March is National Cerebral Palsy Awareness month and Congress just passed a resolution that March 25 is the official National Cerebral Palsy Awareness Day. When there is an injury to the white matter of the brain at birth or before birth, it can cause a comorbidity of both hydrocephalus and cerebral palsy. Cerebral palsy (CP) is a disorder of movement, muscle tone or posture that is caused by injury or abnormal development in the immature brain, most often before birth.” (mayoclinic.com)
During this month of raising awareness of CP, we would like to highlight a guest post written by Lori Poliski
that appeared on the Reaching for the Stars website. Lori is mother to William, who is living with both hydrocephalus and CP, and Claire, William’s older sister. She is also the Founder and current member of the Hydrocephalus Research Guild benefiting Seattle Children’s Hospital and the Children’s Hydrocephalus Support Group in the Pacific Northwest that meets at Seattle Children’s Hospital. Her husband and the children’s father, Paul Gross, is currently the Secretary of the Hydrocephalus Association Board of Directors, after recently completing his term as Chair. He also sits on the National Institutes of Health (NIH) National Institute of Neurological Disorders and Stroke (NINDS) advisory committee and started the Hydrocephalus Clinical Research Network (HCRN). Both Lori and Paul are outspoken advocates for hydrocephalus, raising awareness and funds to find a cure.
“Reaching for the Stars. A Foundation of Hope for Children with Cerebral Palsy” (RFTS, Inc.) is a nonprofit foundation led by parents, with a focus on the Prevention, Treatment and Cure of Cerebral Palsy.
This blog originally appeared on March 10, 2013 on the Reaching for the Stars website as part of their 30 Days of Inspiration, Information and Motivation series marking National Cerebral Palsy Awareness Month. Click here to visit the Reaching for the Stars website and to read the original post.
What is SPML Anyway (and try saying it 3X quickly)?
By Lori Poliski, mother of William
My husband, Paul Gross, and I are parents to our wonderful eight-year-old son William. He was born at 30 weeks and had a pulmonary hemorrhage at two-days old. William developed Hydrocephalus from a bilateral Grade III intraventricular hemorrhage (bleeding in the brain) as well as Spastic Diplegia Cerebral Palsy (SDCP). He spent one month in the NICU at Seattle Children’s Hospital, one month more on the medical floor and has had five brain surgeries – three of which were emergencies — due to hydrocephalus.
As William has grown, he has done a combination of traditional physical therapy, Yoga, Hippotherapy, Feldenkrais, Anat Baniel Method (ABM), worn Ankle Foot Orthotics (AFOs), or night splints plus he had a round of phenol injections for his tight adductors and hamstrings in 2010.
William could walk and run independently around the house and yard and used a walker for longer distances. He rides a bike with big training wheels, loves archery, he is learning to snowboard, and loves Star Wars, Origami, and Legos. He does well in school and has a lot of friends. William has a quirky sense of humor and is kind and loving. He is a typical eight-year-old boy who also happens to have some neurological issues. Hydrocephalus and CP don’t define William.
After a gait study last year, it was recommended that William have the following to help his contractures from getting worse: either a Selective Dorsal Rhizotomy (SDR) – a non-reversible spinal sensory nerve surgery to eliminate spasticity- at age 7, or a SDR first at age 7 or 8 followed by a Single Event Multilevel Surgery (SEMLS) at age 10, which can include traditional tendon lengthenings, tendon transfers and bone osteotomies, or just SEMLS alone at age 7. We wondered if there were other options to consider, and after much research and exploration, we opted to try Selective Percutaneous Myofascial Lengthening (SPML) for William before these much more major surgeries. We felt that SPML would be less invasive with less scarring than SEMLS, and SDR is a higher risk surgery with a longer recovery time because it is a spinal nerve surgery. How about those acronyms?
Selective Percutaneous Myofascial Lengthening, also known as PERCS for percutaneous, is a non-traditional, orthopedic tendon/muscle lengthening/release that is minimally invasive, with minimal scarring, for children and adults primarily with Spastic Diplegia Cerebral Palsy (SDCP).
There are two pediatric orthopedic surgeons in the U.S., to my knowledge, who are regularly doing SPML. They are Dr. Roy Nuzzo, in Summit, NJ, who adapted the procedure initially used for cardiac patients and has over 20 years of experience, and Dr. David A. Yngve in Galveston, TX, who trained with Dr. Nuzzo and has been performing SPML for over six years, having performed over 500 cases.
Dr. Matthew Dobbs at Washington University in St. Louis, MO, also does a PERCS tendon lengthening for patients who have had Selective Dorsal Rhizotomies (SDR) initially with T.S. Park at Washington University.
If you are reading this, you probably know what the medical definition of Spastic Cerebral Palsy is: the most common form of cerebral palsy marked by hypertonic muscles and stiff and jerky movements. One of the secondary problems that can result for a growing child with spastic cerebral palsy is contracture – a permanent shortening (as of muscle, tendon, or scar tissue) producing deformity or distortion which can cause pain, gait and mobility issues and bone misalignment of femurs and tibias, for example.
William had the SPML surgery on his hip adductors, hamstrings, gastrocnemius muscle tendons and tibialis posterior tendons on the inside of his ankles on Monday, February 25, 2013, by Dr. Yngve at the University of Texas Medical Branch (UTMB) in Galveston, TX. It was evident immediately after the surgery that he had “different” legs. He has measured, significant Range of Motion (ROM) gains. His right foot that used to turn inward is able to turn to the right and his legs are straight when he stands. His first small step, with his heel down and with his foot straight, (I will resist moon landing references) for William was beautiful to see. He is now taking giant steps, doing V sits up, and snow angels on the floor. This gives us hope.
