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“It will be a referral centre for pregnant women whose fœtuses are at risk of congenital malformations, whether because of family history, an abnormal ultrasound or exposure to toxic substances,” explains Dr. Valérie Désilets, medical co-director of the new McGill fœtal Diagnosis and Treatment Centre along with Drs. Lucie Morin and Hélène Flageole. (Dr. Désilets and Dr. Morin are from the Department of Obstetrics & Gynecology while Dr. Flageole is from the Department of Pediatric Surgical Services, MUHC.) “The team at the Centre, which includes a number of highly-trained specialists, has set up a complete consultation and treatment service for parents and physicians who need information on diagnosis and prognosis, as well as on possible treatment during pregnancy or after birth.” Parents will have access to a team that includes, more than 30 specialists from three hospitals joined in a single unit: obstetricians, neonatalogists, geneticists, ultra-sound specialists, cardiologists, radiologists, pediatric surgeons, neurosurgeons, radiologists, and other subspecialists from the Royal Victoria, the Montreal Children’s and the Jewish General hospitals. Women referred by their treating physicians first consult clinical geneticists to learn about and understand the congenital anomalies that can affect the fœtus. They are then promptly referred to the appropriate specialists. “The support the Centre offers is crucial to parents and parents-to-be who need answers quickly when they first hear the bad news,” says Lola Cartier, Coordinator of the Centre and genetic counsellor. “What kind of malformations does the fœtus have? Why did it happen? What action can be taken during and after the pregnancy? What kind of quality of life will my child have? Those are the types of questions parents need to ask so they can make an informed decision about whether to continue a high-risk pregnancy and about possible options.” An example of an appropriate informed choice was the one made by a pregnant woman who was carrying babies with the twin-to-twin transfusion syndrome. To help her carry her pregnancy to term, an amnioreduction was performed by doctors. This involved repeated removal of a large amount of amniotic fluid several times during the pregnancy. When her twins were born, despite their prematurity and low birth weight, they were in good health. Another case shows the importance of counselling and appropriate follow-up in the parents’ decision on whether to carry a pregnancy through to term or not. After the ultrasound detected a lung malformation, medical specialists were able to assure the parents that, although their baby would require surgery after birth, there was no significant sign of long-term danger to his health. They therefore decided to continue with the pregnancy, which resulted in a healthy baby boy. Specialists working at the Centre see a growing number of parents annually who need a multi-disciplinary expertise in genetics, obstetrics and pediatric subspecialties. These parents are referred by treating physicians from throughout Quebec. It was to consolidate this expertise and offer better-coordinated support that several services have been brought together into a single unit – services such as genetic counselling, risk evaluation, as well as treatment before, during and after birth. The Fœtal Diagnosis and Treatment Centre offers its services at two MUHC hospitals (the Montreal Children’s and the Royal Victoria) and at the Jewish General Hospital. |
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Article from Infirmières du Québec magazine Click here to download. You will require Adobe Acrobat Reader to view |
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EXIT Equals Life for Baby LioraThe MCH and RVH join forces in creating a success story
The McGill University Health Centre (MUHC) unveiled its latest pediatric success story to the media on April 5 and 6 when they announced they had successfully performed the EXIT Technique (ex-utero Intrapartum Treatment: a specialized method of Cesarean delivery), a surgical first in Quebec. Their reward - giving life and health to a beautiful baby girl. At the heart of this unique procedure was Montreal Children's Hospital (MCH) surgeon, Dr. Jean-Martin Laberge, and Royal Victoria Hospital (RVH) obstetrician, Dr. Samir Khalifé, who teamed up with an impressive array of specialists and nurses from the RVH and MCH (see "List of Team Members Involved in Liora's Birth", below). In total, 42 team members participated in the complex and potentially risky childbirth. The story began at the end of last summer when Esther Castiel, who was 18 weeks pregnant at the time, went to see Khalifé. She learned then that her fœtus had a growth on its neck, which was larger than its head. After a series of ultrasounds, Khalifé diagnosed a teratoma¬a tumoral growth that can be benign or malignant and is found in one in 25,000 births. In newborns, a teratoma