CONGENITAL DIAPHRAGMATIC HERNIA (CDH) 1- Embryology 2- type and pathophysiology 3- clinical presentation 4- diagnosis 5- management

3. THE THORACIC DIAPHRAGM

5. EMBRYOLOGY
■ Simply, it is formed by the fusion of tissues from 4 DIFFERENT SOURCES:
I. Septum transversum (central tendon)
II. Pleuroperitoneal membranes
III. Dorsal mesentery of the esophagus
IV. The body-wall (muscular components from somites (C3-5)

9. PATHOPHYSIOLOGY
■ Most frequently caused by failure of one or both of the
pleuroperitoneal membranes to close the pericardioperitoneal
canals
■ Thus, the peritoneal and pleural cavities are continuous with
one another along the posterior body wall
■ This allows abdominal viscera to enter the pleural cavity,
pushing the lungs and heart
■ In 85% to 90% of cases, the hernia is on the left side

11. TYPES
■ The Bochdalek:
– Most common
– Defect in the posterolateral part of the diaphragm.
– This causes lung hypoplasia.
■ The Morgagni (retrosternal):
– Rare
– Results from a defect in the anterior midline,
■ Hiatal hernias:
– portion of the stomach prolapses through the diaphragmatic esophageal hiatus
– The defect thought to be a result of a congenital shorter esophagus.

12. [B] Bochdalek left posterolateral defect.
[M] Morgagni or anterior.
[H] Hiatus hernia.
[E] large Eventration in the tendinous portion.
[T] a Tear which causes a post-traumatic hernia.
[A] Aorta.

15. CASE SCENARIO
■ Within minutes of birth, a full-term infant boy
develops increasing respiratory distress and
becomes cyanosed. He fails to improve with
upper airway suctioning. The pregnancy was
uneventful. He looks barrel-chested and his
abdomen is scaphoid.

16. Case Scenario
■ Within minutes of birth, a full-term infant boy develops
increasing respiratory distress and becomes cyanosed.
He fails to improve with upper airway suctioning.The
pregnancy was uneventful. He looks barrel-chested and
his abdomen is scaphoid.

18. CLINICAL
PRESENTATION

19. Clinical Presentation
■ The development of symptoms with CDH
correlates with the degree of pulmonary
hypoplasia and pulmonary hypertension. (1)
■ Infants most commonly present with respiratory
distress and cyanosis in the first minutes or
hours of life.(1)
(1)CURRENT Diagnosis &Treatment:Surgery,Chapter 43:
PediatricSurgery

20. Clinical Presentation
(1)CURRENT Diagnosis &Treatment:Surgery,Chapter 43:
PediatricSurgery
The majority of neonates born with a Bochdalek diaphragmatic hernia become symptomatic
at, or shortly after, birth.(2)
 Tachypnoea
 Increased respiratory effort
 Hyperinflated Chest
 Scaphoid abdomen
 Heart sounds are on the right side
 The chest on the side of the hernia may be dull
to percussion (1)
 bowel sounds are not usually appreciated.(1)
Where pulmonary hypoplasia is severe, the neonate becomes cyanosed with severe
respiratory distress within minutes of birth. (2)
(2)Jones Clinical PediatricSurgery 7th Edition

21. Clinical Presentation
■ honeymoon period can happen:(3)
– Which is a period (first 24 to 48 hours after
birth ) of relative stability with high levels of
PaO2 and relatively good perfusion which
followed by progressive cardiorespiratory
deterioration. (3)
(3) Schwartz's Principles of SurgeryChapter 39: PediatricSurgery

22. Clinical Presentation
(2)Jones Clinical PediatricSurgery 7th Edition
Postero-lateral hernia involve: (2)
The left Hemidiaphragm (85%)
Right-sided (13%)
Bilateral (2%)
Associated anomalies occur in up to 40% of patients
Most are minor and do not affect survival.
The most common serious abnormalities are heart defects, which
affect 20–25% of patients.

