Congenital diaphragmatic hernia : 1. Introduction 2. Types 3. Sign and symptoms 4. Diagnosis 5. Treatment 6. Anaesthesia considerations

1. CONGENITAL DIAPHRAGMATIC
HERNIA
MODERATOR : Dr. Mani Kiran
PRESENTER : Anubhav Rana
B.Sc Final year
Dept. of Anaesthesia
PGIMER , Chandigarh

2. INTRODUCTION
• Congenital diaphragmatic hernia (CDH) is a birth defect of
the diaphragm.
• The most common type of CDH is a Bochdalek hernia
• other types include
A. Morgagni hernia,
B. diaphragm eventration and central tendon defects of
the diaphragm.
• Malformation of the diaphragm allows the abdominal organs
to push into the chest cavity, hindering proper lung formation.

3. • CDH is a life-threatening pathology in infants and a major cause
of death due to two complications:
1. pulmonary hypoplasia
2. pulmonary hypertension
• Newborns with CDH often have severe respiratory distress which
can be life-threatening unless treated appropriate
Pulmonary hypoplasia is incomplete development of the lungs, resulting in an abnormally low
number or size of bronchopulmonary segments or alveoli. A congenital malformation, it most
often occurs secondary to other fetal abnormalities that interfere with normal development of the
lungs.
Pulmonary hypertension is when the blood pressure in the arteries of the lungs becomes
abnormally high due to narrowing of the vessels. This strains the right ventricle of the heart and
could lead to heart failure.

5. CLASSIFICATION
1. Bochdalek hernia
2. Morgagni hernia
3. diaphragm eventration and
4. central tendon defects

6. BOCHDALEK HERNIA
• also known as a postero-lateral diaphragmatic hernia
• more than 95% of cases
• characterized by a hole in the postero - lateral corner of the
diaphragm which allows passage of the abdominal viscera into
the chest cavity.
• 80–85% occur on the left side of the diaphragm remaining cases
occur on the right side.

7. • referred to as a Morgagni, retrosternal, or parasternal
hernia
• anterior defect of the diaphragm
• Approx. 2 - 4% of all CDH
• characterized by herniation through the foramina of
Morgagni
• located immediately adjacent and posterior to
the xiphoid process of the sternum
MORGAGNI HERNIA

8. • The xiphoid process or
• xiphisternum or
• metasternum
• A small cartilaginous process (extension) of the lower
part of the sternum
• ossified in the adult human.
• Also referred to as the ensiform process

11. DIAPHRAGM EVENTRATION
• The diagnosis of congenital diaphragmatic eventration is used when
there is abnormal displacement (i.e. elevation) of part or all of an
otherwise intact diaphragm into the chest cavity.
• This rare type of CDH
• occurs because in the region of eventration the diaphragm is thinner,
allowing the abdominal viscera to protrude upwards

12. SYMPTOMS
• difficulty breathing
• fast breathing
• fast heart rate
• cyanosis (blue color of the skin)
• abnormal chest development, with one side being larger
than the other
• abdomen that appears caved in

14. CLINICAL MANIFESTATIONS
• Chronic lung disease
• Pulmonary hypertension
• Gastroesophageal reflux: This is a condition in which acids and fluids from the
stomach move up to the esophagus and can cause heartburn, vomiting, feeding, or
lung problems.
• Failure to thrive: Children with the most serious lung problems are most likely to
have growth problems. Some require special feeding tubes to give enough calories
to grow adequately.
• Developmental delays: These include delays in the ability to roll over, sit, crawl,
stand, or walk.
• Sensorineural hearing loss (SNHL) has been found in 25% of individuals with CDH

15. PATHOPHYSIOLOGY
• It involves three major defects
1. A failure of the diaphragm to completely close
during development
2. Herniation of the abdominal contents into the chest
3. Pulmonary hypoplasia
Hypoplasia hypo- 'under' + plasis ’ formation
• is underdevelopment or incomplete development of a tissue or organ
• refers to an inadequate or below-normal number of cells.

16. DIAGNOSIS
• Can be diagnosed before birth and fetal intervention
• Infants born with diaphragmatic hernia experience respiratory failure due to
both pulmonary hypertension and pulmonary hypoplasia.
• Pulmonary hypoplasia or decreased lung volume is directly related to the abdominal
organs presence in the chest cavity which causes the lungs will be severely undersized,
especially on the side of the hernia.
• Diagnosis can be
1. Prenatal Diagnosis
2. Postnatal Diagnosis

17. PRENATAL DIAGNOSIS
• Color flow Doppler
1. Demonstrate abnormal positioning of the umbilical and portal
veins, which are indicative of liver herniation;
2. Identify right-sided hernias, which can be difficult to detect on
ultrasound examination because of the similar echogenicity of
lung and liver
• Fetal MRI

18. POSTNATAL DIAGNOSIS
• CDH can be detected in a neonate by:
1. Presence of a scaphoid abdomen, diminished breath sounds ipsilateral
to the side of the hernia, and displacement of the heart sounds
contralateral to the hernia.
2. Chest x-ray shows visible bowel gas above the diaphragm
accompanied by a mediastinal shift.

