(From Scoles PV: Spinal deformity in childhood and adolescence. In Behrman RE, Vaughn VC III, editors: Nelson textbook of pediatrics, update 5, Philadelphia, 1989, WB Saunders.)

A careful neurologic examination should always be performed. A subset of curves are associated with an underlying neurologic diagnosis, especially in patients who present in the infant and juvenile years (20% have an associated intraspinal abnormality). The index of suspicion is raised in the presence of back pain or neurologic symptoms, café-au-lait spots, a sacral dimple or midline cutaneous abnormalities (hemangioma, hair patch or skin tag), unilateral foot deformity, or an atypical curve pattern. The examiner should always test the superficial abdominal reflex by lightly stroking the skin on both sides of the umbilicus. Normally, the umbilicus should deviate toward the side that was stroked. Asymmetry of the superficial abdominal reflex (or unilateral absence of this reflex) might suggest syringomyelia as an underlying diagnosis.

Screening for scoliosis facilitates earlier diagnosis and treatment, under the assumption that the natural history may be influenced by bracing, and that earlier identification of cases suitable for surgery reduces the complexity and risks of the surgery. Screening may be accomplished during primary care visits or as part of a school screening program. A consensus statement from professional societies (American Academy of Pediatrics, American Academy of Orthopaedic Surgeons, Pediatric Orthopaedic Society of North America, and the Scoliosis Research Society) states that if screening is to be carried out, this should be at 10 and 12 years of age for girls, and once at 13-14 years for boys. The Adams forward-bend test has been used to identify any asymmetry in the thoracic and/or lumbar region, and an inclinometer (scoliometer, Orthopaedic Systems Inc.) is also used to measure the degree of asymmetry. The number of degrees used for referral typically varies from 5 to 7, and the referral rate from these programs varies from 3% to 30%. School screening remains controversial, and less than half of the states in the USA have formal programs. The U.S. Preventive Services Task Force (USPSTF) has recently recommended against school screening in 2004, and further research is required to inform policy.

The natural history of idiopathic scoliosis varies considerably based on the age at diagnosis. Curvatures in excess of 80 degrees can result in restrictive pulmonary disease, and larger curves in excess of 100-120 degrees can result in a reduced life expectancy, due to cor pulmonale and cardiorespiratory failure. Scoliotic curves diagnosed in infancy through childhood are much more likely than adolescent curves to reach this magnitude. In general, adults with untreated AIS are expected to have similar life expectancy and similar functional activities, and no greater risk of cardiopulmonary complications, when compared with age-matched controls. Back pain may be more common, but it does not appear to be more disabling. Thoracolumbar or lumbar curves are more likely to cause pain during adulthood, particularly if there is an associated translational shift of the vertebrae. Dyspnea is common with thoracic curves of >80 degrees. Thoracic curves <30 degrees rarely progress after skeletal maturity, but those >45 or 50 degrees can continue to progress at approximately 1 degree per year through life. The cosmetic aspects of the deformity may be the most significant concern for the majority of patients with untreated AIS.

Radiographic Evaluation

Standing high-quality posteroanterior (PA) and lateral radiographs of the entire spine are recommended at the initial evaluation for patients with clinical findings that suggest a spinal deformity. On the PA radiograph, the degree of curvature is determined by the Cobb method, in which the angle between the superior and inferior end vertebra (tilted into the curve) is measured (Fig. 671-2). A line is drawn across the superior end plate of each end vertebra, and the angle between perpendicular lines erected from each of these is measured. Although the indications for performing MRI are variable, this modality is helpful when an underlying cause for the scoliosis is suspected based on age (infantile, juvenile curves), abnormal findings on the history and physical examination, and atypical radiographic features (curve patterns and/or specific features). Atypical radiographic findings include uncommon curve patterns such as the left thoracic curve, double thoracic curves, high thoracic curves, widening of the spinal canal, and erosive or dysplastic changes in the vertebral body or ribs. On the lateral radiograph, an increase in thoracic kyphosis or an absence of segmental lordosis might suggest the presence of an underlying diagnosis.

