Infantile Scoliosis Treatment & Management
- Author: Palaniappan Lakshmanan, MBBS, MS, AFRCS, FRCS(Tr&Orth); Chief Editor: Dennis P Grogan, MD more...
The on decision whether to operate on a patient with scoliosis depends on many factors, such as the following:
Time of onset of the curve (early-onset curves most often require surgery)
Degree and site of the curve (a thoracic curve greater than 40° or a lumbar curve greater than 60° in a child aged 10-12 years often requires surgery)
Response to conservative treatment with a brace
Rate of progression of the curve
Acceptability of the cosmetic appearance of the spine to parents and patient
Growing rods without fusion is preferable until combined posterior and anterior fusion can be done. Growing-rod systems (eg, pediatric Isola instrumentation) may be utilized to prevent curve progression; extensions are needed every 6 months to keep pace with the child's growth until the child has adequate trunk length, which is usually between the ages of 11 and 15 years. The child needs a definitive fusion once skeletal maturity is reached; this involves removal of implants and reinstrumentation. If there is evidence of congenital problems or thoracic insufficiency, another type of growing-rod system (eg, the vertical expandable prosthetic titanium rib [VEPTR]) may first be used.[24, 25, 26, 27, 28]
There is, however, an alternative in very young children in whom the curve progresses: a localizer cast can be applied.[29, 30] A localizer cast can hold the curve and prevent it from progressing further. However, application of localizer casts is a dying art, and it requires proper care and frequent change of the plaster. The localizer cast is applied to the child's trunk under general anaesthesia, with traction to the head and neck via a sling across the mandible and the occiput, and countertraction to the pelvis through another sling. The plaster jacket is applied around the trunk, making sure that there is enough room for the hip movements by stopping just below the level of the iliac wings. Superiorly, the plaster goes around the axillae, leaving the arms and the shoulders free. Serial casting has been recently shown in the literature (2012) to delay the surgery by an average of 39 months in moderate-to-severe early-onset scoliosis.
If needed, in higher thoracic curves, a neck support can be included by extending the plaster. However, this is seldom required, and consideration for surgery should be given in such cases. A window is cut in the front for abdominal expansion. After the first cast, a radiograph is performed to confirm better correction of scoliosis. If satisfactory control is not obtained, casting can be tried 1 more time with proper moulding of the cast only if the treating surgeon is confident that the curve is flexible and that the original cast was not applied properly. If the child feels uncomfortable, especially from the breathing point of view, then the cast should be removed immediately.
The localizer cast needs to be changed every 3 months as the child grows in height, and also as the plaster wears off and the cast loosens. The localizer cast is not tolerated well by older children and, hence, is usually suitable for children under 5 years. Localizer casts can produce pressure sores, especially in children with underlying neurological problems. Foreign objects that find their way into the cast can also cause pressure sores in normal children as well asneurologicallyimpairedchildren.
In a retrospective study of the treatment of patients with idiopathic infantile scoliosis, 31 consecutive patients (average age, 25 mo) with a primary diagnosis of idiopathic infantile scoliosis were reviewed. Treatment modalities included bracing, serial body casting, and vertical expandable prosthetic titanium rib (VEPTR). Of the 31 patients, 17 were treated with a brace, 9 of whom had curve progression and subsequently received other treatments. Of the 8 patients who responded to brace treatment, overall improvement was 51.2%. Patients who received body casts had a mean preoperative Cobb angle of 50.4º and had an average correction of 59.0%. Patients who were treated with VEPTR had a mean preoperative Cobb angle of 90º and had an average correction of 33.8%. The study results suggest that body casting is useful in cases of smaller, flexible spinal curves, and VEPTR is a viable alternative for larger curves.
Vertical expandable prosthetic titanium rib
VEPTR is a recent development in the management of severe scoliosis in skeletally immature patients. See images below.
VEPTR usually is indicated in patients with thoracic insufficiency syndrome (TIS). Apart from having a spinal deformity, patients may have a deformity of the thoracic cage, such as fused ribs or a hypoplastic thorax. VEPTR helps rebuild the chest wall and correct the spinal deformity, thereby allowing the lungs to expand to achieve normal functioning. There are a number of types of VEPTR devices, such as the following:
Cradle-to-cradle — Used in cases of fixed or missing ribs, severe scoliosis, and hypoplastic thorax
Cradle-to-lumbar lamina hook — Used when scoliosis involves the lumbar region or when lower ribs are absent
Cradle-to-S-hook — Attaches upper ribs to the pelvis and is useful in cases in which lower ribs are absent and lumbar bones are weak
Pediatric Isola spine system
The Isola system, as seen in the image below, consists of screws with washers that are applied posteriorly to anteriorly; horizontal to the frontal plane of the vertebral body; and parallel to the apex of the curvature. Screws may be applied through the staples. Closed-top end screws are placed first. A rod is then contoured along the curvature and is cut to size, so that it extends about 1 cm beyond the end screws. The rod is passed between the 2 end screws, and open-end screws with staples are then placed in the remaining intervening vertebrae, using the contoured rod as a guide for positioning the screws. Caps are placed on the intermediate screws, and the rod is rotated approximately 180° to obtain both a coronal correction and a sagittal correction.
