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A 20 year study shows that selective dorsal rhizotomy benefits patients long-term

MONTREAL, June 11, 2013 /CNW Telbec/ - Selective dorsal rhizotomy (SDR) is a surgical procedure used to treat spasticity in some children with spastic cerebral palsy. This procedure appears to be effective, but for how long? Researchers from Montreal, Quebec, Canada,* set out to find the answer to this question by studying data from the McGill University Rhizotomy Database on 102 pediatric patients with long-term follow-up. The researchers found that, in the majority of children with spastic cerebral palsy, the benefits of SDR last throughout adolescence and into early adulthood. Details on the study findings are reported and discussed in "Long-term functional benefits of selective dorsal rhizotomy for spastic cerebral palsy. Clinical article," by Dr. Roy W. R. Dudley and colleagues, published today online, ahead of print, in the Journal of Neurosurgery: Pediatrics (http://thejns.org/doi/full/10.3171/2013.4.PEDS12539).

Cerebral palsy (CP) is a term used to describe various disorders of movement, muscle tone, or posture. Caused by abnormal brain development or an injury that affects the part of the brain that controls motor activities, CP first appears before, during, or soon after birth, and can affect a variety of muscles. Spastic cerebral palsy represents the type of CP that manifests increased muscle tension or tone; this increased tone makes the affected muscles very stiff and muscle movements appear spastic. Studies have shown that gross motor development in young people with spastic CP can plateau during childhood and may decline during adolescence. It is therefore essential to improve motor function early in these children and maintain any improvements in muscle function over time so that these children may become productive young adults.

Selective dorsal rhizotomy (SDR) is often performed in children with spastic CP to reduce muscle spasticity. During surgery, nerve fibers carrying sensory impulses from muscles to the dorsal side (the back) of the spinal cord are stimulated electronically at their rootlets to identify which ones are sending messages that induce an exaggerated tone and reflex state called spasticity. Selectively, the fibers that cause spasticity are then cut, leaving fibers that carry normal impulses alone. This is designed to diminish the spasticity of the muscle involved without injuring muscle function. The procedure has proved successful in selected patients, but before the present study there were no published findings on long-term outcomes of SDR based on validated functional assessments and patient stratification.

An examination of the McGill University Rhizotomy Database led researchers to identify 102 children with spastic CP who underwent SDR between the ages of 3 and 10 years and were evaluated by a multidisciplinary team before surgery and periodically afterward. Preoperative data were available for all patients, and postoperative data were available for 97 patients at 1 year, 62 patients at 5 years, 57 patients at 10 years, and 14 patients at 15 years after surgery. Evaluations were made using ratings of the children's activities of daily living, the Ashworth Scale for lower-limb tone, the GMFCS (Gross Motor Function Classification System), the GMFM (Gross Motor Function Measure), and GBTM (group-based trajectory modeling), among other assessment tools.

In evaluating data following selective dorsal rhizotomy (SDR) in these children, the researchers found statistically significant improvements in lower-limb muscle tone, gross motor function, and the ability to perform activities of daily living in the majority of patients. These improvements were durable, lasting throughout adolescence and into early adulthood. The researchers also found that long-lasting improvements in motor function were more likely to be found in higher functioning patient groups, specifically: 1) children with better preoperative gross motor function, such as those who had been able to walk on their own or with assistive mobility devices (GMFCS Groups I through III); 2) children whose spasticity involved only two limbs, rather than three or four; and 3) children whose spasticity involved the adductor muscles of the hip only moderately (Ashworth score lower than 3). The researchers also found that following successful SDR, these children were less likely to need adjunct orthopedic surgeries or Botox injections to control spasticity.

When asked about the take-home message of the study, the senior author, Dr. Jean-Pierre Farmer, stated, "This study has allowed us to confirm that the change in gait pattern induced by the operation is long lasting (likely permanent) and 'protects' children from the natural history of their disease. Whereas the study shows that there are gains in activities of daily living in all patients, it also allows us to establish ambulatory goals that are stratified by the preoperative functional level of the child through predictive indices. The ability to predict the long-term effects of the SDR operation will be extremely valuable for treating teams and families at the time of therapeutic decision-making."

*This paper is the result of a joint program between Montreal Children's Hospital and Shriners Hospitals for Children® — Canada in Montreal. The authors of this report are from Montreal Children's Hospital, Royal Victoria Hospital, Shriners Hospitals for Children® — Canada, McGill University, and Université de Montreal in Montreal, Canada.

