Cong Diaphragmatic Hernia _ Case _ Presentation.pptx

CDH

1. Case Presentation: Congenital Diaphragmatic Hernia (CDH) Presented by: Dr. Ahmed ElhagAly

2. Case Introduction • Patient: Male neonate, born at 38w3d via emergency C-section due to failure to progress • Birth weight: 2500 g • Apgar Scores: 4 at 1 min, 7 at 5 min • Chief complaint: Respiratory distress and cyanosis immediately after birth • NICU Admission: Intubated at birth, placed on mechanical ventilation

3. Antenatal Diagnosis & Workup • 24-week ultrasound: Left-sided CDH suspected, stomach and bowel in thorax • Fetal MRI: - Observed-to-expected lung-to-head ratio (o/e LHR): 30% - Significant pulmonary hypoplasia • Fetal echocardiogram: No major cardiac anomalies • Amniocentesis: Normal karyotype, no genetic abnormalities

4. Birth & Immediate Postnatal Findings • Delivered via C-section due to non-reassuring fetal status • Required immediate intubation due to respiratory distress • Chest X-ray confirmed large left-sided CDH • Initial arterial blood gas (ABG): pH 7.12, pCO2 68, PaO2 45, lactate 4.2 • Pre-ductal SpO2: 80%, Post-ductal SpO2: 88%

5. Comprehensive Clinical Examination • **Cardiovascular:** Tachycardic (HR 170s), normal S1/S2, no murmur • **Respiratory:** Severe respiratory distress, absent breath sounds on left, scaphoid abdomen • **Neurological:** Hypotonic, hypoactive reflexes due to sedation • **Gastrointestinal:** Soft abdomen, bowel sounds auscultated in left chest • **Genitourinary:** Undescended left testicle, hypospadias

6. Expanded Diagnosis & Imaging • **Chest X-ray:** - Mediastinal shift to right - Bowel loops and stomach in thorax • **Echocardiogram:** - Pulmonary hypertension with bidirectional PDA shunt - Small ASD II with left-to-right flow • **ABG Interpretation:** - Respiratory acidosis, hypoxemia, metabolic compensation

7. Step-by-Step Management Plan 1. **Respiratory Support:** - Immediate intubation, avoid bag-mask ventilation - HFOV with inhaled nitric oxide (iNO) for pulmonary hypertension 2. **Cardiovascular Management:** - IV sildenafil, milrinone for PPHN - Dopamine, epinephrine for hypotension 3. **Metabolic & Nutrition Support:** - Total parenteral nutrition (TPN) - NPO until after surgery

8. NICU Course & Surgical Timeline • **Day 1-2:** Stabilization on HFOV + iNO • **Day 3:** Echo shows persistent pulmonary hypertension → Sildenafil started • **Day 5:** Transitioned to conventional ventilation • **Day 7:** Underwent laparotomy & left CDH repair with Gore-Tex patch • **Day 10:** Reintubation for post-op pulmonary hypertension crisis

9. Postoperative Recovery & Complications • **Ventilation:** Extubated on day 18, transitioned to nasal CPAP • **Feeding Intolerance:** Slow introduction of enteral feeds • **Sepsis:** Developed Gram-negative sepsis (Klebsiella), treated with meropenem • **Follow-up Echo:** Improvement in pulmonary hypertension, weaning sildenafil

10. Long-Term Prognosis & Follow-Up Plan • **Pulmonary:** Risk of chronic lung disease, home oxygen possible • **Gastrointestinal:** Monitor for GERD, motility issues • **Neurological:** Risk of developmental delay → Scheduled neurodevelopmental assessment • **Cardiac:** Follow-up echo in 6 months for residual pulmonary hypertension

11. Clinical Discussion & Learning Points • Early stabilization is key to preventing pulmonary hypertension crises • Timing of surgery depends on physiological stability, not age alone • Multidisciplinary approach is needed for long-term care • Advances in fetal intervention (FETO) may improve outcomes in future cases