| Diaphragmatic hernia|
|By Joel Mills - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=946353|
Diaphragmatic hernia Main page
Synonyms and Keywords: Congenital diaphragm hernia, Diaphragm hernia, Hernia through the diaphragm.
Diaphragmatic hernia is a congenital anomaly that occurs due to failure of the diaphragm to close leading to herniation of the abdominal content into the thoracic cavity leading to pulmonary hypoplasia. It most commonly presents in the neonates as respiratory distress. It is classified according to the site of herniation into anterior, posterolateral, and hiatal diaphragmatic hernia. It can cause complications in both the GIT and the cardiopulmonary circulation. It can be diagnosed antenatally in the week 24 and after birth, it is apparent on chest x-ray. The mainstay of treatment is surgical correction which must be done when the infant is completely stabilized.
- In 1679, the first case of congenital diaphragmatic hernia was reported by Riverius.
- In 1888, the first procedure to repair congenital diaphragmatic hernia in an adult was done and in 1889, the first procedure on an infant was done.
- In 1905, the first successful operation was done but the success rate remained below 60% until 1925.
- In 1940, A set of diagnostic criteria for diaphragmatic hernia were suggested.
- In 1950, the transthoracic approach was suggested instead of the transabdominal approach.
Posterolateral (Bochdalek) diaphragmatic hernia
- It is the most common subtype.
- Most commonly occurs on the left side and rarely occurs bilaterally.
Anterior (Morgagni) diaphragmatic hernia
- In anterior diaphragmatic hernia, the intestine bulges into the thorax through the anterior midline.
- Most of the cases of anterior diaphragmatic hernia occur on the right side.
- The intestine finds its way to the thorax through the esophageal hiatus.
- More common in the adults than in neonates.
- The herniated viscera decrease the area needed by the lung to develop appropriately.
- According to the degree of herniation, the degree of pulmonary hypoplasia is determined.
- The herniated viscera impairs the development of the bronchial tree.
- As a consequence, the alveoli development is also slowed.
- The development of the pulmonary artery tree is halted too resulting in excessive masculinization of the arteries.
- The increased pulmonary pressure leads to increased pressure on the right ventricle leading to the development of a right to left shunt.
- As most of the cases of diaphragmatic hernias are unilateral, the pulmonary hypoplasia is also usually unilateral, but it can be bilateral if the mediastinum is pushed by a massive unilateral hernia.
- The abnormal alveolar development can lead to hypoxemia leading to pulmonary vasoconstriction which aggravates the condition.
Risk factors of diaphragmatic hernia include:
Differentiating Diaphragmatic hernia from other Diseases
- Congenital diaphragmatic hernia has no sex predilection.
- 90% of the congenital diaphragmatic hernias present in the neonatal period.
According to USPSTF, there is no screening recommended for diaphragmatic hernia.
Natural history, complications and prognosis
- 90% of the cases of congenital diaphragmatic hernia presents in the neonatal period.
- If left untreated, congenital diaphragmatic hernia can be fatal due to pulmonary hypoplasia and severe pulmonary hypertension.
- Pulmonary hypoplasia
- Pulmonary artery hypertension
- In severe cases, ventricular hypoplasia
- The prognosis is mainly dependent on the size of the defect and the degree of the herniation.
- The presence or absence of liver herniation on fetal MRI is the most reliable prediction of the postnatal survival.
- The estimation of fetal lung volume and lung area to head circumference is a useful indicator of the prognosis of the disease in the absence of liver herniation.
- The mortality ranges from 25-60% of the cases.
History and Symptoms
CDH most often presents in the neonatal period as:
- Rapid breathing (tachypnea)
- Shortness of breath (dyspnea)
- Increased work of breathing (labored breathing)
- Intolerance to feeding due to inadequate oxygenation.
- Adrenal insufficiency: it is a common association with congenital diaphragmatic hernia.
- Heart rate: tachycardia
- Respiratory rate: tachypnea
- Blood pressure: The infant may have hypotension due to compression of the ventricles
- The chest may have a barrel shape.
- Absent breath sounds at the side of the hernia
- Peristaltic sounds in the chest
- The heart sounds may be displaced to the other side of the chest.
- The heart sounds may reveal the murmurs of associated cardiac anomalies.
- The abdomen is scaphoid due to herniation of the abdominal content into the thoracic cavity.
- Amniocentesis: karyotyping should be obtained to rule out associated chromosomal abnormalities.
- Maternal alpha-fetoprotein is decreased in CDH
- Arterial blood gas: ABG may show hypoxemia, metabolic acidosis, and hypercapnia.
- The infant should be investigated for the presence of associated chromosomal abnormalities.
- CDH can be diagnosed in the 24th week.
- In addition to the hernia, ultrasound can show polyhydramnios and absence of the gastric air bubble
- Left sided CDH is far more common than right sided CDH, while bilateral CDH is rare.
- The presence of liver herniation is associated with worse prognosis and survival.
- The estimation of lung area to head circumference ratio can give an idea about the prognosis (especially if there is no liver herniation). The lower the ratio, the worse the prognosis.
- Bowel loops in the thoracic cavity (can be further demonstrated through placement of a feeding tube)
- Shifting of the mediastinum to the contralateral side
- Echocardiography must be done to exclude associated cardiac anomalies and to exclude the presence of an associated ventricular strain from the pressure.
- Preoperative stabilization of the patient is important as performing the operation in an unstable infant is associated with higher morbidity and mortality rates.
- Preoperative stabilization measures include:
- Mechanical ventilation: The aim is to ensure proper oxygenation without causing any barotrauma to the lung. Peak inspiratory pressure should be always less than 30 cm H2O
- Nasogastric tube insertion: The aim is to decompress the bowel and to give more space for the lung
- Blood pressure support: using vasopressors or isotonic fluid: The aim is to keep the mean blood pressure above 50 mmHg.
- High-frequency oscillatory ventilation: It is useful in improving the oxygenation without increasing the pressure in the airways or the alveoli.
- Extracorporeal membrane oxygenation: It is used as the last resort in patients resistant to all the conventional methods of ventilation.
- Inhaled nitric oxide: The hypoxemia resulting from lung compression can cause hypoxemia that may result in pulmonary vasoconstriction and hypertension.
- The procedure must not be done unless the infant is stable.
- Most of the operations are done through a subcostal incision.
- The repair can be done in one of two ways depending on its size:
- Primary closure: it involves repairing the defect using non-absorbable sutures. It is used if the defect is relatively small.
- Patch closure: using a prosthetic or fascial patch to close the defect. It is useful especially with large defects but increases the risk of infection.
- Endoscopic procedures through the thorax or the abdomen have been reported to have lower mortality rates but have higher recurrence rates.
- If CDH repair is associated with an abdominal wall defect, silo closure may be tried temporarily until the CDH is stabilized.
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