Here is a complete, structured management guide for CDH, tailored to this case.
Management of Congenital Diaphragmatic Hernia (CDH)
Based on this ultrasound finding at 33 weeks, management is divided into three phases: Antenatal (remaining pregnancy), Neonatal Stabilization, and Surgical Repair, followed by long-term follow-up.
PHASE 1 - Antenatal Management (Now, at 33 Weeks)
1. Urgent Referral to a Tertiary/Fetal Medicine Centre
The single most important step right now. As stated in Creasy & Resnik's Maternal-Fetal Medicine: "Diagnosis of CDH should prompt referral to a tertiary center that is experienced in assessing this anomaly and managing CDH in the perinatal period." A centre with a Level III NICU and pediatric surgical team is mandatory.
2. Confirm and Characterize the Diagnosis
A detailed workup at the tertiary centre includes:
| Investigation | Purpose |
|---|
| Detailed anomaly ultrasound | Confirm CDH; identify which organs are herniated (stomach, bowel, liver); assess liver position (liver-up vs liver-down - prognostic) |
| Lung-to-Head Ratio (LHR) / O/E LHR | Key prognostic tool - measures contralateral lung area divided by head circumference; expressed as observed/expected (O/E LHR) for gestational age |
| Fetal MRI | Better visualization of lung volumes, liver position, and extent of herniation than ultrasound alone |
| Fetal echocardiography | Rule out congenital heart defects (associated in ~30% of CDH cases); detect early pulmonary hypertension signs |
| Chromosomal/Genetic testing | Array CGH or karyotype - CDH is associated with trisomies and microdeletions; associated anomalies raise mortality to >85% |
Prognostic interpretation of O/E LHR (Sabiston Textbook of Surgery):
- O/E LHR < 25% = severe CDH, ~15% survival without intervention
- O/E LHR 25-35% = moderate CDH
- O/E LHR > 45% = mild CDH, survival approaches 80-90%
3. Prenatal Intervention - FETO (Fetoscopic Endoluminal Tracheal Occlusion)
This is the only available fetal therapy for severe CDH. A small balloon is placed endoscopically to block the fetal trachea (usually at 27-29 weeks gestation), which causes lung fluid accumulation and stimulates lung growth. The balloon is removed several weeks later.
Evidence: The landmark TOTAL (Tracheal Occlusion to Accelerate Lung Growth) randomized controlled trial showed that in
severe CDH (O/E LHR < 25%), FETO improved survival to discharge from
15% to 40%. The moderate CDH trial (O/E LHR 25-35%) did not show benefit. A
2026 systematic review (PMID: 41319474) confirms prenatal imaging and this prognostic stratification remain central to management.
Important for this case: At 33 weeks, the window for FETO (ideally 27-29 weeks) has already passed. Therefore, antenatal management will focus on delivery planning rather than fetal intervention.
Complications of FETO: Preterm labor, PPROM, premature birth, fetal demise - which is why timing matters.
4. Multidisciplinary Counselling
Parents must be counselled by maternal-fetal medicine, neonatology, pediatric surgery, and genetics. Informed decision-making about resuscitation options should be discussed given the prognosis, especially if additional anomalies are identified.
5. Delivery Planning
- Delivery should occur at the tertiary centre (do NOT deliver at a peripheral hospital)
- Timing: CDH itself is not an indication for preterm delivery; aim for term unless obstetric indications arise
- Mode: Vaginal delivery is generally acceptable; caesarean section only for obstetric indications
- A neonatal resuscitation team (neonatologist, pediatric surgeon, NICU) must be present at delivery
PHASE 2 - Neonatal Stabilization (Immediately After Birth)
Key principle: CDH is a PHYSIOLOGIC emergency, not a surgical emergency. Stabilize first, operate later.
