Infantile and Juvenile Scoliosis: The Crooked Path to Diagnosis and Treatment

Article Outline

• ABSTRACT
• Classification of Scoliosis
• Idiopathic Scoliosis
• Congenital Scoliosis
• Neuromuscular Scoliosis
• Syndromes with Spine and Chest Deformities
• Physical and Radiologic Examination
• Orthosis/Bracing—The First Approach to Scoliosis Treatment
• Serial Casting
  • Cast application
• Halo Traction
• Standards of Care Applied to all Surgical Patients
  • Preoperative phase
  • Intraoperative phase
• Instrumentation of the Posterior Spine with Growing Rods
• VEPTR Implants
  • Procedure description
  • Hybrid rib-to-spine and rib-to-lumbar lamina VEPTR construct
  • Rib-to-pelvis VEPTR construct
  • Rib-to-rib VEPTR construct
• Other Types of Surgical Interventions for Correction of Infantile and Juvenile Scoliosis
  • In-situ fusion
  • Hemi-epiphysiodesis
  • Hemivertebral resection
  • Multiple level anterior and/or posterior spinal fusions
• Postoperative Phase
• Conclusion
• Examination
  • Infantile and Juvenile Scoliosis: The Crooked Path to Diagnosis and Treatment
    • Purpose/Goal
    • Behavioral Objectives
    • Questions
• Answer Sheet
  • Infantile and Juvenile Scoliosis: The Crooked Path to Diagnosis and Treatment
• Learner Evaluation
  • Infantile and Juvenile Scoliosis: The Crooked Path to Diagnosis and Treatment
    • Purpose/Goal
    • Objectives
    • Content
    • Test Questions/Answers
    • Learner Input
• Acknowledgements
• References
• Copyright

ABSTRACT 

Most cases of scoliosis are diagnosed and treated during adolescence; many are detected in school screening programs. For a small percentage of children, however, the onset of scoliosis occurs much earlier than adolescence.

Infantile scoliosis (ie, onset from birth to two years of age) and juvenile scoliosis (ie, onset from three to nine years of age) involve very different diagnoses and treatment regimens than adolescent scoliosis. Early onset scoliosis may resolve with growth or may require nonsurgical treatment (eg, orthosis, body cast); surgical intervention (eg, halo traction, growing rods, vertical expandable prosthetic titanium rib); or a combination of both. AORN J 90 (September 2009) 347-376. © AORN, Inc, 2009.

Key words:  scoliosis , infantile scoliosis , juvenile scoliosis , idiopathic scoliosis , congenital scoliosis , neuromuscular scoliosis , orthosis , halo traction , serial casting , growing rods , vertical expandable prosthetic titanium rib

Scoliosis is an abnormal lateral and rotational curvature of the vertebral column. One segment of the spinal column is a vertebra. Humans usually have 33 vertebrae—seven cervical; 12 thoracic; five lumbar; five sacral (fused into one bone, the sacrum); and four coccygeal (fused into one bone, the coccyx).

The term scoliosis refers to a sideward (ie, right or left) curve in the spine and is derived from the Greek word skol, meaning twists and turns.¹ There are historical references to scoliosis in ancient Hindu materials dating as far back as 3500 BC to 1800 BC and from the writings of Hippocrates around 400 BC.¹, ², ³ The prevalence of the condition is only estimated because scoliosis is not a medical condition for which reporting is mandated. According to the National Scoliosis Foundation, an estimated 6 million people have scoliosis in the United States.³

The Scoliosis Research Society, an international group that studies spinal deformities, has standardized the classifications of deformity and the associated terminology (Table 1).⁴ For the purposes of this article, onset of scoliosis from birth to two years of age is considered infantile, onset from three to nine years of age is considered juvenile, and onset from 10 years to 17 years is considered adolescent. Adult scoliosis has an onset of 18 years and older. Some spine surgeons advocate replacing the age-related terms infantile and juvenile with the broader terms early-onset scoliosis and late-onset scoliosis, depending on whether the onset is before or after five years of age.¹, ²

Table 1. Scoliosis Research Society Glossary of Terms¹

1. Lenke LG; SRS Terminology Committee and Working Group on Spinal Classification. Revised glossary of terms. Scoliosis Research Society. http://www.srs.org/professionals/glossary/glossary.php. Accessed July 1, 2009.

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Classification of Scoliosis 

Eighty percent of patients with scoliosis are classified as having idiopathic scoliosis because a cause cannot be determined. The remaining cases of scoliosis are divided into two types: nonstructural (ie, functional) and structural.¹, ³

Nonstructural scoliosis may be considered compensatory or postural because it may be related to posture, pelvic tilt, flexion deformities at the hip or knee, or to a leg length discrepancy.³ In nonstructural scoliosis, when the patient is seated or recumbent, the lateral curve of the spine resolves.³ Other causes of nonstructural scoliosis are tumors, disc herniation, or spondylolisthesis (ie, forward displacement of a lumbar vertebra on the one below). In these situations, removal of the cause resolves the scoliosis.¹, ³, ⁵

Structural scoliosis may be related to

This article addresses idiopathic scoliosis and scoliosis related to structural causes.

Scoliosis curves are classified as primary (ie, major) or secondary (ie, compensatory/minor). A primary scoliosis curve is a more structural curve with less flexibility and greater potential for clinical deformity. A secondary scoliosis curve is a compensatory curve that balances the primary curve. It is more flexible and is less structural and less deforming in nature.⁵ This distinction is important for treating scoliosis curves because correcting the primary curves results in an improvement of the compensatory curves. Scoliosis treatment depends on three factors: the cause of the condition, the severity of the condition, and the age of the patient at the time of presentation.

The age of onset has important implications for the management of scoliosis. Typically, a teenager with a 60-degree, idiopathic curve is managed quite successfully with a posterior instrumented spinal fusion. If a young child with the same 60-degree curve were managed in the same manner, the anterior spine would continue to grow while the posterior spine remained fused. This would lead to crankshaft phenomenon, which is a rotation of the spine around the posterior fusion mass, resulting in a progressive increase in the spinal deformity.⁶, ⁷ Loss of growth in the spine may result in a foreshortened trunk and a loss of chest-cavity volume, resulting in a loss of normal lung growth that could potentially lead to future serious cardiopulmonary compromise. For this reason, other interventions have been developed for managing early onset scoliosis. These treatments take into consideration the future growth of the child's spine as well as growth of the child's rib cage and lungs.