23. Clinical Presentation
(2)Jones Clinical PediatricSurgery 7th Edition
Retrosternal hernia:(2)
Usually asymptomatic unless strangulation occurs.
 Usually Protrudes into the inferior mediastinum
May protrude into the pericardial cavity and cause
cardiac tamponade (Very rare) Presents as
Cardiorespiratory distress in the neonatal period

24. DIAGNOSIS

25. PRENATAL DIAGNOSIS
Most congenital diaphragmatic hernias are diagnosed
well before birth, on antenatal ultrasonography. (2)
(2)Jones Clinical PediatricSurgery 7th Edition

26. Prenatal Diagnosis
■ Diagnosis of congenital diaphragmatic hernia (CDH)
can be achieved as early as 15 weeks’ gestation. (3)
■ US findings:
– herniated abdominal viscera in the chest
– changes in liver position
– mediastinal shift away from the herniated viscera
(3) Schwartz's Principles of SurgeryChapter 39: Pediatric
Surgery

27. Prenatal Diagnosis
Arrows point to the location of the diaphragm. Arrow head points to the stomach, which is
in the thoracic cavity. (3) Schwartz's Principles of SurgeryChapter 39: Pediatric
Surgery

28. Prenatal Diagnosis
What is the benefit of Prenatal Diagnosis?(3)
– optimizes prenatal and postnatal care of both the mother and fetus
– allows the mother-to-be to be transferred to a tertiary paediatric surgical centre
before birth.
(3) Schwartz's Principles of SurgeryChapter 39: Pediatric
Surgery

29. Prenatal Diagnosis
■ Case 1:
– Fetal diagnosed with CDH in week 15 of gestation.
■ Case 2:
– Fetal diagnosed with CDH in week 23 of gestation.
■ Which case have better prognosis ?Why? (3)
– Case 2, early antenatal diagnosis is associated with worse outcomes
(3) Schwartz's Principles of SurgeryChapter 39: Pediatric
Surgery

30. Prenatal Diagnosis
(2)Jones Clinical PediatricSurgery 7th Edition

31. Postnatal Diagnosis
diaphragmatic eventration : Elevated thinned out diaphragm, Bowel loops in chest
and Mediastinal shift

32. Postnatal Diagnosis
(2)Jones Clinical PediatricSurgery 7th Edition
congenital diaphragmatic hernia (Bochdalek type). Multiple bowel loops fill the left pleural
cavity, and the heart is displaced to the right.

33. Postnatal Diagnosis
■ the diagnosis of CDH is made by chest x-ray
■ In left-sided defects: (2)
– Loops of bowel may be seen in the left chest.
– The heart is deviated to the right.
– The hypoplastic left lung appears small and is further compressed by the mass effect of the
herniating liver, gut and spleen.
■ Sometimes the appearance may be difficult to distinguish from basal lung cysts, in which case
a repeat chest x-ray is performed after a nasogastric tube has been inserted, the tip of which
may be seen in the chest (2)
(2)Jones Clinical PediatricSurgery 7th Edition

34. Postnatal Diagnosis
■ a barium study will show bowel within the thoracic cavity when there is a diaphragmatic
hernia.(2)
■ Right-sided hernias can be difficult to distinguish from a diaphragmatic eventration. (1)
– This can be differentiated by an MRI scan
(2)Jones Clinical PediatricSurgery 7th Edition
(1)CURRENT Diagnosis &Treatment:Surgery,Chapter 43:
PediatricSurgery

35. Postnatal Diagnosis
■ Once the diagnosis of a CDH is confirmed, additional radiographic and US
examinations should be carried out to search for associated anomalies.