can reach enormous proportions, and may even weigh as much as the baby. It puts the health of the baby in jeopardy because of its tendency to block adjacent structures, to rupture, to bleed, and more. It is important to remove the growth as soon as the baby is born, or shortly after. Castiel and her husband immediately met with the members of the Fœtal Diagnostic and Treatment Team of the MUHC, including Lola Cartier, Genetics Counselor and Coordinator of the team. Ultimately, because of their religious beliefs and the encouraging advice they received, the parents decided not to terminate the pregnancy. During the weeks that followed, the teratoma grew at a faster rate than the baby, who they named Liora, preventing her from swallowing normally. This resulted in an excessive accumulation of amniotic fluid, which could have led to premature labor. Despite repeated aspirations of this excessive fluid and of the cysts that developed in the teratoma, there was still no guarantee of a risk-free childbirth. The care team was forced to rethink their childbirth strategy. It had to be a technique that would prevent the teratoma, almost as large as the baby itself, from choking the infant during birth. This is when specialists opted for the EXIT Technique. At that time, a dozen specialists in obstetrics, pediatric surgery, anesthesia, neonatology, pediatric cardiology and medical imaging from the MCH and RVH were called in, as were many specialized nurses that played an important role in the coordination of clinical activities and operating-room logistics. The coordination aspects were the responsibility of nurses Julie Goudreau and Francine Asswad, from the Birthing Centre of the RVH, and nurses Martine Lestage and Linda Sand, from the operating room of the MCH, where the procedure took place. The EXIT Technique involved Liora's mother being deeply anesthetized to enable a complete uterine relaxation and to maintain blood flow between the placenta and the fœtus while the airway was secured. A Cesarean section was then made to expose the whole uterus, during which the placenta was protected in order to prevent it from being damaged. Next, a little opening was created in the uterus to facilitate the insertion of a uterine stapler and the surgeon's finger. Following a pre-established line, a larger opening was then made in the uterus to make it possible for the head, neck, upper torso and arms of the fœtus to be pulled out, all the while preventing any trauma to the teratoma, which could result in a rupture or a hemorrhage. The lower body, including the umbilical cord, remained inside the uterus. A continual perfusion of a warm saline solution kept the fœtus warm inside the uterus. Even though the fœtus was anesthetized through the placenta, other anesthetic agents were injected in the arm of the child in order to proceed with the key element of the EXIT Technique. This key element involved intubating Liora while she was still benefiting from the oxygen supplied by the umbilical cord. Guided by a flexible bronchoscope, the breathing tube was passed by the mass, and once secured, a clamp was applied to the cord, and then Liora was born. It took a total of 39 minutes between the time Liora's head came out of the uterus to the time the cord was clamped. Three hours after the birth, and once the baby was stabilized by the neonatal team, Dr. Louis Beaumier, MCH surgeon, and Laberge, started the procedure of removing the teratoma. This took almost four hours. Because the growth was in the neck region it did end up affecting the digestive tract and larynx of Liora, who was transferred to the Neonatal Intensive Care Unit of the MCH after the surgery. She remained there for three months. Castiel stayed under the care of the obstetrical nurses from the RVH for one night at the Children's. She was then transferred to the RVH for postpartum care. During the April presentation to the media Castiel and her husband said the following to the press: "God has sent us an extremely difficult challenge, but He has also put us in the hands of the best doctors and nurses. December 21 of the Year 2000, which was the first day of Hanukkah (the Festival of Lights), brought us another miracle, the birth of a little girl that never would have been able to live without the cooperation and the extraordinary commitment of dozens of doctors and nurses¬without a doubt the best and the most dedicated in their field. There aren't enough words to thank them." After successfully completing this surgical technique, members of the team are more than ready to face the challenges of other fœtal malformations needing surgical procedures before birth. They all agree, though, that preparing for this type of procedure will be much easier once the MUHC partners are all at one site. Dr. Jean-Martin Laberge was named Personnalité de la semaine (This Week's Personality) by the La Presse daily newspaper April 15, 2001. He willingly shares this honour with all his team members and Liora's parents.
