Fetal Care Program

A congenital diaphragmatic hernia, commonly referred to as CDH, results from the failure of the diaphragm's closure as the baby develops. They are usually diagnosed prenatally on an ultrasound. Once the diagnosis is made, you will see a specialist throughout your pregnancy and have further imaging. This will likely include more ultrasounds and an MRI; helping determine the severity of the CDH. The hole can be either on the diaphragm's left, right or both sides. All intra-abdominal organs can be found in the chest. This displaces the heart and affects lung growth. The morbidity from CDH comes from pulmonary hypoplasia (very small, underdeveloped lungs).

The baby will need extracorporeal membrane oxygenation (ECMO) support. ECMO is a cardiopulmonary bypass machine that some babies with CDH need until their lungs can grow. We are fully equipped with the technology, expertise and healing touch to provide this specialized treatment.

Here is a breakdown of the process:

• Delivery is usually planned between 37- and 38-week gestation age
• Immediately after birth, the baby will be assessed and likely need a breathing tube
• Your team will decide whether the baby needs ECMO support; only babies with very severe CDH need ECMO support Once your baby stabilizes, they will undergo surgical treatment

Once your baby’s surgery is complete, there is a long road to recovery as the lungs continue to develop and grow. While your baby may experience feeding difficulties, with about 25% will need a feeding tube and possibly another surgery for reflux, our compassionate team will stay by your family’s side no matter what challenges may arise — every step of the way.

In gastroschisis, a congenital disorder of the anterior abdominal wall, the intestines are located outside the abdominal wall. Extensive bowel injury during fetal life can occur secondary to exposure to the amniotic fluid. Infants can be born prematurely, but this is not usually associated with abnormal chromosomes or anomalies in other organ systems. There is a 10% chance of having other intestinal problems, though. The overall outcome for infants with gastroschisis is quite good, with severe or life-threatening problems happening only rarely.

If your obstetrician advises it, these babies can usually be delivered vaginally. They will be taken to the NICU and examined by a surgeon.

If there is sufficient abdominal capacity, we would reduce the intestines at the bedside. However, we often place a silastic silo at the bedside in the NICU, reducing the intestines over several days to a week. After all the intestines are safely reduced into the abdominal cavity, the infant is brought to the operating room for final abdominal wall closure.

The baby may then be in the hospital for at least another few weeks as the bowels start to work normally again. They are given nutrition through a special IV until they can eat independently.

The overall long-term outcome is quite good in babies with gastroschisis. However, there are a small number of babies who never absorb nutrients well and may require total IV nutrition for prolonged periods, which may lead to severe liver damage.

With an omphalocele, the intestines are outside the abdomen but covered with a thin membrane that protects the intestines; however, omphalocele can be associated with chromosomal and anatomic anomalies that are associated with morbidity more than the omphalocele itself. When the baby is born the bowel will be covered, and the baby will be assessed to look for other anomalies. If the baby is otherwise stable, doctors will decide whether the omphalocele can be closed immediately or at another time. If the omphalocele is very large, it is more likely that the defect will be closed when the baby is older. Dressings will be applied daily and changed frequently until skin has grown over the membrane. The timing of surgical repair depends on size of the defect and other associated anomalies.

A pulmonary sequestration/congenital pulmonary adenomatous malformation (CPAM) is an abnormal cystic growth within the baby’s lung which can be diagnosed prenatally. In over 90% of these lesions, we do not see problems during fetal life. As the baby grows, the mass often appears smaller as the baby enters the third trimesterand approximately 10% of CPAMs seem to disappear.

Most babies have no symptoms at birth, and most are discharged home with their parents. The babies come to our surgery clinic around 2 to 3 months of life. We then discuss getting more pictures of the lesion, which usually involves a computed tomography (CT) scan.

For most asymptomatic lesions, we still recommend surgical removal due to the risk of infection and malignant degeneration later in life. Surgery timing is usually between 6 and 12 months of age. In many lesions, a complete anatomic lobectomy is required. The rest of the baby's lungs will grow and compensate for the removed part. We have been removing most of these lesions thoracoscopically, through very small incisions, whenever possible.

Another congenital disorder of the anterior abdominal wall, an omphalocele, is when the intestines are outside the abdomen but covered with a thin membrane. This protects the intestines; however, omphalocele can be associated with chromosomal and anatomic anomalies. These can cause more morbidity than the omphalocele itself.

When the baby is born, a bowel seal will be covered, and then the baby will be assessed to look for other anomalies. If the baby is otherwise stable, the decision will be made on whether the omphalocele can be closed in the newborn stage or at another time. If the variceal is very large, it is more likely that the defect will be closed when the baby is older. Dressings will be applied daily and changed frequently until skin has grown over the membrane. The surgical repair timing depends on the defect's size and other associated anomalies.