Cooper the Trouper: On a Mission to Mobilize

Cooper the Trouper: On a Mission to Mobilize

Previously published in Rite Up, 2024 – Issue 3.

by Kristi Shewmaker

“I can’t wait for you to see the little tricycle up in the ceiling,” BJ said to his wife, Ashley, when they arrived at Scottish Rite for Children in January 2023. Ashley and BJ Burton drove from their home in College Station with their 2-month-old son Cooper to see Scott Oishi, M.D., FACS, hand surgeon and director of the Center for Excellence in Hand.

BJ was referring to the “Flying Machine,” a giant dirigible, or airship, powered by aviators and teddy bears that hangs from the ceiling in the Atrium of Scottish Rite’s Dallas campus. The “Flying Machine” has delighted children and families since 1978. It represents mobility for the patients of Scottish Rite. BJ remembered it fondly from his time at the hospital as a child when he received treatment for a rare form of congenital arthrogryposis, a condition that affects the joints and causes contractures that limit movement. His case affected his hands and feet.

When Ashley was 30 weeks pregnant, a 4D ultrasound revealed that Cooper’s hands were, in BJ’s words, “drifting.” Ulnar drift is a hand deformity that occurs when the joints contract and cause the fingers to bend toward the pinky finger, a symptom of arthrogryposis. “We noticed his hands first, so we started to prepare ourselves,” BJ says. When Cooper was born, BJ saw that his feet were deformed as well. “I knew they weren’t clubfeet because I had clubfeet,” he says. “The people at the hospital didn’t know, and they were spitting out all kinds of disheartening things. Obviously, that’s not what you want to hear less than 24 hours into the birth of your child.”

At Scottish Rite, where BJ had found hope years ago, Dr. Oishi evaluated Cooper and confirmed a diagnosis of arthrogryposis. The Burtons learned that when Dr. Oishi came to Scottish Rite, his mentor was hand surgeon Marybeth Ezaki, M.D., who was BJ’s physician when he was a child. Scottish Rite for Children was the first pediatric orthopedic facility in the United States to employ full-time hand surgeons. “It was really comforting for me,” BJ says. “Knowing that I got great care at Scottish Rite, I knew that care would transition to Cooper as well.”

For Cooper’s hands, Dr. Oishi prescribed splints to wear at night and at nap time to stretch his fingers and keep his thumbs from tucking into his palms. For his feet, Dr. Oishi referred the family to Anthony I. Riccio, M.D., pediatric orthopedic surgeon and director of the Center for Excellence in Foot.

Dr. Riccio diagnosed Cooper with congenital vertical talus, a rare, complex foot deformity that causes the sole of a child’s foot to flex in a convex position, forming a rocker-bottom appearance. A bone in the ankle, the talus, connects the lower leg bone, or tibia, to the foot. “The talus is normally positioned at about a 90 degree angle to the tibia, but in a congenital vertical talus, the talus points straight down,” Dr. Riccio says. “Because the talus is the central and most important bone in the ankle and foot, the bones around it fold up on either side, resulting in a V-shaped foot.” Affecting 1 in 10,000 births, the condition can occur in children with no other conditions, as well as those who have a variety of syndromes, like arthrogryposis. “We had never heard of vertical talus,” BJ says. “I knew that Cooper would face challenges, and I was torn apart because I felt responsible.”

“I was terrified that he wasn’t going to be able to walk,” Ashley says, “but I remember Dr. Riccio saying, ‘He will be able to walk. He will be able to play baseball,’ and that meant a lot to us.” The Burtons are a baseball family. BJ coaches varsity at A&M Consolidated, a local high school.

Cooper would need surgery on both feet, but first, Dr. Riccio prepared him for the operation by gently stretching and realigning his feet through manual manipulation and casting. For two months, the Burtons drove back and forth from College Station every week as Cooper received a series of seven casts. When Cooper was 6 months old, he underwent surgery. Dr. Riccio ensured that his joint was aligned, stabilized it with a pin and then released his Achilles tendon, which brought his foot into the correct position. Cooper came out of surgery with a long cast on each leg. After six weeks, the casts were removed, and he wore a boots and bar brace, which is comprised of special shoes, or boots, that are connected by a metal bar that kept his feet in the corrected position. He wore the brace 23 hours a day until he pulled up to stand when he was 10 months old.

To promote walking, Cooper transitioned to wearing an ankle foot orthosis (AFO) on each foot during the day and the boots and bar at night. “He started walking along the couch and pushing his little push toy,” Ashley says. And then, at 14 months old, Cooper walked on his own. “We thought he was going to be severely behind developmentally, but he’s right on track,” she says.

Today, Cooper is walking all over the place. “Nobody would ever guess he went through bilateral foot surgery,” BJ says. “He’s already hitting a ball off a tee and running to the front door like it’s a base, and then he comes back with a huge smile on his face!”