Figure 671-2 A-C, Cobb angles measurements.

(From Morrissy, RT, Weinstein, SL: Lovell & Winter’s pediatric orthopaedics, ed 6, Philadelphia, 2006, Lippincott Williams & Wilkins.)

Treatment

Options for treatment include observation, bracing, and surgical treatment. Treatment decisions are based on the natural history of each curvature, which relates to age (degree of skeletal maturity or growth remaining), the magnitude of the curve, and occasionally associated diagnoses and/or medical considerations. A positive family history does not help predict the behavior of an individual curve. Observation is always indicated for curvatures <20 degrees. Curves of larger magnitude may be treated by bracing or surgery. The risk of curve progression depends on the amount of growth remaining, the curve’s magnitude, and gender. More than one parameter must be used when determining the amount of growth remaining, and both clinical (age, annual growth velocity, menarchal status, Tanner stage) and radiographic (Risser sign, skeletal age, maturation of olecranon apophysis) measures are available. Curves are more likely to progress if there is significant growth remaining (premenarchal, Tanner stage I or II, Risser 0 or 1). Premenarchal girls with curves between 20 and 30 degrees have a significantly higher risk of progression than do girls 2 yr after menarche with similar curves. Boys with curvature of the same magnitude appear to have similar risks of progression when judged by other maturation standards; however, the assessment of skeletal maturity in boys is more difficult.

Bracing

The goal of bracing is to prevent progression of the deformity, thereby reducing the need for surgery. The efficacy of bracing in AIS remains controversial, but most centers in North America offer a brace to selected patients with progressive curvatures. In AIS, the typical indications for bracing are a curve >30 degrees, or a curve from 20-25 degrees that has progressed >5 degrees, in a skeletally immature patient (Risser 0,1, or 2). Bracing is thought to be ineffective in curves >45 degrees. An “in brace” correction of 50% in Cobb angle is desirable.

The success of bracing is thought to increase with greater time spent in the brace, and the ideal program includes 23 hours in the brace per day. Protocols vary between 16 and 23 hours per day, recognizing that full compliance is difficult to achieve in the adolescent population. Bracing is commonly used as a temporizing measure for infantile and juvenile scoliosis given the high likelihood of significant progression, and the need to delay more definitive treatment. The success rate is much lower for infantile and juvenile curvatures, but the goal is to delay progression.

There are several options for braces (Fig. 671-3). The Milwaukee brace, employing longitudinal traction from the skull to the pelvis with lateral compression of the chest wall, can be adjusted for growth and thus is a good brace for patients with infantile or juvenile scoliosis. Underarm braces (the Boston or Wilmington braces) are less obvious and so are often preferred by adolescents. The Charleston brace provides a corrective force and is used only at night.

Figure 671-3 Various brace types.

(From Morrissy, RT, Weinstein, SL: Lovell & Winter’s pediatric orthopaedics, ed 6, Philadelphia, 2006, Lippincott Williams & Wilkins.)

Surgery

Surgical treatment is indicated for the majority of patients with infantile or juvenile idiopathic scoliosis and in selected patients with AIS, when other methods of treatment have failed to control the deformity, and when further progression would be expected to result in unacceptable cosmesis and/or physiologic abnormalities. Such deformities are typically treated by a definitive spinal arthrodesis (fusion). The majority of progressive infantile and juvenile curves ultimately require a spinal arthrodesis, and the goal is to delay the definitive procedure until the pulmonary system and thoracic cage have matured and the trunk height has been maximized.

An alternative strategy is required for curvatures that progress despite bracing. In some centers, serial casting under general anesthesia is employed for selected infantile and juvenile curves to gain correction into a “braceable” range, followed by application of a spinal orthosis. The other option for progressive curvatures is a “growing rod” construct. A spinal rod (or 2 rods) is attached to anchors at the top and at the bottom of the curvature, and distraction forces are applied to achieve correction. The rods hold the spine in the corrected position, and they must then be lengthened every 6 months to maintain correction. Many curves have been controlled for years using such a protocol, and definitive fusion is delayed until a more optimal age.

