Congenital Posterolateral Diaphragmatic Hernia and Other Less Common Hernias of the Diaphragm in Infants and Children

1
Congenital Posterolateral Diaphragmatic Hernia
and Other Less Common Hernias of the Diaphragm in
Infants and Children
2
A. CONGENITAL POSTEROLATERAL DIAPHRAGMATIC HERNIAS

• The diagnosis of a congenital diaphragmatic
• hernia can be made in utero.
• Infants and older children who are found to have
  a congenital diaphragmatic hernia are not as
  severely affected as the neonate and should have
  100 predicted survival.

3
Embryology

• The Bochdalec hernia is a classic posterolateral
  defect of diaphragm caused by failure of the
  pleuroperitoneal canal to close at 8 weeks
  gestation.
• The defect occurs on the left side 80 of the
  time and is occasionally bilateral.
• The hole can be 1-2 cm in size to total absence
  of hemidiaphragm.

4

• Fig 50-1

5
Embryology

• When the intestine return to the abdomen from the
  yolk sac, the abdominal organ will herniate to
  the chest at 10 weeks gestation
• The mediastinum will be pushed into contralateral
  side and both lungs will be hypoplasia.

6
Embryology

• 72 of posterolateral diaphragmatic hernia
  fetuses have extradiaphragmaic abnormalities,
  such as congenital heart disease, neural tube
  defect, genitourinary, skeletal, craniofacial
  anomaly, abdominal wall defectetc.

7
Embryology

• Prognostic factors based on prenatal ultrasound
  is problematic.
• Prenatal diagnosis was ever considered a poor
  prognostic factor, but the significance decreased
  due to increased numbers of ultrasound.
• Observation of the liver in the chest is a poor
  prognostic factor.

8
Embryology

• Fetal abdominal circumference is less than fifth
  percentile is a poor prognostic factor.
• Metkus et al reported that the size of
  contralateral lung measured as a ratio of lung
  area to head circumference was the only absolute
  predictor of survival.

9
Presentation

• It may cause respiratory distress, feeding
  intolerance.
• Some patients have no symptoms.
• The diagnosis is suspected if the abdomen is
  scaphoid or heart sound can be heard in the right
  chest.
• Chest radiography can reveal air-filled loops of
  intestines or an NG tube.

10

• table 50-1

11

• Fig 50-2, -3

12
Presentation

• Any baby with respiratory distress at birth who
  is suspected to have a Bochdalec hernia must be
  intubated and ventilated. Mask bagging can
  increase abdominal distention and should be
  avoided.
• Mechanical ventilation with 100 FiO2 and low air
  pressure(lt25 cm H2O with 5 cm of PEEP) should be
  used.

13
Presentation

• ECMO, dopamine (low dose), dobutamine, 5
  albumin( 10-15 ml, IV bolus, for systemic
  hypotension) may be necessary.
• If a Bochdalec hernia is found in an older infant
  or a child incidentally, the hernia should be
  repaired at the time.

14
Management

• NO therapy(to control pulmonary hypertension) and
  partial liquid (perfluorocarbon) ventilation may
  be used.
• Hyperventilation and alkalosis were ever used to
  control pulmonary hypertension and persistent
  fetal circulation, but they are avoided to
  decrease ventilator-induced lung injury now.

15
Management

• If these are not useful, the ECMO is indicated.

16

• Table 50-2

17
Operative Correction

• A newborn must be stabilized at least 72 hours
  before operation.
• If vital signs deteriorate, a contralateral
  pneumothorax is suspected and a 10F chest tube
  must be placed.
• The correction is done through a paramedian
  incision.

18
Operative Correction

• Fig 50-5

19
Operative Correction

• Extralobar pulmonary sequestration is removed.
• A small defect is repaired with permanent suture
  and Teflon pladgets.
• A large defect is repaired with
  polytetrafluoroethylene membrane.

20
Operative Correction

• If a hemidiaphragm is absent completely, then a
  polytetrafluoroethylene membrane must be sutured
  on the ribs anteriorly and posterolaterally. The
  median portion of the membrane is sutured to the
  contralateral diaphragmatic leaf and the adventia
  overlying the esophagus and the aorta.

21
Operative Correction

• The use of chest tube is controversial.
  Suggestions include one chest tube, no chest
  tube, bilateral chest tubes, under water seal,
  and tube exposure to the atmosphere.
• Associated abdominal anomalies must be corrected
  during the operation.

22
Extracorporeal Menbrane Oxygenation(ECMO)

• Standard criteria have not established, but in
  generally include AaDO2lt 600 for 12 hours oxygen
  indexgt40 acute deterioration(pH lt7.15 or
  PaO2lt55mmHg) for 2 hours failure of conventional
  management and progressive barotrauma.

23
Extracorporeal Menbrane Oxygenation(ECMO)

• Contraindications include preexisting
  intraventricular hemorrhage weightlt2000 g and
  congenital or neurologic abnormalities
  incompatible with a normal life.

24
Factors in Survival

• ABG pHlt 7.0 with a PCO2gt100 is a poor predictor.
• Toulokian et al reported a scoring system based
  on radiographic findings to predict the outcome.
  The findings include side of the hernia, location
  of the stomach, relative volume aerated lungs,
  and presence of a pneumothorax.

25
Factors in Survival

• Preoperative and postoperative AaDO2 gt500 mmHg
  correlated little chance of survival.
• Wilson et al reported that best postductal PCO2
  and the oxygenation-ventilation index (PCO2/mean
  airway pressure X respiratory rate X 100) were
  predictors of mortality. Infants who did not
  respond to conventional mechanical ventilation
  (best postductal PO2lt100 best postductal
  PCO2gt40, with ventilation indexgt1000) did not
  benefit from ECMO.

