Treatment for cartilage hair hypoplasia aims to help the patient adapt to their surroundings and prevent and treat any complications. At the Paley Institute, we can successfully address the orthopedic difficulties, namely short stature due to shortened femurs and tibias.
At the Paley Institute, we perform extensive limb lengthening to correct the short stature. Extensive limb lengthening involves four segment lengthening: simultaneous lengthening of both femurs and tibias in each leg. The major advantage to four segment lengthening is that it maximizes the amount of length gained while minimizing time spent in the fixator.
The lengthening surgery involves an osteotomy (surgical cut to the bone) separating the bone into segments, followed by application of an external fixator or an internal lengthening device. The internal lengthening device we utilize here at the Paley Institute is the PRECICE intramedullary nail. Both external fixators and the PRECICE internal nail perform lengthening by slowly distracting the bone segments. As the bone is distracted, new bone will form in the gap. For more information on the biology of limb lengthening, see the Limb Lengthening Center.
During the lengthening process the patient is expected to stay locally and attend daily physical therapy (PT) as well as follow up appointments every two weeks. It is important to receive daily PT and attend all follow up appointments to avoid developing complications from the lengthening. The successful outcome of limb lengthening is dependent on the entire team effort; everyone at the Paley Institute is dedicated to the sole goal of a safe and predictable outcome.
Close clinical follow up is crucial during lengthening for patients with cartilage hair hypoplasia. Due to an increased susceptibility to infection, patients must be closely monitored throughout treatment. At the Paley Institute we work closely with various specialists at St. Mary’s Medical Center and the Palm Beach Children’s Hospital, including immunologists, geneticists, and physical therapists, in order to ensure safe and consistent treatment.