Congenital Diaphragmatic Hernia (CDH)

Delivery Room Presentation

• Antenatal diagnosis usually known (polyhydramnios, shifted mediastinum on ultrasound)
• Postnatal: Scaphoid abdomen, severe respiratory distress, barrel chest
• Diminished breath sounds on the affected side (usually left)
• Heart sounds shifted to the contralateral side

Immediate Delivery Room Management

• DO NOT use bag-mask ventilation (forces gas into herniated bowel, further compressing lungs)
• Immediate endotracheal intubation at delivery
• Insert a large-bore replogle/orogastric tube (10-12 Fr) to continuous low suction to decompress stomach and bowel
• Gentle ventilation protocol (PIP < 25 cmH2O) to avoid pneumothorax in the hypoplastic lung

NICU Stabilization

• Secure venous and arterial access (umbilical lines preferred)
• Strict management of Pulmonary Hypertension (PPHN): echocardiogram, Inhaled Nitric Oxide (iNO), milrinone
• Avoid severe hyperventilation: Permit mild hypercapnia to protect lungs
• Early evaluation for ECMO if oxygenation failure despite iNO and HFOV
• Surgical repair is DELAYED until respiratory parameters and pulmonary hypertension are stable (usually days 3-7)

Perioperative & Wound Protocol

• Strict optimization of acid-base balance (pH > 7.30) and normothermia (36.5-37.5 C) before surgical transport.
• Pain Management: Morphine infusion 10-20 mcg/kg/hr or Fentanyl infusion 1-2 mcg/kg/hr; utilize FLACC/NIPS scales.
• Avoid excessive traction on bowel or silo structures; inspect mesenteric color Q1H for ischemia.
• Wound care: Sterile saline packing or specialized dressings strictly per Pediatric Surgery direction.
• Do NOT administer nephrotoxic drugs post-op until urine output is > 1.5 mL/kg/hr.

Standardized Discharge & Follow-Up Criteria

• Adequate weight gain explicitly > 15-30g/day over a continuous 3-5 day period.
• Maintenance of temperature in an open crib with standard clothing for 48 hours.
• Successful completion of the 90-120 minute Infant Car Seat Challenge.
• Caregiver successfully completed CPR training, rooming-in, and independent medication administration.
• Referrals placed for High-Risk Infant Follow-Up Clinic, Early Intervention (PT/OT), and appropriate sub-specialists.

Diagnostic & Management Algorithm

• CDH Delivery Room Algorithm: [START] Known CDH infant born -> [ACTION] Immediate Intubation (DO NOT BAG/MASK) -> [PROCEDURE] Insert Replogle tube -> [MONITOR] Pre-ductal SpO2 -> [GOAL] Permissive Hypercapnia & Target SpO2 85%+

References

1. Logan JW, Rice HE, Goldberg RN, Cotten CM. Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies. J Perinatol.
2. Lally KP. Congenital diaphragmatic hernia - approaches to management. Semin Pediatr Surg.