With regard to AIS, surgical treatment is usually indicated for skeletally immature patients with progressive thoracic curves >45 degrees and skeletally mature patients with thoracic curves >50-55 degrees. Lumbar curves are more likely to progress, and surgical stabilization may be offered for curves as low as 35-40 degrees if there is a significant shift of the trunk relative to the pelvis and lower extremities. The goals of surgery are to arrest progression of the deformity, to improve cosmesis, and to minimize the number of vertebral segments that are stabilized. These are achieved through a spinal fusion or arthrodesis, and implants are used to apply mechanical forces to the bony elements to affect correction and to hold the spine in the corrected position until the spine fuses. Postoperative immobilization is usually not required. Options for bone grafting include autograft (iliac crest) or allograft, and in recent years most surgeons have used cancellous allograft with or without enhancers such as demineralized bone matrix.

The most common procedure is an instrumented posterior spinal fusion, and the typical spinal implant construct includes 2 rods anchored to the spine by hooks, wires, and/or screws (Fig. 671-4). In the last few years there has been significant interest in using a construct with pedicle screws at each level. Although correction of the axial deformity (rotation of the rib cage) is more pronounced with this technology, it remains unclear whether patient outcomes are improved. An anterior release and fusion, performed through a thoracotomy or thoracolumbar exposure, is indicated for isolated thoracolumbar and lumbar curves, to improve correctability of stiffer curves, and to prevent curve progression (“crankshaft”) from continued anterior growth of the spine in selected patients with considerable growth remaining. A construct with multiple pedicle screws can allow the surgeon to avoid an anterior approach in many larger or stiffer curves (more powerful correction) and in younger patients (stiff enough to resist anterior spinal growth and prevent crankshaft). Thoracoscopic surgery has also been used to perform an anterior release with or without instrumentation and fusion, but this technique has been used much less often since the advent of thoracic pedicle screws. For idiopathic thoracolumbar and lumbar curves, an anterior fusion with instrumentation (usually screws in the vertebral body connected to 1 or 2 rods) can be done as an alternative to save lumbar motion segments.

Figure 671-4 A, Preoperative standing posteroanterior radiograph of a 14 yr old girl who was skeletally immature and developed a 68-degree right thoracic and a 53-degree left lumbar scoliosis. Her trunk was shifted to the right, and the left shoulder was slightly depressed. B, Based on the risk of future progression, she was treated by an instrumented posterior spinal fusion from T3 to L3, with correction of the right thoracic curve to 20 degrees and the left lumbar curve to 10 degrees. Coronal spinal balance was restored, and shoulder height was maintained.

There has been an interest in developing techniques to tether spinal growth, most commonly by placing metallic staples across each disk space on the convexity of a curvature. The goal is to slow or arrest growth on the convexity while allowing growth to proceed on the concavity, thereby preventing progression and possibly resulting in permanent correction without the need for an arthrodesis. The indications for this technique, and the long-term results, remain to be determined.

Bibliography

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de Lind van Wijngaarden RFA, de Klerk LWL, Festen DAM, et al. Scoliosis in Prader-Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype. Arch Dis Child. 2008;93:1012-1016.

Dolan LA, Weinstein SL. Surgical rates after observation and bracing for adolescent idiopathic scoliosis: an evidence based review. Spine. 2007;32:S91-S100.

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671.2 Congenital Scoliosis

David A. Spiegel, John P. Dormans

Congenital scoliosis results from abnormal growth and development of the vertebral column, likely due to intrauterine events at or about the 6th wk of gestation. There can be a partial or complete failure of formation (wedge vertebrae or hemivertebrae), a partial or complete failure of segmentation (unilateral unsegmented bars), or a combination of both (Fig. 671-5). One or more bony anomalies can occur in isolation or in combination.

Figure 671-5 The defects of segmentation and formation that can occur during spinal development.

