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e472 Copyright © SLACK Incorporated FEATURE ARTICLE Pediatric Scoliosis and Kyphosis: An Overview of Diagnosis, Management, and Surgical Treatment Diane Dudas Sheehan, ND, APN, FNP-BC; and John Grayhack, MD, MS ABSTRACT Evaluation of pediatric spinal deformity requires knowledge of special orthopaedic test- ing and radiographic interpretation. The determination of recommendations for treatment of spinal abnormalities in children can be challenging and at times complex, as treatment options are dependent upon a variety of factors. The etiology of scoliosis or kyphosis, pres- ence or absence of vertebral anomalies, symptoms, magnitude of the curve, physiologic/ skeletal age, and evidence of and risk of progression all require consideration and play a role in the shared decision-making process. This article provides an overview of relevant information and includes research outcomes to support the care of pediatric patients with spinal deformities. [Pediatr Ann. 2017;46(12):e472-e480.] P rimary care providers (PCPs) of- ten screen their pediatric patients for scoliosis and kyphosis when performing complete physical exami- nations during routine wellness visits. Identification of an abnormal spinal con- dition in infants, toddlers, children, and adolescents can generate a referral to an orthopaedic specialist. The specialist’s input may, at the very least, alleviate pa- tient and family anxiety or, if necessary, provide for more treatment options and appropriate management with the po- tential to mitigate progression and long- term consequences. This article defines scoliosis and kyphosis in pediatric pa- tients, reviews the differential diagnoses, categorizes the potential natural history and issues, examines treatment options, and examines shared patient/family- provider decision-making for treatment choice. It describes the complexity of the surgical decision (in the case of se- vere spinal abnormalities), explains the risks and benefits of surgery, considers postsurgical management, and reviews published historical complication rates of these procedures SPINAL MISALIGNMENT SYMPTOMS AND CLINICAL PRESENTATION Most patients with scoliosis or ky- phosis are asymptomatic, without com- plaints or conspicuous signs. Some patients or families may recognize a prominence of the back or uneven shoulders or waist, or report back pain. Scoliosis and kyphosis are typically not painful conditions. Back pain is primar- ily muscular or postural in nature, most often improving with core strengthening activities or physical therapy. It is un- usual for significant back pain to inter- rupt daily activities or sleep, and rare to have unrelenting symptoms or associat- ed neurologic signs or symptoms such as numbness and tingling in the extremities and bowel or bladder control issues. If any of these atypical symptoms are re- ported they may be associated with an Diane Dudas Sheehan, ND, APN, FNP-BC, is an Assistant Professor, Department of Physical Medicine and Rehabilitation, Feinberg School of Medicine, Northwestern University; and a Family Nurse Practi- tioner, Division of Orthopaedic Surgery and Sports Medicine, Ann & Robert H. Lurie Children’s Hospital of Chicago. John Grayhack, MD, MS, is an Associate Professor, Department of Orthopaedic Surgery, Feinberg School of Medicine, Northwestern University; and an Orthopaedic Surgeon, Division of Or- thopaedic Surgery and Sports Medicine, Ann & Robert H. Lurie Children’s Hospital of Chicago. Address correspondence to Diane Dudas Sheehan, ND, APN, FNP-BC, Division of Orthopaedic Sur- gery and Sports Medicine, Ann & Robert H. Lurie Children’s Hospital of Chicago, 225 E. Chicago Avenue, Box 69, Chicago, IL 60611; email: [email protected]. Disclosure: The authors have no relevant financial relationships to disclose. doi:10.3928/19382359-20171113-01

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Page 1: Pediatric Scoliosis and Kyphosis: An Overview of Diagnosis ...1cb... · Pediatric Scoliosis and Kyphosis: An Overview of Diagnosis, Management, and Surgical Treatment Diane Dudas

e472 Copyright © SLACK Incorporated

FEATURE ARTICLE

Pediatric Scoliosis and Kyphosis: An Overview of Diagnosis, Management, and Surgical TreatmentDiane Dudas Sheehan, ND, APN, FNP-BC; and John Grayhack, MD, MS

