Canadian First: SickKids and Sinai Health System repair spina bifida in-utero

In a Canadian first, a team from Mount Sinai Hospital and The Hospital for Sick Children (SickKids) has repaired a form of spina bifida in a fetus at 25 weeks gestation. The baby’s mother, Romeila Son, recovered very well from surgery, and a bouncing baby girl, Eiko, weighing 2.45 kg, was born on August 19 at 36 weeks gestation at Mount Sinai Hospital. Since her birth, Eiko has needed no further intervention for this usually debilitating condition. This is the first case in Canada where the mother has not had to travel to the United States for this specialized surgery.

Dr. Greg Ryan heads Canada’s largest and most experienced fetal therapy program at Mount Sinai Hospital and Dr. James Drake heads Canada’s largest and most established neurosurgical centre for the postnatal repair of spina bifida at SickKids. Working in collaboration with colleagues from Vanderbilt University in Nashville who pioneered the procedure, a team of 24 clinicians led by Drs. Ryan and Drake successfully carried out the 2.5 hour in-utero procedure at Mount Sinai on June 4, 2017.


From left: Dr. Michael Apkon, President & Chief Executive Officer, SickKids; Dr. Paige Church, Head of spina bifida clinic at Holland Bloorview Kids Rehabilitation Hospital; Dr. James Drake, Head of Neurosurgery, SickKids;  Premier of Ontario, Kathleen Wynne; Dr. Greg Ryan, Head of Fetal Medicine program, Mount Sinai Hospital; Romeo Crisostomo, Eiko’s dad; Romeila Son, Eiko’s mom; baby Eiko Crisostomo; Dr. Mathew Sermer, Obstetrician and Gynaecologist-in-Chief, Sinai Health System; Dr. Gary Newton, President and Chief Executive Officer, Sinai Health System.

What is spina bifida?

Myelomeningocele is a form of spina bifida, affecting approximately 120-150 babies in Canada each year. It is caused when the spinal column fails to close early in fetal development, causing permanent damage to the baby’s spinal cord and nervous system.

Babies with spina bifida can have varying degrees of paralysis of their lower limbs and many will need mobility supports such as leg braces, crutches or wheelchairs. Over 80 per cent of children with spina bifida will require a shunt to be inserted to relieve pressure on their brain, which must remain in place for their entire life, and some affected children will have a negative neurocognitive outcome. Between 15-30 per cent of children with spina bifida do not survive into adulthood and less than 50 per cent live independently as adults. A third of adults with spina bifida require substantial lifelong daily support. The emotional, social and financial impact for these affected individuals, their families and society as a whole, is enormous.

Better treatment option for babies

The Management of Myelomeningocele Study (MOMS), a groundbreaking trial published by Vanderbilt University, Children’s Hospital of Philadelphia and the University of California, San Francisco, in the New England Journal of Medicine in 2011, showed that, in babies who underwent the in-utero procedure:

  • Brain malformations were reversed by one-third
  • The need for walking aids or a wheelchair was halved
  • Their need for brain shunts was reduced by half

Consequently, the Society of Obstetrics and Gynaecologists of Canada (SOGC) now mandates that any pregnant woman whose fetus has spina bifida must be counselled about this treatment option.

“Although fetal surgery will not be appropriate for all fetuses with spina bifida, it is extremely encouraging that, for some, it may preserve neuromotor function, reverse brain herniation and reduce their need for a brain shunt,” said Dr. Greg Ryan, head of the fetal medicine program at Mount Sinai Hospital, part of the Frances Bloomberg Centre for Women’s and Infants’ Health. “However, it also entails some maternal and fetal risks, particularly that of premature labour, which must be carefully balanced.”

“We regularly see children who have been affected by spina bifida at SickKids,” said Dr. James Drake, head of the division of neurosurgery and senior associate scientist at SickKids. “It is my hope that our capacity to perform this in-utero surgery here in Toronto will mean that we can optimize their clinical outcomes, and reduce the degree of medical challenges these children will face.”

Collaboration counts

“I am extremely proud of the collaboration between Mount Sinai Hospital and SickKids – which has resulted in a terrific outcome for this baby girl,” said Dr. Ryan.”Having this kind of clinical capacity here in Ontario will really change the range of options available to parents who have been given a diagnosis of spina bifida during pregnancy.”

