Babies with CDH are typically unable to breathe alone and do not get enough oxygen-they will require the help of a respirator (also called a ventilator). Some babies who can not be managed successfully on the ventilator may require a temporary lung bypass machine. Once the baby's condition has improved (this may take up to a week or longer), the operation (to bring intestines down into the abdomen and close the hole in the diaphragm) will be performed.
It may seem strange to wait that long to perform the operation (since, after all, it is the hole in the diaphragm that caused the problem). However, pulmonary hypoplasia is the true problem and lung function needs to be corrected first. Operating too soon may upset the very delicate balance. The operation requires an incision in the skin and the muscles of the abdomen. All of the abdominal contents (including small and large bowel, stomach, spleen and sometimes liver) are removed from the chest and placed back into the abdominal cavity, and the hole in the diaphragm is repaired. Sometimes, the hole in the diaphragm is very large and may require an artificial patch to be placed.
After surgery the baby will be kept in the neonatal intensive care unit until full recovery. Many babies will require ventilator assistance for a number of days to weeks after surgery, until the lung has time to recover and improve its function.
The future for babies with diaphragmatic hernia is improving. The outcome for these babies is primarily based on the degree of lung underdevelopment and prevention of injury to those fragile lungs at birth. All babies will require a strict follow-up with the pediatric surgical service, to assist the long-term management of problems that may be part of the condition. Many babies may have problems requiring medications for lung therapy. Other babies have feeding problems from the malpositioned stomach in the chest during development, which leads to difficulty eating and with weight gain. Gastroesophageal reflux is another condition that may occur.
Many times babies require additional feedings through the placement of a feeding tube to give more calories in order to grow and become healthier. This detailed follow-up also provides the opportunity to ensure proper development. Early problems identified during follow-up with feeding, growth, or failure to meet normal milestones, are treated early. Early intervention with speech therapy and occupational therapy often helpss these babies improve muscle strength and coordination.
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