Dr. Riccio says, “The outcome is exactly what I hoped it would be — it’s a perfectly aligned foot.” Cooper will continue to wear the AFOs as he grows and the boots and bar at night until he is 4. Because the condition can recur, Dr. Riccio will monitor Cooper’s feet as he gets older, and Dr. Oishi will continue to treat his hands. “We definitely found the best of the best at Scottish Rite for everything,” Ashley says. BJ concurs, “The moment we got there, we just knew that this was the place we were supposed to be.”

Expert Care for Extra Fingers and Toes

Expert Care for Extra Fingers and Toes

Published in Rite Up, 2023 – Issue 2. 

“Holden is very much the baby of the family,” says Ashley, his mother. “He likes being the center of attention and being constantly held. He’s also a little mischievous.” The youngest of three children, Holden, of Dallas, turned 1 in April and, according to Ashley, is into everything. “He’s in that ‘I now can get myself anywhere I want to be, so I’m going to explore anywhere I can’ phase,” she says. But, for the first nine months of his life, he could not wear a shoe on his left foot.

Holden was born with polydactyly, a condition in which a baby is born with extra digits. In Holden’s case, he had an extra pinkie finger on each hand and an extra toe on his left foot that sat on top of his other pinkie toe.

Ashley was prepared for the extra toe because her doctor had spotted it in her 20-week ultrasound. He said that they would have a pediatrician at her delivery who would put a clamp on it, and it would fall off like an umbilical cord. The two extra fingers were a surprise. “When Holden was born, the pediatrician said that given the number of extra digits and the way some of them were connected, they weren’t confident clamps would work,” Ashley says. Within 24 hours, they were referred to Scottish Rite for Children.

Hand surgeon Chris Stutz, M.D., evaluated Holden when he was just 3 weeks old. A leader in training hand surgeons from around the world, Scottish Rite was the first pediatric orthopedic facility in the nation to employ full-time hand surgeons. Dr. Stutz recommended surgery to remove the three extra digits at 9 months of age. The timing of surgery was scheduled late enough in Holden’s development to mitigate the risk of complication due to anesthesia and early enough to ensure that he could learn to walk.

“The idea of handing over your 9-month-old baby for surgery is terrifying,” Ashley says, “but it was so much easier knowing that he was with people who specialized in that.”

Holden came out of surgery with soft wraps on his hands and a hard cast on his foot. “As soon as the cast came off, we put him in a hard-soled shoe,” Ashley says. “His ability to stand and move around has been really great.”

“While this is a common procedure for our team, it is uncommon for the patient and family,” Dr. Stutz says. “We were happy to provide the comfort, confidence and expertise for this wonderful, sweet family.”

“We loved our Scottish Rite experience,” Ashley says, “and we’re glad that Holden can move on with being a 1-year-old who can wear shoes!”

Read the full issue.

Hand Camp Around the World

Hand Camp Around the World

Scottish Rite for Children is world-renowned for its patient-centered care for children with orthopedic conditions, and our Center for Excellence in Hand is committed to caring for children with hand and upper limb conditions.

Focused on providing innovative treatment to help patients live active and independent lives, Occupational Therapist and Certified Hand Therapist Amy Lake, OTR, CHT, recently shared her knowledge at the 2023 World Symposium on Congenital Malformations of the Hand and Upper Limb. Working with a team from around the world, Lake served as co-chair. She shared her research and insight surrounding patient involvement in Hand Camp as well as the impact therapeutic camps have on children with hand differences. 

For more than 20 years, Scottish Rite has created a therapeutic and safe environment for patients with congenital hand differences through Hand Camp. Hand camp provides an opportunity for children with upper limb differences and their families to meet others facing the same challenges while enhancing confidence and self-esteem.

Lake’s research evaluates the positive benefits these camps provide to this patient population. Results showed that the participation in camp enhances their self-esteem and overall hand function, which promotes participation in extracurricular activities.

Her published study and passion for treating the whole child is inspiring hospitals all over the world to start their own hand camps. We are so proud to have Amy as part of our team sharing the incredible things happening at Scottish Rite.

What is Arthrogryposis?

What is Arthrogryposis?

Arthrogryposis is a rare condition that occurs in one out of 3,000 births. It involves curved or hooked joints and limited range of motion for joints of the hands, wrists, knees, feet, shoulders and hips. Most children with arthrogryposis have normal intelligence and a sense of touch.

The full name is Arthrogryposis Multiplex Congenita.

  • Arthro = joints
  • Grypo = curved
  • Multiplex = different forms
  • Congenita = present at birth

Arthrogryposis is also known as Multiple Congenital Contractures.

  • Multiple = many
  • Congenital = at birth
  • Contractures = limited joint motion

In some cases only a few joints are affected and the range of motion is nearly normal. In severe cases many joints are involved, including the jaw and back.

The most common form is Amyoplasia.

  • A = absent
  • Myo = muscle
  • Plasia = abnormal growth or development

This condition involves multiple contractures in all of the limbs. The joint contractures are often severe and affect the same joint in both limbs.

What are the causes?
In most cases the cause cannot be identified. Joints that are unable to move before birth can result in joint contractures. When joints are not moved for a period of time, extra connective tissue develops. This fixes the joint in a stiff or locked position. It also causes the tendons connecting to the joint to not stretch to their normal length making normal joint movement difficult.

