Congenital Diaphragmatic Hernia Flashcards
What is congenital diaphragmatic hernia ?
An abnormal opening in the diaphragm that allows part of the abdominal organs to migrate into the chest cavity, occurring before birth.
vast majority of Congenital diaphragmatic hernia occurs on which side ?
Left side
Associated chromosomal abnormalities include
trisomy 18, trisomy 13, 12p), Trisomy 21, and Turner syndrome
Congenital diaphragmatic hernia may occur as:
Nonsyndromic: as an isolated defect Syndromic:
Syndromic congenital diaphragmatic hernia examples are
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.
What is a diaphragm?
It is a musculotendinous structure that separates the organs in the abdominal cavity from those in the chest.
Different types of congenital diaphragmatic hernia
Bochdalek hernia − Morgagni’s hernia − Hiatal hernia Central tendon hernia − Eventration of diaphragm
Morgagni’s hernia is
The Morgagni defect occurs posterior to the sternum and results from failure of sternal and costal fibers to fuse
The hiatus hernia …
occurs through the esophageal hiatus
Central tendon hernia is
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
Types of CDH
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
The hiatus hernia is divided into three types , Namely …
◦ Sliding
◦ Rolling
◦ Mixed sliding and rolling
• A hernia sac is present in 10–20 % of congenital diaphragmatic hernias True or false
True
four embryonic structures the diaphragm is derived from
− The pleuroperitoneal membranes
− The mesoderm of the body wall
− The esophageal mesenchyme
− The septum transversum
pathophysiology of congenital diaphragmatic hernia involves:
− Pulmonary hypoplasia − Pulmonary hypertension − Pulmonary immaturity − Pulmonary surfactant deficiencies
Clinical features prenatally , Antenatal ultrasound will reveal
− Polyhydramnios
− An absent intra-abdominal gastric air bubble
− Mediastinal shift
− Hydrops fetalis
Clinical features after birth
− 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
Patients may present outside of the neonatal period with:
− Respiratory symptoms
− Intestinal obstruction
− Bowel ischemia and necrosis following volvulus
Diagnosis
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.
Management
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
The main problem in congenital diaphragmatic hernia is
Persistent pulmonary hypertension and pulmonary hypoplasia
Types of diaphragmatic hernia
Bochdalek hernia − Morgagni’s hernia − Hiatal hernia Central tendon hernia − Anterolateral hernia − Eventration of diaphragm − Latrogenic diaphragmatic hernia − Traumatic diaphragmatic hernia
Eventration of the diaphragm
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
Eventration of the diaphragm leads to an abnormal elevation of one leaf of an intact diaphragm as a result of:
− Congenital aplasia or maldevelopment of the diaphragm
− Paralysis of the phrenic nerve
Associated anomalies of Eventration of the diaphragm?
− Cleft palate − Congenital heart disease − Situs inversus − Undescended testicle
Eventration of the diaphragm is divided into two types:
Congenital − Acquired
The defect in congenital eventration can be:
− Partial − Diffuse
Clinical features of eventration of diaphragm
asymptomatic
• Recurrent chest infections
Diagnosis is made through ?
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.
Eventration of diaphragm treatment
diaphragmatic plication. This makes the lax diaphragm taut and moves it to a lower position
