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What Is Congenital Diaphragmatic Hernia: Illustrated

An illustrated explanation of postnatal surgery to repair congenital diaphragmatic hernia. http://www.chop.edu/cdh 0:03 What is congenital diaphragmatic hernia? 0:31 How does congenital diaphragmatic hernia impact lung development? 0:50 How is congenital diaphragmatic hernia diagnosed? 1:05 What predicts the outcome in congenital diaphragmatic hernia? 1:12 What is pulmonary hypoplasia? 1:42 What is pulmonary hypertension? 1:55 How is congenital diaphragmatic hernia surgically repaired after birth? 2:28 Why is it important to follow babies born with congenital diaphragmatic hernia over the long term? Congenital diaphragmatic hernia (CDH) is a birth defect that occurs when the diaphragm — the flat muscle that separates the heart and lungs in the chest from the liver, stomach, intestine and other organs in the abdomen — doesn’t close properly as the baby forms in the womb. The hole in the muscle may be on the left or right side, and small or large. If the hole is large enough, the intestine, stomach, spleen and liver can move up into the chest. There, they can crowd the heart and lungs and keep the lungs from growing normally. CDH occurs in 1 of every 3,000 live births. Most of the time, the condition is diagnosed during pregnancy. To ensure an accurate CDH diagnosis and the best outcome, it is important to visit a fetal therapy center experienced in evaluating and caring for pregnancies affected by CDH. Since 1995, the Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment at Children’s Hospital of Philadelphia (CHOP) has been at the forefront of understanding, diagnosing and treating CDH. The center’s team has cared for more babies with CDH than any other team in the U.S. Expectant mothers from all 50 states — and more than 70 countries — have traveled to Philadelphia for care at the center. Because it is a large, heavy organ, if the liver moves up into the chest, it can cause particular problems. The center uses sophisticated imaging to look at liver placement and lung size – the two biggest predictors of how a baby will do. Many babies with CDH develop a condition called pulmonary hypoplasia, which means small lungs. When they are born, they may have trouble getting enough air. Babies with CDH may also develop high blood pressure in the lungs (pulmonary hypertension). CHOP monitors and treats this condition very carefully. In most cases, the pregnancy is followed closely by the center’s obstetrical team until planned delivery. Babies are born in CHOP’s Garbose Family Special Delivery Unit (SDU), the world’s first birth facility in a freestanding pediatric hospital which was designed specifically for healthy mothers carrying babies with known birth defects. After delivery, the baby is immediately handed to a waiting team of doctors and nurses and stabilized by a specialized Newborn/Infant Stabilization Team and Neonatal Surgical Team — the only team of its kind in the world. The baby is taken to CHOP’s Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU), ranked among the best in the nation. Once the baby shows improvement, it is time for surgery to repair the diaphragm. At CHOP, the anesthesiologists and surgeons perform the operation at the bedside in the N/IICU, as it’s safer for the baby not to be transported to another location. The baby is placed under general anesthesia. The surgeons make an incision below the ribs and gently bring the liver, stomach and intestines into place. They then repair the diaphragm using stitches and, often, a Gore-Tex patch. The baby will recover in the N/IICU for weeks to months. As babies grow, their brains and lungs grow as well. In fact, the child’s lungs will continue to grow for several years. CHOP’s Pulmonary Hypoplasia Program follows these children into adulthood, providing specialized follow-up care.