36. MANAGEMENT

37. Management
■ in utero correction of diaphragmatic hernia and fetoscopic tracheal occlusion
has been achieved in a research setting.
■ However, the techniques are complex and the indications are still being
refined.
■ No improvement survival, nor reduced morbidity, compared with current
postnatal techniques.
(2)Jones Clinical PediatricSurgery 7th Edition

38. Management
■ Initial treatment :
• involves intensive cardiorespiratory support and insertion of a nasogastric
tube to prevent bowel dilatation within the chest.
• Care must be taken to avoid hyperinflation and barotrauma of the small
hypoplastic lungs.
• High-frequency oscillatory ventilation (HFOV) in combination with nitric
oxide has improved survival rates.
(2)Jones Clinical PediatricSurgery 7th Edition

39. Management
■ Ventilation with a facemask should be avoided as this may force air into the stomach,
increasing its volume at the expense of the already compromised lungs.
■ Vigorous endotracheal ventilation should also be avoided because of the risk of causing
barotrauma or tension pneumothorax.
(2)Jones Clinical PediatricSurgery 7th Edition

40. Management
■ Sudden deterioration of the neonate’s condition during initial resuscitation
suggests a developing tension pneumothorax.
– may necessitate prompt release by needle aspiration or insertion of an
intercostal drain.
■ How to avoid this complication ?
– strict avoidance of hyperventilation and limited inflation pressures
(2)Jones Clinical PediatricSurgery 7th Edition

41. Management
■ The key to success is careful gentle ventilation that minimizes injury to the
hypoplastic lungs.
(2)Jones Clinical PediatricSurgery 7th Edition

42. Management
(2)Jones Clinical PediatricSurgery 7th Edition
• The primary focus of management is achieving adequate ventilation with control of
pulmonary hypertension.
• Surgery to return the herniated contents to the abdominal cavity and to repair the defect in
the diaphragm is only performed once the neonate’s condition has stabilized.

43. Management
■ Surgery generally involves repair through a transverse or subcostal abdominal
incision on the same side as the defect.
■ Thoracoscopic surgery has a role in selected cases, usually with smaller
diaphragmatic defects.
(2)Jones Clinical PediatricSurgery 7th Edition

44. Management
■ the hernia can be repaired in one of three ways:
– Primary Repair: If the opening in the diaphragm is small and the amount of diaphragmatic
muscle is adequate, is adequate muscle for closure, a single layer of nonabsorbable
horizontal mattress sutures can be used (3)
– Patch Repair: If the opening in the diaphragm is large or a significant portion of the diaphragm
is absent, the surgeon will require additional tissue or material to close the defect. Often, a
synthetic biocompatible material will be used to patch the opening(4)
– Muscle-Flap Repair: In some patients, the surgeon will close the hole in the diaphragm with a
flap of muscle-the internal oblique or latissimus dorsi from the infant's abdomen/back (4)
(4)University of California, San Francisco (UCSF)(3) Schwartz's Principles of SurgeryChapter 39: Pediatric
Surgery

45. Management
■ Survival rates post-surgery of more than 80%
■ The major cause of death remains pulmonary hypoplasia and pulmonary
hypertension
– due to the small pulmonary vascular bed and to the changing resistance of
the pulmonary arterioles
(2)Jones Clinical PediatricSurgery 7th Edition

46. Management
■ Anterior diaphragmatic hernia :
■ Repair is still advisable because of the risk of strangulation of the bowel that
protrudes through the defect.
■ Commonly performed as a laparoscopic procedure
(2)Jones Clinical PediatricSurgery 7th Edition

47. Management
■ Recurrence: (1)
– occurs in 10%-20% of infants
– is most common when a prosthetic patch is used for the repair
– It should be considered in any child with a history of CDH who presents with
new GI or pulmonary symptoms.
(1)CURRENT Diagnosis &Treatment:Surgery,Chapter 43:
PediatricSurgery

48. 1 2 3
4 - University of California, San Francisco (UCSF)
http://www.pedsurg.ucsf.edu/conditions--procedures/congenital-diaphragmatic-hernia.aspx

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