Neuromuscular Diseases, Neuromuscular Scoliosis
Conditions
Brief summary
This study was sponsored by Federal state budgetary institution Russian scientific center for traumatology and orthopedics n.a. acad. G.A. Ilizarov of the Ministry of Health of Russian Federation. The study will take place at this center. It is expected to enroll 70 patients aged 6-25 years with NMD, all of whom will be subjected to thoracic and lumbar spinal deformity surgery. Patients will be divided into two groups depending on skeletal maturity. The degree of skeletal maturity will be determined on the basis of an X-ray study of spinal and pelvic bones. The type of surgical correction will depend on the group.
Detailed description
First group of patients: local dorsal access. Cranial access: at the level of posterior Th2 5 structures and caudally at the level of L4-S2 vertebrae with exposure of posterior upper spines of iliac wings. Skeletization of vertebrae will be done within transverse processes. Pedicle support points are set bilaterally cranially at each segment level for the space of 3 segments. Caudal support base is formed with pedicle screws in lateral masses of the sacral bone or iliac crests at the level of L5-S1 vertebrae. Control fluorography is performed in two standard planes in order to control screw position. Two dynamic rods are formed on the basis of the distance between screw heads allowing 2 cm for distraction; these are two rods connected with a longitudinal connector and bent following the normal sagittal spine profile. After that, channels are formed on both sides under m. erector spinae in the cranial/caudal direction. Pre-bent rods with connectors are placed into the prepared channels. The metal construct is stabilized with internal set screws. If necessary, the construct may be stiffened with crosslinks. Control fluorography is performed in two standard planes in order to control implant position and evaluate correction. The intervention completes with local dorsal spinal fusion with an autobone at the level of base screws. The wound is sewn up in layers tightly. Group two: dorsal access to the posterior column is gained in accordance with the preoperative plan. The access length depends on the instrumentation area. Vertebral skeletization is performed within transverse processes. Subgroup 1 - pedicle support points are set bilaterally at each segment level. Subgroup 2 - pedicle support points are set bilaterally in every second segment. Segmented instrumentation of 3 cranial segments to prevent dislocation of metal construct elements at the level of Th2-4 or Th3-5 vertebrae is a special measure. Similar to group I, caudal support base is formed with pedicle screws in lateral masses of the sacral bone or iliac crests at the level of L5-S1 vertebrae. Control fluorography is performed after that in two standard planes in order to control screw position. Segmented facetectomy is recommended for additional spine mobilization. Pre-bent rods are placed into support points. Deformity correction is performed by means of a translational maneuver, segmented distraction (concave side) and compression (convex side). The metal construct is stabilized with internal set screws. If necessary, the construct may be stiffened with crosslinks. Control fluorography is performed in two standard planes in order to control implant position and evaluate correction. The intervention completes with dorsal spinal fusion with an autobone along the implants. The postoperative bed is subjected to Redon drain. The drainage is exteriorized to the skin via a separate puncture. The wound is sewn up in layers tightly.
Interventions
All patients will be implanted Medtronic CD Horizon Legacy 4.5 and 5.5 under X-ray control (C-arm), intraoperative spinal deformity surgery safety control and standard anesthetic support.
Sponsors
Study design
Eligibility
Inclusion criteria
* Signed and dated informed consent for participation in the study (separate informed consents for child and parents/caregivers), obtained before conducting any study procedures * Verified and documented diagnosis (neuromuscular desease, NMD) with the results of neurologic examination * Deformity of thoracic and/or lumbar spine associated with NMD, which requires surgical intervention
Exclusion criteria
* Patient unwillingness or inability to follow study procedures * Absence of signed and dated informed consent for participation in the study (as for child and for parents/caregivers) * Patient participation in another clinical study, which can influence the results of this study * Life expectancy \<12 months * Concomitant diseases, which preclude patient participation in this study according to doctor's opinion
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Lordotic Curve L1-S1 | before surgery | Lordotic curve L1-S1 before surgery in Cobb degrees |
| Kyphotic Curve Th5-Th12 | before surgery | Kyphotic curve Th5-Th12 before surgery in Cobb degrees |
| First Scoliotic Curve | before surgery | First scoliotic curve before surgery in Cobb degrees |
| Second Scoliotic Curve | before surgery | Second scoliotic curve before surgery in Cobb degrees |
| Tilt of the Pelvis Relative to the Horizon | before surgery | tilt of the pelvis relative to the horizon before surgery in Cobb degrees |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| First Scoliosis Curve | 10 days after surgery | First scoliotic curve after surgery in Cobb degrees |
