Congenital Diaphragmatic Hernia

Question 1

What is a Congenital Diaphragmatic Hernia?

Answer 1

A developmental discontinuity of the diaphragm that allows abdominal viscera to herniate into the chest

Question 2

What are the 2 factors that can cause a CDH?

Answer 2

1) Genetic
2) Environmental

Question 3

What are the environmental factors that can cause a CDH?

Answer 3

1) Vitamin A deficiency
2) Thalidomide
3) Anticonvulsants
4) Quinines

Question 4

What are the types of CDH?

Answer 4

1) Postero-lateral Hernia (Bochdalek)
2) Anterior Hernia (Morgagni-Larrey)
3) Central Hernia
4) Diaphragm Agenesis
5) Diaphragmatic Eventration

Question 5

What is a Postero-lateral CDH also called?

Answer 5

Bochdalek

Question 6

What is an Anterior CDH also called?

Answer 6

Morgagni-Larrey

Question 7

Which type of CDH is the most common?

Answer 7

Postero-lateral Hernia (Bochdalek)

Question 8

Which CDH type involves the central tendon?

Answer 8

Central Hernia

Question 9

What is Diaphragmatic Eventration?

Answer 9

Abnormal elevation of congenitally thin, hypoplastic but intact diaphragm

Question 10

What is an isolated CDH?

Answer 10

Diaphragmatic hernia that occurs without any other major congenital anomalies or genetic syndromes.

Question 11

What is a complex CDH?

Answer 11

CDH that occurs with other congenital anomalies

Question 12

Which has a better survival rate: Isolated or Complex CDH?

Answer 12

Isolated

Question 13

Which is more common: Isolated or Complex CDH?

Answer 13

Isolated

Question 14

How can you diagnose a CDH prenatally?

Answer 14

1) Fetal ultrasound screening
2) Fetal MRI

Question 15

How can you diagnose a CDH Postnatally?

Answer 15

1) Chest x-ray
2) CT or MRI
3) GI contrast study
+ May present with respiratory distress

Question 16

How can you diagnose a CDH in Infants and children?

Answer 16

1) Incidental finding on chest x-ray
2) CT or MRI
3) GI contrast study

Question 17

What is the prognostic factor if the CDH is associated with other congenital anomalies?

Answer 17

Poor

Question 18

What are the prognostic factors if the CDH is associated with right sided defects?

Answer 18

1) Poor survival rate SR
2) Higher ECMO (Extracorporeal Membrane Oxygenation) rate = Poor prognosis
3) Higher needed patch repair rate

Question 19

What is the prognostic factor if the CDH is associated with Liver herniation?

Answer 19

Poor survival rate

Question 20

What are the prognostic factors if the CDH is associated with low Fetal lung volume?

Answer 20

Poor survival rate

Question 21

What are the prognostic factors if the CDH is associated with high Fetal lung volume?

Answer 21

Good survival rate

Question 22

Low Lung area to head circumference ratio (LHR) with a CDH is more indicative of:

Answer 22

Morbidity NOT mortality

Question 23

CDH Prenatal Management?

Answer 23

1) Screening for associated abnormalities
2) Fetal echocardiography
3) Genetic studies
4) Family counselling
5) In utero fetal therapy
6) Delivery planning

Question 24

What type of In utero fetal therapies can we do for prenatal CDH?

Answer 24

1) Investigational procedures
2) Fetoscopic Endoluminal Tracheal Occlusion (FETO)

Question 25

CDH Postnatal Management?

Answer 25

1) Optimizing cardiorespiratory status 2) Achieving hemodynamic stability 3) Screening for associated malformations

Question 26

What parameters should be optimized before we can do elective surgery?

Answer 26

1) Reduce lung compression 2) Ventilatory support 3) Cardiovascular support 4) Correction of acid-base status 5) Correction of pulmonary hypertension 6) Surfactant 7) Hydrocortisone

Question 27

How can we reduce lung compression in postnatal CDH?

Answer 27

1) Immediate intubation post-delivery 2) NG Tube

Question 28

How can we offer ventilatory support in postnatal CDH?

Answer 28

1) High-Frequency Oscillatory Ventilation (HFOV) in most cases 2) ECMO in severe cases 3) Liquid ventilation

Question 29

How can we offer cardiovascular support in postnatal CDH?

Answer 29

1) Proper venous access 2) IV fluids 3) Inotropic agents

Question 30

How can we correct pulmonary hypertension in postnatal CDH?

Answer 30

1) Maintain MAP ≥50 mmHg 2) Reverse right-to-left shunting 3) Inhaled Nitric Oxide (iNO) 4) Sildenafil

Question 31

What are the types of operative management for CDH?

Answer 31

1) Open 2) MIS (Minimally Invasive Surgery) a) Thoracoscopy (through the chest) b) Laparoscopy (through the abdomen)

Question 32

What are the pros of MIS (Minimally Invasive Surgery) for CDH?

Answer 32

1) Lower overall surgical stress 2) Lower Postoperative pain 3) Better respiratory compliance 4) Higher survival rate 5) Decreased length of hospital stay 6) Avoidance of thoracotomy associated complications

Question 33

What are the cons of MIS (Minimally Invasive Surgery) for CDH?

Answer 33

1) CO2 insufflation leading to: a) Hypercapnia and acidosis b) Pulmonary hypertension c) Right-to-left shunting 2) High Recurrence rate

Question 34

Mortality and morbidity of CDH are mainly related to:

Answer 34

1) Severity of lung hypoplasia 2) Pulmonary hypertension 3) Associated anomalies 4) Prematurity