Congenital Diaphragmatic Hernia

Published on 10/06/2015 by admin

Filed under Pediatrics

Last modified 10/06/2015

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Congenital Diaphragmatic Hernia

Patricia R. Chess, MD

Basic Information

Definition

Congenital diaphragmatic hernia (CDH) is a diaphragmatic defect resulting from failure of the posterolateral portion of the diaphragm to develop, usually involving the foramen of Bochdalek, resulting in herniation of abdominal contents into the chest cavity. This leads to various degrees of pulmonary hypoplasia.

Synonyms

Congenital hernia of the diaphragm
D-hernia
Diaphragmatic hernia

ICD-9-CM Code

756.6 Congenital diaphragmatic hernia

Epidemiology & Demographics

• Prevalence is 1 case per 3000 live births.
• Most cases are sporadic.
• There is a 2% recurrence risk, and an autosomal recessive inheritance pattern usually is seen in recurrent cases.
• The mortality rate is up to 50%, even with aggressive support.

Clinical Presentation

History

• Prenatal: polyhydramnios, lack of stomach bubble, loops of intestine seen in the chest by ultrasound
• Respiratory distress may occur in the delivery room, and mild symptoms may delay the diagnosis.
• Patients are at high risk for pneumothorax with positive-pressure ventilation because of pulmonary hypoplasia.

Physical Examination

• Respiratory distress
• Decreased left-sided breath sounds (90% of hernias are left sided)
• Heart sounds shifted to right
• Scaphoid abdomen

Etiology

• Most cases are sporadic; an autosomal-recessive pattern has been described in recurrent cases.
• Approximately 40% are associated with chromosomal, genitourinary, renal, cardiac, or central nervous system anomalies.

Diagnosis

Differential Diagnosis

• Bronchogenic cyst
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