1. CONGENITAL DIAPHRAGMATIC HERNIA
Dr Osama Bawazir
Assistant Professor , Consultant Pediatric surgeon
FRCSI, FRCS(Ed), FRCS (glas), FRCSC,
FAAP,FACS.

2. CDH-Epidemiology 1 in2000- 4000 births
95.8% posterolateral defects (Bochdalek)
84.4% left side
13.2% right side
2.4% bilateral
Morgagni and pars sternalis hernias rare
1/3 will have associated anomalies.

3. Anatomy
Septum transversum
Pleuroperitoneal membranes

4. Pathophysiology Combination of events… Physical compression
Pulmonary hypoplasia bilaterally (ipsilateral>contralateral)
Smaller bronchi
Less branching
Decreased alveolar SA
Pulmonary hypertension
Lack of pulmonary arterioles
Increased thickness of muscle
Abnormally high vascular reactivity of vessels
Right to left shunt

5. Diagnosis Prenatally U/S – can diagnose 50%, 50% “normal”,
as early as 15 weeks
Polyhydramnios
Absent or intrathoracic stomach bubble
Mediastinal or cardiac shift away from hernia
Assess other organs
Amniocentesis for chromosomes, alpha fetoprotein (18 weeks)

6. Diagnosis
Postnatal
RD in the first 24hours. scaphoid abdomen (majority)
After 24h with vomiting, cough, cyanosis, gastric outlet obstruction, herniation of liver, spleen, bowel.

7. Prognostic Indicators
*Herniated organs (liver)
*Lung to head ratio (LHR)
<0.6 poor, % survive, > % survive
Associated anomalies
Birth weight and Apgar score ( the CDH study group)
Measurement of L/T ratio
L/T normal ~ 0.52, severe < 0.26

8. Importance of Prenatal Dx
Check for associated anomalies
Plan for delivery at tertiary perinatal centre +/- ECMO
Options for parents
Prenatal therapies, termination, treatment after delivery

9. Case CXR Male, 36 week GA, 30 yr. old G1P0 SVD
Apgars 31,75,910, ETT in DR
BW – 2,700 g
Normal pregnancy
U/S – 28 week  CDH
Amnio – Normal, 46XY
On exam
Scaphoid abdomen
Bowel sounds over left chest
CXR

10. What's next?!

11. Management SVD Initial resuscitation Supplemental O2 No bag & mask
ETT with mechanical ventilation
Ventilation strategies*
Watch for pneumothorax (usually contralateral)
NG tube
Fluid status
Inotropic support
Delayed surgery ( until patient is stable).

12. CMV Paralysis Amp and gent NG tube Inotropic support
Require high setting, high FiO2
High OI= (OI= MAP×FiO2×100/PaO2)
Paralysis
Amp and gent
NG tube
Inotropic support

13. Ventilation Strategies
Prevent conditions that raise pulmonary vascular resistance (hypoxemia, acidosis, hypotension and hypercarbia)  hyperventlation to control hypoxemia
Aim: pH > 7.45, PCO2 < 35 , PaO2 > 60
 barotrauma.
Wung et al. showed that some of the mortality in CDH infants was,infact,due to ventilator-induced lung injury
Gentle ventilation with permissive hyper-capnea
Low PIP, pressure-limited ventilation, minimal set RR, minimal sedation and tolerance of high PCO2 > 50  survival>90%

14. Ventilation Strategies
HFOV
Good in animal models
little benefit
Nitric oxide
Selective pulmonary vasodilator
Result in Pt with CDH have been discouraging
Surfactant
ECMO

15. Preoperative management
If alternative ventilator strategies fail-consider Extracorporeal Membrane Oxygenation (ECMO)
Failure:
PaO2<40mmHg in 100% FiO2
Pulmonary HTN persists
Decide failure early

16. ECMO criteria
No strict criteria can accurately predict high mortality

17. ECMO in CDH 1st ECMO for CDH by german et al.in 1977
Treat infant post CDH repair
Now ECMO is strategy of pre-op stabilization.(only 5% post repair in 2001)
ECMO provides effective short-term support
The overall survival reported to ELSO is 52% only.(?? 64% in90 &52% in 01)
Survival was similar in 2 studies (one use ECMO in 50% and the other 1%)

18. Mode of ECMO in CDH Traditionally VA ( hemodynamically unstable)
VV is an acceptable initial mode for CDH .
No difference between R & L side CDH in regard to failure of VV and need to conversion to VA (Dimmitt et al) {??R CDH low venous drainage  VA}
Infants with CDH tend to had smaller veins (frenkner et al)

19. CDH surgery on ECMO
Risk of bleeding (early report significant hemorrhagic complications  low survival )
Close coagulation monitoring bleeding can be avoided.
Aminocaproic acid { 5% re-exploration for bleeding Vs 26% , circuit change (downard et al)}
CDH study group data
54% repaired on ECMO 30% following ECMO
Survival was 49% in infant repair on ECMO Vs 83% after ECMO

20. Long-term outcome
GERD
Neurological outcome

21. Pt put on ECMO
Start wean of ECMO in 10 days
Had surgical repair then in 24 hr had de-cannulation

22. Surgical Repair First repair in 1940 (Ladd & Gross)
1970’s – Early intervention
Emergent surgery to alleviate the compression of the “good” lung
Current practice allows a period of stabilization prior to repair
CDH is a physiologic emergency, not a surgical emergency
Pulmonary hypertension is the primary determinant of mortality

23. Surgical Repair

24. New Advances In utero Surgical repair - >70% mortality
Tracheal ligation
27-28 weeks GA
Causes distention and secondary hyperplasia
Good results in 40% of patients
Ventilation strategies

25. Thank You