Open spina bifida (OSB), a birth defect that occurs in approximately 1 in 1,500 births in the United States, results from failure of the spine to close at some point along its length during fetal development. The defect leads to injury and loss of spinal cord tissue at and below the defect. Research showed that antenatal treatment of OSB via open fetal surgery, which requires opening the maternal abdomen and uterus, can improve the outcome of babies affected with OSB. However, the mother is at risk for rupture of the uterus (uterine rupture) and requires delivery by cesarean section for the affected pregnancy and for any subsequent pregnancy.
Doctors at The Fetal Institute, Miami and the USFetus, in collaboration with colleagues in Brazil, have developed a minimally-invasive (fetoscopic) technique to treat fetuses with OSB, which does not require opening the uterus. This allows the mother to deliver vaginally and avoid the risk of uterine rupture. The doctors are currently conducting a clinical trial to validate this new fetoscopic technique. For further information, please click here.
I respect and share the mission of The TTTS Foundation of providing babies and mothers in need with the best possible options for treatment.
Their website serves as the first point of information for families seeking treatment for TTTS, including TAPS, SIUGR and TRAP.
The TTTS Foundation does amazing work! I have admired over the years how much they have helped families in need and how effectively they have helped spread awareness. I share in the passion of The TTTS Foundation in helping families affected by these conditions.
I am truly honored and very pleased to be a member of the Medical Advisory Board of The TTTS Foundation and help to fulfill their mission.
– Dr. Eftichia V. Kontopoulos
TTTS Foundation Medical Advisory Board
Dr. Kontopoulos is an internationally recognized fetal surgeon. Since 2017, Dr. Kontopoulos is member and Co-Founder of The Fetal Institute Miami, where she practices Maternal-Fetal Medicine, Fetal Therapy and Fetal Surgery.
For more information about TTTS, including TAPS, SIUGR and TRAP, please go to tttsdfoundation.org.
Parents Lauren and Ryan were helped by The TTTS Foundation when Lauren was diagnosed with TTTS. She had laser surgery at 18 weeks. She developed post laser TAPS, a form of TTTS, and had a second laser surgery. Precious little Collin and Caden were born at 27.4 weeks and are now safe at home with their big sister Juliana.
In collaboration with The Fetal Institute Miami, the National Institute of Perinatology in Mexico (INPer) successfully performed a Fetal Laser Photocoagulation Surgery. The goal is to create the first public National Center for Fetal and Perinatal Surgery in Mexico
Dr. Ruben Quintero from The Fetal Institute Miami and the specialists from the National Institute of Perinatology, “Isidro Espinosa de Los Reyes” (INPer), successfully performed a fetal laser photocoagulation surgery. The procedure was carried out in a patient with a twin pregnancy of 20 weeks’ gestation.
This intervention allowed correcting an alteration known as Twin-Twin Transfusion Syndrome (TTTS), a specific and unique complication of multiple gestations with a single placenta that causes both fetuses to pass blood to each other in an unbalanced way through placenta vascular communications, which puts the lives of both fetuses at risk.
On January 29, 2021 the intervention was carried out by a team of specialists from The Fetal Institute Miami and Maternal-Fetal Medicine Specialists from INPer. In Mexico, there are approximately 32,000 twin births; of these, 30% are monochorionic (development of twins with a single placenta), of which up to 20%, about 1,800 pregnancies, will develop TTTS.
Minimally-invasive surgery consisted of making an incision in the skin of the mother to insert a cannula into the uterine cavity, where a camera and the laser fiber were introduced.
Through the camera, a direct view of the placenta is obtained through an endoscope. After identifying the vascular communications, they are coagulated with laser, eliminating all vascular connections between the fetuses and dividing the single placenta into two independent placental territories.
In order to offer specialized and high-quality care that patients and their babies require, INPer has the project of creating the first public National Center for High-Specialty Perinatal Surgery with the support of The Fetal Institute Miami, directed by the renowned specialists in fetal surgery Dr. Rubén Quintero and Dr. Eftichia Kontopoulos.
The collaboration between both institutes aims to train and expand INPer’s fetal medicine specialists’ skills, weighing ethics and humanism values, becoming the first public hospital in Mexico to treat pathologies during pregnancy with minimally-invasive techniques.
The Quintero’s TTTS staging system has basically been adopted all over the world it is a very practical way of assessing patients with twin twin transfusion syndrome it has very clearly defined parameters that anyone can follow and it has withstood the test of time. I published this in 1998 and many years later it still holds and allows us to evaluate and tell patients how severe their particular condition is.