“I would like to congratulate everyone at Mount Sinai Hospital and SickKids who teamed up to perform Canada’s first in-utero spina bifida surgery,” said the Honourable Kathleen Wynne, Premier of Ontario. “The incredible story of baby Eiko’s journey into this world shines a bright light on our world-class health care professionals. They’re talented, innovative, and making life better for people in this province — starting even before they’re born. Congratulations to little Eiko and her family. I wish them a healthy, happy future.”

Mount Sinai Hospital and SickKids recently collaborated on another in-utero surgical first, repairing a congenital heart defect called transposition of the great arteries, in May, 2017. The team from SickKids and Mount Sinai Hospital is Canada’s only provider of fetal surgery.

Read the original press release of this Canadian First here.

*Feature photo: From left: Premier of Ontario, Kathleen Wynne; Dr. James Drake, Head of Neurosurgery, SickKids; Romeo Crisostomo, Eiko’s dad; baby Eiko Crisostomo; Romeila Son, Eiko’s mom; ; Dr. Greg Ryan, Head of Fetal Medicine program, Mount Sinai Hospital.

Related Stories

SickKids and Sinai Health System are two of Ontario’s 23 research hospitals that contribute to a healthier, wealthier, smarter province. Look for other RESEARCH SPOTLIGHT posts on our Healthier, Wealthier, Smarter blog or join the conversation about why health research matters for Ontario on Twitter, using the hashtag #onHWS.

Research Spotlight: Sinai Health System

Short-term insulin therapy successfully induces remission in Type 2 diabetes

A new study lead by researchers at Mount Sinai Hospital, part of Sinai Health System, has shown that early intervention with short-term intensive insulin therapy for four weeks can successfully induce a remission of Type 2 diabetes that lasts for up to one year thereafter.

The study was led by Dr. Ravi Retnakaran, an endocrinologist with Leadership Sinai Centre for Diabetes at Mount Sinai Hospital, and has just been published in BMJ Open Diabetes Research and Care. The findings of Dr. Retnakaran and his team show that the earlier that short-term intensive insulin therapy was administered after the diagnosis of diabetes, the more successful it was in sustaining a 48 week remission.

Dr. Ravi Retnakaran, Mount Sinai Hospital
Dr. Ravi Retnakaran, endocrinologist with Leadership Sinai Centre for Diabetes at Mount Sinai Hospital

Short-term intensive insulin therapy is typically administered for a period of two to four weeks and can decrease insulin resistance, reduce glucagonemia, improve pancreatic beta-cell function and induce a remission that can last up to one year in just under 50% of patients. The study showed that those who had been diagnosed within the preceding two years and then undergone the treatment had the longest sustained remission. Patients who had sustained remission had better baseline beta-cell function that was preserved across the one year after stopping the treatment.

On the road to longer term remission

“This study points us in a very important direction in our quest to address one of the most prevalent chronic diseases today. The first point is the clear benefit of short-term intensive insulin therapy, and the second is the importance of early intervention within the first few years after diagnosis.   Our quest is to better understand the factors that determine remission so that we can offer long term solutions for our patients. The current study tells us that one key factor is early intervention with short-term insulin therapy during a window of opportunity that only exists in the first few years after diagnosis,” said Dr. Retnakaran.

Home to world-class diabetes researchers and research centres

Dr. Retnakaran, an endocrinologist with Leadership Sinai Centre for Diabetes at Mount Sinai Hospital and an investigator with the Lunenfeld-Tanenbaum Research Institute, collaborated with Drs. Bernard Zinman and Caroline K. Kramer, both of Leadership Sinai Centre for Diabetes at Mount Sinai Hospital and Lunenfeld-Tanenbaum Research Institute, in conducting a major clinical trial known as RESET-IT that aims to induce remission of Type 2 diabetes. The institute ranks amongst the top diabetes research centres in the world.

More than three million Canadians have Type 2 diabetes and the World Health Organization recently warned that rates have quadrupled since 1980, with 422 million people worldwide living with diabetes. The rate is expected to double in the next 20 years.

Patients interested in participating in the RESET IT trial should contact: Ms. Haysook Choi at Mount Sinai Hospital at (416) 586-8778 or haysook.choi@sinaihealthsystem.ca.

 

Sinai Health System is one of Ontario’s 24 research hospitals that contribute to a healthier, wealthier, smarter province. Look for other RESEARCH SPOTLIGHT posts on our Healthier, Wealthier, Smarter blog or join the conversation about why health research matters for Ontario on Twitter, using the hashtag #onHWS.