Congenital diaphragmatic hernia
- Published 8 January 2009
A female infant aged 5.5 months of Balkan origin arrived at the emergency department of our hospital with fever of 38.8°C, tachypnoea of 60 breaths per minute, heart rate of 110 beats per minute and oxygen saturation of 100%. The patient was admitted to the Paediatric Clinic and underwent full laboratory and culture tests, which were negative for infection. Initial chest x ray at admittance showed a consolidation of the left upper lobe of the left lung and the presence of air in the left hemithorax, which was believed to be probably the lower gastrointestinal tract. The patient was given a few millilitres of diluted gastrografin to swallow by mouth for the exclusion of tracheo-oesophageal fistula and localisation of the abdominal contents (stomach). It was decided to move the patient to a paediatric hospital but she died during transport. At autopsy bowel loops (left colonic flexure) were found in the left hemithorax and the consolidation was compressed atelectatic lung.
Congenital diaphragmatic hernia (CDH) is a life-threatening condition that occurs with a frequency of approximately one per 3000 births, when the fetal diaphragm fails to develop normally. The survival rate for infants with hernias of the foramen of Bochdalek varies from institution to institution; but overall, it has remained at approximately 50% for nearly half a century, despite advances in neonatal intensive care, anaesthesia and surgery. CDH is believed to result from incomplete fusion of the pleuroperitoneal membrane and passage of the abdominal contents into the chest. A historical review of the literature on this subject shows a wide divergence of opinion on the aetiology of the various types of CDH and on the recommended treatment. A variety of theories regarding its causes and the optimal way of approaching it have been published from the mid-19th century through the 20th century and are reviewed in this article. It was believed that, after birth, progressive compression of the lung and shift of the mediastinum by gaseous expansion of the herniated intestine was the sole pathological mechanism. Bochdalek’s hernia was reported by Lawrence as a “congenital deficiency of the muscular fibers of the left half of the diaphragm with displacement of the stomach”.2 The patient died of double pneumonia. This was followed by several case reports in the last half of the 19th century. Dittel reported a unilateral defect in the diaphragm of an infant who died soon after birth.3 The stomach, small intestines and part of the larger omentum were lying in the left pleural cavity; both the phrenic nerves were normal.
This article has been adapted from Kotis A A K, Tsikouris P, Guindaglia L. Congenital diaphragmatic hernia Emergency Medicine Journal 2008;25:261