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Vendredi 6 avril 2001
 Une naissance peu banale MATHIEU PERREAULT La Presse
Le cordon ombilical de Liora Benhamou n'était pas encore coupé qu'elle avait déjà subi sa première chirurgie. Quand elle est née à l'Hôpital de Montréal pour enfants, le 21 décembre dernier, Liora avait une tumeur de 1,4 kilo au cou. Une équipe de 42 personnes, dont 13 médecins, ont sorti le haut de son corps du ventre de sa mère pour installer un tube dans la trachée du bébé. Puis l'obstétricien l'a sortie complètement, a coupé le cordon ombilical, avant de laisser la place aux chirurgiens qui ont enlevé la tumeur. Sans le tube dans sa trachée, qu'il a fallu 40 minutes pour installer, Liora serait morte étouffée en quelques minutes, selon le Dr Jean-Marie Laberge, chef de la chirurgie pédiatrique. Cette première application québécoise d'une technique utilisée depuis huit ans aux États-Unis, qui permet d'opérer un bébé encore relié au cordon ombilical, est la dernière étape avant la chirurgie fœtale, a expliqué le Dr Laberge, hier en marge d'une conférence de presse à l'hôpital de la rue Tupper. Aux États-Unis, surtout à Philadelphie, des chirurgiens opèrent depuis huit ans des fœtus affligés de diverses malformations, puis les remettent dans le ventre de leur mère pour que celle-ci mène la grossesse à terme. Les parents de la petite Liora, qui ne devrait garder qu'une voix rauque comme séquelle, ont souffert le martyre de juillet à décembre. Au départ, la grossesse d'Esther Castiel s'annonçait aussi bien que ses trois précédents. Mais une échographie à l'hôpital Royal Victoria a montré une anomalie. Le verdict a été brutal: c'était un «bébé de Turner», qui mourrait à la naissance, se rappelle le père, Jean-Noël Benhamou, avec un brin d'amertume. «Sur le coup, j'ai pleuré, pour la première fois en six mois, se souvient l'étudiant en droit de 29 ans (sa femme en a 31). Puis je me suis rendu compte que c'était ma femme qui avait du trouble, et qu'il fallait que je sois fort.» Étant juif pratiquant, le couple montréalais a d'emblée écarté l'avortement et demandé une contre-expertise à l'Hôpital de Montréal pour enfants. Après quelques visites, le diagnostic a changé: il s'agissait en fait d'un tératome, une tumeur bénigne formée de tissus aussi divers que des cellules cérébrales, intestinales et osseuses. Au fil de la grossesse, du liquide tumoral a été drainé pour ralentir la croissance de la tumeur, l'une des plus grosses jamais enlevée avec cette technique, selon le Dr Laberge. À partir de 29 semaines, l'équipe a été sur le qui-vive, au cas où le cœur du bébé faiblirait face à la demande sanguine importante de la tumeur. L'opération a été effectuée peu après la 32e semaine, quand la prématurité a cessé de poser un problème. «La tumeur était tellement grosse qu'il fallait la tenir pour ne pas qu'elle comprime les poumons du bébé et l'empêche de respirer», commente le Dr Laberge. Selon le Dr Laberge, il n'aurait pas été possible de remettre Liora dans le ventre de sa mère et de poursuivre la grossesse, parce que l'ablation a duré quatre heures, près de trois fois plus que la durée maximale d'une chirurgie fœtale. Une anesthésie spéciale a empêché l'utérus de commencer ses contractions sous le choc opératoire. Liora, qui portait hier une charmante robe verte et une veste lavande, n'avait pour seule trace de son opération et de ses trois mois d'hospitalisation qu'une joue droite plus basse que la gauche. Certains nerfs du côté gauche ont été détruits ou endommagés par la tumeur ou l'opération. «Mais nous avons bonne confiance qu'elle s'en remettra et n'aura pas le sourire de Jean Chrétien», a blagué le Dr Laberge. Elle doit être nourrie par un tube introduit dans son estomac, parce qu'elle est incapable de prendre beaucoup de lait à la fois, mais les médecins croient qu'elle s'alimentera normalement d'ici quelque temps. Quant à ses parents, ils sont prêts à avoir d'autres enfants si c'est possible. Copyright © 2000-2001 Cyberpresse Inc., une filiale de Gesca. Tous droits réservés. |
