Surgical Reconstruction for Poland Syndrome: Beau's Story
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Heather gave birth to twins, Beau and Ellie, at a hospital near her home in Lancaster, Pennsylvania. Ellie arrived first; three hours later, Beau was born. When he was placed on Heather’s chest, she could see something was different about Beau’s left hand. Rather than being five individual digits, the fingers on his left hand were fused together. Joy over the birth of her children suddenly turned to worry.
“There we were trying to be happy and celebrate and we were suddenly thinking about what the future would be like for Beau,” recalls Heather. “We knew we had to find answers and get him seen by someone as soon as possible.”
While Heather and the babies recovered from the delivery, her husband, Steven, searched the web about Beau’s condition and found information about Apurva S. Shah, MD, MBA, an orthopaedic surgeon at Children’s Hospital of Philadelphia (CHOP) who specializes in pediatric hand and upper extremity surgery and has extensive training and expertise in congenital hand differences (anomalies). He contacted CHOP and, within a week of Beau’s birth, the family was at CHOP for an evaluation.
Diagnosis of Poland syndrome
After a thorough examination, Dr. Shah diagnosed Beau with Poland syndrome, a non-inherited disorder that affects the development of the hand and arm on one side of the body. The condition caused Beau to have a complex hand abnormality called symbrachydactyly. Not only were the fingers on his left hand fused together, some of the bones in the fingers were severely underdeveloped. Beau also lacked part of his chest muscles (called the pectoralis major), and the bones in his left forearm were shorter than those in his right arm.
Dr. Shah recommended surgery to reconstruct Beau’s hand. The surgery was risky; there was a chance Beau could lose one of his fingers, given how underdeveloped it was. But Heather and Steven wanted to give Beau the greatest possible function.
“We wanted Beau to be able to interact with the world and be able to use what he has with the best of his ability,” says Heather.
Surgical hand reconstruction improves function
Dr. Shah planned to repair Beau’s hand in two stages. In the first operation, Dr. Shah planned to separate the thumb and also separate the middle finger from the ring finger. During the second operation, Dr. Shah planned to separate the remaining fingers. Beau returned to CHOP for a follow-up appointment at 9 months, then had the first surgery at age 1 and the second surgery six months later. At the end of the two surgeries, Beau had five separate digits.
Beau is now 2 years old and has five well-functioning digits. He sees a physical therapist at a hospital near his home every few months and will have follow-up appointments with Dr. Shah every year. Dr. Shah is very pleased with the way Beau’s hand has healed, and his high level of function. Dr. Shah is awed by the support and love Beau receives from his parents.
“Beau’s resilience and his family’s unrelenting support have together brought him to this remarkable point,” says Dr. Shah.
Beau’s favorite things to do are play with cars and trucks, dance, sing songs and, most of all, pal around with his twin sister and his big brother, Brady.
“I’m just very thankful that Dr. Shah is at CHOP and we had the opportunity to have him operate on Beau,” says Heather. “He was wonderful.”
