1. MODERATOR : DR NEBIYU SHITAYE (MD, Assistant professor
of general surgery and consultant paediatrics surgeon)
PRESENTOR : Dr.Worku M.(GSR3)

2. INTRODUCTION
• Congenital diaphragmatic
hernia (CDH) is a
developmental defect of
the diaphragm that allows
abdominal viscera to
herniate into the chest.
• Herniated contents often
include
 The left lobe of the liver,
 The spleen, and
 Almost the entire
gastrointestinal tract.
 The stomach
 The kidney

3. EPIDEMIOLOGY
• The reported incidence of CDH is estimated to be
between 1 in 2000 to 5000 births.
• However, the overall incidence of CDH may be
underestimated.
• Approximately one third of infants with CDH are
stillborn which is less well documented.
• When stillborns are counted with live births, females
appear to be more commonly afflicted than males.
• Infants with isolated CDH are typically male and one
third are associated with a major congenital
anomaly.

4. EPIDEMIOLOGY…
Bochdalek(8%)
Left sided(80/85%)
Right
sided(20/13%)
Morgagni (2 %) Left sided (10%)
Right sided(90%)
Bilateral(rare/2%)
Bilateral(not
uncommon/rare)

5. Embryology
• Incompletely understood
involves multiple, complex
cellular and tissue
interactions.
• The precursors of diaphragmatic
structure begin to form during
the fourth week of gestation.
• As the embryo begins to form,
the septum transversum
migrates dorsally and
separates the
pleuropericardial cavity from
the peritoneal cavity.
• At this point, the pleural and
peritoneal cavities still
communicate.

6. • The septum transversum
interacts with the
pleuroperitoneal folds (PPF)
and mesodermal tissue
surrounding the developing
esophagus and other foregut
structures, resulting in the
formation of primitive
diaphragmatic structures.
Diaphragm develop from the fusion of
four embryonic components:
Anteriorly by the septum transversum,
Dorsolaterally by the pleuroperitoneal
folds (PPF),
Dorsally by the crura from the
esophageal mesentery, and
Posteriorly by the body wall mesoderm

7. Associated malformations
• Incidence of associated
malformations in infants with a
CDH ranges from 10% to 50%.
1) Skeletal defects (32% )
2) Cardiac anomalies (24%)
3) Anatomic anomalies of the
tracheobronchial tree (18% )
 Congenital tracheal stenosis,
 Trifurcated trachea.
4) Neural tube defects
Abnormalities noted stillborn
group
Anencephaly,
Myelomeningocele, and
Hydrocephalus
5) Other midline
developmental anomalies
Esophageal atresia,
Omphalocele, and
Cleft palate.

8. Clinical Presentation
• At birth
• Immediate respiratory distress with associated low Apgar
scores to
• An initial stable period and
• A delay in respiratory distress for 24 to 48 hours.
• Initial signs associated with respiratory distress include
 Tachypnea,
 Chest wall retractions,
 Grunting,
 Cyanosis, and
 Pallor.

9. • On physical examination
 Scaphoid abdomen
 Increased chest diameter.
 The point of maximal cardiac impulse is often
displaced, suggesting mediastinal shift.
 Bowel sounds may be auscultated within the
chest cavity with a decrease in breath sounds
bilaterally.
 Chest excursion may be reduced, suggesting a
lower tidal volume.

10. DIAGNOSIS
Prenatal Diagnosis -discovery is 24 weeks
• The diagnosis of a CDH by prenatal ultrasound (US) -
accurate in 40%to 90% of cases.
 Obstetrical ultrasonography-signs of fetal
diaphragmatic hernia.
 Displacement of the mediastinum,
 The absence of a stomach bubble in the abdomen,
and
 The presence of abdominal organs in the chest
 Polyhydramnios has been reported present in up to
80% of pregnancies with associated CDH.
 Identifying the gallbladder in the fetal chest may be the
most reliable sign in these cases.

11. Postnatal x-ray

12. TREATMENT
• PREOPERATIVE CARE
• Resuscitation
• CDH is a physiologic emergency and not a surgical emergency.
• Resuscitation begins with endotracheal intubation and
nasogastric tube insertion.
• Arterial and venous access should be acquired through the
umbilicus
• Proper temperature regulation, glucose homeostasis, and
volume status , maintain adequate oxygen delivery.
• Infants should be properly sedated, and any combination of
agents, including midazolam, fentanyl, or morphine, can be
used.
• Muscle paralysis is strongly discouraged because of its
untoward consequences on ventilatory mechanics and
potential morbidity.

13. SURGICAL MANAGEMENT
• Operative Repair- Transabdominally
VS Thoracoscopic
1) Position of patient
 The infant is positioned supine,
elevating the thoracolumbar spine.
 Both the upper abdomen and chest
are prepared as the operating fields.
2) Incision
 An ipsilateral subcostal incision is
made 1 cm below the costal margin.
 To create a muscle flap with the
transversalis abdominis muscle,
make the incision 2 cm below the
costal margin.

14. 3) Exposed and Reduction
• The defect is exposed after
the stomach is
decompressed, and the
viscera are reduced from
the chest.
• Reduction of the liver and
spleen may be challenging,
but can be accomplished by
careful manual reduction
using downward pressure
from above.

15. 4) True hernia sac Mx
 If a true hernia sac exists, it
must be excised to ensure
proper healing of the
defect.
Primary repair is
accomplished with
interrupted, simple, non-
absorbable sutures.

16. 6) If a prosthetic patch is required,
1-mm polytetrafluoroethylene
(PTFE) is preferred.
 Laterally, the patch can be
anchored to the ribs.
 An ipsilateral thoracostomy
tube is unnecessary unless
there is concern for
 Bleeding or
 An air leak.

17. Prognostic Criteria
• The factors most consistently associated with a poor
outcome on prenatal ultrasound are
(1) the presence of liver herniation into the chest; and
(2) a low lung-to-head ratio (LHR).
100% survival with an LHR greater than 1.35,
61% survival with an LHR between 0.6 and 1.35, and
0% survival with an LHR less than 0.6.
3) The observed to expected LHR (OE LHR)
OE LHR <25% is associated with an 18% survival
OE LHR >45% correlates with 89% survival.
4) Older patients who present later in life have a much
better prognosis

18. Follow-Up Guidelines

19. REFERENCES