When treatment begins before birth

More than a year ago, together with experts from medical centers in Brazil and the United States, you performed the first prenatal surgery for fetal encephalocele in Europe and the third in the world. A baby girl was born in late August. How is the little patient doing today?

Dr. hab. Magdalena Litwińska: She celebrated her first birthday in August, and her mother sent us some photos. The little girl is absolutely delightful, and we are very happy to be part of her life. We believed that Julka had a good prognosis and could grow up to lead a happy life. Her mother wrote to us to thank us for our tremendous commitment. And it really was a huge effort, not just on our part but on the part of the entire hospital. Given the pioneering nature of this surgery, we had to obtain all necessary approvals, which were granted through an expedited procedure. Organizing the entire procedure was a major challenge. That's what happens when you perform an operation for the first time. You never quite know what to expect. However, we had every reason to believe that the little patient would benefit from it. That was our goal, and I think we achieved it. 

Is the little girl still being monitored regularly by your center?

Dr. hab. Ewelina Litwińska: Of course. The patient is under close follow-up by a large multidisciplinary team of specialists. For us, this is also a matter of scientific knowledge and further education. We need to learn from each case so that we know how best to proceed if another patient with the same condition comes to us. 

Dr. hab. Magdalena Litwińska: The girl is also being cared for by Dr. hab. Tymon Skadorwa from the Department of Pediatric Neurosurgery at UCK WUM. 

What was the nature of the girl's condition? 

Dr. hab. Magdalena Litwińska: She had a meningoencephalocele. This is a condition in which part of the brain of an unborn baby is located outside its normal anatomical position. With each passing week of pregnancy, the condition progresses and the defect becomes more severe. In this patient's case, we were already seeing signs of what is known as microcephaly. The baby's head circumference was decreasing, which could have led to severe neurological damage. Most cases of this kind of defect are associated with a very poor prognosis. 

Dr. hab. Ewelina Litwińska: Two months before the operation, we had completed a short clinical placement in the United States. We discussed the fact that the world's first prenatal surgery for fetal encephalocele had been performed in Brazil. It was a purely theoretical discussion, but just a few weeks later, a patient with exactly this condition came to our center. 

How did the operation proceed, and what did it involve?

Dr. hab. Magdalena Litwińska: Prenatal operations at our center are fetoscopic, meaning minimally invasive procedures. We open the patient's abdomen as we would for a cesarean section. We exteriorize the uterus so that it is outside the abdominal cavity and insert the instruments. We operate through the uterine muscle. In the case of fetal encephalocele, the hernia sac was first dissected free, and then a patch was sutured in place to close the opening in the skull. The skin over the patch was then sutured. 

How long did the operation take, and how many people were involved? 

Dr. hab. Ewelina Litwińska: We expected it to be a lengthy procedure. We thought it might take five to seven hours. In the end, however, it lasted three and a half hours. Many people were involved. In addition to the two of us, three specialists from a center in Houston, Texas, took part, including Professor Michael A. Belfort, a legend in the field of fetal surgery. We were also joined by doctors from Brazil and the United Kingdom. 

The fetal encephalocele operation was pioneering and exceptional. What types of intrauterine surgery do you perform on a regular basis?

Dr. hab. Magdalena Litwińska: We treat three main conditions for which prenatal intervention can improve the prognosis. We will be treating one of them later today. We will perform a fetoscopic procedure for twin-to-twin transfusion syndrome (TTTS). This procedure marked the beginning of complex intrauterine surgery. The second type of operation we perform involves placing a balloon in the fetal trachea in cases of congenital diaphragmatic hernia. The third type of procedure is prenatal surgery for fetal spina bifida. 
Dr. hab. Ewelina Litwińska: It is worth adding that prenatal surgery for spina bifida provided the foundation for performing surgery in the case of fetal encephalocele. Only centers that routinely perform complex intrauterine procedures can consider treating even more unusual conditions, such as encephalocele.

What does prenatal surgery for spina bifida involve?

Dr. hab. Magdalena Litwińska: Our aim is to close the spine defect. Through this opening, the spinal cord protrudes and is exposed to the toxic effects of amniotic fluid, as well as the risk of mechanical injury. The procedure is performed in collaboration with and under the supervision of a neurosurgeon. Our task is to untether the spinal cord and then close the tissues over the defect in layers. 

Dr. hab. Ewelina Litwińska: We also try to delay delivery as long as possible, ideally until after 34 weeks of pregnancy. In cases of spina bifida, following intrauterine surgery, vaginal delivery is also possible. 

What are the outcomes of this type of surgery? Is the baby born healthy, or is further medical care required?

Dr. hab. Magdalena Litwińska: Even after a successful operation, the baby still requires special care after birth. Parents are aware of this. It is not the case that the defect is completely cured in the womb and the baby is born healthy. The child will still have health problems, but they will be less severe.

You mentioned congenital diaphragmatic hernia. What exactly does this condition mean?

Dr. hab. Ewelina Litwińska: The diaphragm is a muscle that separates the abdominal cavity from the chest cavity. In congenital diaphragmatic hernia, there is a defect in the diaphragm. It develops early in fetal development. As the pregnancy progresses, abdominal organs move through this opening into the chest. And that is precisely where the problem lies. The displaced organs compress the lungs, preventing them from developing properly. 

