Complications with scoliosis surgery common for Angelman patients
Prevalence of scoliosis also high in these people, underscoring risks
Spinal surgery for scoliosis carries a high likelihood of complications, including serious ones, among people with Angelman syndrome and a related disorder known as Prader-Willi syndrome, a study highlights.
“These findings provide additional evidence to support the idea that surgical intervention in patients with scoliosis with underlying AS [Angelman syndrome] or PWS [Prader-Willi syndrome] should be approached with special caution because dramatically increased complication rates appear to exist,” the researchers wrote.
The study, “Angelman and Prader-Willi Syndromes: Sister Imprinting Disorders With High Complication Rates Following Spinal Deformity Surgery,” was published in the journal Orthopedics.
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Angelman syndrome is caused by mutations in the copy of the UBE3A gene that is inherited from an individual’s biological mother. PWS is a related condition caused by mutations affecting genes on the paternal chromosome 15.
While Angelman symptoms differ from those of Prader-Willi, children with both of these disorders are at high risk of scoliosis, an abnormal sideways curve of the spine.
Spinal surgery is often recommended for advanced scoliosis to help correct the abnormal curving, which is painful and can affect breathing and the heart. While a few studies have reported high complications associated with spinal surgery in these patients, there isn’t much published data on outcomes.
“Relatively little literature exists on the subject of surgery in patients with scoliosis with underlying AS or PWS. However, scoliosis is a significant comorbidity in these syndromes, with reported prevalence rates as high as 70% in AS and 83% in PWS,” the researchers wrote.
Scientists with a Texas hospital reported outcomes for five people with Angelman syndrome and 15 with PWS who underwent scoliosis surgery at their center between 2000 and 2018. Their age at surgery ranged from 4 to 21 years old. Most of the surgeries were spinal fusions, procedures to connect the individual bones of the spine (vertebrae) in order to provide stability and prevent abnormal curving.
Nearly all of the patients experienced complications following the surgery, including four of the five Angelman patients.
Serious complications were reported in three of the Angelman patients, including deep infections, abnormal buildup of fluid near the surgical site (seroma), and problems with stitches or other issues in keeping surgical wounds closed. Complications in the fourth patient were more minor issues related to closure of surgical wounds.
Serious complications also were reported among six of the 15 PWS patients. One died as a result of a serious infection that progressed into sepsis, a life-threatening reaction to an infection, with disseminated blood clotting within blood vessels. Five people with PWS, but none with Angelman, also experienced complications during the surgery itself.
Collectively, these data suggest a high risk of complications associated with scoliosis surgery in people with Angelman or PWS. The researchers noted that the risk among these patients seems particularly high even compared to other complex health disorders that can cause scoliosis.
Numerous factors likely contribute to a complications risk for Angelman patients, the researchers said. For example, they noted that seizures, a common Angelman symptom, can lead to complications from surgery. Angelman and PWS patients often struggle with communication, which may cause problems with post-surgical care, they added.
Despite the high risk, the researchers said people with Angelman or PWS still should be considered for spinal surgery — indeed, they noted that it’s impossible to say how these complications might compare to health issues that arise if scoliosis goes unaddressed. Instead, the investigators said it’s important for clinicians planning to perform spinal surgery on Angelman or PWS patients to be aware of the high complication risk and be proactive about trying to reduce the risk.
“The first step in reducing the extraordinarily high complication rates found in these populations is recognition of the high-risk setting,” the team wrote.
Steps to limit complications could include the use of preventive antibiotics and having a two-surgeon team for the operation itself, the scientists said. They also stressed the importance of paying close attention to wound closure, and suggested more frequent check-ups after surgery to assess how the surgical wound is healing.
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