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Idiopathic Scoliosis 

The most common of all spine deformities in children is idiopathic scoliosis.⁵ The diagnosis of idiopathic scoliosis is one of exclusion after ruling out other causes.⁸ Although the cause of idiopathic scoliosis is unknown, there is a genetic predisposition for development of idiopathic scoliosis.⁹ Infantile idiopathic scoliosis is more prevalent in males and usually is detected by six months of age. It is most common in children of European descent. Infantile idiopathic scoliosis has been estimated to account for only 0.5% of all cases of idiopathic scoliosis in the United States.³

Infantile idiopathic scoliosis curves normally occur in the thoracic spine and are more often convex to the left.³, ⁶ Many of the diagnosed cases of infantile scoliosis spontaneously resolve without treatment by the time the child reaches three years of age.³, ⁶, ¹⁰ Persistent curves can become severe, requiring extensive treatment. Initially, these children are treated with bracing. If compliance with the bracing regimen is a problem or if the curve progresses, serial casting is the next option. The decision to perform surgery is based primarily on the inability to control a curve with nonsurgical interventions.⁹

Juvenile idiopathic scoliosis comprises about 10% to 15% of all cases of idiopathic scoliosis in children.¹¹ Boys are affected slightly more than girls at the younger end of the age spectrum, and the spinal curve is often left-sided. At the upper end of the age spectrum, juvenile idiopathic scoliosis is more like adolescent idiopathic scoliosis, with more girls being affected than boys, and the spinal curve is more often right-sided (Figure 1).¹¹ Some clinicians consider this form of scoliosis to be a malignant subtype of adolescent idiopathic scoliosis (ie, with a tendency to progress to severe or very severe deformity that compromises respiratory function) because of its high rate of curve progression and the need for surgical treatment.³ Curves of 20 degrees or more, especially those demonstrating progression, should be treated rather than observed.⁵ Juvenile curves of 30 degrees or more tend to continue to worsen without treatment; nearly 95% of children with juvenile curves of 30 degrees or more eventually require surgical treatment.¹¹

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• Figure 1. 

  X-ray of a girl with juvenile idiopathic scoliosis in which the curve is right-sided.

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Congenital Scoliosis 

Congenital scoliosis is a fixed spinal deformity present at birth and caused by a deformity in the bony structure of one or more vertebrae resulting in longitudinal spine growth imbalance. The condition affects approximately one in 10,000 Americans,³ and girls are affected more often than boys (60% versus 40%).³, ⁵ Congenital scoliosis (Figure 2, Figure 3, Figure 4) is classified as

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• Figure 2. 

  A computed tomography scan showing a child with combined failures of segmentation and formation.

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• Figure 3. 

  A three-dimensional computed tomography scan showing a lateral view of hemivertebra with resulting spine angulation.

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• Figure 4. 

  A three-dimensional computed tomography scan showing a posterior view of hemivertebra with bone fusion.

Depending on the location of the abnormality and the potential for affecting spinal growth, the deformity can be relatively static and benign or progressive and quite malignant in severity, leading to rapid curve progression. The location of the defect in the vertebra will determine the type of deformity: scoliosis, lordosis, kyphosis, or a combination (Table 2).

Table 2. Types of Deformities in Congenital Scoliosis

1. Stedman's Medical Dictionary. 28th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2006.

Congenital scoliosis is likely to worsen during growth, and the most severe deformities involve the thoracolumbar region of the spine.¹² Patients with this condition require extensive radiologic examinations including three-dimensional computed tomography (CT) scans and magnetic resonance imaging (MRI) scans of the spine. Early detection is important to rule out progressive types of deformities and intraspinal pathology, which can lead to neurological deficits. It is important to rule out other associated spinal cord anomalies, such as Arnold-Chiari malformation; syrinx; lipoma of the cord; tethered cord; diastematomyelia (ie, congenital length-wise division of the spinal cord); and other pathological conditions⁸ (Table 3). Some congenital scoliosis deformities require surgical interventions not addressed in this article, including vertebral osteotomies, short segment fusions, hemivertebrectomy, and surgical correction of the associated neurological conditions.

Table 3. Pathological Conditions Associated with Scoliosis

1. Stedman's Medical Dictionary. 28th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2006.

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Neuromuscular Scoliosis 

Neuromuscular scoliosis is a term that includes the neuropathic (ie, involving the nervous system) and myopathic (ie, involving the muscles) categories of scoliosis.³, ¹³, ¹⁴ This form of scoliosis is associated with many conditions such as cerebral palsy, spina bifida, muscular dystrophy, spinal muscular atrophy, and paralysis from spinal cord injury.¹⁵, ¹⁶ The spinal curve is secondary to muscle imbalance. Children with neuromuscular diseases generally are in one of two categories—spastic or flaccid.¹³, ¹⁴, ¹⁶ Children with spasticity have very tight muscles and poor brain control over their muscles, and they exhibit rigid posturing. Children with flaccidity have weak muscle control and appear “floppy.”¹⁴

The likelihood of neuromuscular scoliosis developing varies with the underlying diagnosis. The age of onset and severity of scoliosis is strongly related to the degree of neuromuscular involvement. Children who are paraplegic or quadriplegic before age 10 and children who have spina bifida with a high level of paralysis have a 100% chance of developing neuromuscular scoliosis.¹⁴ In contrast, among patients with cerebral palsy, patients with spastic quadriplegia have a 60% to 70% chance of developing neuromuscular scoliosis; a child with hemiplegic cerebral palsy has only a 5% chance.¹⁴ The age of onset also varies with etiology related to the progression of symptoms. A child with spinal muscular atrophy presents with a spinal deformity at a younger age than a child with muscular dystrophy.¹⁴ In general, the greater the degree of neuromuscular involvement, the greater the likelihood of scoliosis occurring and the greater the severity.¹³ Early detection is important, children with neuromuscular involvement should be checked annually and assessed for the development of spinal deformities.¹⁴

Scoliosis in a child with neuromuscular disease is only one facet of his or her disease. These children may have complex conditions with multisystem involvement and difficulty performing activities of daily living. Potential comorbidities include

