What is the treatment for congenital lobar emphysema (Verges disease) in infants and young children?

Treatment of Congenital Lobar Emphysema (Verges Disease)

Surgical lobectomy is the definitive treatment for congenital lobar emphysema (CLE) in infants and young children presenting with severe respiratory distress, particularly those under 2 months of age, while conservative management may be considered only for older infants (>2 months) with mild symptoms and normal bronchoscopy findings. 1, 2

Clinical Presentation and Diagnosis

The majority of patients with CLE present with severe respiratory distress requiring urgent intervention:

• 57% of patients are symptomatic at birth, with dyspnea (57%), cyanosis (43%), and recurrent respiratory infections (28.5%) as the primary presenting features 2
• All symptomatic children except newborns typically have a history of pulmonary infection before diagnosis 1
• The condition is frequently misdiagnosed as pneumothorax or pneumonia, leading to delayed treatment 3, 4

Diagnostic imaging should include:

• Chest radiography showing hyperaeration of the affected lobe with mediastinal shift and compression atelectasis of adjacent lobes 1, 2
• Thoracic CT scan as the primary radiologic method (diagnostic in 86% of cases) 1, 2
• Pulmonary perfusion scan demonstrating loss of perfusion in the affected lobe (positive in 88% of cases) 2

Surgical Management

Immediate surgical lobectomy is indicated for:

• All infants under 2 months of age regardless of symptom severity 2
• Infants older than 2 months presenting with severe respiratory symptoms (dyspnea, cyanosis, significant respiratory distress) 2
• Patients with progressive respiratory compromise or mediastinal shift 1

Surgical outcomes are excellent:

• All patients in reviewed series had uneventful postoperative courses with no mortality 1, 2
• Dramatic improvement occurs immediately after surgical removal of the affected lobe 4
• The affected lobe demonstrates significantly greater alveolar diameters compared to normal lung tissue, confirming the pathologic nature requiring removal 1

Conservative Management

Conservative management may be considered only in highly selected cases:

• Infants older than 2 months with mild to moderate respiratory symptoms 2
• Normal bronchoscopic findings excluding other airway pathology 2
• Asymptomatic or minimally symptomatic children identified incidentally 5

Critical caveats for conservative management:

• Radiological abnormalities persist despite symptom improvement during follow-up periods of 3 months to 4 years 2
• Families must be extensively counseled about the disease and warning signs of deterioration 2
• Close follow-up is mandatory, as patients can suddenly decompensate requiring emergency surgical intervention 4
• Only 6 of 11 symptomatic children showed spontaneous improvement with conservative management 5

Common Pitfalls to Avoid

Do not mistake CLE for pneumothorax or pneumonia, which leads to inappropriate treatment and delayed definitive management 3, 4

Do not delay surgery in severely symptomatic infants attempting conservative management, as the majority have respiratory distress too severe to avoid surgical intervention 1

Do not use positive pressure ventilation aggressively before surgery, as this can worsen hyperinflation and cause cardiovascular compromise 3

Ensure proper anesthetic management during surgery, as intraoperative oxygen desaturation may occur requiring modification of surgical approach (partial lobectomy if needed) 1

Anatomic Distribution

The left upper lobe is most commonly affected (57%), followed by right middle lobe (28.5%) and right upper lobe (14.5%) 2. Congenital cardiac anomalies occur in approximately 14.5% of cases and should be evaluated preoperatively 2.