Plenária: Kypros Nikolaides Hérnia Congénita Diafragmática

PLENÁRIA HÉRNIA CONGÉNITA DIAFRAGMÁTICA DR. KYPROS NIKOLAIDES

Kypros Nicolaides nasceu no Chipre e estudou Medicina no King’s College London. Atualmente, é professor no King’s College Hospital e diretor da sua unidade de investigação e do Harris Birthright Centre, uma unidade clínica e de investigação na área da Medicina Fetal, no mesmo hospital. Kypros Nicolaides fundou o Fetal Medicine Centre, cujos lucros revertem para uma fundação que subsidia investigação científica e bolsas de formação para médicos de todo o mundo. É, ainda, o protagonista de um episódio da série documental da Netflix The Surgeon’s Cut, que acompanha o seu quotidiano como médico.

Autor de mais de 1400 artigos publicados e vencedor de vários prémios internacionais, Kypros Nicolaides é considerado um dos “pais” da Medicina Fetal, tendo mais de 40 anos de experiência dedicados à cirurgia fetal, ao diagnóstico pré-natal e à investigação para prevenir complicações na gravidez. Foi pioneiro no desenvolvimento de técnicas cirúrgicas que permitiram salvar a vida de centenas de bebés, nomeadamente, a Oclusão Traqueal Fetoscópica, utilizada no tratamento de fetos com hérnia diafragmática.

How does it feel to perform surgery on beings that are still under development? Is it still nervewrecking or extensive training and experience beats the uneasiness?

Fetal surgery is difficult to perform because the patient is very small and it is always done by the use of endoscopes that are introduced through the mother’s abdomen into the uterus and these endoscopes are 2-3 millimetres in diameter. People performing fetal surgery need to have very extensive training, quite often for many years and that’s why there are only a few super specialist centres in the world that carry out these procedures.

How does the procedure for correction of diaphragmatic hernia work? What are the greatest difficulties and critical points to be aware of?

In diaphragmatic hernia the presence of liver and bowel in the chest compresses the lungs and prevents them from developing normally and the high proportion of babies will die soon after birth because their lungs would not have developed. Earlier attempts at fetal surgery in the nineteen eighties and early nineties involved opening up the uterus and then opening up the baby and trying to pull the liver and the bowel down to the abdomen and then stitching up the defect as you would in postnatal life. Unfortunately, the results from these surgeries were very poor. The method that we have developed twenty years ago involved the introduction of the thin endoscope inside the uterus and then entering the baby’s mouth going down to the oropharynx and then under the epiglottis, through the vocal cords and into the trachea. We then deposit a balloon into the trachea, which prevents the escape of the fluid from the lungs. Normally in fetal life, babies’ lungs are producing fluid, which is constantly escaping into the amniotic cavity. By inserting the balloon, the fluid is retained within the lungs, the lungs are stretched and this stretch stimulates growth. Also as the lungs expand some of the bowel from the chest will be pulled back into the abdominal cavity. The procedure is carried out at around 27 weeks and then we need to repeat the fetoscopy at around 34 weeks to go in and now puncture the balloon and remove it from the throat otherwise if the baby is born with the balloon in the throat it will die soon after birth because it will not be able to breath.

Since the usual treatment for congenital diaphragmatic hernia is surgery after the baby is born, what are the main advantages of performing this surgery in utero? Are the rates of survival and prognosis better than the traditional approach?

In most of the babies with diaphragmatic hernia the condition is not very severe and these babies have an operation after birth. However, in the group of babies that have severe diaphragmatic hernia, if we wait for surgery after birth the baby will die. It will die soon after birth and before even the action of having a surgery. In these cases of severe diaphragmatic hernia it is important to try to do a procedure before birth to stimulate fetal growth so that the babies survive after birth and then they will have a definite postnatal surgery. We have spent many years developing the technic and then in the last ten years we have been carrying out the multi-centres study in various centres in Europe and the United States and we have recently found out from this trial that intrauterine surgery in case of severe diaphragmatic hernia improves substantially the rate of survival. So, in summary, the surgery postnatally is the method of treatment for the majority of cases but in the subgroup with the severe disease fetal surgery is essential to improve survival.

What advice do you have for future doctors, in particular, those with an interest in obstetrics and gynecology and fetal surgery?

Obstetrics and gynaecology is a very interesting and diverse field. Some people will be very much interested in surgery for cancer for example, and they will become the gynaecology oncologists, others are interested in fertility and there are many developments in this field where you can be involved in in vitro fertilisation. Others are interested in general gynaecology and some are interested in obstetrics. Small group of people are allowed to specialise in fetal medicine so the beginning of the training is the same for everybody. They do obstetrics and gynaecology and then you

subspecialise in the field of interest. I think the field is extremely exciting and provided you love it then it is worthwhile because it is so very specialised and you’re helping babies survive that would otherwise have died.

Do you feel like an innovative surgeon? Looking back on your career, what do you feel was your main contribution to your field of work?

I have been involved in fetal medicine from the very beginning, soon after I qualified in medicine. The King’s College Hospital where I qualified was the pioneering centre in fetal medicine in the world, so I entered this field and then I have carried out an extensive research over the last few decades. I have described the series of different signs with which you can diagnosed fetal abnormalities. For example, in the nineteen eighties, I described how the shape of the head of babies with spina bifida is different than normal and I called that the lemon and the cerebellum is curved and I called that the banana sign. With these signs it is possible to diagnose the condition more easily. In the early nineteen nineties, I described how in babies with Down syndrome and many other chromosomal abnormalities, there is an increase in the collection of the fluid behind the fetal neck that can be seen by ultrasound easily in the 12 week of pregnancy. It is called nuchal translucency and the scan is now carried out throughout the world as a method of identifying the pregnancies at risk of chromosomal and other abnormalities. I introduced fetal blood sampling for the diagnosis of fetal anaemia and then the fetal blood transfusions. I introduced the use of shunts putting drainage tubes in the thorax that drain the fluid in cases where the babies have plural effusions into the amniotic cavity and putting shunts in the bladder to drain the urine that is retained in babies with obstructive uropathy. In the early nineties I described the endoscopic laser surgery for the separation of babies with twin to twin transfusion syndrome identical twins where one of the twins is haemorrhaging into the other and then in the early two thousands we introduced the endoscopic placement of the balloon in the fetal trachea.