Pediatric Connection A PUBLICATION OF THE PEDIATRIC MUSCULOSKELETAL DEPARTMENT OF HOSPITAL FOR SPECIAL SURGERY VOLUME 2 – ISSUE 1 SUMMER 2009
MEDICAL STAFF
Cerebral Palsy
Pediatric Orthopedists John S. Blanco, MD Shevaun M. Doyle, MD Daniel W. Green, MD
by David M. Scher, MD
Cathleen L. Raggio, MD Leon Root, MD David M. Scher, MD Roger F. Widmann, MD Pediatricians H. Susan Cha, MD Lisa S. Ipp, MD Stephanie L. Perlman, MD Pediatric Rheumatologists Alexa B. Adams, MD Thomas J.A. Lehman, MD Emma Jane MacDermott, MD
Cerebral palsy (CP) has been recognized as a distinct condition for more than a century. During much of that time, there were limited options for improving the lives of affected children. Today, advances in medicine, science, and technology have all contributed to dramatic progress in our ability to improve function and enhance the lives of children with CP. In young children with CP who are just learning to walk, many of our efforts are directed at managing the spasticity of the muscles that impairs muscle function. When young children with spastic muscles are learning to walk, their muscles become tight and their legs can not get into the right position at the right time. One of the most effective ways to decrease spasticity is with the use of Botox (botulinum toxin). Botox works by blocking some of the activity at the neuromuscular junction to “turn off ” a portion of the signal moving from the nerve to the muscle. Another commonly used and effective technique for managing spasticity is brace (orthotic) treatment. An appropriately prescribed orthosis counteracts the spastic tone in the calf muscles that causes the foot to point down. Other techniques such as nerve surgery (rhizotomy) and direct delivery of medication to the central nervous system (intrathecal Baclofen) enable us to manage muscle spasticity.
Pre-Op
Post-Op
Between age five and adolescence, we can realign surgically the bones and joints of the lower extremities to improve how children walk. Over the past two decades, dramatic advances help us to understanding the science of gait, and allow us to use modern surgical techniques to optimize a child’s gait. Much of the theory behind this process unfolded with gait analysis technology (see related article). The concept used to describe abnormal gait in ambulatory children with CP is called “lever-arm dysfunction”. This is akin to turning a bolt with a wrench that is too short and made of rubber. A wrench that is longer and rigid is more effective than one that is short and floppy. The extremities of children with CP become malpositioned, analogous to the short, floppy wrench. By improving alignment, we can provide children with their optimal power for walking. (Continued on page 3)
CONTENTS Cerebral Palsy . . . . . . . . 1 Lyme Disease . . . . . . . . 1 Measuring Walking: Gait Analysis . . . . . . . . . . 2 The Connection Inspection . . . . . . . . . . . . 3 Question of the Quarter . . . . . . . . . . . 4
Lyme disease by Emma Jane MacDermott, MD
Lyme disease as a cause of childhood arthritis is something we see frequently at HSS. Lyme disease is very familiar to those living in the northeastern United States. The ticks that carry the disease are found in this area, and up to 90% of cases occur here. However, Lyme disease is not caused by ticks; rather it is caused by a small spiral shaped organism called Borrelia burgdorferi that lives for part of its life cycle (Continued on page 2)
Measuring Walking: Gait Analysis by Sherry I. Backus, MSPT
Since ancient times, people have been watching other people walk. But today, there are options beyond just using your eyes to evaluate how individuals, both children and adults, move. Over the past 50 years, researchers and clinicians have used film, video, and now digital technology to “capture” and record walking patterns to optimize treatment options and choices. Current technology uses high-speed digital cameras that capture data at 100 frames per second (more than three times faster than your average camcorder) and can be set up to 1,000 frames per second for sports activities! With the use of specialized reflective markers placed on the legs, pelvis, and torso, the cameras record the positions of the child as he or she walks across the gait analysis laboratory. At Hospital for Special Surgery, this happens at the Leon Root, MD Motion Analysis Laboratory. In addition to documenting how the body moves, we also record the activity of selected muscles during walking with the electrodes placed over (or in) the muscles. The simultaneous recording of muscle and movement patterns allows clinicians and researchers to understand what is working well and what is not. The end results of the joint angle movement patterns (kinematics) and the muscle activity patterns (kinesiologic electromyography), in combination with a clinical examination and appropriate radiographic studies, allow the medical team to make treatment recommendations. While we see all ages of individuals in the Leon Root, MD Motion Analysis Laboratory, the largest percentage of our clinical cases are children and adults with cerebral palsy whose surgeons and clinical team are considering surgery or other treatment options. The specific documentation of walking patterns helps dramatically in the decision process. Gait analysis is one of the many specialized tools available to help provide the best care for individuals with altered walking patterns. Sometimes it takes a computer and a team of engineers, physical therapists, and physicians to really know what the “eye” sees when a child walks. 2