The diagnosis of neuromuscular scoliosis is usually made when a caregiver notices a problem with the child's ability to sit in his or her wheelchair. When the curve is present, it continues to worsen as a result of posture and gravity. Modifications may be made to the wheelchair, and the child may be braced and padded to prevent skin breakdown, but the curve will continue to progress. The change in pelvic obliquity (ie, horizontal angulation of the pelvis in the frontal plane) results in an imbalance requiring the child to use one or both hands for support, and thus functionally losing the use of one or both arms.¹⁴

As the curve grows larger, it will begin to affect the child's pulmonary function. Eventually pulmonary capacity is reduced by collapse of the thorax and elevation of the diaphragm. This may be manifested by the child developing recurrent pneumonia, especially if the patient has coexisting muscle weakness secondary to neuromuscular disease. For some children with neuromuscular involvement (eg, Duchenne muscular dystrophy), this deterioration of pulmonary function may require earlier surgical intervention depending on skeletal indications (eg, Risser sign).¹⁶

Patients with more involved neuromuscular issues usually progress to needing some type of spinal stabilization. During their juvenile years, these children's curves usually can be controlled by bracing and nonsurgical interventions, but these measures usually fail at the onset of the adolescent growth spurt.¹³, ¹⁴, ¹⁶ Surgical options to treat neuromuscular scoliosis generally are divided into two groups based on whether patients are ambulatory with curves that are more idiopathic in nature or wheelchair dependent with large “C” shaped paralytic curves. In ambulatory patients with less involved neuromuscular issues and a pelvis that is generally level, the fusion extends from the levels above and below the curve, similar to the treatment for idiopathic scoliosis. In nonambulatory children with more involved neuromuscular issues, the fusion usually extends from the upper thoracic area to the sacrum to correct the pelvic obliquity and achieve a stable sitting position.¹⁴

Surgery enables nonambulatory patients to sit erect, improving functions of daily living. For patients with pulmonary involvement, surgery can help lessen occurrences of pneumonia and decrease swallowing and aspiration problems. The general outcome is an overall improvement in their quality of life.

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Syndromes with Spine and Chest Deformities 

As with neuromuscular scoliosis, children with syndromes present with a wide variety of complexities in addition to their spine and chest deformities. The age of onset is dependent on the type of syndrome and associated congenital defects. For example, children with syndromes that include congenital rib fusions require earlier intervention related to pulmonary issues than children who develop syndrome-related scoliosis at a later age. Many of these children present with cardiac, pulmonary, neurological, renal, and other associated medical issues that complicate their care (Table 4). Surgical team members need to be aware of the syndromic comorbidities that may require special interventions. These may include

Table 4. Terms Used with Neuromuscular Disorders or Syndromes of the Spine or Chest

1. Baum VC, O’Flaherty JE. Anesthesia for Genetic, Metabolic, and Dysmorphic Syndromes of Childhood. Philadelphia, PA: Lippincott Williams & Wilkins; 1999.

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Physical and Radiologic Examination 

The initial evaluation of a child with scoliosis should include a detailed history and physical examination. The clinician reviews the family history because of the genetic basis for many causes of scoliosis. As a three-dimensional condition, scoliosis requires careful analysis in both sagittal and coronal planes.⁹, ¹² The clinician first performs an Adams forward-bending test—named after William Adams, MD, who first described the maneuver—to assess spinal movement and rotational prominences such as pronounced rib hump.⁹ The majority of patients with early-onset scoliosis require an MRI scan for a complete spinal evaluation to rule out causes related to neuropathology.¹⁷

Standard radiological tests that are performed for diagnosing scoliosis are standing posterior-anterior and lateral x-rays of the spine. The lateral views are important for assessing the three-dimensional properties of scoliosis to determine whether the child has an associated kyphosis, lordosis, or junctional curve in the sagittal plane. Supine, maximum side-bending x-rays to the left and right are taken to assess the flexibility of the curve. These films help determine the levels of instrumentation for idiopathic scoliosis by assessing the flexibility of the spine. The bending films also may be helpful in determining whether a patient would benefit from an anterior spinal release before undergoing a posterior instrumented correction.

The Cobb method, a universally agreed upon measurement, has been in use since 1948 to measure the magnitude of spinal curves.³ The radiologist, surgeon, or resident draws lines parallel to the end plates of the vertebral bodies at the two end vertebrae of the curve to be measured. He or she draws a second line perpendicular to each of the lines. The angle formed by the intersection of these lines is the Cobb angle (Figure 5). There is a standard measurement error of three to five degrees based on the way the x-ray was taken, the position of the patient, and the way the lines are drawn. This error factor should be taken into account since the Cobb measurement may not be exactly the same each time the spine is measured.¹⁸ The Cobb angle is useful as a monitoring tool to measure progression or correction of scoliosis curves.¹⁹ A limitation of the Cobb angle is that it does not measure the amount of vertebral rotation or alignment of the spine, nor does it account for the degree of tilt on the endpoint vertebrae. Rotation is assessed by looking at other indicators on the films, such as vertebral pedicle alignment to the midline.

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• Figure 5. 

  Cobb angle measured on an x-ray of a patient with nonstructural scoliosis related to a syrinx.

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Orthosis/Bracing—The First Approach to Scoliosis Treatment 

Descriptions of scoliosis and its treatment have been mentioned in literature throughout the ages. Past treatments included everything from posture corrections and exercises to plaster jackets and mass-produced corsets.³ In the 19th and early 20th centuries, treatments for scoliosis primarily included the use of braces and other devices that stabilized the spine and reduced or prevented further disfigurement.³

The terms brace and orthosis are interchangeable; orthosis is the professional term and brace is the lay term. Thoracic lumbar sacral orthosis (TLSO) is a descriptive term, which applies to any orthosis that addresses the thoracic, lumbar, and sacral portions of the spine (Table 5). The type of orthosis used is regional, based primarily on the physician's personal preference and the availability of skilled orthotists.

Table 5. Types of Orthoses for Treatment of Scoliosis¹

Table courtesy of Todd Dewees, CPO, staff orthotist, Shriners Hospital, Portland, OR.

1. Manual of Brace Treatment for Idiopathic Scoliosis. Milwaukee, WI: Scoliosis Research Society; 2003. http://www.srs.org/professionals/bracing_manuals. Accessed July 1, 2009.

