Functional surgery can improve walking ability in people with Charcot-Marie-Tooth disease (CMT), and patients are generally satisfied with the outcomes, a study indicates.
The study, “Long‐term walking ability and patient satisfaction after lower limb functional surgery in patients affected by charcot‐marie‐tooth disease: A retrospective study,” was published in the Journal of the Peripheral Nervous System.
CMT is caused by defects that affect the peripheral nervous system (the parts of the nervous system outside the brain and spinal cord). This can lead to symptoms including limb abnormalities and hampered walking ability.
At present, management of CMT is largely focused on addressing these symptoms. This can take the form of rehabilitation (i.e., physical therapy), but surgery has also been used to help reduce walking difficulties in CMT.
The basic idea behind such surgery, termed functional surgery, is to correct defects in the bones and tissues of the foot and/or legs, ultimately allowing for more stable biomechanics and, as such, easier walking.
Surgical techniques for CMT have substantially improved in recent decades. However, there is a dearth of published data on the effects of surgery for people with CMT, in large part because the disease is so rare.
In the study, a group of Italian researchers retrospectively analyzed data from 63 CMT patients, who underwent functional surgery at least once between 1967 and 2018. The group included 28 males and 35 females, with a mean age of 41.6 years. Three-quarters had demyelinating types of CMT, 20% had axonal CMT, and 5% had other types.
Most (about 60%) of the surgeries occurred in 2010 or later, and most (73%) of the people assessed were adults at the time of surgery (the rest were children). Nearly half (43%) of those assessed required more than one surgery.
To assess walking ability, the researchers conducted phone interviews in 2018–19. Based on these interviews, they assigned individuals scores on the Walking Handicap Scale (WHS) and on the patient-reported Global Impression of Change Scale (pGIC).
Both of these are standardized measures: the WHS measures walking ability on a scale from one to six, and the pGIC measures how well patients believe a treatment is working, on a scale from one to seven. Higher scores represent better walking ability and better perceptions of treatment, respectively.
While the full spectrum of possible WHS scores was represented in the analyzed sample, most people assessed reported good walking ability with three-quarters scoring five or more on the WHS.
A significant association was found between WHS score and age — older individuals were more likely to have walking difficulties than their younger counterparts. This association was strongest among those with demyelinating types of CMT.
“Our results indicate that gait efficiency was high in patients who underwent FS [functional surgery] during their lifetime, irrespective of the age at which the first surgery was performed, the number of interventions, and the kind of surgical procedure,” the researchers concluded.
Similarly, all possible pGIC scores were represented in the group, but most (78%) had a pGIC score of four, indicating that the treatment made symptoms at least “somewhat better.” These scores were not significantly correlated with age, CMT type, or walking ability (as assessed by WHS).
“The satisfaction rate was very high in this sample,” the researchers wrote. “Until now, the satisfaction rate of operated patients related to foot FS has never been investigated.”
Of the 63 patients, five (8%) experienced complications after surgery, including complications from surgical wounds and tendon detachment.
“Clinical complications of FS were uncommon,” the investigators wrote.
The researchers noted that, over the time period assessed, surgical techniques changed. Specifically, in the 1970s and 1980s, surgeries focused nearly exclusively on bones. Then, starting at around the turn of the millennium, surgeries started to focus more on soft tissue (muscle, tendons, etc.), in addition to bones.
“These new approaches allow for conservative or surgical options, which down the line preserve foot functionality as best as possible,” the researchers wrote.
However, the study design impaired the researchers’ ability to accurately determine whether modern surgical interventions are better at improving walking efficiency. They also could not compare walking efficiency in these patients with those who received no surgery. An additional limitation was the study’s small sample size.
Overall, “these results confirm the validity of FS in the management of acquired foot deformities in CMT patients,” the researchers concluded, but additional studies are needed to address the limitations.
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