Lyme disease (Continued) inside some species of ticks. When a tick bites to feed, the Lyme-causing organism can be transferred to the blood stream of the animal, usually a deer or a mouse. If the infected tick then feeds on a child, Lyme disease may result. Lyme disease presents in 3 stages – local disease, followed by disseminated (or widespread) disease, and ultimately chronic / late disease. Classic Lyme disease presents with a rash occurring at the site of the tick bite. This rash may be completely red, but usually develops a pale area in the center that makes it look like a bull’s eye; this is known as a ‘target lesion’. Symptoms of early disease occur within days or weeks of the tick bite and resemble the flu. In fact, early disease is dismissed often as a viral infection in children and therefore is not treated. If the Lyme disease is not recognized or remains untreated, children commonly progress to ‘late disease,’ often many months later. This can involve the heart, nervous system, and very commonly the joints. Up to 70% of untreated patients develop arthritis and Lyme arthritis can affect any joint. Arthritis often involves one large joint, like the knee, which rapidly becomes markedly swollen and painful, but symptoms may be more subtle. While not everyone that is bitten by a tick gets Lyme disease, and not everyone that gets Lyme disease gets arthritis, in an endemic area, children displaying symptoms should be tested and treated appropriately. The question often arises of what to do for a child who has been found with an embedded tick, but has displayed no symptoms. In these cases, a Lyme test should be performed, and it is considered appropriate to begin medication, which can be stopped if repeat Lyme testing is negative. The blood test for Lyme involves a two step process. First, a screening test is performed which is very sensitive and detects any Borrelia organisms including those causing Lyme disease, but also many others that occur normally. The second phase of the Lyme test, known as a Western blot, is done on all patients with positive results; this tests very specifically for Borrelia Burgdorferi. Treatment is with antibiotics; the type used depends on the age and medical allergies of the child. The duration of treatment differs based on the stage of disease. A longer course is given if patients have late symptoms. Sometimes fluid can be removed from a swollen, painful joint to give symptomatic relief; and anti-inflammatories can be given to help patients feel better. A small proportion of children continue to have some symptoms despite extensive antibiotic treatment. It is thought that this does not represent persistence of the Borrelia organism in the body, but rather persistent stimulation of the inflammatory system, and as such is best treated with the battery of medications that we use for inflammation. In an endemic area, awareness is essential. Check children for ticks, particularly during the summer months. Any suspicious rashes should be evaluated by a physician. Finally, don’t ignore unexplained joint complaints. If you have any queries about Lyme arthritis in children, we would be happy to help you.
Cerebral Palsy (Continued) In non-ambulatory children, we focus on keeping the hips located, the pelvis level, and the spine straight in order to promote good sitting balance. By keeping the hips located in the sockets, we prevent painful arthritis and maintain hip motion so that a child who cannot walk can still lie down, sit, and stand. Advances in our understanding of the shape of the hip in children with CP have optimized our surgeries in order to restore the normal anatomy. After Treatment When scoliosis develops in children with CP, it can interfere also Before Treatment with a child’s ability to sit evenly and remain comfortable in a wheelchair. Scoliosis surgery in children with CP is among the most challenging spinal procedures, due to the reduced strength of the bone and the severity of the curve. New implants and surgical techniques that have developed over the last decade have enhanced greatly our ability to perform these surgeries safely and reliably. While technological and medical advances have improved remarkably our ability to care for children with CP, the sub-specialization of caregivers, such as pediatric orthopedists, physical therapists, neurologist, physiatrists, engineers, orthotists, and nurses, is equally important. Today there are multiple centers around the world where people from many different disciplines focus their efforts on CP research and clinical care. In doing so, they gain clinical experience and knowledge in the management and treatment of this condition with the greatest impact on the lives of children with CP.
