What is the treatment for a newborn diagnosed with congenital diaphragmatic hernia (CDH)?

Treatment of Congenital Diaphragmatic Hernia in Newborns

Immediate intubation at birth with gentle ventilation using low tidal volumes (3.5-5 mL/kg) and minimal peak inspiratory pressures is the cornerstone of initial management, followed by delayed surgical repair only after medical stabilization. 1, 2

Initial Stabilization and Respiratory Management

Ventilation Strategy:

• Minimize peak inspiratory pressure and avoid large tidal volumes (target 3.5-5 mL/kg) to prevent ventilator-associated acute lung injury—this is a Class I recommendation 3, 1
• The hypoplastic lung in CDH has reduced functional residual capacity and is highly susceptible to barotrauma and volutrauma 1
• Maintain oxygen saturations between 92-95% to avoid hypoxemia while preventing oxygen toxicity 3, 1
• High-frequency oscillatory ventilation is a reasonable alternative (Class IIa, Level A) when conventional ventilation fails due to poor lung compliance and inadequate gas exchange 3, 1, 4

Critical Pitfall:

• Do NOT use surfactant therapy in CDH—it has been associated with increased need for ECMO, higher incidence of chronic lung disease, and increased mortality 4

Management of Pulmonary Hypertension

Pulmonary hypertension occurs in 63% of CDH cases and carries 45% mortality, making it the critical determinant of survival 1, 4, 5

Inhaled Nitric Oxide (iNO):

• Do NOT use iNO routinely in CDH 4
• Use iNO selectively only in infants with severe PH and suprasystemic pulmonary vascular resistance causing critical preductal hypoxemia (Class IIa, Level B) 3, 1, 4
• Use cautiously in patients with suspected left ventricular dysfunction, as lowering pulmonary vascular resistance can worsen pulmonary edema 3, 4

Escalation of Therapy:

• ECMO is recommended for patients with severe PH who fail medical therapy (Class I, Level B) 3, 1, 4
• Prostaglandin E1 may be considered to maintain ductal patency and improve cardiac output in infants with suprasystemic PH or right ventricular failure (Class IIb, Level C) 3, 1, 4

Surgical Repair Timing and Approach

Timing:

• Delay surgery to allow optimal medical stabilization—surgery is NOT an emergency 5, 2, 6
• Perform appropriate preoperative assessment and stabilization before surgical intervention 5

Surgical Approach:

• Laparoscopic repair is preferred in hemodynamically stable infants without significant comorbidities (in-hospital mortality 0.14%) 1, 5
• Open surgical approach (laparotomy) is recommended for unstable patients with complicated CDH 1, 5
• Large defects (>8 cm or >20 cm²) require mesh reinforcement 1, 5
• Avoid tackers near the pericardium due to risk of cardiac complications 5

Diagnostic Confirmation

• CT scan of chest and abdomen is the gold standard for diagnosing CDH 1, 5
• Echocardiography is obligatory to evaluate pulmonary hypertension severity, which predicts survival 5
• Initial chest X-ray may be normal in up to 62% of cases, particularly with right-sided hernias 1

Long-Term Follow-Up Requirements

Multidisciplinary follow-up is essential as 87% of survivors have associated morbidity 2, 7, 6:

• Gastroesophageal reflux (up to 62% incidence) 1, 5
• Ongoing pulmonary sequelae 2, 7, 6
• Neurodevelopmental deficits 2, 7
• Gastrointestinal disorders 2, 7
• Evaluation for long-term PAH-specific therapy should follow standard pediatric PH recommendations 3, 1