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A First for Quebec: MUHC Team employs new procedure to deliver fœtus at risk Montreal, April 5, 2001 – “It was teamwork that ensured the successful delivery of this very special baby,” explained Dr. Jean-Martin Laberge, Director of General Surgery at the Montreal Children’s Hospital of the McGill University Health Centre. At a press conference today, Dr. Laberge and a team of MUHC doctors and other health care professionals briefed media on the first Quebec use of the EXIT procedure to deliver Baby Liora at the MCH on December 21, 2000. Preparation for this event began in late summer when Royal Victoria-based MUHC obstetrician Dr. Samir Khalife was consulted by the mother who was in her 18th week of pregnancy and had just learned that her fœtus had a large neck mass (larger than the size of the fœtus’s head), which was subsequently determined to be a teratoma. Following meetings with the obstetrician, pediatric surgeons and the genetic counsellor Lola Cartier, the parents decided that they wanted to maintain the pregnancy. The challenge for their health care team was to identify a way of delivering the baby that would prevent the teratoma, which was well over half the size of the fœtus, from suffocating the baby during birth. The EXIT Procedure The team headed by Dr. Laberge decided on a process known as the EXIT procedure. A modified cesarean section was performed by Dr. Khalife and the upper body of the fœtus was extracted from the womb while the lower body remained in utero and the fœtus retained its link to the placenta. The uterus was kept relaxed using special anesthetic techniques. Supporting the mass, the airways were assured by inserting a breathing tube and adjusting its position. Then the cord was clamped and Baby Liora was born. Altogether 39 minutes elapsed between the emergence of the head and the birth. The key to this procedure was that the baby was intubated to assure the airways while she was still attached to the maternal blood supply. Neonatology specialists, who were part of the team from the time the planning started, were in the operating room to stabilize the baby after delivery. Three hours after the birth the surgery to remove the teratoma began. The mass had affected the neck area where it was attached to the fœtus and Baby Liora had hurdles to overcome. These include problems with her gastrointestinal system and larynx. Following the surgery, Liora was admitted to the MCH’s neonatal intensive care nursery where she stayed for the next three months. She was recently released and there is no doubt that she is a lovely child with a bright future. Nurses Played a Key Role “I think that we should emphasize the important role played by the nursing personnel in this situation,” said Dr. Khalife. “Not only are they pivotal people in the ongoing care of the baby, but their contribution was essential to the logistics side of this complicated case.” “The biggest challenge was coordinating clinical activities on two different sites. The success of the procedure was due to the extraordinary team work prior to, during and after the big day. Both teams, nursing and medical, were focussing on the optimal care for both mother and baby”, said Julie Goudreau, assistant head-nurse in Obstetrics and Perinatal Unit at the Royal Victoria Hospital. “We now feel ready more than ever to handle other fœtal malformations that may require an operation before birth,” noted Dr. Laberge. In addition to Dr. Laberge and Dr. Khalife, the team included a dozen of specialists in obstetrics, pediatric surgery, anesthesia, neonatology, cardiolgy, as well as many specialized nurses and a genetic counsellor. A number of these individuals are members of the McGill Fœtal Diagnosis and Treatment Group, a team of 30 specialists who provide a complete consultation and treatment service for parents and physicians who need information on diagnosis or prognosis as well as possible treatment during and after birth. “We spent several weeks planning the most intricate details of the procedure, including the position of the equipment and the 28 persons who would be in the OR at one time or another,” said the members of the team. “Logistics and planning will certainly be facilitated when the MUHC is on one site.” As for Liora’s parents, they are delighted with their baby daughter and thankful to everyone who was involved in her care. “ God gave us something very difficult to deal with, but He also placed us in the hands of the best doctors and nurses. December 21, 2000 the first day of Hanukkah (the Festival of Lights) was marked by a miracle, the birth of a little girl who would never have seen the light of day without the cooperation and the extraordinary commitment of several doctors and nurses, who are without a doubt the best and the most devoted in their profession. No words can adequately express our thanks to them.” |
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