What can be done in such cases before the baby is born?

Dr. hab. Ewelina Litwińska: We can use minimally invasive intrauterine therapy. The procedure is performed fetoscopically and involves placing a balloon in the fetal trachea. This blocks the fetal airway, trapping lung fluid inside the alveoli. In this way, we stimulate fetal lung development. So, in cases of diaphragmatic hernia, we do not repair the hernia itself or suture the defect. Our goal is to promote the best possible development of the fetal lungs so that, after birth, the baby has the greatest possible chance of surviving long enough to undergo hernia repair surgery and of achieving the best possible outcome from that operation. Intrauterine intervention for diaphragmatic hernia is therefore not a definitive treatment. It serves as a bridge to surgical repair after birth. 

You will also be performing an operation for twin-to-twin transfusion syndrome (TTTS) today. What does this condition involve?

Dr. hab. Magdalena Litwińska: TTTS occurs only in monochorionic twin pregnancies, in which the babies share one placenta but have two separate umbilical cord insertions. The two umbilical cord insertion sites are connected by blood vessels through which blood flows. In TTTS, this blood flow is unequal, putting the lives of both babies at risk. One becomes the recipient, meaning that it receives too much blood, while the other becomes the donor and suffers from hypovolemia, meaning that it has too little blood. Prenatal surgery is therefore life-saving and constitutes a definitive treatment. If it is successful, we have a chance of both babies being born healthy. In cases of twin-to-twin transfusion syndrome, we use fetoscopic laser ablation to seal the abnormal vascular connections between the twins. 

Of all the procedures you perform, which are the most common? 

Dr. hab. Magdalena Litwińska: We most frequently perform laser ablation for twin-to-twin transfusion syndrome. Although this is a rare complication, we see quite a lot of patients because they come to our clinic from all over Poland and from abroad. Prenatal operations for spina bifida and diaphragmatic hernia are less common. In both cases, we have very strict eligibility criteria, and we must have difficult conversations with parents about their expectations and what we can realistically offer. The point is that the treatment is not definitive and, like any procedure, carries risks.

What risks are associated with the prenatal operations we have been discussing?

Dr. hab. Magdalena Litwińska: The greatest challenge is the risk of preterm delivery. This can happen for many different reasons, including complications that arise during the procedure. It is important to remember that we have two (or sometimes three) patients here: the pregnant woman and the fetus or fetuses we are trying to help. We present each of our patients with all available options. And we always decide whether or not to proceed with surgery together with the patient. In the case of twin-to-twin transfusion syndrome, the situation is straightforward. If we do not perform the operation, the babies will die or be born in an extremely serious condition. With spina bifida, the situation is more complicated. We now have a substantial body of published evidence indicating that children with spina bifida who undergo prenatal surgery, compared with those who do not, have greater motor independence, are less likely to require ventriculoperitoneal shunt placement after birth, and ultimately enjoy a better quality of life.

Dr. hab. Ewelina Litwińska: In cases of diaphragmatic hernia, we know that when the defect is severe, only about 10 percent of babies survive without prenatal surgery. If the operation is performed, however, we can increase their chances of survival to almost 50 percent. The mother must therefore be aware of the very high risk that a baby with severe diaphragmatic hernia may not survive. Prenatal surgery nevertheless allows us to maximize the baby's chances. 

How do you see the future? What other congenital conditions might become treatable through surgery before birth? 

Dr. hab. Ewelina Litwińska: During our most recent visit to the United States, we discussed surgery for gastroschisis with Professor Belfort. This is a condition in which the fetal intestines are located outside the abdominal cavity. The first intrauterine procedures have already been performed to return the intestines to the abdominal cavity so that the baby can be born healthy. It is important to remember, however, that this condition can present in different ways. In some cases, it is uncomplicated and does not require intrauterine intervention. Surgeons can deal with it after birth, and the baby will also be healthy. 

Dr. hab. Magdalena Litwińska: Everyone likes the phrase "pioneering surgery." However, we cannot perform operations simply to boost our egos or showcase spectacular procedures. That is why we must select patients for intrauterine surgery only when their babies stand to derive real benefits from it.

Few women know that intrauterine surgery is even an option. How do patients find their way to your clinic?

Dr. hab. Ewelina Litwińska: We provide education and organize conferences and training sessions for doctors. Education is our second great passion. We are delighted when doctors subsequently contact us and request consultations. Today, for example, we have received referrals for patients from Lower Silesia and Konin. 

Dr. hab. Magdalena Litwińska: Yes, educating doctors is the most important thing. They want to learn; we just need to share our knowledge with them and make ourselves available. We are also active on social media, but our followers there are mostly patients, particularly those who already know about us. 

Can we say that patients in Poland have access to prenatal surgery at the highest international standards? How would you assess the center here? 

Dr. hab. Ewelina Litwińska: I would rate it very highly. It should be emphasized that there are very few centers in the world like the University Center for Women's and Newborn Health, where we offer the full range of intrauterine procedures. 

Dr. hab. Magdalena Litwińska: That's right. We perform only those procedures whose effectiveness has been confirmed by large-scale studies. We collaborate with leading centers around the world.