and sent home with a prescription for amoxicillin. After several days, his symptoms did not improve and he presented to the emergency department. A chest radiograph was obtained that demonstrated a large consolidation in his left lung (Figure 1). He was treated for pneumonia with IV ceftriaxone and discharged home with a prescription of oral Cefdinir. After several weeks, the patient continued to exhibit increased work of breathing, fussiness and nasal flare. A follow up chest radiograph demonstrated a persistent large consolidation in the left lung (Figure 2).

A CT of the chest was obtained that revealed a large left congenital diaphragmatic hernia with a large amount of abdominal contents in the the thoracic cavity (Figures 3 & 4). The patient was taken to the operating room and underwent a thoracoscopic repair of the left congenital diaphragmatic hernia that was of the Bochdalek subtype. A follow-up postoperative chest radiograph demonstrated a large left pneumothorax and a hypoplastic left lung (Figure 5).

Figure 1. Frontal chest radiograph. For a larger image, please click Gutovich 1 .

Figure 2:Frontal chest radiograph. For a larger image,please click Gutovich 2 .

Figure 3: Noncontrast enhanced CT scan of the chest. For a larger image, please click on Gutovich 3 .

Figure 4: Noncontrast enhanced coronal reformatted image from CT scan of the chest. For a larger image, please click on Gutovich 4 .

Figure 5: Frontal chest radiograph. For a larger image, please click on Gutovich 5 .

Discussion

Congenital diaphragmatic hernias are a result of a pleuroperitoneal foramen and are more common to occur in males than females (1.5:1.0). Additionally, they are more frequent on the left than right (5:1). Posterior hernias are known as Bochdalek hernias, while anterior hernias are termed Morgagni hernias. Symptoms include severe respiratory distress immediately after birth. A diagnosis is usually made based on a chest radiograph that typically demonstrates bubbly lucencies in the chest that resemble bowel. Treatment is surgical repair of the pleuroperitoneal foramen and reduction of herniated contents back into the abdomen. Survival rate for patients with this entity range between 47 percent and 93 percent with prognosis usually related to the degree of lung hypoplasia on the affected side.

References

Johnson AM et al: Congenital anomalies of the fetal/neonatal chest. Semin Roentgenol. 39(2):197-214, 2004

Taylor GA et al: Imaging of congenital diaphragmatic hernias. Pediatr Radiol. 39(1):1-16, 2009

Clark RH et al: Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group. J Pediatr Surg. 33(7):1004-9, 1998

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