ABSTRACT

Evaluation of pediatric spinal deformity requires knowledge of special orthopaedic test-

ing and radiographic interpretation. The determination of recommendations for treatment

of spinal abnormalities in children can be challenging and at times complex, as treatment

options are dependent upon a variety of factors. The etiology of scoliosis or kyphosis, pres-

ence or absence of vertebral anomalies, symptoms, magnitude of the curve, physiologic/

skeletal age, and evidence of and risk of progression all require consideration and play a

role in the shared decision-making process. This article provides an overview of relevant

information and includes research outcomes to support the care of pediatric patients with

spinal deformities. [Pediatr Ann. 2017;46(12):e472-e480.]

Primary care providers (PCPs) of-ten screen their pediatric patients for scoliosis and kyphosis when

performing complete physical exami-nations during routine wellness visits. Identification of an abnormal spinal con-dition in infants, toddlers, children, and adolescents can generate a referral to an orthopaedic specialist. The specialist’s

input may, at the very least, alleviate pa-tient and family anxiety or, if necessary, provide for more treatment options and appropriate management with the po-tential to mitigate progression and long-term consequences. This article defines scoliosis and kyphosis in pediatric pa-tients, reviews the differential diagnoses, categorizes the potential natural history

and issues, examines treatment options, and examines shared patient/family-provider decision-making for treatment choice. It describes the complexity of the surgical decision (in the case of se-vere spinal abnormalities), explains the risks and benefits of surgery, considers postsurgical management, and reviews published historical complication rates of these procedures

SPINAL MISALIGNMENT SYMPTOMS AND CLINICAL PRESENTATION

Most patients with scoliosis or ky-phosis are asymptomatic, without com-plaints or conspicuous signs. Some patients or families may recognize a prominence of the back or uneven shoulders or waist, or report back pain. Scoliosis and kyphosis are typically not painful conditions. Back pain is primar-ily muscular or postural in nature, most often improving with core strengthening activities or physical therapy. It is un-usual for significant back pain to inter-rupt daily activities or sleep, and rare to have unrelenting symptoms or associat-ed neurologic signs or symptoms such as numbness and tingling in the extremities and bowel or bladder control issues. If any of these atypical symptoms are re-ported they may be associated with an

Diane Dudas Sheehan, ND, APN, FNP-BC, is an Assistant Professor, Department of Physical Medicine

and Rehabilitation, Feinberg School of Medicine, Northwestern University; and a Family Nurse Practi-

tioner, Division of Orthopaedic Surgery and Sports Medicine, Ann & Robert H. Lurie Children’s Hospital

of Chicago. John Grayhack, MD, MS, is an Associate Professor, Department of Orthopaedic Surgery,

Feinberg School of Medicine, Northwestern University; and an Orthopaedic Surgeon, Division of Or-

thopaedic Surgery and Sports Medicine, Ann & Robert H. Lurie Children’s Hospital of Chicago.Address correspondence to Diane Dudas Sheehan, ND, APN, FNP-BC, Division of Orthopaedic Sur-

gery and Sports Medicine, Ann & Robert H. Lurie Children’s Hospital of Chicago, 225 E. Chicago Avenue, Box 69, Chicago, IL 60611; email: [email protected].

Disclosure: The authors have no relevant financial relationships to disclose.doi:10.3928/19382359-20171113-01

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underlying pathology and further testing is likely warranted.

Some children, adolescents, or family members may notice an asymmetry with concern for scoliosis such as uneven shoulders, a prominence on one side of the back, a more pronounced scapula, amplified concave flank region, one hip appearing higher than the other, or an overall imbalance or leaning toward one side. Parents may notice a stooped posture or posterior prominence relat-ing to a possible exaggerated kyphosis, or the family or patient may be bothered by their physical appearance.1-4 These family concerns and patient signs may prompt a visit to their PCP.

