The document discusses current management strategies for congenital diaphragmatic hernia (CDH), including the shift from immediate surgical repair to physiological stabilization and delayed repair based on determinants like pulmonary hypertension and lung hypoplasia. CDH has a varied etiopathogenesis and is associated with a 60-70% survival rate, with significant prognostic indicators such as liver herniation and lung area-to-head ratio. Surgical techniques and management approaches are detailed, including prenatal imaging, initial management procedures, and various repair methods that ensure better outcomes in critically ill infants.

1. Current concepts in the
management of Congenital
Diaphragmatic Hernia
Sanjay Khope
MS. M. Ch., FACS, FIAPS, FISPU
Pediatric Surgeon

2. Shift of Management Strategies
•From immediate Repair
•To management of Pulmonary
Hypertension
•Physiological Stabilization
•Delayed Surgical repair

3. Determinants
•Lung Hypoplasia
•Remodeled Pulmonary vasculature
•Ventricular dysfunction
•Genetic Syndromes-

4. incidence
• 1:3000 live births
• Survival remains 60-70%
• Pulmonary Hypertension and Lung Hypoplasia are key determinants
• Associated Cardiac anomalies 20%
• Syndromes
• Gastrointestinal anomalies
• Polyhydramnios
• Birth weight

5. Etiopathogenesis
• CDH complex developmental defect
• Etiologically heterogeneous 80% cause not known
• Congenital Heart disease 20%
• Numerous Syndromes are associated with CDH
• Cornelia de Lange
• Pallister-Killian
• Marfans’s Syndrome
• Environmental Factors-retinol signaling pathway: infants with CDH
have low levels of retinol and retinol binding protein

6. Etiopathogenesis
• Failure of diaphragm to fully
develop during
embryogenesis
• Or weakening of one of its
part
• Or failure to connect with
one another could
predispose to CDH
• Premature return of the
mid-gut into the abdominal
cavity with resultant
increase in the abdominal
pressure disrupting
diaphragmatic development

7. Anatomy
•Septum Transversum
•Pleuro-peritoneal folds
•Esophageal mesentery
•Musculature

8. Types of CDH
• 85-90 % Left side
• Bochdalek hernia 90% postero-lateral
• Morgagni( Right)
• Central
• Bilateral

9. Types of CDH
Bochdalek defect Morgagni defect

10. Clinical features
Respiratory distress
Scaphoid Abdomen
Central cyanosis
Late presentation
a) Recurrent respiratory
tract infections
b) failure to thrive
c) Masquerading as
pneumonia

11. Initial Management
Planned delivery
Resuscitation
Intubation
Ventilation
IV access
Fluids
ABG
Echo once stable

12. Initial Management
Resuscitation Intubation

13. Prenatal imaging
• Ultrasound
• Diagnosis is particularly difficult before 24 weeks
• Even after 24 weeks diagnosis is a challenge
• 25% of cases are missed and 11% of cases it is diagnosed Postnatally
• Suspected by absence of stomach in the normal intra abdominal
location
• Intra thoracic mass due to liver, bowel or stomach
• Some times indirect evidence due to abnormal cardiac axis ,
mediastinal shift or polyhydramnios

14. Role of fetal MRI
• To confirm the diagnosis in equivocal cases
• Assessment of prognosis of CDH
• Presence of liver herniation-poor prognostic indicator
• Review of 407 liver up and 303 liver down
• worst survival in 45.4% survival in liver up cases compared with 73.9%
liver down cases Also 80% liver up required ECMO compared to 25%
liver down cases and over all survival rate was 45% compared with
93% liver down cases.(Mullasery D et al. Ultrasound Ob Gyn. 2010;
33:609-140)

15. Prognosis
• Liver up
• Lung hypoplasia- ipsilateral lung more affected than contra lateral
• Sonographic measurement of Lung area –to- head ratio ( LHR).No
survivors with LHR ˂ 0.6
• Survival 100% if LHR was ˃1.35
• Low LHR ˂ 1.0 predicted increasing use of ECMO( 75%) and lower
survival 35%
• LHR has predictive value when measured at 24 weeks or after

16. Prognosis
• Measurement of fetal lung volume at 34 weeks gestation on fetal
MRI
• Fetal lung volume ˃20-25 ml and absence of Bad prognostic indicator
like liver up-SPONTANEOUS DELIVERY
• Infants who survived had high FLV compared to those who died
• Fetal lung-to-thorax ratio
• Fetal intervention /FETO/ are some of the options at 28 weeks
• High incidence of PROM and delivery despite best care
• Prenatal steroids

17. Associated malformations
• 40-60%
• CARDIAC
• RENAL
• GASTROINTESTINAL
• CNS
• AMNIOCENTESIS FOR GENETIC SYNDROMES

18. Surgical details
• Anesthetic considerations
• Precarious condition for transport
• Surgery in NICU IN VERY CRITICAL STABILIZED CASES
• Subcostal incision
• Reduce the contents
• Identify sac/ ICD
• Assess the repair modality
• Diaphragm substitutes-Dacron patch, fascia, Abdominal muscle flaps

19. LEFT POSTERIOR CDH

22. DIAPHAGMATIC DEFECT AFTER REDUCING THE CONTENTS

23. Completed repair

24. Large CDH
• Primary repair
• Synthetic patch
• Toldt’s fascia flap( the small medial muscle remnant, TF, peritoneum, and
retroperitoneal connective tissue, was mobilized carefully from the
ipsilateral kidney and adrenal gland, and the repair completed with
interrupted sutures using non absorbable material
• Split abdominal wall muscle flap- The split abdominal wall muscle flap is
performed by downward rotation of the internal oblique and transversalis
abdominal wall muscles. This repair requires that the initial subcostal
incision be positioned at least 4 to 5 cm below the costal margin, low
enough to insure an adequate length of muscle will be available to fill the
defect.

25. Abdominal muscle Pedicle flaps
•Split abdominal muscle flap with internal oblique
and transversalis
•Natural (autologous) substitute
•Repair more anatomical
•No second surgery for issues related to prosthesis,
recurrence rare
•Abdominal wall hernias not common
•12 cases with excellent results.

26. THANK YOU