Figure 1






Congenital diaphragmatic hernia (CDH) was first described in the medical literature in the early 18th century and is characterize by the herniation of abdominal contents (bowel and spleen, liver, kidneys etc) into the pleural cavity (Figure 1). The first successful surgical repair of a CDH was carried out by Dr Robert E. Gross, a surgeon at the Harvard Medical School and Children’s Hospital in Boston, Massachusetts.


There are 3 basic types of CDH: 1. Bochdalek hernia (occurring at approximately 6 weeks’ gestation, most common type occurring in approximately 85% of cases), associated with a posterolateral defect of the diaphragm (Figure 2), 2. Morgagni hernia, usually anterior through a defect in the parasternal region (Figure 2), and 3. Hiatus hernia. CDH can be associated with other congenital abnormalities such as variable degree of pulmonary hypoplasia and left ventricular hypoplasia.



Figure 2: Anatomical diaphragmatic defects associated with different types of Congenital Diaphragmatic Hernia.



CDH occurs in 1 of every 2000-3000 live births and accounts for 8% of all major congenital anomalies. Risk of occurrence of isolated non-syndromic CDH in future siblings is approximately about 2%.  Familial CDH is rare, with incidence of less than 2% of all cases but both autosomal recessive and autosomal dominance pattern of inheritance have been reported. Syndromic CDH occurs in Cornelia de Lange syndrome and Fryns syndrome.


CDH carries a high mortality after birth and has been reported to be in the range of 40-62%. Best chance of a cure is early reparative surgery. In cases of CDH associated with pulmonary hypoplasia, surgical repair can be performed with ECMO support during and after surgery to allow for the hypoplastic lung to grow and developed after surgical repair.


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Images prepared by Mr Chong Chee Fui, Consultant Cardiovascular & Thoracic Surgeon, Thoracic Unit, Department of General Surgery, RIPAS Hospital, Brunei Darussalam.

All images are copyrighted and property of RIPAS Hospital.