Diaphragmatic Hernia

Definition
A condition characterized by a defect in the diaphragm, through which the abdominal viscera migrate into the chest during fetal life

Incidence
1 in 2,000 5,000 births 80% left side 20% right side Bilaterality is rare Associated anomalies in 40% of cases

Types
Bochdalek hernia (most common)

Hernia of foramen of Morgagni

Eventration of the diaphragm

Diaphragm seen from below

(B) Bochdalek left posterolateral defect (M) Anterior or Morgagni type (H) Hiatus for oesophagus and hiatal hernia (E) Large eventration in the tendinous portion of the right copula (T) A tear causing post-traumatic hernia (A) Abdominal aorta

Pathophysiology

Embryology
Failure of closure of the pleuroperitoneal canal, which occurs during 8th gestational week.

Consequently, the abdominal viscera herniates into the thorax, which causes pulmonary hypoplasia by compressing the growing lung.

Congenital diaphragmatic hernia

Pulmonary hypoplasia Left ventricular hypoplasia Surfactant deficiency

Hypoxemia Hypercapnea Acidosis Persitent fetal circulation Right to left shunting

Pulmonary arterial vasoconstriction

Pulmonary hypertension

Symptoms

Most cases are symptomatic at or shortly after birth

- Respiratory distress including tachypnea, grunting, cyanosis and tachpnea

- Feeding intolerance

Signs

1. Inspection
- Scaphoid abdomen - Decreased chest movement on the affected side

2. Auscultation
- Decreased air entry - Heard bowel sounds are heard in the chest - Heart sounds are distant or displaced on the affected side

Other presentations
Asymptomatic Non-specific eg. repeated chest infection Signs of intestinal obstruction or bowel necrosis following volvolus

Antenatal diagnosis Ultrasonography

As early as 25th week of gestation 1. Polyhydramnios 2. Bowel loop within the chest 3. Absent or intrathoracic gastric bubble 4. Mediastinal shift 5. Lung to head ratio (LHR): an important indicator of severity

Laboratory
- Maternal serum -FP may be decreased
- Amniocentesis may show decreased Lecithin to Sphingomyelin ratio and associated chromosomal anomalies

Postnatal diagnosis X-ray

Bowel loop in the chest Mediastinal shift Small portion of the lung on the ipsilateral side Deficient of the bowel gas in the abdomen

CT
distinguish between congenital diaphragmatic hernia and a cystic lung lesion (eg, congenital cystic adenomatoid malformation).

Differential diagnosis Congenital cystic lung diseases ( eg. Congenital cystic adenomatoid malformation)
Mediastinal cystic lesions ( eg. Cystic teratoma, Bronchogenic & thymic cysts )

Other congenital thoracic lesions (eg. Hiatal hernia & primary agenesis of the lung )

Possible antenatal markers of severity

Early gestational age at diagnosis on U/S Lung-to-head ratio (LHR) at 24-26 weeks gestation <1.0 Small fetal lung volume on 3D U/S and MRI >50% liver in chest on right side Liver on chest on left side Stomach and spleen on chest in left side No hernial sac (difficult to confirm on scanning)

Pre-operative preparation
Immediately following delivery, the infant is intubated (bag and mask ventilation is AVOIDED) to avoid air swallowing and distension of the gut. A nasogastric tube is passed to decompress the stomach and to avoid visceral distention which compresses the lung. Adequate assessment involves continuous cardiac monitoring, arterial blood gas and systernic pressure measurements, urinary catheterization to monitor fluid resuscitation, and both preductal (radial artery) and postductal (umbilicai artery) oximetry.

Pre-operative preparation (cont.)

Pressure limited ventilation should be used. Peak inspiratory pressures should be less than 30 cm H2O to avoid barotraumas to the hypoplastic lung.
Alternative means of support (e.g. inhaled nitric oxide [iNO]) highfrequency oscillatory ventilation [HFOV], ECMO) should be considered for the patient who fails to stabilize on conventional ventilation;

High-frequency oscillatory ventilation (HFOV): means the use of

high respiration rate with small tidal volume to improve oxygenation of the fragile hypopiastic lung to maintain preductal oxygenation while avoiding hyperventilation and barotrauma.

ECMO: means the use of extracorporeal membrane oxygenator

(artificial lung) in case failure to respond to maximal medical treatment.

ECMO
A life support system that employs partial heart-lung bypass providing rest to the lungs for long periods of time during which it is hoped that the lung and, in particular, the lung vasculature will mature.

Timing of surgery
It had been gradually shifted from an emergency repair to a policy of stabilization using a variety of ventilatory strategies prior to operation.

Operation
Reduction of the content & repair of the diaphragm
A subcostal incision is made. The abdominal viscera are examined, and the hernia is reduced by gentle traction. A hernia sac is sought and excised if found. Following careful dissection to the posterior leaf of the diaphragm, primary repair of the diaphragm can be accomplished in a single layer using nonabsorbable sutures.

Operation (cont.)
Reduction of the content & repair of the diaphragm
If the diaphragmatic defect is large enough to preclude primary closure, a Gore-Tex patch can be used. If abdominal closure may interfere with chest wall or diaphragmatic compliance or lead to abdominal compartment syndrome, then a temporary silo with delayed primary closure of the fascia or skin can be safely accomplished.

Use of chest tubes and whether to use suction are controversial. It is preferred to use a chest tube but limit suction to 5 cm H2O.

Postoperative
Transfer to ICU Warming IV fluids & antibiotics Ventilatory supports Chest X-ray to detect pneumothorax

Complications
1. 2. 3. 4. Recurrence Gastroesophageal reflux Small bowel obstruction may occur secondary to adhesions or intussusception Increased risk of neurodevelopmental problems and growth retardation

Prognosis
Poor prognosis in:
Polyhydramnios Right-sided CDH Intrathoracic stomach and liver LHR < 1.0

Eventration of the diaphragm

A developmental anomaly that results in muscular aplasia of the diaphragm.

associated with congenital myopathies or intrauterine infections

as a result of phrenic nerve injury during forceps delivery or surgery

Clinical picture
Ranges from asymptomatic to severe respiratory distress May present later with repeated attacks of pneumonia, bronchitis or bronchiectasis May present with GIT symptoms of vomiting or epigastric discomfort later in childhood

Diagnosis
Chest X-ray:
Elevated diaphragm with a smooth, unbroken outline

Treatment
Asymptomatic patients are treated expectantly Symptomatic patients: Surgery once the condition is stabilized Plication of the diaphragm

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