Dudley RWR, Parolin M, Gagnon B, Saluja R, Yap R, Montpetit K, Ruck J, Poulin C, Cantin MA, Benaroch, TE, Farmer JP. Long-term functional benefits of selective dorsal rhizotomy for spastic cerebral palsy. Clinical article. Journal of Neurosurgery: Pediatrics, published online, ahead of print, May 28, 2013; DOI: 10.3171/2013.4.PEDS12539.

Disclosure: The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Our Patients
Background Information

Sara: 4 years old from Montreal
Sara was born at term following a normal pregnancy. However, due to complications at birth Sara had to fight for survival. On her fourth day of life, she had seizures and it is at this time that her parents realized that they were facing a new reality, very different one from what they had imagined. "Until the age of two her parents would tell themselves, she is still just a baby, she will grow out of it, she will be able to catch up", recalls Steve Tanguay, Sara's father. He went on to say: "However, between the ages of two and three, the reality of what we were facing hit us hard, our little one did not walk, her capacity was limited. She had cerebral palsy with dysphasia. We had little hope and even fewer answers. We had to mourn the child that we had hoped for ".

One evening, Mr. Tanguay saw a news report on television which featured Dr. Jean-Pierre Farmer who spoke about the type of neurosurgery he practiced at The Montreal Children's Hospital. "I cried for my child because it was the first time I saw the potential of what was possible for her. It was a revelation" recalls Mr. Tanguay.

At a follow up appointment with neurologist Dr. Chantal Poulin, the family got more information about the selective rhizotomy surgery. Dr. Poulin then referred the family to Dr. Farmer.

During the first assessment, Dr. Farmer asked that the family return later as Sara was not yet old enough to undergo surgery. During the second evaluation, Dr. Farmer confirmed that the operation would be in a few months. A few weeks later, the family received a call telling them that the surgery could be advanced if they wished, they did not hesitate.

"If we had the chance to return to Shriners Hospital for children for more intensive therapy, we would. We felt good and supported when we were there. It was not painful experience, it was actually fun ", says Mr. Tanguay.

Sara is a girl who has beautiful strengths: she is a tiny ball of love. She is magnetic and has a lot of determination. She stretches and walks every day and actively participates in her treatment. She loves cartoons on television, loves stories books and she like to invent stories.

"The first time I walked hand in hand with my daughter, I felt so happy because a few months earlier it was something that simply seemed impossible", concluded Mr. Tanguay.

Ali Ammar from Montreal
Ali Ammar was born in Lebanon in 1989, half of his body paralyzed. His doctors believed he would never walk and would be confined to a wheelchair for his entire life. In the early 1990s, when he was only 18 months old, his family fled the civil war in Lebanon to settle in Canada.  Ali was referred to Shriners Hospital for Children®- Canada as a toddler for spastic dysplasia, a type of cerebral palsy. He was then evaluated by Jean-Pierre Farmer, M.D., neurosurgeon, and it was determined that Ali was an ideal candidate for a specialized type of neurosurgery (a new technique at the time), a selective dorsal rhizotomy. The surgery took place in 1992 at The Montreal Children's Hospital of the McGill University Health Centre and was followed with six weeks of intense rehabilitation at Shriners Hospitals for Children®- Canada, where Ali took his first steps at three years old.

In 2004, in order to improve his gait, Ali had additional surgery on his ankles and Marie-Andrée Cantin, M.D., orthopaedic surgeon at CHU Sainte-Justine and accredited surgeon at Shriners Hospitals for Children, completed two surgeries on his right foot.  The surgeries, rehabilitation and Ali's efforts proved to be extremely successful and now Ali walks completely unaided.

Today, Ali heading to University. He has also overcome the real-life obstacles of spastic dysplasia and has even played the lead role in Romeo Eleven, an award-winning film by Montreal director Ivan Grbovic. The film offers a rare glimpse into the life of someone with a physical disability and the struggles portrays a shy teenager looking for love on the Internet. The film has been presented in twenty festivals around the world, has won six international awards and brought Ali in Paris and Lyon in France, Karlovy Vary in Czech Republic and Toronto in Canada.  Ali`s motto: "When you really want something, you can succeed."

Jayden, 3 ½ from Chicoutimi
Jayden was born premature at 30 weeks. When he turned one, he began daycare where some of his developmental delays became apparent. He was unable to stand or even sit by himself.  This worried his parents Marie-Michèle and Alex, and they were not sure what the future held for their baby.