Further correction can be accomplished by opening the vertebral spaces with a Cobb elevator after tightening 1 of the intermediate screws. At this stage, further correction can also be accomplished by applying distraction between the screw connector bodies. The disk space that is created can now be filled completely with bone graft material. Vertebral screws are compressed centrally, starting from the top of the screw to the bottom, and then to the top of the next screw. The final compression is applied across the apical vertebrae.
Rods are inserted to prevent progression of the curve, and the rods are extended every 6 months to keep pace with the child's growth. Hooks are used as anchors on the upper part of the curve, and pedicle screws are used in the lower part of the curve. At the apex of the curve, the muscle is not dissected, so as to maintain the blood supply to the bone at the apex.
See the list below:
Improve the general condition of the child by providing appropriate nutrition and making sure the child is capable of undergoing general anaesthesia
Treat any infections appropriately
Require flu prophylaxis for any child with lung problems
Require pneumonia prophylaxis for any child on a ventilator
Operative details for VEPTR
See the list below:
The patient is placed in the lateral decubitus position.
Two incisions are made: a large J-shaped incision medial to the border of the scapula and curving anteriorly, and a small incision made distally so as to apply the distal end of the prosthesis to the spine.
The prosthesis is applied over the rib cage and beneath the skin and muscle.
After the proximal and distal ends are attached, the device is distracted with the aid of expansion pliers.
Intraoperative spinal cord monitoring (somatosensory evoked potentials) has been found to be useful in these cases. If somatosensory evoked potentials change intraoperatively, then decreasing the VEPTR expansion may resolve the issue. Spinal cord monitoring can decrease the incidence of neurologic complications following excessive surgical correction.
The surgical wounds need to be protected with padding to prevent injury. The prosthesis needs to be expanded every 4-6 months as the child grows. Expansion requires that a small incision be made at the site of distraction. When the child stops growing, the device can be removed, and other definitive procedures, such as rib spreading, may be necessary.
The risks associated with anesthesia include bleeding; pneumonia; wound complications (eg, poor healing, infection); device-related problems, such as allergic reaction to metal, or bending, breaking, or loosening of the device; and neurologic deficit due to stretching of the spinal cord from expansion. Crankshaft phenomenon is a complication following isolated posterior fusion surgeries, in which the unfused anterior vertebral bodies continue to grow and cause lordosis and scoliosis. Crankshaft phenomenon is seen when the spine is skeletally immature, and typically occurs during the 2 peak growth velocity periods (ie, from 0-5 years of age and from 10-15 years of age).
Paralysis: Paralysis is the most feared complication of surgery for scoliosis. A survey conducted by the Scoliosis Research Society determined that the incidence of acute neurologic complications resulting from the treatment of scoliosis to be 0.72%.  In infantile scoliosis, because neural axis involvement is significant, the risk of neurologic injury is greater if not recognized preoperatively.
Infection: Infection is a risk with all surgical procedures; antibiotic prophylaxis is essential.
Pseudoarthrosis: Pseudoarthrosis is a failure of the spine to fuse and is more common in adults than in adolescents.
Decompensation: Decompensation occurs because of overcorrection of the spinal curve, in which the curvature of the spine loses its flexibility, causing the patient to lean to 1 side.
Flat-back syndrome: Flat-back syndrome is seen less often now because of technical improvements since the Harrington rod. In this condition, patients have decreased lumbar lordosis and need to hyperextend their hips to stand or need to adopt a flexed-hip-and-knee gait, leading to increased back fatigue.
Low-back pain: There is a risk of low-back pain, especially after lower distal level fusion. This may result from unfused levels of the spine or degeneration of the fused spine.
Rod fractures: Rod fractures can happen in 15% of the cases.  The preoperative magnitude of scoliosis or kyphosis is not associated with the risk of rod fractures. However, the risk factors for rod fractures are prior fracture, single rods, stainless steel rods, small-diameter rods, proximity to tandem connectors, short tandem connectors, and preoperative ambulation. Repeat fractures are common, especially with single rods. Rod replacement, with larger-diameter rods if appropriate, may be a preferred strategy over connecting the broken rods as fractures signal fatigue of the rod.
The complications associated with casting are an increase in the peak inspiratory pressure, which can result in increased respiratory complications as shown by Dhawale et al in 2013. Subclavian thrombosis after casting has also been reported in the literature recently.
Outcome and Prognosis
More favorable outcomes have been associated with male sex, a left-sided curve, a low initial curve measurement, an RVAD of less than 20° in the initial radiograph, and the onset of scoliosis in the first year of life.
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