The goal of bracing is to slow the inevitable progression of the curve and allow the child to grow until a more definitive treatment, such as surgery, is performed (Figure 6a, Figure 6b).⁷, ²⁰ Whether a child is a candidate for a brace is contingent on the flexibility of the curve, as determined by the bending radiographs.²⁰ A brace will do little good for a curve that is rigid and does not correct (ie, get smaller) on the bending films. A brace rarely corrects scoliosis permanently. Braces also do not work well for a congenital deformity but may be used to correct compensatory curves.

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• Figure 6a. 

  X-ray of a patient with infantile scoliosis without a brace.

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• Figure 6b. 

  X-ray of the same patient with the curves controlled by a brace.

Children usually are instructed to wear the brace full-time, removing the brace only for bathing and special occasions. Patients with thoracolumbar and lumbar curves are more successfully treated with a brace; one treatment option is nighttime bracing (ie, wearing the brace 10 to 12 hours at night). Cervical curves are not typically treated with a brace. As the child grows, new braces need to be fabricated every 12 to 18 months. Most braces function by exerting pressure on the rib cage; therefore, physicians are concerned about pulmonary function and lung development in young children. Modifications may be made to braces for children with special needs (eg, gastric reflux, feeding tubes, colostomies).²⁰

At Shriners Hospital, Portland, Oregon, the Boston and Providence nighttime braces are the most commonly used orthoses for treating juvenile idiopathic scoliosis. The next most common is the soft Boston-type orthosis for treatment of neuromuscular low-tone curves and infantile scoliosis. The low-profile scoliosis or kyphosis corrective orthosis is used less frequently, but is the preferred device for treating Scheuermann's kyphosis. The Milwaukee brace, one of the first braces developed for scoliosis treatment, is less popular because of its cosmetic design. This brace is made of leather and metal with a high neck ring and shoulder supports. It is the only brace that can manage curves in the upper spine and may include a chin extension if needed.²⁰ It takes a skilled orthotist to make it fit comfortably.

Approximately 70% of TLSOs are measured, not molded. If a child is progressing from serial casting to a TLSO, the mold for the orthosis will be made in surgery at the time of the final cast application. Whether the brace is measured or molded, it typically takes about two weeks to construct (Figure 7).

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• Figure 7. 

  Sample braces (back row, from left) Milwaukee brace with mandibular extension; Providence brace for a double curve; Boston brace for a left-thoracic/right-lumbar curve; and Boston brace for a right-thoracic/left-lumbar curve. (Front row, from left) posterior view of a Boston brace; posterior view of a soft Boston brace; low-profile, corrective kyphosis brace; and Providence brace for a lumbar curve.

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Serial Casting 

A cast can be more efficient than bracing to manage spinal curves, with the added advantage of patient compliance since it cannot be removed. Many parents find casting preferable to bracing because a cast eliminates the difficulty of putting a brace on an uncooperative young child.²¹ Indications for casting include noncompliance with bracing or a progression of the curve despite bracing. After a course of serial casting, the deformity may correct sufficiently to allow bracing to be reinstituted.

In some cases, casting may be a definitive treatment. Research in the United Kingdom demonstrated that treating noncongenital scoliosis with serial casts beginning at 12 months of age with an average curve of 32 degrees resulted in a reduction of scoliosis to less than 10 degrees at maturity.²¹ Patients who started treatment at 18 months or later with larger curves averaging 52 degrees achieved less correction but maintained their deformities at a similar degree of magnitude. This means their curves did not progress, which is an achievement in this age group. Casting, with the goal of curing the scoliosis, requires frequent cast changes under anesthesia during the period of rapid growth for children younger than age two.¹⁰, ²¹ Children older than two years of age require less frequent cast changes and may have a higher rate of recurrence. A brace is still needed after the casting treatment to maintain correction while the child is growing.¹⁰, ²¹

Cast application 

The surgeons at Shriners Hospital, Portland, use the Cotrel casting technique, commonly referred to as a Risser cast.² If the patient is undergoing a body cast application for the first time, teaching that includes cast care and play therapy is initiated for the patient and his or her family members. Radiographs of the spine (eg, posterior-anterior, lateral, bending films) are performed to determine the degree of scoliosis needing correction and where the corrective forces should be applied. X-rays may be performed out of the cast to evaluate the curve progression.

For children who have already been in a cast, the cast is removed before surgery to allow time for bathing and skin assessment. The nurse fits the patient with a silver-impregnated T-shirt. Silver has antimicrobial properties that decrease skin breakdown during the eight to 12 weeks that the patient will wear the cast. The patient is transferred to a modified pediatric casting table (ie, Mehta table), a specially designed table that allows circumferential body cast application while traction is applied to the patient's head and pelvis to allow for correction of the scoliosis (Figure 8, Figure 9). Special care is taken to position the patient on the table with a head support, padding to the arms, slings for the feet to suspend the legs, a shoulder support, and perineal support to hold the patient suspended.

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• Figure 8. 

  Pediatric body casting table designed and built by Rob Eldridge, a registered technician in prosthetics and orthotics at Shriners Hospital, Portland, OR.

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• Figure 9. 

  Charles d'Amato, MD, FRCSC, pediatric orthopedic spine surgeon, applies a body cast with traction while the patient is under general anesthesia.

When the surgical team has finished positioning the patient, the table pad is lowered to allow complete access for cast application. Traction is applied with a halter traction device (ie, chin/occipital strap) and a series of soft straps wrapped around the waist just above the iliac crests then tightened with a ratchet to improve spinal alignment. The cast application requires multiple participants.

A combination of stockinette, cotton cast padding, felt, and casting foam are used to pad and protect bony areas and pressure points. Surgeons' preferences for the molding cast materials vary. Some surgeons prefer plaster because they believe that plaster achieves a better mold and correction, although plaster takes longer to dry. Other surgeons prefer rapid-dry, stretchable fiberglass, which also is used for orthotic molds.

When the surgeon has applied the base layer, he or she obtains correction with a combination of traction and manual pressure. When the base layer has hardened, the radiology technologist takes an anterior-posterior radiograph of the spine to assess correction. After the surgeon has reviewed the radiograph, he or she completes the cast application. The surgeon then applies a final layer of colored fiberglass; the patient and/or family members are allowed to select the color before the procedure. The surgeon uses a cast saw to trim the cast to allow full movement of arms and legs, and creates an abdominal hole for abdominal expansion (Figure 10). For patients with more involved comorbidities, care is taken to allow access to gastric tubes or other abdominal stomas. The surgeon then performs a neurological assessment of the patient during recovery from anesthesia when the patient is able to respond to verbal commands. The nurse finishes the cast before the patient is discharged, making sure to pad any potential problem areas with moleskin. The cast is changed every eight to 12 weeks until the child is eligible for a brace or has a surgical intervention.