21. TREATMENT
• The first step in management is orogastric tube placement and
securing the airway (intubation).
• Extracorporeal membrane oxygenation (ECMO)
• Diaphragm eventration is typically repaired thoracoscopically, by a
technique called plication of the diaphragm.
• Plication basically involves a folding of the eventrated diaphragm
which is then sutured in order to “take up the slack” of the excess
diaphragm tissue.

23. GENERAL ANAESTHETIC APPROACH
• Patients are often intubated while in the NICU prior to transfer to
the OT
• In OT intubation is done as rapid-sequence intubation with propofol.
• Mask ventilation avoided to avoid the risk of gastric insufflation.
• Neuromuscular blocking agents (NMBAs) to be avoided for
intubation as there is evidence of lung function deterioration with
administration without any apparent added benefit.
• As with intubation, NMBAs are not recommended given decreased
lung compliance from the loss of spontaneous ventilation
• .

24. • An arterial line for intraoperative monitoring and is preferentially placed in
the right radial artery to provide preductal measurements
• Venous access obtained in upper extremities as the reduction of
herniated viscera will increase abdominal compartment pressures,
potentially decreasing the flow of the inferior vena cava.
• Central lines may be helpful as they can be used postoperatively to
administer vasoactive medications.
• A naso or orogastric tube should be in place and set to low continuous
suction for gastric decompression
• Anesthesia maintained through high-dose intravenous (IV) opioids.

25. • Volatile anesthetics used as a supplement.
• Agents should be given cautiously to avoid compromising cardiac output.
• Nitrous oxide is avoided, to avoid the risk of expansion within the thoracic
cavity or herniated viscera.

26. INTRAOPERATIVE MONITORING
• Monitoring include
1. pre and postductal pulse oximetry,
2. Arterial line - The arterial line is needed for invasive BP monitoring
and arterial blood gas analysis
3. the standard monitors for heart rate,
4. noninvasive blood pressure (BP) cuff,
5. temperature,
6. end-tidal CO2
7. five-lead EKG.

27. • Labs to monitor include hemoglobin/hematocrit, glucose, and ABGs.
• It is also important to regularly monitor the nonoperative lung field for
evidence of pneumothorax as this may occur secondary to
excessive ventilatory pressures

28. VENTILATION
• General goals for ventilation include the following:
• Preductal SpO2 between 85% to 95%
• Postductal SpO2 > 70%
• PIP < 25 cmH2O with a PEEP set between 3 to 5 cmH2O
• FiO2 < 50%, titrated to preductal SpO2 goals
• Respiratory rate between 40 to 60 breaths per minute
• PaCO2 between 50 to 70 mm Hg
• the pH of 7.25 and above

29. CARDIOVASCULAR
• Maintain adequate cardiac output and blood pressure through the use of inotropes,
vasopressors, and fluids.
• Target blood pressure ranges should be based on the neonate’s gestational age.
• Most patients with CDH have concomitant adrenal insufficiency based on low random
cortisol measurements; hypotension in this population generally responds well to stress-
dose hydrocortisone.
• Long-term administration of steroids increase the risk of mortality and sepsis.
• Fluid boluses of 10 to 20 cc/kg if the patient is hypovolemic.
• Dextrose-rich maintenance fluids for caloric and hemodynamic requirements.

30. PULMONARY HYPERTENSION
• Commonly used medications include
1. inhaled nitric oxide (NO) and
2. Milrinone.
• If requiring PH treatment, NO can be initiated for those patients with a normal functioning
LV, whereas milrinone may be more appropriate for those neonates with comorbid LV
diastolic dysfunction.
• Milrinone in this setting can additionally act as a lusitrope to increase LV filling and reduce
the left-to-right shunt via the PFO.
• If no response is seen with these agents, then a second-line agent may be
administered instead, such as a prostacyclin or a phosphodiesterase inhibitor.

31. POSTOPERATIVE MANAGEMENT
• In the post-anesthesia care unit, the patient will need continued hemodynamic monitoring
and supportive care.
• Lung fields to be regularly assessed for any evidence of pneumothorax, hemorrhage, or
atelectasis.
• patient will be intubated and mechanically ventilated.
• Ventilatory and hemodynamic support is weaned as tolerated.
• Main management - opioids, an epidural, and acetaminophen if liver function tests are
normal.
• Monitoring for return of bowel function and initiating enteral feeding as tolerated is
encouraged for optimal postoperative recovery.

32. THANK YOU