Immediate Resuscitation
- Immediate intubation at delivery - do NOT attempt bag-mask ventilation (risks gastric distension and further lung compression)
- Insert a nasogastric tube for gastric decompression
- Avoid nitrous oxide (causes bowel gas expansion)
- Limit peak inspiratory pressure < 25 cmH₂O to prevent barotrauma
- Target preductal SpO₂ 85-95%; accept mild hypoxemia rather than over-ventilate
Ventilatory Strategy - "Gentle Ventilation" (Gentilation)
The mainstay of CDH management per Sabiston Textbook of Surgery:
- Permissive hypercapnia: allow PaCO₂ up to 60-65 mmHg (post-ductal)
- Pressure-limited ventilation with low tidal volumes
- High-Frequency Oscillatory Ventilation (HFOV) if conventional ventilation fails - provides better gas exchange with less barotrauma
- Avoid aggressive ventilation that worsens pulmonary hypertension
Treatment of Pulmonary Hypertension
This is the key driver of mortality:
| Treatment | Mechanism |
|---|
| Inhaled Nitric Oxide (iNO) | Selectively dilates pulmonary vasculature, reduces right-to-left shunting |
| Sildenafil (PDE-5 inhibitor) | Pulmonary vasodilation (oral/IV) |
| Prostacyclin (PGI₂) / Treprostinil | Pulmonary vasodilators for refractory PH |
| Milrinone | Inodilator - reduces pulmonary hypertension and supports cardiac function |
| PGE₁ | Maintains ductal patency in refractory cases |
ECMO (Extracorporeal Membrane Oxygenation)
Used when the baby fails to stabilize despite maximal medical therapy:
- Venoarterial (VA) ECMO preferred for combined cardiac and respiratory failure
- Allows the lungs to "rest" while maintaining systemic perfusion
- Bridging therapy until the baby is stable enough for surgery
- As noted in Miller's Anesthesia: "Unstable neonates with CDH may require ECMO and inhaled nitric oxide" before surgery can be planned
PHASE 3 - Surgical Repair
Timing
- Not an emergency - delay surgery until hemodynamic stability is achieved
- In stable infants: defer repair 48-72 hours after birth to allow pulmonary vascular lability to settle (Sabiston)
- In ECMO-dependent infants: timing is debated; some favour repair during ECMO, others after decannulation (bleeding risk is higher on ECMO)
Surgical Approach
Open repair (most common) via ipsilateral subcostal incision:
- Reduce herniated viscera back into the abdomen
- Excise hernia sac (if present)
- Identify and mobilize diaphragm leaflets
- Close the diaphragmatic defect
Closure technique:
- Primary repair with interrupted non-absorbable sutures - preferred when possible
- Patch repair (Gore-Tex / Surgisis) for large defects where primary closure would be under tension - patch repairs have higher recurrence rates but are necessary for large defects
Laparoscopic repair: feasible in stable infants with smaller defects; associated with higher recurrence rates than open repair; not suitable in hemodynamically unstable infants.
Thoracotomy approach: occasionally used for right-sided CDH.
If abdominal domain is lost after reduction: temporary abdominal silo or skin-only closure with delayed fascial repair.
PHASE 4 - Long-Term Follow-Up
Survivors of CDH require lifelong monitoring due to significant chronic morbidities (Sabiston):
| System | Complication |
|---|
| Respiratory | Bronchopulmonary dysplasia, chronic lung disease, recurrent respiratory infections |
| Cardiovascular | Persistent pulmonary hypertension (PPHN), right ventricular dysfunction |
| GI | Gastroesophageal reflux disease (GERD), foregut dysmotility, feeding difficulties |
| Musculoskeletal | Scoliosis, pectus excavatum, chest wall deformity |
| Neurodevelopmental | Developmental delay, hearing loss, seizures (especially after prolonged NICU care/ECMO) |
| Recurrence | Hernia recurrence - higher with patch repairs, requires surveillance |
| Growth | Growth retardation |
A
2025 systematic review (PMID: 40100453) confirms that CDH survivors have significant long-term morbidities beyond childhood, reinforcing the need for structured multi-specialty follow-up.
Summary Flowchart for This Case
33-week scan → CDH suspected
↓
URGENT referral to tertiary centre
↓
Detailed US + MRI + Fetal echo + Genetics
↓
FETO: NOT possible at 33 wks (window 27-29 wks)
↓
Plan delivery at tertiary centre (with NICU + peds surgery)
↓
At birth: Immediate intubation + NG tube
↓
Gentle ventilation + treat pulmonary hypertension
↓
If fails → ECMO
↓
Stabilise 48-72 hrs → Surgical repair
↓
Long-term NICU + multidisciplinary follow-up
Overall survival with modern neonatal care at high-volume centres: 65-90% for isolated CDH (Sabiston Textbook of Surgery). If associated with chromosomal anomalies or other structural defects, mortality exceeds 85%.