Akron Children’s Hospital surgeons, Stephanie Russo, MD, PhD and Drew Engles, MD, are giving patients with arthrogryposis a new way to move their joints thanks to an innovative procedure called, carpal wedge osteotomy and flexorpronator slide.
Arthrogryposis, also called arthrogryposis multiplex congenita (AMC), is a rare condition that causes stiff or contracted joints at birth and limits range of motion in more than one joint (hands, feet, hips, knees, elbows, shoulders, wrists, fingers, toes). There is no cure for AMC, but surgery may help improve function for some patients. However, many patients rely on physical and occupational therapies to improve quality of life.
“To my knowledge, Akron Children’s is only the second place in the country doing this specific combination of procedures for arthrogryposis,” said Dr. Russo, pediatric hand and peripheral nerve surgeon at Akron Children’s. “I studied under Dr. Zlotolow, an expert in the AMC field, during my fellowship so I’m excited to bring that experience to Akron Children’s. Working with Dr. Engles, we do a thorough pre-op assessment to make sure a child is a good candidate for the procedure because there are a lot of variables to consider like how much control the patient has over their fingers and whether or not they need wrist flexion to feed themselves or perform other daily activities.”
For Joseph “JoJo” Krejsa (4), he proved to fit the candidate profile perfectly.
When JoJo was born, his elbows were locked, shoulders turned in, wrists contracted and fingers curled in. At just 9 days old, he met Dr. Engles who determined arthrogryposis was the cause of his condition. At 3 weeks old, JoJo began working with occupational therapist, Margaret Norin, who he still sees 2 times a month. Over the past 4 years, JoJo has had a several surgeries, numerous splintings and countless hours of therapies to help him build strength, fine motor and problem-solving skills.
JoJo’s biggest fan and advocate, his mom, has always been open to helping her son maximize his abilities, but has been cautious about surgeries.
“Before doing surgeries, we always try to see if we can improve his active and passive range of motion and increase strength enough for self-care or self-feeding with therapies,” said Maria, JoJo’s mom. “It’s always scary sending your child into surgery, but especially if you’re not sure it will really help him improve.”
Knowing JoJo’s condition and his family’s concerns, Dr. Engles asked Dr. Russo to meet with JoJo’s family to discuss the new procedure.
“We were really nervous to do the new surgery because I was afraid it would take away the function he had with that right hand,” said Maria. “After meeting with Dr. Russo, we found her to be wonderful, examining JoJo carefully and considering how his presentation would affect function… We also knew she had been part of cutting-edge surgery with Dr. Zlotolow so we felt blessed to have her and our trusted Dr. Engles work on JoJo.”
Drs. Engles and Russo performed a combination of procedures – flexor-pronator slide, carpal wedge osteotomy, camptodactyly release, and transposition of the EPL muscle – on JoJo’s right hand. The 6-hour surgery worked to help loosen JoJo’s finger flexion and bring his wrist up to a neutral position while keeping wrist motion.
Three months post surgery and JoJo’s right hand is open. He can also hold a mouse and use his fingers. While progressing, occupational therapy remains important to helping JoJo gain independence, improve joint motion and avoid muscle atrophy.
“The best part of this surgery for me is, for the first time, I can walk along with JoJo and hold his hand,” added Maria.
JoJo’s Akron Children’s team continues to monitor his progress to determine if his left hand will be a candidate for the procedure, too.
“We couldn’t be happier with the outcome JoJo has achieved,” said Dr. Russo. “I’m grateful to have a partner like Dr. Engles to work closely with so we can find new and better ways to positively impact patient lives. I am proud that together we are advancing hand, nerve and upper extremity surgery at Akron Children’s.”
Contact Akron Children’s orthopedics team at 330-543-3500.