Congenital Diaphragmatic Hernia

Question 1

What is congenital diaphragmatic hernia ?

Answer 1

An abnormal opening in the diaphragm that allows part of the abdominal organs to migrate into the chest cavity, occurring before birth.

Question 2

vast majority of Congenital diaphragmatic hernia occurs on which side ?

Answer 2

Left side

Question 3

Associated chromosomal abnormalities include

Answer 3

trisomy 18, trisomy 13, 12p), Trisomy 21, and Turner syndrome

Question 4

Congenital diaphragmatic hernia may occur as:

Answer 4

Nonsyndromic: as an isolated defect Syndromic:

Question 5

Syndromic congenital diaphragmatic hernia examples are

Answer 5

Cornelia de Lange syndrome is an autosomal dominant syndrome with characteristic facial features, hirsutism, developmental delay, and congenital diaphragmatic hernia.

• Fryns syndrome is an autosomal recessive condition that includes congenital diaphragmatic hernia, along with hypoplasia of the distal digits and other variable abnormalities of the brain, heart, and genitourinary development.
• Pallister–Killian syndrome (tetrasomy 12p mosaicism) includes coarse facial features, aortic stenosis, cardiac septal defects, abnormal genitalia, and congenital diaphragmatic hernia.

Question 6

What is a diaphragm?

Answer 6

It is a musculotendinous structure that separates the organs in the abdominal cavity from those in the chest.

Question 7

Different types of congenital diaphragmatic hernia

Answer 7

Bochdalek hernia
− Morgagni’s hernia
− Hiatal hernia
Central tendon hernia
− Eventration of diaphragm

Question 8

Morgagni’s hernia is

Answer 8

The Morgagni defect occurs posterior to the sternum and results from failure of sternal and costal fibers to fuse

Question 9

The hiatus hernia …

Answer 9

occurs through the esophageal hiatus

Question 10

Central tendon hernia is

Answer 10

This hernia is a rare diaphragm defect involving the central tendinous portion of the diaphragm.

Question 11

Anterolateral hernia:

Answer 11

Occurs anterolaterally through a defect in the muscular part of the diaphragm.

Question 12

Eventration of diaphragm:

Answer 12

There is incomplete mascularization of the diaphragm. This leads to elevation of a portion or all of the intact diaphragm that is thinned as a result of incomplete muscularization

Question 13

Latrogenic diaphragmatic hernia:

Answer 13

Occurs as a result of diaphragmatic injury during surgery.

Question 14

Traumatic diaphragmatic hernia:

Answer 14

Occurs following penetrating and blunt abdominal and thoracic trauma

Question 15

Types of CDH

Answer 15

Bochdalek hernia: The most common type of congenital diaphragmatic hernia is a posterolateral hernia

− Morgagni’s hernia: The Morgagni defect occurs posterior to the sternum and results from failure of sternal and costal fibers to fuse

− Hiatal hernia: The hiatus hernia occurs through the esophageal hiatus

Central tendon hernia: This hernia is a rare diaphragm defect involving the central tendinous portion of the diaphragm.

− Anterolateral hernia: Occurs anterolaterally through a defect in the muscular part of the diaphragm.

− Eventration of diaphragm: There is incomplete mascularization of the diaphragm. This leads to elevation of a portion or all of the intact diaphragm that is thinned as a result of incomplete muscularization

− Latrogenic diaphragmatic hernia: Occurs as a result of diaphragmatic injury during surgery.

− Traumatic diaphragmatic hernia: Occurs following penetrating and blunt abdominal and thoracic trauma

Question 16

The hiatus hernia is divided into three types , Namely …

Answer 16

◦ Sliding

◦ Rolling

◦ Mixed sliding and rolling

Question 17

• A hernia sac is present in 10–20 % of congenital diaphragmatic hernias True or false

Answer 17

True

Question 18

four embryonic structures the diaphragm is derived from

Answer 18

− The pleuroperitoneal membranes
− The mesoderm of the body wall
− The esophageal mesenchyme
− The septum transversum

Question 19

pathophysiology of congenital diaphragmatic hernia involves:

Answer 19

− Pulmonary hypoplasia − Pulmonary hypertension − Pulmonary immaturity − Pulmonary surfactant deficiencies

Question 20

Clinical features prenatally , Antenatal ultrasound will reveal

Answer 20

− Polyhydramnios
− An absent intra-abdominal gastric air bubble
− Mediastinal shift
− Hydrops fetalis

Question 21

Clinical features after birth

Answer 21

− A scaphoid abdomen
− A barrel-shaped chest
− Signs of respiratory distress (retractions, cyanosis, grunting respirations) in the first minutes or hours of life
− Poor air entry on the affected side, with a shift of cardiac sounds

Question 22

Patients may present outside of the neonatal period with:

Answer 22

− Respiratory symptoms
− Intestinal obstruction
− Bowel ischemia and necrosis following volvulus

Question 23

Diagnosis

Answer 23

arterial line in the umbilical artery or in a peripheral artery (radial, posterior tibial) for continuous blood pressure measurement and to frequently assess pH, PaCO2, and PaO2

Chromosomal studies.

• A chest radiograph
• An echocardiography

Urinary catheterization to monitor urine output and fluid resuscitation.

Question 24

Management

Answer 24

Infants should be intubated and ventilated
nasogastric or an orogastric tube should be placed as soon as possible to help decompress the stomach

• Place an umbilical vein catheter to allow for administration of inotropic agents and hypertonic solutions such as calcium gluconate.

Exogenous surfactant

Inhaled nitric oxide

Surgical repair

Question 25

The main problem in congenital diaphragmatic hernia is

Answer 25

Persistent pulmonary hypertension and pulmonary hypoplasia

Question 26

Types of diaphragmatic hernia

Answer 26

Bochdalek hernia
− Morgagni’s hernia
− Hiatal hernia
Central tendon hernia
− Anterolateral hernia
− Eventration of diaphragm
− Latrogenic diaphragmatic hernia
− Traumatic diaphragmatic hernia

Question 27

Eventration of the diaphragm

Answer 27

is a disorder in which all or part of the diaphragmatic muscle is replaced by fibroelastic tissue.

• Eventration of the diaphragm leads to an abnormal elevation of one leaf of an intact diaphragm

Question 28

Eventration of the diaphragm leads to an abnormal elevation of one leaf of an intact diaphragm as a result of:

Answer 28

− Congenital aplasia or maldevelopment of the diaphragm

− Paralysis of the phrenic nerve

Question 29

Associated anomalies of Eventration of the diaphragm?

Answer 29

− Cleft palate − Congenital heart disease − Situs inversus − Undescended testicle

Question 30

Eventration of the diaphragm is divided into two types:

Answer 30

Congenital − Acquired

The defect in congenital eventration can be:

− Partial − Diffuse

Question 31

Clinical features of eventration of diaphragm

Answer 31

asymptomatic

• Recurrent chest infections

Question 32

Diagnosis is made through ?

Answer 32

Ultrasound
Fluoroscopy
Chest X-ray

The affected hemidiaphragm should be at least two intercostal spaces higher than the other side.

• Fluoroscopy is considered the most reliable way to document diaphragmatic paralysis. Paradoxical movement of the affected diaphragm is seen during screening.

Question 33

Eventration of diaphragm treatment

Answer 33

diaphragmatic plication. This makes the lax diaphragm taut and moves it to a lower position