26
Future Prospects

• In utero correction of defect can allow
  sufficient growth of lung for survival in fetal
  lambs, but the technique did not improve outcome
  in humans.
• Antenatal tracheal occlusion (with balloon or
  clip) can reverse the lung hypoplasia in lambs,
  but the outcome of the technique remains to be
  seen in humans.

27
Long-Term Follow-Up

• Jeandot et al reported survivors after 1-2 years
  have a persistent reduced perfusion but improved
  ventilation.
• Vanomo et al reported no clinical impairment but
  some reduced exercise tolerance and minor
  ventilatory impairment.
• Anterior chest wall deformity, scoliosis, and G-E
  reflux were also noted by others.

28
Long-Term Follow-Up

• Recurrence may develop, especially in those
  babies who require patch repair.

29
B. MISCELLANOUS CONGENITAL DIAPHRAGMATIC HERNIAS
30
B-1. Hernias through the Central Tendon of the
Diaphragm

• If it occurs on the right side, a mushroom-like
  projection of liver can be seen. This projection
  will be misinterpreted a diaphragmatic tumor.
  Distinguishing can be done by creating a
  pneumoperitoneum. Air appears around the liver
  protrusion can be seen. Operation is not
  necessary.

31
B-1. Hernias through the Central Tendon of the
Diaphragm

• If the hernia occurs in the left side, the
  stomach may herniate, a air-containing cyst can
  be seen on the top of the diaphragm. The hernia
  may associated with partial absence of
  pericardium, so cardiac symptoms may occur. The
  operation is done through an abdominal approach
  in infants and children but through thoracic
  approach in older children and adults.

32
B-2. Paraesophageal Hernia in Infants and Older
Children

• It is congenital or acquired in infants and
  children.
• The acquired variety is usually a complication of
  operation of G-E reflux.
• UGI series reveals herniated stomach and other
  abdominal organs.
• In some children, anemia may occur from gastritis
  or esophagitis vomiting from obstruction or
  incarceration.

33
B-2. Paraesophageal Hernia in Infants and Older
Children

• Operation should be done in all cases and should
  include reduction of herniated organs
  approximation of the crura. Anti-reflux procedure
  or gastropexy is also suggested to carry out.

34
B-3. Morgagni Hernia

• It is an anterior retrosternal diaphragmatic
  hernia.

35
B-3. Morgagni Hernia

• Anatomy
• 1. Lack of fusion or muscularization of
• pleuroperitoneal membrane anteriorly can
• lead a defect as known as Morgagni
• foramen or space of Larrey .
• 2. The internal mammary artery may pass
• through the space to rectus sheath and
  become
• the superior epigastric artery.

36
B-3. Morgagni Hernia

• Anatomy
• 3. The peritoneum is intact so there
• is usually a true hernia sac.
• 4. Left side is less involved because of
• protection of the pericardium.
• 5. The ligamentum teres is the medial
• border of the hernia.

37

• Fig51-1

38
B-3. Morgagni Hernia

• INCIDENCE
• 1. It is uncommon.
• 2. Although it is congenital, it is found less
  
• in a child than in a adult.
• 3. It is more in women and obese people.
• 4. Right side (90), left side (2), bilateral
  (8).

39
Morgagni Hernia

• SYMPTOMS
• 1. 1/3 patients have no symptom.
• 2. Most frequent symptoms are cramping
• pain, constipation from partial colonic
• obstruction.
• 3. Gastric volvulus or small intestine
• obstruction are less frequent.

40
Morgagni Hernia

• SYMPTOMS
• 4. Complete obstruction, incarceration, or
• strangulation is rare.
• 5. Cardiorespiratory symptoms are less
• common than GI symptoms.
• 6. Trauma, exercise and pregnancy can cause
• the occurrence.

41
Morgagni Hernia

• Diagnosis
• 1. Chest radiograph reveals
• pericardiophrenic density that is solid or
  
• contain air.
• 2. The lateral view reveals retrosternal
• opacity.
• 3. Pericardial cyst, lobulated pneumothorax,
  fat
• pad, mediastinal tumor or bronchogenic
• carcinoma may mimic the hernia.

42

• Fig51-2

43
Morgagni Hernia

• Diagnosis
• 4. Contrast study of colon and UGI can
• confirm the diagnosis.
• 5. CT scan can reveal fat or a hollow organ.
• 6. MRI is not necessary.

44
Morgagni Hernia

• Surgical repair
• 1. All symptomatic adults should undergo
• repair.
• 2. Some reports showed asymptomatic adults
• should undergo repair to avoid
• incarceration and stranulation.
• 3. It is reasonable that patients with only
  omentum
• herniation or high operative risk do not
  need
• operation.

45

• Fig.51-7

46
Morgagni Hernia

• Surgical repair
• 4. Repair can be done through paramedian,
• subcostal or upper median incision
• transabdominally.
• 5. A small defect can be closed primarily with
  
• interrupted suture.
• 6. Prosthetic patches are necessary to close
• larger defects.

47
Morgagni Hernia

• Surgical repair
• 7. It is not necessary to enter or to drain
  the
• the pleural space.
• 8. Lapatroscopy and thoracoscopy are ever
• used.
• 9. The mortality and morbidity are low.
• 10. Recurrence is rare.

48
Thank You!