The causes for limitation of joint movement are:

  • muscles do not develop properly.
  • muscle diseases.
  • fever during pregnancy and viruses that may damage cells that transmit nerve impulses.
  • decreased amount of amniotic fluid.
  • the central nervous system and spinal cord do not form correctly.
  • the tendons, bones, joint or joint linings may develop abnormally.
  • a genetic cause in 30% of the cases.
    • Several genetic patterns have been recognized, but most cases are rare and recurrence varies with the type of genetic disorder.

What are the goals?
To assist your child in developing and achieving goals to maximize independence in activities of daily living and accessing his or her environment.

What is the treatment?
Treatment is tailored to the individual by a multi-disciplinary team of physicians (pediatrician, orthopedic surgeon, neurologist, geneticist), nurses, physical therapists and occupational therapists.

Treatments may include physical therapy, occupational therapy and/or surgery.

  1. The goal of physical therapy is to increase the range of motion and strength through functional activities and exercises. Assistive devices to help achieve goals may include splints, braces, crutches and wheelchairs. Parents are encouraged to become active participants in a therapy program and to continue therapy at home on a daily basis.
  2. The goal of occupational therapy is to learn activities of daily living such as feeding, bathing, toileting and dressing with assistive devices. These assistive devices include long handled utensils, hairbrushes, toothbrushes, bath brushes, toileting aids, etc.
  3. The goal of surgery is to correct the alignment of the upper and/or lower extremities so that the activities of daily living, such as standing and walking are possible. In some cases, tendon transfers are done to improve muscle function. For severely affected children, the extremities are positioned in a fixed standing or sitting position. The pros and cons for both positions will be discussed prior to scheduling surgery.

What to expect?
In most cases, the outlook is a positive one. The condition does not worsen with age. Therapy and other available treatments can bring about substantial improvement. The vast majority of children survive and live a normal life span. Many excel because they become experts at meeting and tackling challenges and are able to lead productive, independent lives as adults.

Polydactyly in Children and What Parents Need to Know

Polydactyly in Children and What Parents Need to Know

Polydactyly is a word that means “many fingers.” There are many forms of this common diagnosis. Some extra fingers are on the thumb side, some on the pinkie finger side and some in the middle of the hand. Polydactyly of the feet is also common. Some forms of polydactyly run in families; others come as new “surprises.” It is rare for the extra fingers to be fully formed normal fingers. Some of these fingers may be very small, floppy and have nonfunctional fingertips. Others will have bones and joints, and some may even have tendons and some movement. Some forms of polydactyly are “isolated,” meaning that there is nothing else the matter with the baby. Other forms of polydactyly may have other conditions such as webbing, twisting or angling of other fingers. In rare cases, there may be problems in parts of the body other than the hand. Polydactyly is common, it is not painful and it is not an emergency to treat.

How can polydactyly be treated?

The treatment of polydactyly depends on the exact shape and form of the extra digit, whether there are other problems in the hand and whether there are other more general problems for the baby. If the extra finger parts are very small, the treatment may be simply tying off or putting clips on the base of the finger to allow it to dry up and fall off (similar to the way the stump of the umbilical cord dried up, separated and fell off on its own). Any finger that is too big for this will require surgery that will be done in the operating room and done with general anesthesia for your baby. Some polydactyly cases require delicate reconstructive surgery to rebuild the best parts to save for your child’s best hand function. There is no hurry in doing this, because it is safer to wait until the baby is older. The risk of anesthesia is higher for a little baby and lower for an older baby. Your doctor will discuss with you the exact plan for the polydactyly type of your child.

Learn more about our treatment for hand disorders.

On The Move: Unstoppable

On The Move: Unstoppable

Article previously published in Rite Up, 2022 – Issue 2.

It is not uncommon to see Michael, of Redmond, Washington, posing like his favorite superheroes. He loves playing Minecraft™, riding his bike, swimming, wearing costumes, acting, dancing and singing. In January 2021, this highly active and vivacious young man had a life-changing surgery performed on his left forearm by pediatric orthopedic experts from our hand and limb lengthening and reconstruction teams at Scottish Rite for Children.

“I have no words to describe the blessing it has been for Michael and myself,” Berenice, Michael’s mother, says. “Scottish Rite is simply amazing.”

Michael was born premature at 23 weeks and stayed in the NICU for four months. At 3-months-old, the family realized he had a bump on his left arm and worked with Michael’s doctors to investigate the issue. Following a biopsy, it was determined that Michael had congenital pseudoarthrosis, which caused a deformity in the bones in his left forearm.There are very few teams that could successfully manage the complexities and intricacies of Michael’s treatment, but Scottish Rite for Children could. “To me Scottish Rite is a place where miracles happen, where the prayers of many parents come true,” Berenice says. “The doctors really care, and all the staff understand you and want to help in any way they can. Somehow you know your child is in the best hands, and you trust they are going to be okay.”

 

Watch Michael’s Story and learn more about Scottish Rite’s extraordinary care.

Read the enitre issue.