China News Service, June 28 (Chen Jing Shi Jiaqi) Spinal muscular atrophy (SMA) is a genetic disease that seriously affects the quality of life.
Spinal plastic surgery has long been regarded as a "forbidden zone" for spinal muscular atrophy.
The reporter learned on the 28th that a child with severe spinal muscular atrophy with a scoliosis of 176 degrees was successfully corrected in Xinhua Hospital affiliated to Shanghai Jiaotong University School of Medicine.
The postoperative X-ray showed that the child's scoliosis angle was reduced to 82 degrees.
Sitting up from the bed, this most common action, Mengyuan (a pseudonym), a 13-year-old child with spinal muscular atrophy with SMA, waited 10 years.
Mengyuan’s mother said: “Her life has always been lying on the bed, begging the doctor to save her, and more importantly, let her walk the future with dignity.”
Professor Yang Junlin, director of the Department of Pediatric Orthopedics and Spine Center of Shanghai Xinhua Hospital, told reporters that SMA patients have normal mental development and sensation, and their life expectancy is between 2 and 10 years. Some mild patients can survive to adulthood and close to normal life.
However, they face many complications of the bones and respiratory system, especially scoliosis caused by muscle weakness, which compresses the chest cavity.
When Mengyuan was born 9 months old, her hands were weak and weak. Everyone felt that she was calcium-deficient.
But after one year old, the child still can't stand and walk. After a few steps, he will suddenly bend down and his legs cannot be fully straightened.
Mengyuan's parents took her on a long journey to seek medical advice.
Genetic testing revealed that Mengyuan suffered from spinal muscular atrophy, SMA, and his expected life was 5 years old.
By 2020, Mengyuan’s scoliosis will become more and more serious, and the cardiopulmonary function will deteriorate accordingly.
After many inquiries, the parents brought Mengyuan to Shanghai Xinhua Hospital for help.
In the past two years, as the Shanghai Children’s Rare Disease Diagnosis and Treatment Center, Xinhua Hospital has established an SMA multidisciplinary diagnosis and treatment team.
Professor Yang Junlin told reporters that the child’s scoliosis angle was 176 degrees, the pelvis was severely tilted, the vertebral body was highly rotated, and the concave side of the thorax was severely deformed with a "fin-like" appearance.
The 13-year-old child weighs only 24 kg and is about 135 cm tall.
According to reports, the child's deformed rib cage squeezed the lungs' space for activity, resulting in poor breathing and making speech impossible.
Yang Junlin told reporters that such a child has extremely poor muscle strength, and that the intubation and extubation of the operation is a matter of life and death.
Surgery faces huge challenges.
Multidisciplinary experts recommend that patients continue traction therapy, increase nutrition and functional exercises, and consider surgical treatment after lung function and physical fitness have improved.
The parents of Mengyuan insisted on bearing all the consequences, hoping that the expert would perform the operation on the child.
The operation has begun, and there are many difficulties.
Before the operation, nine doctors, nurses, etc. worked together to complete the correct positioning.
With everything ready, the expert must complete the two key surgical procedures of nail placement and orthopedics within 3 hours.
In fact, the nail path for thoracic spine placement is only a few centimeters wide and the deformity is serious. A little carelessness may cause spinal cord injury and paralysis; or damage the pleura and cause complications.
Yang Junlin's team accurately inserted 12 screws for the child.
It is reported that the child's orthopedic rate reached 53%, and the intraoperative blood loss was only 300 ml.
Professor Yang Junlin said in an interview with reporters that SMA is a rare genetic disease, in addition to patient treatment, prevention is also an important part.
The population carrying rate of spinal muscular atrophy reaches one in fortieth, and a simple blood test can detect who is the carrier.
The expert also bluntly said that the incurable of SMA does not mean that it cannot be treated. A new concept and confidence that comprehensive treatment can improve the condition should be established.
The expert said that with existing technical conditions, most patients can receive surgical treatment. When the degree of scoliosis is greater than 60 degrees, it is recommended that patients undergo surgery as soon as possible to improve their quality of life.
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