| Second Scoliotic Curve | 10 days after surgery | Second scoliotic curve after surgery in Cobb degrees |
| Tilt of the Pelvis Relative to the Horizon | 10 days after surgery | tilt of the pelvis relative to the horizon after surgery in Cobb degrees |
| Kyphotic Curve Th5-Th12 | 10 days after surgery | Kyphotic curve Th5-Th12 after surgery in Cobb degrees |
| Lordotic Curve L1-S1 | 10 days after surgery | Lordotic curve L1-S1 after surgery in Cobb degrees |
Countries
Russia
Participant flow
Participants by arm
| Arm | Count |
|---|---|
| Neuromuscular Scoliosis Scoliosis surgery: All patients will be implanted with telescopic connector Medtronic CD Horizon Legacy 4.5 and 5.5 under X-ray control (C-arm), intraoperative spinal deformity surgery safety control (NIM Eclipse, Stealth Station S7) and standard anesthetic support. | 71 |
| Total | 71 |
Baseline characteristics
| Characteristic | Neuromuscular Scoliosis |
|---|---|
| Age, Categorical <=18 years | 65 Participants |
| Age, Categorical >=65 years | 0 Participants |
| Age, Categorical Between 18 and 65 years | 6 Participants |
| Age, Continuous | 14 years STANDARD_DEVIATION 3.605 |
| Neuromuscular scoliosis | 71 Participants |
| Race/Ethnicity, Customized Russia | 71 participants |
| Region of Enrollment Russia | 71 Participants |
| Sex: Female, Male Female | 26 Participants |
| Sex: Female, Male Male | 45 Participants |
Adverse events
| Event type | EG000 affected / at risk |
|---|---|
| deaths Total, all-cause mortality | 1 / 71 |
| other Total, other adverse events | 0 / 71 |
| serious Total, serious adverse events | 1 / 71 |
Outcome results
First Scoliotic Curve
First scoliotic curve before surgery in Cobb degrees
Time frame: before surgery
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Neuromuscular Scoliosis | First Scoliotic Curve | 71.4029 degrees | Standard Deviation 27.60483 |
Kyphotic Curve Th5-Th12
Kyphotic curve Th5-Th12 before surgery in Cobb degrees
Time frame: before surgery
| Arm | Measure | Value (MEAN) |
|---|---|---|
| Neuromuscular Scoliosis | Kyphotic Curve Th5-Th12 | 28,7029 degrees |
Lordotic Curve L1-S1
Lordotic curve L1-S1 before surgery in Cobb degrees
Time frame: before surgery
| Arm | Measure | Value (MEAN) |
|---|---|---|
| Neuromuscular Scoliosis | Lordotic Curve L1-S1 | 53,8829 degrees |
Second Scoliotic Curve
Second scoliotic curve before surgery in Cobb degrees
Time frame: before surgery
| Arm | Measure | Value (MEAN) |
|---|---|---|
| Neuromuscular Scoliosis | Second Scoliotic Curve | 44,7125 degrees |
Tilt of the Pelvis Relative to the Horizon
tilt of the pelvis relative to the horizon before surgery in Cobb degrees
Time frame: before surgery
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Neuromuscular Scoliosis | Tilt of the Pelvis Relative to the Horizon | 14.7729 degrees | Standard Deviation 10.19554 |
First Scoliosis Curve
First scoliotic curve after surgery in Cobb degrees
Time frame: 10 days after surgery
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Neuromuscular Scoliosis | First Scoliosis Curve | 31.4667 degrees | Standard Deviation 19.21541 |
Kyphotic Curve Th5-Th12
Kyphotic curve Th5-Th12 after surgery in Cobb degrees
Time frame: 10 days after surgery
| Arm | Measure | Value (MEAN) |
|---|---|---|
| Neuromuscular Scoliosis | Kyphotic Curve Th5-Th12 | 21,7486 degrees |
Lordotic Curve L1-S1
Lordotic curve L1-S1 after surgery in Cobb degrees
Time frame: 10 days after surgery
| Arm | Measure | Value (MEAN) |
|---|---|---|
| Neuromuscular Scoliosis | Lordotic Curve L1-S1 | 49,6700 degrees |
Second Scoliotic Curve
Second scoliotic curve after surgery in Cobb degrees
Time frame: 10 days after surgery
| Arm | Measure | Value (MEAN) |
|---|---|---|
| Neuromuscular Scoliosis | Second Scoliotic Curve | 28,5533 degrees |
Tilt of the Pelvis Relative to the Horizon
tilt of the pelvis relative to the horizon after surgery in Cobb degrees
Time frame: 10 days after surgery
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Neuromuscular Scoliosis | Tilt of the Pelvis Relative to the Horizon | 6.1514 degrees | Standard Deviation 5.85862 |
First Scoliotic Curve
First scoliotic curve 12 months after surgery in Cobb degrees
Time frame: 12 months after surgery
| Arm | Measure | Value (MEAN) |
|---|---|---|
| Neuromuscular Scoliosis | First Scoliotic Curve | 31,6614 degrees |
Kyphotic Curve Th5-Th12
Kyphotic curve Th5-Th12 12 months after surgery in Cobb degrees
Time frame: 12 months after surgery
| Arm | Measure | Value (MEAN) |
|---|---|---|
| Neuromuscular Scoliosis | Kyphotic Curve Th5-Th12 | 22,9581 degrees |
Lordotic Curve L1-S1
Lordotic curve L1-S1 12 months after surgery in Cobb degrees
Time frame: 12 months after surgery
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Neuromuscular Scoliosis | Lordotic Curve L1-S1 | 51.7405 degrees | Standard Deviation 15.08844 |
Second Scoliotic Curve
Second scoliotic curve 12 months before surgery in Cobb degrees
Time frame: 12 months after surgery
| Arm | Measure | Value (MEAN) |
|---|---|---|
| Neuromuscular Scoliosis | Second Scoliotic Curve | 28,5750 degrees |
Tilt of the Pelvis Relative to the Horizon
tilt of the pelvis relative to the horizon 12 months after surgery in Cobb degrees
Time frame: 12 months after surgery
| Arm | Measure | Value (MEAN) |
|---|---|---|
| Neuromuscular Scoliosis | Tilt of the Pelvis Relative to the Horizon | 7,0513 degrees |