The Connection Inspection... Born with cerebral palsy, Matthew Russo of Brooklyn, New York had been going to physical therapy since he was nine months old to improve his joint range of motion and strengthen his coordination and balance. Often misperceived as a form of mental retardation or a condition related to cognitive dysfunction, cerebral palsy is described more accurately by impairment of motor skills, ranging from mild to severe. Matthew’s condition, which is considered mild, does not impact his performance in the classroom, but presents challenges when keeping up with his peers physically. After two years in leg braces failed to help Matthew meet these challenges, his mother, Linda Cesaria, turned to Dr. David M. Scher at Hospital for Special Surgery. Matthew Russo
“His examination was so thorough,” says Ms. Cesaria. “He explained everything about Matthew’s condition, was compassionate, and made Matthew feel relaxed. I felt very comfortable and confident choosing Dr. Scher to help correct Matthew’s problem.”
After Matthew had a physical examination, radiographic evaluation, and a gait analysis (which utilized digital technology to measure how Matthew’s joints were moving throughout all three planes of space), Dr. Scher recommended that the seven-year-old undergo a series of surgeries designed to straighten his hips and release tightness in the muscles of his legs. The procedure, which is known as a single event multi-level surgery (SEMLS), encompassed eight surgeries under one anesthesia. The surgeries included a varus rotational osteotomy (VRO), which placed his hip bone in better alignment with his socket and shifted his legs outward to place them in a stronger position for growth, and several lengthening procedures of the muscles in his lower extremities. “After the initial shock of Matthew being told that he needed surgery, he was a trooper and such a good sport about it,” says Ms. Cesaria. “Post-surgery,” she continues, “both of Matthew’s legs were in casts and he had to use a wheelchair for eight weeks. When the casts were removed, he had to use a walker for six weeks.” After one year, Matthew returned to HSS to have plates removed from his hips, a common overnight procedure. He then required a walker for an additional four weeks. Throughout the entire process, his attitude impressed even Dr. Scher. “He’s got a great spirit,” says the surgeon. “After the surgery and during his rehabilitation, he was quite mature and an exceptionally hard worker. I think that’s much of the reason behind his quick recovery and his excellent result.” Matthew, now 10, still wears braces for six hours a day and goes to physical therapy twice a week, but the Little League outfielder, whose favorite player is Derek Jeter, is keeping up with his peers better than ever before. “Our season is just beginning, but I hope we make the playoffs,” says the three-sport athlete. “Throughout both of the surgeries, the staff was wonderful,” says Ms. Cesaria. “Not only did they take such excellent care of my son, they also were wonderful to me. Two years later, I often still talk about the wonderful experience we had. Because of the excellent care they provided, HSS made a very stressful time for my family a lot better. I will forever be thankful and grateful for what Dr. Scher had done for my son.” 3
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Pediatric Connection
A PUBLICATION OF THE PEDIATRIC MUSCULOSKELETAL DEPARTMENT OF HOSPITAL FOR SPECIAL SURGERY
Question of the Quarter What is a flexible flat foot (pes planus)? by Shevaun M. Doyle, MD
When standing, children with flexible flat feet do not have arches along the inner borders of their feet. However, when they stand up on tip toe or sit down with their feet dangling off the examining table, they reconstitute well formed arches. Most flexible flat feet are painless, do not impede a child’s athletic ability or normal growth pattern, and do not cause long term deformity or disability. A majority of infants with flat feet will develop arches, even when standing, by 5 years old; this is due to tightening of the soft tissue structures in the feet that occurs with weight bearing activities and walking. Special shoes, orthotics, or foot pads are contraindicated in most cases.
Q& A
www.hss.edu/peds Editor Shevaun M. Doyle, MD For inquiries, please call (877) HSS-1KID or e-mail: Peds@hss.edu Photography by Brad Hess ©2009 Hospital for Special Surgery. All rights reserved.
The HSS Pediatric Musculoskeletal Department: Bottom row L-R: David M. Scher, MD; Roger F. Widmann, MD; H. Susan Cha, MD. Second Row: Alexa B. Adams, MD; Leon Root, MD; Cathleen L. Raggio, MD; Stephanie L. Perlman, MD; Arkady Blyakher Third Row: Lisa S. Ipp, MD; Shevaun M. Doyle, MD; John S. Blanco, MD; Daniel W. Green, MD; Thomas J.A. Lehman, MD; Emma Jane MacDermott, MD