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• Figure 10. 

  Completed body cast with abdominal hole for abdominal expansion.

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Halo Traction 

Patients with severe, inflexible curves sometimes require more extensive measures to improve spinal alignment. Halo traction uses the patient's own body weight to achieve some correction of rigid curves. Counterweights attached to the halo are used to provide additional traction force.², ²² The goal is to decrease the curvature in preparation for other interventions such as a cast application or instrumentation with a growing rod or vertical expandable prosthetic titanium rib (VEPTR™).

An open-back halo ring is placed on the patient under general anesthesia. Halo size is determined by the circumference of the patient's head measured 1 cm above the eyebrows and ears. When the patient's head circumference is in the upper limits of a halo ring size, the large ring size is used.²³

The surgeon may place temporary non-penetrating pins to position the halo before inserting the skull pins. The surgeon may inject a local anesthetic with epinephrine after the circulating nurse preps the patient's skin with povidone-iodine paint. The surgeon positions the skull pins on or below the equator of the skull in a balanced manner to maximize fixation, using four to eight pins based on the size of the patient and anticipated traction force. More pins are used in small children to help distribute the load because their bones are thinner. Usually only four skull pins are needed for patients older than age eight. The surgeon finger-tightens the sterile pins until they advance through the skin, after which the surgeon advances the pins with a torque-limiting screwdriver through the outer table of the skull. The surgeon takes care to ensure that the skin is not under tension. The surgeon then tightens locking nuts on the ring, taking care not to advance the skull pins. A traction bar attached to the halo is used with a series of ropes, pulleys, and weights to maximize traction therapy. The circulating nurse applies an antibiotic or povidone-iodine ointment to the pin sites. After the patient emerges from anesthesia, the postoperative nurse performs regular neurological checks for cranial nerve function to monitor the patient for any changes or deficits.

The patient sleeps in a circle electric bed (Figure 11) with the head of the bed elevated and weights applied for the duration of their traction treatment. As time progresses, the weight amount is increased as the surgeon deems necessary. The child usually remains in traction for four to eight weeks. Circle electric beds are considered antiquated and are difficult to obtain. Shriners Hospital, Portland, has two circle-electric beds but has been unable to obtain more, so patients frequently have to wait for a bed to become available.

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• Figure 11. 

  Circle-electric bed for halo traction.

Members of the physical therapy department at Shriners Hospital, Portland, have been extremely creative in developing modified wheelchairs, walking frames, tricycles, and suspension frames for shower stalls that allow the patients some degree of mobility while continuing traction therapy (Figure 12, Figure 13, Figure 14). Regular radiographs are taken to monitor spinal correction in traction. The degree of correction obtained can speed up or delay the impending surgical procedure.

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• Figure 12. 

  Tricycle adapted for halo traction.

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• Figure 13. 

  Four-point walker adapted for halo traction.

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• Figure 14. 

  Standard wheelchair adapted for halo traction.

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Standards of Care Applied to all Surgical Patients 

When nonsurgical interventions fail, some patients require a surgical procedure to limit progression of their spinal deformity. With infantile and juvenile scoliosis, the challenge for spine surgeons is to stop the progression of the curve without limiting future growth of the spine. Shriners Hospitals are dedicated to helping children maximize their capabilities and live happy, healthy, productive lives. This dedication includes an adherence to the National Patient Safety Goals established by the Joint Commission. All patients undergoing surgical procedures at Shriners Hospital, Portland, receive the following standards of care.

Preoperative phase 

Before the patient arrives in the preoperative area, the preoperative nurse reviews the patient's electronic medical record verifying allergies and reviewing medications and the history and physical examination. When the patient arrives in the preoperative area, the nurse greets the patient and his or her family members and asks them to verify the patient's name and date of birth as they appear on the patient's wristband and surgical consent. The nurse confirms the patient's NPO status, allergies, the presence of a weight-based emergency medication form, and the medication administration record from the Reconciliation of Medications Across the Continuum form. The nurse takes the child's developmental age into consideration and offers age-appropriate options of care whenever possible. For instance, the nurse gives every child a toy to accompany him or her to surgery, and if a cast is to be applied, the nurse asks the child or parent to select a color for the cast. The preoperative nurse verifies that all consents needed for the procedure (eg, blood administration consent, vendor consent, surgical consent, interhospital transfer consent to the pediatric intensive care unit [PICU] at the adjacent university hospital) are present and complete. If the surgical consent has not been completed before the patient's arrival, the nurse contacts the attending surgeon. After completing all required informed consents, the surgeon and the patient or a family member perform the surgical site marking, ensuring that the surgeon's initials will be visible after prepping and draping. The nurse then completes the secondary preoperative checklist, which was initiated by the inpatient care nurse, and completes a report for the circulating nurse. The preoperative nurse then takes time to address the patient's and family members' questions and concerns.

The circulating nurse arrives in the preoperative area to assess the patient. After introducing herself or himself to the patient and family members, the nurse reviews the patient's medical record (eg, history and physical assessment, laboratory results, informed consents, preoperative checklist). The nurse then assesses the patient (eg, musculoskeletal needs, NPO status) and describes the surgical phase. After answering any questions that the patient or family members have, the nurse reassures the family that they will be provided with updates throughout the surgical procedure.

The preoperative nurse then gives the circulating nurse a verbal hand-off report and the circulating nurse develops a patient-specific care plan to fit the patient's needs and reflect interventions necessitated by the procedure being performed (Table 6). After the anesthesia care provider performs a brief preoperative reassessment, the circulating nurse and anesthesia care provider transport the patient to the OR. The preoperative nurse may accompany the patient to the OR and assist as a second circulator to start the surgical procedure.

Table 6. Nursing Care Plan for Pediatric Patients Undergoing Surgery for Scoliosis

The attending surgeon, fellow, or resident is responsible for obtaining and verifying the patient's x-rays and verifying that implants and equipment needed for the procedure have been communicated to the surgical team. The circulating nurse ensures that these are available for the procedure.

Intraoperative phase 

On arrival in the OR, the circulating nurse assists the anesthesia care provider in placing all routine monitoring equipment, after which the anesthesia care provider administers a general anesthetic. The circulating nurse remains with the patient through the induction. Children are given a choice in selecting their method of care whenever possible within the constraints of patient safety. Some patients have an IV catheter inserted in the preoperative area with their family members present; others elect to have a mask induction with the IV placed after they are asleep.

When the anesthesia care provider has indicated that the patient is ready to be positioned, the attending surgeon supervises the positioning of the patient. The surgical team then performs an initial time out while the patient is still visually identifiable, verifying correct patient, correct position, correct procedure, x-rays, antibiotic administration, and availability of equipment and implants. A final safety time out is performed when the patient is prepped and draped just before the scrub person passes the scalpel to the surgeon. The circulating nurse records the time out in the patient's electronic perioperative record.

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Instrumentation of the Posterior Spine with Growing Rods 

When scoliosis cannot be controlled by serial casts or braces, growing rods can be used to maintain spine length until a definitive correction can be performed when the patient nears skeletal maturity. The purpose of the growing rod procedure is to allow for continued, controlled growth of the spine.²⁴, ²⁵ Growing rods are manufactured by several companies; the type used is based primarily on the surgeon's choice. One commonality of the systems used is the availability of pediatric pedicle screws and hooks.

The OR is prepared as for a posterior spinal fusion. After general anesthesia is induced, the electroneurology technologist places the neurological leads for somatosensory-evoked potentials (SSEPs) and motor-evoked potentials (MEPs) to monitor the patient's spinal cord function during the procedure. The circulating nurse inserts a latex-free, temperature-monitoring, indwelling urinary catheter. When all lines and monitors are secured, surgical team members position the patient in the prone position on a special spine table. The spine table allows modifications based on the patient's size with interchangeable chest, hip, and thigh pads. The table also adapts for either solid leg support or a sling support that allows versatility for positioning patients with contractures or deformities. Smaller patients may be placed in the prone position on a radiolucent table with chest rolls and gel pad support of the extremities. The patient's arms are placed in the prone flexed position unless the patient has physical limitations.

The anesthesia care provider uses size-appropriate, prone face pillows with endotracheal tube access to support the patient's head and neck, taking care to prevent ocular pressure. The circulating nurse ensures that all of the patient's bony prominences are well padded. The nurse then preps the patient's skin, after which the surgeon and scrub person drape the patient in the usual sterile fashion. Usually, the circulating nurse and scrub person set up an autotransfusion device. The circulating nurse measures blood loss during the surgery and, if needed, processes the collected red blood cells so the anesthesia care provider can return them to the patient. Fluoroscopy may be used to confirm the desired levels of fixation.

The surgeon makes a midline incision down to the superficial fascia. He or she splits the fascial layer distally and proximally at the desired fixation levels, with the fascia remaining intact over the mid-portion so the rods will be placed on top of the intact fascia. The surgeon performs a subperiosteal dissection along both sides of the spinous process and out over the lamina to the pedicles of the spine. Usually, the surgeon inserts four multi-axial pedicle screws into the desired two distal vertebrae. For the proximal portion of the construct, the surgeon strips the posterior aspects of the vertebrae and uses hooks (eg, pedicle, transverse process, sublaminar) for proximal fixation. The surgeon cuts the titanium rods with extra length distally for future lengthenings and may contour the rods to give some correction and to reconstruct the sagittal plane contour. The surgeon connects the rods to the screw and hook construct. The surgeon can then compress or distract the hooks and pedicle screws to obtain correction of the scoliosis.

After correction is achieved, the surgeon tightens all components using a torque-limiting screwdriver. The surgeon may place one or more cross-links between the rods for additional stability. The surgeon may use fluoroscopy throughout the procedure to evaluate implant placement, especially during pedicle screw insertion. The surgeon obtains autograft bone using a high speed burr or Capner gouge. This autograft bone provides a fusion mass to supplement decortication of the screw and hook fixation sites. If indicated, the surgeon may supplement with allograft. The surgeon copiously irrigates the wound with normal saline and then closes the surgical incisions. The radiology technologist takes an x-ray after the procedure is complete to assess correction and verify the location of the implants.

Before discharge, the team fits the patient for a non-corrective orthosis for stability until bone fusion is achieved at the implant sites. The patient wears the orthosis for three months or until bone fusion is demonstrated. Some patients continue to use soft braces for sports or day braces to help maintain correction.

Lengthening of the growing rod, which is a much simpler procedure, usually is performed every six months as the patient continues to grow. The surgeon makes small incisions over the distraction lock sites. The surgeon loosens the lock and uses a distractor to obtain the desired increase in length of both rods. The procedure is performed under fluoroscopy and a postoperative x-ray is taken to assess correction.

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VEPTR Implants 

Vertical expandable prosthetic titanium rib implants are approved by the US Food and Drug Administration to mechanically stabilize and expand the thorax in skeletally immature patients with thoracic insufficiency syndrome (TIS).⁶, ²⁴ Thoracic insufficiency syndrome describes the inability of the thorax to support lung growth or normal pulmonary function.²⁶, ²⁷ Thoracic insufficiency syndrome may occur in patients with congenital and noncongenital spine deformities (Figure 15).

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• Figure 15. 

  Congenital scoliosis demonstrating fusion from the thoracolumbar junction to the sacrum. Thoracic insufficiency syndrome is related to abdominal crowding/displacement.

Congenital cases involve children with spinal or chest wall deformities that limit the growth of the chest wall or spine severely enough to impair pulmonary function.²⁶ When rib fusions or a spinal deformity limit chest wall growth, the lungs fail to develop. Breathing is compromised by decreased lung capacity and rigidity of the chest wall. This process is progressive, and if left untreated, may even result in death. In cases of neuromuscular scoliosis, breathing is compromised by the inability of the chest wall muscles to support respiratory effort. The VEPTR device in these children may prevent collapse of the thorax as the spinal curve increases.

There are contraindications, however, to the use of the VEPTR. The device is contraindicated in patients who

The goals of treatment with the VEPTR device are to increase thoracic volume, obtain thoracic symmetry, and improve thoracic function. The long-term goal is to maintain these improvements throughout the patient's growth phases while maintaining spinal alignment and allowing spinal growth. At Shriners Hospital, Portland, the VEPTR surgery is performed primarily

Expansion surgery is performed every six months. This requires a less invasive surgical procedure with two small incisions over the distraction clip sites. The distraction clip is removed, the device is expanded, and a new clip is placed. Revisions of the construct may be necessary as the child grows, but revisions usually are timed with scheduled expansions.

A variety of VEPTR-based expansion thoracoplasty techniques can be used to increase chest volume and allow for growth of the lungs while indirectly correcting the scoliosis without fusing the spine. Construct options include

Procedure description 

The VEPTR-based expansion thoracoplasty procedure is performed under general anesthesia. Some patients undergoing VEPTR procedures require fiber optic intubation because of difficult airway management issues related to the syndromes treated with the VEPTR procedure. After general anesthesia is induced, the electroneurology technologist places the electrodes for SSEPs and MEPs for spinal cord monitoring. The surgical team positions the patient in the prone position with rolls placed transversely under the upper chest and pelvis on a radiolucent OR bed, taking care to keep the patient's abdomen free. The circulating nurse preps the patient's skin, after which the surgeon and scrub person drape the surgical site in standard sterile fashion. The circulating nurse and scrub person set up an autotransfusion device, and if needed, the circulating nurse processes the collected red blood cells and the anesthesia care provider returns them to the patient.

Hybrid rib-to-spine and rib-to-lumbar lamina VEPTR construct 

The surgical approach to these two procedures is similar. The surgeon makes two small incisions overlying the indicated spinous processes using fluoroscopy for guidance. The surgeon makes the first incision at the lumbar site to the laminar pedicles. He or she then inserts screws into the pedicles or hooks around the pedicles, then verifies the position with fluoroscopy. The surgeon makes the second incision over the desired rib-fixation point down to the fascia. The surgeon undermines the fat layer so that the fascial incision can be made in a plane, distinct from that of the skin incision to allow better coverage of the implants. Typically, the surgeon identifies the third and fourth ribs on the affected side and places two VEPTR cradles over the ribs (Figure 16).

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• Figure 16. 

  Postoperative x-ray of a patient with cat eye syndrome who underwent rib-to-spine vertical expandable prosthetic titanium rib implantation.

The surgeon inspects the chest for a pneumothorax and, if needed, places a chest tube, which the circulating nurse and scrub person connect to a water-seal drainage reservoir. The length of the proximal and distal extension devices takes into consideration the need for future expansion procedures. The surgeon contours the lower portion as needed and cuts the extension rods to the appropriate length. The surgeon passes the rod through a tunnel created under the muscle and subcutaneous tissues using a 20-Fr chest tube. The surgeon then connects the rod to the rib-cradle construct and then to the pedicle screws.

Distraction is then carried out. For bilateral cases, the surgeon duplicates the procedure on the opposite side of the spine. After the rod is distracted and correction is obtained, the surgeon performs the final tightening. The surgeon secures the extension rods with a distraction lock and may use transverse bars, extensions, and connectors to allow variations of attachment options. The surgeon applies a bone graft to the lumbar portion of the construct to promote bone fusion.

Rib-to-pelvis VEPTR construct 

If the VEPTR construct is rib-to-pelvis, the surgeon makes an incision over the iliac crest with an additional incision in the iliac crest apophysis. The surgeon widens the incision with a Freer elevator and a 90-degree pelvic hook from the VEPTR set. A rib-pelvis hybrid extension device is chosen that allows for future expansions. The surgeon contours the lower portion into lordosis and cuts the device to the appropriate length. The surgeon uses vaginal packing forceps to tunnel under the muscle and subcutaneous tissues from the superior wound to the lower wound. The surgeon then puts a 20-Fr chest tube into the proximal portion of the wound, passes the VEPTR extension subcutaneously, and secures the extension from the pelvic hook to the rib cradle using a variety of connection options. Distraction is then carried out. If the procedure is to be done bilaterally, the surgeon performs the same steps on the opposite side (Figure 17). When distraction and correction have been achieved, the surgeon performs the final tightening.

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• Figure 17. 

  Postoperative x-ray of a patient with arthrogryposis who underwent bilateral rib-to-pelvis vertical expandable prosthetic titanium rib implantation.

Rib-to-rib VEPTR construct 

A rib-to-rib construct allows for attachment from the superior rib to the inferior ribs using opposing rib cradles (Figure 18). The surgeon takes special care to examine the pleura for tears, and if needed, places a chest tube, which is connected to a water-seal drainage reservoir.

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• Figure 18. 

  Postoperative x-ray of a patient who underwent bilateral rib-to-rib and bilateral rib-to-pelvis vertical expandable prosthetic titanium rib implantation.

After irrigation and wound closure, the surgeon injects local anesthetic along the wound edges to assist with postoperative pain control. The surgeon and scrub person apply sterile dressings in a protective double layer and place compressive bandages on the incisions. The radiology technologist takes a postoperative anterior-posterior chest x-ray to assess the construct and evaluate for pneumothorax. Patients undergoing primary VEPTR with chest wall expansion are transferred to the PICU. Patients undergoing revisions are transferred to the standard postanesthesia care unit (PACU).

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Other Types of Surgical Interventions for Correction of Infantile and Juvenile Scoliosis 

Four other surgical techniques (ie, in-situ fusion, hemi-epiphysiodesis, hemivertebral resection, multiple level anterior and/or posterior spinal fusions) also may be used for this age group. Complications may be associated with these procedures in young children, however, because growth of the spine may result in curve progression or rotational deformities.¹¹, ²⁴

In-situ fusion 

In-situ fusion, meaning fused “where it is,” may be performed via an anterior or a posterior approach. This involves a limited correction of the curve and is performed to prevent further serious deformity of the spine.¹¹, ²⁴ This technique is used almost exclusively for short segment, congenital deformities.

Hemi-epiphysiodesis 

Hemi-epiphysiodesis is performed to limit growth on one side of the spine while allowing corrective growth on the other side.¹¹, ²⁴ Although past reports of the use of this technique have been disappointing, there has been a recent resurgence using staples in the anterior spine as a substitute for bracing in juvenile and adolescent scoliosis.

Hemivertebral resection 

Hemivertebral resection is performed in cases of congenital vertebral deformity. The approach may be anterior via a lateral thoracotomy approach, posterior, or combination anterior/posterior procedure. When the hemivertebra is removed, the vertebrae proximal and distal to the deformity are fused together with instrumentation. Only a short segment of the spine needs to be corrected with instrumentation (eg, two to three levels).

Multiple level anterior and/or posterior spinal fusions 

Multiple level anterior and/or posterior spinal fusions are rarely performed in cases of infantile and juvenile scoliosis. When the patient nears skeletal maturity, a definitive spinal fusion with instrumentation, autograft, or allograft will be performed. Posterior approach with instrumentation is the most common surgery performed for spinal fusion. Anterior approach with instrumentation is generally used for single thoracic or single lumbar curves. Some patients with more rigid curves require an anterior release followed by a posterior spinal fusion with instrumentation.¹¹, ²⁴ In cases in which a posterior fusion must be performed when the patient is still expected to grow significantly, anterior release and fusion to promote anterior growth arrest may be indicated to prevent twisting of the spine with later loss of correction. This problem is known as a crankshaft phenomenon.

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Postoperative Phase 

Teamwork and interactions between the OR team and the PACU nurses are crucial. The assigned PACU nurse frequently is present in the OR at the completion of the surgical procedure to assist with transferring the patient to the stretcher or postoperative bed or crib. The PACU nurse reviews the patient's electronic medical record before the procedure is completed and receives a verbal hand-off report from the circulating nurse and the anesthesia care provider with information pertinent to postoperative patient care. Family members are allowed in the PACU when the patient has emerged from the anesthetic. The PACU nurses then address any questions or concerns expressed by the patient or family members before completing the hand-off report to the receiving medical/surgical unit nurse.

If the patient is being transferred to the PICU of the university hospital, the circulating nurse gives a full telephonic report to the receiving PICU nurse. The transport team, which includes the anesthesia care provider, two certified pediatric advance life support RNs, the surgical fellow or resident, and a certified nurse's aide, is fully briefed by the anesthesia care provider and circulating nurse. If the circulating nurse does not accompany the patient during transport, the pediatric RNs give a copy of the circulating nurse's report to the PICU RN to answer any questions that arise. On arrival in the PICU, the anesthesia care provider, transport RN, and surgical resident or fellow give a full patient care hand-off report to the PICU physician and PICU RN.

When stable, the patient returns from the PICU to the Shriners Hospital, Portland, Oregon, inpatient nursing unit. The PICU nurse gives a hand-off report to the inpatient nurses including all the information pertaining to the patient's postoperative intensive care course. The receiving nurse performs a nursing assessment and a member of the medical staff performs a readmission history and physical examination and completes a progress note. The patient remains on the inpatient unit until he or she meets discharge criteria.

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Conclusion 

Early onset scoliosis is a complex problem requiring a dedicated treatment approach on a long and potentially “crooked” path. Nonsurgical treatments such as orthosis or casting require patient and family compliance, with ongoing modifications and/or replacements as the child grows. Surgical treatment with halo application results in weeks and possibly months of hospitalization for traction therapy. After initial instrumentation with growing rod or VEPTR implants, surgical procedures are required every six months to lengthen the rods. Revisions and/or replacement of instrumentation may be needed as the child grows and because of metal fatigue, resulting in implant breakage or displacement.

The child's caregivers bear a great deal of responsibility for the care and treatment of a child with early onset scoliosis, but the multidisciplinary medical and surgical team provides invaluable support to the family. It would be an arduous task without a concentrated effort of physicians, nurses, orthotists, therapists, and numerous other ancillary staff members sharing the responsibility. Interventions begin from the time the child is diagnosed with idiopathic, congenital, neuromuscular, or syndrome-related scoliosis. This is an ongoing process until the child's scoliosis resolves or, more likely, he or she has reached the age where corrective spinal fusion with instrumentation can be performed. These children are committed to a regimen that may encompass their entire childhood.

For children with a syndrome or neuromuscular disease, treatment for their scoliosis may be one component of a series of measures that serve to improve the quality of their lives and to a variable degree, the quantity of their years. Children with thoracic insufficiency syndrome, some of whom would not have survived without intervention, are able to grow to adulthood with increased thoracic volume and improved pulmonary function. The goal of medical and surgical care at Shriners Hospitals is to send these children to adulthood breathing easy and proudly standing or sitting tall and erect.

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Examination 

Infantile and Juvenile Scoliosis: The Crooked Path to Diagnosis and Treatment 

Purpose/Goal 

To educate perioperative nurses about caring for infants and children undergoing treatment for infantile or juvenile scoliosis.

Behavioral Objectives 

After reading and studying the article on infantile and juvenile scoliosis, nurses will be able to

Questions 

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Answer Sheet 

Infantile and Juvenile Scoliosis: The Crooked Path to Diagnosis and Treatment 

Event #09305

Session #1139

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Learner Evaluation 

Infantile and Juvenile Scoliosis: The Crooked Path to Diagnosis and Treatment 

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This evaluation is used to determine the extent to which this continuing education program met your learning needs. Rate these items on a scale of 1 to 5.

Purpose/Goal 

To educate perioperative nurses about caring for infants and children undergoing treatment for infantile or juvenile scoliosis.

Objectives 

To what extent were the following objectives of this continuing education program achieved?

Content 

To what extent

Test Questions/Answers 

To what extent

Learner Input 

What other topics would you like to see addressed in a future continuing education article? Would you be interested or do you know someone who would be interested in writing an article on this topic?

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Acknowledgements 

The authors acknowledge and thank the Board of Directors and administrative team at Shriners Hospital, Portland, Oregon, for recognizing the importance of nursing education and the value of nursing publications. Without this emphasis on education and research, articles of this nature would not be possible. The authors also thank the following people from Shriners Hospital, Portland, Oregon: Ivan Krajbich, MD, FRCSC, pediatric orthopedic spine surgeon, and Charles d'Amato, MD, FRCSC, pediatric orthopedic spine surgeon, for providing their expertise and assistance in the writing of this article; Todd DeWees, CPO, staff orthotist, for providing the table on orthotics and answering a multitude of questions; Harlan Pine, media specialist, and Brian Demmings, photographer, from media services for the numerous supporting photographs and x-rays used to illustrate key points; Larry Jacobson, MD, chief of anesthesia, for helping improve and shorten the article with his keen editing skills; Herb Hostler, physical therapist, for modifying the tricycle, wheelchair, and walking frame illustrated in the halo traction portion of the article; and Bill Weide, network specialist from information services for technical support.

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References 

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