The Quintero staging system allows both the colleagues and the patients to know how severe their particular TTTS case is. People have asked me how did you come up with this classification system? What I realized when I started evaluating patients with twin twin transfusion syndrome is that not all the cases presented in the same way. I decided to analyze what was the difference in the different presentations. For example, we will get a phone call from a colleague saying, “hi I have a patient has a mild case of TTTS” or “I have a patient with a severe case of TTTS” so we didn’t know what that meant. By noting that they don’t present in the same fashion, I basically came up with a classification that helps us say how severe is the disease for each particular case and the staging system that I proposed as five stages:
In the first stage an ultrasound you can still see the bladder of the donor twin they all have regardless of the stage the discordant in amniotic fluid volume one more than eight centimeters deep and the other less than two centimeters deep regardless with days but in stage 1 you can still see the bladder of the donor twin.
In stage 2, after an hour of observation and ultrasound you cannot see the bladder of the donor twin, though a stage 2 patient is more severely affected than a stage 1 patient.
In stage 3, the blood flow studies of either twin are very abnormal. When we say very abnormal that means that they are not above a certain percentile or some other norm that you can look on a table, not they are either present or absent the blood flow studies the anomalies in the blood flow studies. So in stage 3 either the umbilical artery shows no blood flow at the end of the cardiac cycle, is called absent end diastolic velocity also revered as a AEDD or the umbilical vein shows bumpy positive flow or the ductus venosus which is a vessel that runs through the liver of the fetus those either no flow or backwards flow in a segment of the cardiac cycle we’re supposed to always show polar flow.
Either twin can have any of these abnormal Doppler studies so you can have stage 3 donor for stage 3 recipient for stage 3 donor recipient.
In stage 4 the babies have gone into heart failure one or both that is shown on ultrasound with fluid that accumulates that extravasation different parts of the body the level of the scalp at the level of the chest at the level of the belly so there is fluid accumulation and the baby that means the baby went into heart failure
In stage 5 one or both babies have passed away.
Now the staging system has several features that are very important to note they may not be apparent when you first read the staging system but they have a purpose. First of all I call this stage not type. This suggests or implies that babies can have different degrees of severity meaning it’s the same condition but one one patient can be more severely affected than another or less affected than the other.
Because of that, the Quintero staging system also uses Roman numerals that also implies that the higher the Roman numeral the more severe the disease. The third aspect that is characteristic of the system is that all the parameters that we evaluate are either present or absent and that makes it very practical to use. In virtually every ultrasound lab in the world they have the Quintero staging system and it’s very easy to follow because either the bladder is visible or not visible or the Doppler are abnormal or they’re not abnormal, or there’s hydrops or there’s no hydrops. This is very easy and very practical.
The importance of the staging system is not only that they didn’t establish a common language among physicians but also that it helps us evaluate the prognosis when either nothing is done or when symptomatic treatment is offered. Symptomatic treatment is when you simply take off fluid from the sac of the recipient. The more advanced the Quintero state stage was for that particular patient the worse the prognosis you know for that patient. It always works. This is the origin and the importance of the Quintero staging system.
Fetal surgery draws principally from the fields of surgery, obstetrics and gynecology, and pediatrics– especially the subspecialties of neonatology (care of newborns, especially high-risk ones), maternal-fetal medicine (care of high-risk pregnancies), and pediatric surgery.
It often involves training in obstetrics, pediatrics, and mastery of both invasive and non-invasive surgery, meaning it takes several years of residency, and at least one fellowship (usually more than one year), to be able to become proficient. It is possible in the U.S. to become trained in this approach whether one started in obstetrics, pediatrics, or surgery.
Because of the very high risk and high complexity of these cases, they are usually performed at Level I trauma centers in large cities at academic medical centers,- offering the full spectrum of maternal and newborn care, including a high level neonatal intensive care unit (level IV is the highest) and suitable operating theaters and equipment, and a high number of surgeons and physicians, nurse specialists, therapists, and a social work and counseling team. The cases can be referred from multiple levels of hospitals from many miles, sometimes across state and provincial lines. In continents other than North America and Europe, these centers are not as numerous, though the techniques are spreading.
Most problems do not require or are not treatable through fetal intervention. The exceptions are anatomical problems for which correction in utero is feasible and may be of significant benefit in the future development and survival of the fetus. Early correction (prior to birth) of these problems will likely increase the odds of a healthy and relatively normal baby.
The Fetal Institute provides patients with the latest and most advanced resources in the field of Maternal-Fetal Medicine and Fetal Therapy. This includes high-resolution ultrasound imaging, non-invasive serum screening and prenatal testing (NIPT), as well as comprehensive invasive prenatal diagnostic and therapeutic techniques. Fundamental to the philosophy of the Institute, all diagnostic and therapeutic options are performed using either a non-invasive or a minimally invasive approach, which results in better pregnancy outcomes.