(From McMaster MJ: Congenital scoliosis. In Weinstein SL, editor: The pediatric spine: principles and practice, ed 2, Philadelphia, 2001, Lippincott Williams & Wilkins, p 163.)

As the spine (including the neural elements) and the viscera are formed around the 6th week in utero, patients with congenital scoliosis often have visceral and intraspinal anomalies as well. Once a congenital spinal anomaly is diagnosed, a priority is to rule out malformations in other organ systems. Genitourinary abnormalities are identified in 20-40% of children with congenital scoliosis and include unilateral renal agenesis, ureteral duplication, horseshoe kidney, and genital anomalies. Approximately 2% of these patients have a silent obstructive uropathy that may be life threatening. Renal ultrasonography should be performed early on in all children with congenital scoliosis, and other studies (CT, MRI) may also be required. Cardiac anomalies are identified in 10-25% of patients. A careful cardiac examination should be performed; some clinicians recommend routine echocardiography.

Approximately 20-40% of patients have an intraspinal anomaly. Infants with cutaneous abnormalities overlying the spine might benefit from ultrasonography to rule out an occult spinal dysraphic condition. MRI is usually recommended during the course of treatment. Spinal dysraphism is the general term applied to such lesions (Chapters 585 and 598). Examples include diastematomyelia, split cord malformations, intraspinal lipomas (intradural or extradural), arachnoid cysts, teratomas, dermoid sinuses, fibrous bands, and tight filum terminale. Cutaneous findings that may be seen in patients with closed spinal dysraphism include hair patches, skin tags or dimples, sinuses, and hemangiomas. Most of these lesions become clinically evident through tethering of the spinal cord, the symptoms of which include back and/or leg pain, calf atrophy, progressive unilateral foot deformity (especially cavovarus), and problems with bowel or bladder function.

The risk of progression depends on the growth potential of each anomaly, which can vary considerably, so close radiographic follow-up is required. Progression of these curves is most pronounced during periods of rapid growth, namely, the first 2-3 yr of life and during the adolescent growth spurt. The most severe form of congenital scoliosis is a unilateral unsegmented bar with a contralateral hemivertebra. In this anomaly, the spine is fused on 1 side (unsegmented bar) and has a growth center (hemivertebra) on the other side at the same level. A rapidly progressive curve is seen, and all patients usually require surgical stabilization. A unilateral unsegmented bar is also associated with significant progression and in most cases will require surgical intervention. An unsegmented bar might not be radiographically apparent, but the adjacent ribs on the concavity may be fused, providing a clue to the diagnosis. An isolated hemivertebra must be followed closely, and many of these will be associated with a progressive deformity that requires surgical intervention. In contrast, an isolated block vertebra has little growth potential and rarely requires treatment.

Early diagnosis and prompt treatment of progressive curves are essential. Bracing is not indicated for most congenital curves due to their structural nature, except in rare cases in which the goal is to control a flexible, compensatory curvature in another area of the spine. The treatment of progressive curves is preemptive spinal arthrodesis, and both anterior and posterior spinal fusion is often required. Other procedures that are employed in selected patients include an isolated posterior spinal fusion (sometimes an in situ fusion), convex hemiepiphysiodesis (only 1 side of the spine is fused to allow some correction of the deformity with growth), and partial or complete hemivertebra excision (usually in the lumbar spine). Spinal arthrodesis is ideally performed before a significant deformity has developed because intraoperative correction is difficult to achieve and the risk of neurologic complications is high.

When multiple levels of the thoracic spine are involved, especially in the presence of fused ribs, a progressive 3-dimensional deformity of the chest wall can impair lung development and function, resulting in a thoracic insufficiency syndrome. This syndrome is best described as the inability of the chest wall to support normal respiration. A thoracic insufficiency syndrome may be seen in patients with several recognized conditions, such as Jarcho-Levin syndrome (spondylocostal or spondylothoracic dysplasia) and Jeune syndrome (asphyxiating thoracic dystrophy). There is interest in treating these difficult cases with an experimental technique called expansion thoracoplasty,

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