Dr. Yngve is really wonderful both as a surgeon and a human being — kind, knowledgeable, deliberate and passionate about his work. Although reserved about what he is doing for kids with cerebral palsy, I think it is revolutionary given the other interventions with higher risk and longer recovery that were our recommended options.
Okay, let’s break down the unwieldy acronym of SPML:
- Selective refers to select areas of tightness caused by spasticity.
- Percutaneous – an instrument developed for eye surgeries is used to make small (2-3 mm) incisions in the areas of tightness/spasticity aided by a nerve block using ethanol on the obturator nerve and to make small cuts to the fascia. Percutaneous refers to a procedure performed through skin using a needle etc.
- Myofascial – myo means muscle and fascia is defined as connective tissue made of collagen that covers a muscle or a group of muscles.
- Lengthening – surgically creating a relaxation or lengthening of a muscle.
Many ask why SPML has been around for 30 years and isn’t standard of care practice/protocol in pediatric orthopedics or why don’t people know about it? Why does it seem that kids with varying degrees of Spastic Diplegia are primarily receiving recommendations of only traditional tendon and muscle lengthenings and osteotomies? Here is my opinion:
There are clinicians and there are clinicians/researchers. Both are important. Dr. Nuzzo and Dr. Yngve are clinicians, not clinical researchers. To be a clinician researcher requires significant time, training, collaboration, and funding. You need a Principal Investigator (PI), data collection resources, access to clinical epidemiology resources such as biostatisticians and clinical collaborators to power the study for something like SPML.
And if you want to know if it is replicable, you need to know that there are enough surgeons who are willing to try it and they have to have the same training. You have to have sufficient credibility with your peers in this procedure. You have to be good enough and have enough time and interest in research to write up sufficient numbers of case reports that you get published in peer-reviewed publications. You have to actively recruit people who want to train in the technique and swim upstream from their comfort zone of doing what they were originally trained.
Dr. Yngve, in my opinion, is focusing his energy on taking care of his patients instead of trying to convince his colleagues that this is the future for orthopedic treatment for Spastic Diplegia. He performs over 100 SPML procedures annually.
We are cautiously optimistic and know it is early, but we are committed to the six weeks of physical therapy (PT) 3x a week plus a forever home PT program to ensure whatever increase in range of motion he has now gets even better. He did PT and alternative therapy religiously before but his contractures were getting worse as he put height and weight on his frame. Now he has a real chance to try to stretch those tight muscles to keep up with his long bone growth and keep the contractures away.
Yes, SPML doesn’t fully address the spasticity that his brain injury causes but it does buy him time. Dr. Yngve’s data of 28 patients from six years ago show that approximately 90% have maintained or increased their ROM gains from SPML. About 10% might need a “redo” in an area. The beauty of SPML is that there is minimal scarring as opposed to traditional SEMLS where all that cutting of tendons and muscles can cause scar tissue that can make contractures harder to address if future surgery is needed.
As William’s neurologist told us a month ago, novel therapies that are not backed by evidence-based medicine cannot be dismissed or there wouldn’t be novel treatments or any improvements in treatment! Sometimes you take a leap when swimming against the medical tide of advice.
So after two-days post op and now eleven days later, we are SPML converts despite the lack of research studies on SPML. There are a few, and I have provided links below.
What swayed us finally was an email response from Dr. Edwin Spencer, who is a board-certified orthopedic surgeon for shoulders in Knoxville, TX. His daughter Savannah, who has Spastic Diplegia, had the SPML procedure by Dr. Yngve. I asked why he chose this over the traditional treatments that were backed by evidence-based medicine. He said he reviewed everything and wanted the least invasive procedure with the best outcome. His daughter, who was in a wheel chair, is now walking with a walker. You can see her video on YouTube in partnership with UTMB and with Dr. Yngve being interviewed.
I thought if a board-certified orthopedic surgeon who is published in orthopedic journals, chose SPML with Dr. Yngve for his own daughter — we would try it for our son. It is only one data point but it was the final one that pushed us to try SPML. The procedure for the traditional SEMLS recommended was bilateral femoral derotations, lengthenings for bilateral psoas, adductors, hamstrings, gastrocs and casts for six weeks.
The SEMLS would have entailed an all day surgery under anesthesia, five to six days in the hospital, six weeks out of school and a six-month to a one-year back to baseline period with intensive PT. It seemed obvious to us to try SPML first.
Here are my pros for SPML vs. SEMLS for William:
1. It is one hour under anesthesia vs. eight hours.
2. Less time in the hospital post op (outpatient day surgery vs. 5 days).
3. Less blood loss.
4. Less scar tissue, which is important if the procedure needs to be repeated with growth or loss of ROM gains.
5. Less pain and recovery time.
6. Back to baseline strength in one to two months vs. six months to a year.
7. PT regimen is 3x a week for six weeks for SPML and AFOs after casts are removed four weeks post op, which is less than SEMLS.
8. Back to school one week later vs. six to eight weeks.
9. More mobility with walking after surgery supported by a walker vs. reclining on a chair or a bed for six weeks. (Less weakening).
As the parents of a child with Spastic Diplegia who researched all of the surgical interventions exhaustively for six months with careful review of published outcomes studies, interviews with doctors, parents and patients for each procedure, we ask you to not dismiss SPML simply based on its lack of evidence-based medicine especially if you are considering a traditional orthopedic surgery.
And at some point, despite the uncertainty, parents have to accept and trust in themselves, and their medical advisors, in whatever medical decisions they make for their children and family, ultimately because they want what is best. It is all we can do.
Lori Poliski and Paul Gross
Please email me if you would like more information or to see before and after videos and pictures of William. firstname.lastname@example.org
SPML Research Links from Lori Poilski: