Congenital Diaphragmatic Hernia Unlike Many

Congenital Diaphragmatic Hernia

Unlike many problems that can plague newborns but usually clear up on their own or with mild medical intervention, congenital diaphragmatic hernia (CDH) is a major surgical emergency. Prompt diagnosis and treatment are the keys to survival, especially in babies that are born prematurely. How severe the hernia is usually directs the course of action that is taken. For example, some very small hernias may not even be noticed at birth, but larger hernias will cause severe and immediate respiratory problems and distress. A congenital diaphragmatic hernia occurs when the diaphragm has a hole in it that allows the contents of the abdomen to push into the chest cavity. This can include the stomach, bowel, and intestine, as well as the liver and other organs. When this takes place these organs can be damaged, but they also stop the lungs from growing to their normal size and working properly. Because of this, respiratory distress occurs and this is not something that will be resolved on its own. Instead, surgical intervention is required to close the hole in the diaphragm to ensure that the organs stay where they should be and the lungs have a chance to mature correctly and function normally. Often, CDH is called a Bochdalek hernia, to honor Victor Alexander Bochdalek who contributed greatly to the findings of this problem and the knowledge of it. Within the United States, CDH can be expected in one out of every 2000-4000 live births and makes up 8% of all of the major congenital anomalies. Internationally, the numbers are about the same (www.emedicine.com,2004).

Mortality rates are a concern for this condition, as the mortality rate is estimated to be somewhere between 25-60%. However, it is difficult to judge what the actual mortality rate is for those that have the hernia surgically corrected, because this percentage includes what is called 'hidden mortality.' This is the percentage of babies with CDH that die before they can even be transferred to the surgical unit for intervention. In other words, the mortality rate may not accurately reflect the rate of babies with this condition that are surgically treated but still die from the hernia or the trauma to their bodies during surgery. If the stomach is found to be in the chest cavity, the outcome is often poorer, as well. It is also possible that this issue with the stomach means that the baby developed the hernia at an earlier gestational age and therefore there has been more time for pulmonary hypoplasia to take place, further reducing the infant's chances for survival. Congenital diaphragmatic hernia also has some recurrence risk for future siblings, but it is only around 2%. In general, CDH is not something that is seen to run in the family and only does so in very rare cases. There are also other syndrome and conditions that are associated with CDH including De Lange syndrome and Fryns syndrome. In addition to this, there are malformations of the cardiovascular, gastrointestinal, and genitourinary systems that can be related to CDH (www.emedicine.com,2004).