Shortly after his entry in daycare, Jayden began physiotherapy at the local rehabilitation center in their community. This is when his therapist informed the parents about a hospital in Montreal that could potentially perform surgery that could help their son. During the time, they also met a family whose son had undergone the surgery close to 20 years ago, with positive results.

The day of February 5, 2013 was marked with excitement and anxiety for the family. The family went to Shriners Hospital for Children - Canada so Jayden could be evaluated and a decision would be rendered if he would benefit from the surgery. The evaluations lasted all day and in the end, it was confirmed that Jayden was in fact an ideal candidate for a selective dorsal rhizotomy.

Jayden had surgery on June 5, 2013 at The Montreal Children's Hospital and was transferred on June 10, 2013 to Shriners Hospitals for Children-Canada for post-operative rehabilitation which lasts 6 weeks.

Jayden's family has created a Facebook page for people to check on his progress under: Jayden, le petit combatant or www.facebook.com/JaydenParalysieCerebrale. (French only).

Video with caption: "Video: Jayden". Video available at: http://stream1.newswire.ca/cgi-bin/playback.cgi?file=20130611_C7010_VIDEO_EN_27843.mp4&posterurl=http://photos.newswire.ca/images/20130611_C7010_PHOTO_EN_27843.jpg&clientName=H%26%23244%3Bpital%20Shriners%20pour%20enfants%20%28Canada%29&caption=Video%3A%20Jayden&title=H%26%23212%3BPITAL%20SHRINERS%20POUR%20ENFANTS%20%28CANADA%29%20%2D%20Groundbreaking%20techniques%20brought%20forth%20in%20the%201990s%20change%20the%20lives%20of%20children%20and%20allow%20them%20to%20grow%20to%20be%20autonomous%20adults&headline=Groundbreaking%20techniques%20brought%20forth%20in%20the%201990s%20change%20the%20lives%20of%20children%20and%20allow%20them%20to%20grow%20to%20be%20autonomous%20adults

Video with caption: "Video: Sara". Video available at: http://stream1.newswire.ca/cgi-bin/playback.cgi?file=20130611_C7010_VIDEO_EN_27844.mp4&posterurl=http://photos.newswire.ca/images/20130611_C7010_PHOTO_EN_27844.jpg&clientName=H%26%23244%3Bpital%20Shriners%20pour%20enfants%20%28Canada%29&caption=Video%3A%20Sara&title=H%26%23212%3BPITAL%20SHRINERS%20POUR%20ENFANTS%20%28CANADA%29%20%2D%20Groundbreaking%20techniques%20brought%20forth%20in%20the%201990s%20change%20the%20lives%20of%20children%20and%20allow%20them%20to%20grow%20to%20be%20autonomous%20adults&headline=Groundbreaking%20techniques%20brought%20forth%20in%20the%201990s%20change%20the%20lives%20of%20children%20and%20allow%20them%20to%20grow%20to%20be%20autonomous%20adults

Video with caption: "Video: Ali Ammar". Video available at: http://stream1.newswire.ca/cgi-bin/playback.cgi?file=20130611_C7010_VIDEO_EN_27845.mp4&posterurl=http://photos.newswire.ca/images/20130611_C7010_PHOTO_EN_27845.jpg&clientName=H%26%23244%3Bpital%20Shriners%20pour%20enfants%20%28Canada%29&caption=Video%3A%20Ali%20Ammar&title=H%26%23212%3BPITAL%20SHRINERS%20POUR%20ENFANTS%20%28CANADA%29%20%2D%20Groundbreaking%20techniques%20brought%20forth%20in%20the%201990s%20change%20the%20lives%20of%20children%20and%20allow%20them%20to%20grow%20to%20be%20autonomous%20adults&headline=Groundbreaking%20techniques%20brought%20forth%20in%20the%201990s%20change%20the%20lives%20of%20children%20and%20allow%20them%20to%20grow%20to%20be%20autonomous%20adults

SOURCE: Hôpital Shriners pour enfants (Canada)

For further information:

Stephanie Tsirgiotis
Communications Officer
Public Relations and Communications
The Montreal Children's Hospital
514-412-4307

Emmanuelle Rondeau
Manager
Public Relations
Shriners Hospitals for Children-Canada
514-207-1057 erondeau@shrinenet.org