Congenital diaphragmatic hernia

2.  Defect in the developing diaphragm of the
fetus leading to herniation of abdominal
viscera into the thorax
 Incidence – 1 in 2000 to 5000 live births
 90% of the defects are posterolateral and 85%
of these are on left sided.
 Left = 80%
 „
Right = 20%
 Bilateral is very rare
 „
2% risk to first-degree relatives

3.  Unknown
 ?Genetic
 „
Drug/insecticides
„„
Nitrofen
„„
Phenmetrazine
„„
Thalidomide
„„
Quinine
 „ Vitamin A deficiency

4.  28% incidence
 Very common in stillborn infants
 Most common
 Neural tube defects
 Cardiac Defects
 Esophageal atresia
 Omphalocele
 Trisomy 13, 18, 21

5.  4th week
 4 components
1. Septum transversum–
Central Tendon
2. Pleuroperitoneal
membranes–
dorsolateral
3. Esophageal mesentery
–dorsal crura
4. Intercostals –muscular
portion
Complete closure by 8th
Week

6.  Results from failure of closure of the pleuro
peritoneal canals, which occurs normally in
the human fetus at 8–10 weeks’ gestation.
 The abdominal viscera herniate in to the
affected thorax, creating a shift of the
mediastinum to the contralateral side and
hypoplasia of both the ipsilateral and
contralateral lung.

7.  Adequate intrathoracic space is
required for normal pulmonary growth
othewise
◦ Structurally immature lungs
◦ Abnormal pulmonary vasculature

8.  „
Left CDH
◦ Left lobe of liver
◦ Spleen
◦ Stomach
 Right
◦ Liver
◦ Abnormal hepatic veins to right atrium
 Both
◦ Large and Small bowel

9.  The most common types
1) Bochdalek Hernia
2) Morgagni Hernia
3) Eventration of diaphragm/ Central tendon
defects of diaphragm

10.  It is life threatening pathology in infants, and
a major cause of death due to
1) Pulmonary hypoplasia
2) Pulmonary hypertension.
leads to severe respiratory distress>>>
death..

11.  Also known as a posterolateral diaphragmatic
hernia.
 >95% cases
 Majority occur on left side of diaphragm(85
%)
 Characterized by Hole in the postero-lateral
corner of the diaphragm which allows
passage of abdominal viscera into chest
cavity.

12.  Rare anterior defect of diaphragm
 Referred to as Morgagni , retrosternal , or
parasternal hernia.
 2% of all CDH cases
 Majority on right side.
 Characterized by herniation through the
foramen of morgagni which is located
immediately adjaceent to the xiphoid process
of sternum.

13.  Central tenden defect
 Central portion of diaphragm is absent
 So abdominal viscera can herniate through
this defect in thoracic cavity.

14.  Pulmonary Hypoplasia
 Small lungs
 Caused by compression
 Prevented growth and maturation
 Pulmonary Hypertension
 Abnormal Lung Vasculature
 The high pressure in the lungs limits blood flow to
the lungs

15.  Hernia is on one side
 Both sides are underdeveloped
 Fewer than normal alveoli (sacs for oxygen transfer)
 Abnormal pulmonary blood vessels on both
sides
 Pulmonary hypertension

16.  Prenatal ultrasound
 Polyhydramios(80%)
 High-Resolution ultrasound in the first
trimester can visualize the diaphragm
 Important to evaluate for presence of liver
or bowel in the thorax
 Stomach at level of heart
 Absence of stomach in abdomen
 Observe for other anomalies
 If ultrasound is positive, consider MRI

17.  Clinically
Narrow abdomen
Large chest
Respiratory distress

18.  CXR
 Ultrasound only if diagnosis in
doubt
 GI Contrast studies in difficult
cases

19.  Eventration of Diaphragm
 Elevated hemidiaphragm
 Congenital Pulmonary Adenomatous
Malformations (CPAMs)
 Lung agenesis

20.  Associated anomalies
 Cardiac (excl. single ventricle)
47% survival
 „„
Single ventricle
5% survival
 Ultrasound Studies
 Lung to Head ratio (LHR)*
<0.8 fatal
<0.8 but >1.0 poor prognosis
>1.0 good prognosis

21.  It is sonographic measure to identify fetuses
with CDH that have poor prognosis
 The lung area contra lateral to the CDH
obtain by taking the product of the longest
two perpendicular linear measurements of
the lung. The product is divided by head
circumference to obtain the LHR
 Lung area= length1x length2
 LHR =lung area/Head circumference

22.  Respiratory distress at birth
◦ Cyanosis, tachypnea
 Scaphoid abdomen
 Mediastinal shift
 Breath sounds absent on affected side
 Bowel sounds auscultated in the chest

23.  If neonate is distressed:
◦ Immediate intubation
 Avoidance of bag-mask ventilation
 Nasogastric tube placement with gastric
decompression

24.  Surfactant
 Inhalation of Nitric Oxide

25.  High mortality for emergent surgery
 Best survival is delayed repair when patient
become stable.
 Serial Echocardiography
 Evidence of resolution of pulmonary hypertension

26.  Introduced in 1977
 Formerly called “ECMO”
 Criteria for use
◦ Inadequate delivery of Oxygen
◦ „
„
Predicted 80% mortality without ECLS

27.  Thoracosopic Repair
◦ Minimally invasive repair
◦ Best suited for stable, near-term patients
◦ Surgery is done through small incisions in the chest
 Open repair through abdomen
◦ Can be done on less stable patients
◦ Well-suited for complete absence of the diaphragm

28.  Subcostal Incision
 pull abdominal organs back into the
abdomen
 Close the defect
 Patch Repair of defect
◦ Gortex
◦ Mess repair

29.  Developmental delay
 GERD
 Chronic lung disease
 Hernia recurrence

30.  Fetoscopic tracheal occlusion (FETO)
 Developed at UC San Francisco
 Criteria
◦ LHR <0.8 or <1.0 with liver up
◦ 28 weeks EGA
◦ PROM, prematurity common
◦ 50% survival
 In Utero repair has been abandoned