SCOLIOSIS AND KYPHOSIS: SCREENING AND EVALUATION

Scoliosis and kyphosis screening may be performed on all patients regard-less of symptoms. Upon presentation to the PCP, spinal alignment and flexibility may be assessed by inspection, palpa-tion, and an Adam’s forward bend test. This test is performed while the patient stands with their back to the provider, without shoes, feet spaced shoulder-width apart, legs and knees straight, bending to 90 degrees at the waist with arms dangling and palms together. The provider assesses for a significant rota-tional prominence of one side as mea-sured using an inclinometer centered along the spine from the upper thoracic region to the sacrum, noting the asso-ciated spinal region and documenting the correlating angle. An asymmetry or angle of trunk rotation measurement of greater than 7 degrees may warrant a ra-diographic examination or referral to a spine specialist.

All patients should then be assessed from the side for posture while in an up-right position, along with forward flex-ion and extension to demonstrate extent and flexibility of spinal kyphosis. Any

significant increase in kyphosis of the spine should be noted. As sagittal align-ment may prove rather difficult to judge, initial radiographic examination may include posteroanterior (PA) and lateral views.

When findings on physical exami-nation raise the suspicion of scoliosis or kyphosis, other key areas should be carefully assessed. The examiner should note the lower extremity range of mo-tion at the hips, knees, and ankles; mus-cle strength including hip, knee, and toe flexion and extension, hip abductors, and hip adductors; evidence of joint hypermobility; and appraisal of any leg length discrepancy (if noted, measure-ment of each leg from the anterior supe-rior iliac spine to the medial malleolus to determine difference). An examination for skin lesions (ie, café-au-lait spots) and midline cutaneous anomalies (ie, dimple, tuft of hair) should also be per-formed. The examination should also include testing the patient’s sensation, reflexes, and gait patterns.2,4

Assessment of children with neu-romuscular conditions may be chal-lenging for various reasons. In young children, cognitive and communicative skills may be limited. Posture, spinal alignment, and spine flexibility may need to be assessed in lying or sitting positions versus standing. The Adam’s forward bend test may not be feasible, warranted, or reliable; therefore, the test may need to be modified (eg, a family member supporting the child if there is evidence of weakness) or eliminated al-together if not reproducible. Clinicians should interview the child to assess for complaints of imbalance and pain, and include the caregiver’s perception of the child’s discomfort, difficulties with sitting, or issues maintaining positions. For a child whose primary mode of mo-bility is a wheelchair, the supportive seating system should be reviewed and

documented. Other key physical exami-nation considerations include typical positioning of upper and lower extremi-ties while supine and upright, assess-ment of muscle atrophy, Galeazzi test to assess for hip dislocation, the presence of contractures, pelvic obliquity, exces-sive lordosis, and spasticity or increased tone.5

RADIOGRAPHIC EVALUATION FOR DIAGNOSIS AND TREATMENT OPTIONS

Radiographic studies are usually in-dicated when physical examination re-veals 7 degrees or more of asymmetry on the Adam’s forward bend test, as this may be associated with a spinal curve of 20 degrees or more. Imaging of the en-tire spine should include PA and lateral views of the full spine, including the cer-vical, thoracic, and lumbar regions in the most upright position possible (standing preferred). If a leg-length discrepancy is identified on physical examination, a block may be placed beneath the foot of the shorter limb to level the pelvis (the size and position should be documented in the technique portion of the X-ray report). From the PA radiograph of the spine, overall alignment is assessed in addition to the Cobb angle (the angle be-tween the most tilted cephalad and cau-dal vertebrae of the curve), along with the vertebral shape and development. The magnitude of this curve is used to classify scoliosis in terms of natural his-tory, risk of progression, and treatment options4,6 (Figure 1).

The lateral radiograph includes as-sessment of the vertebral size, shape, or wedging, along with sagittal alignment. Hypokyphosis, or flattening of the tho-racic spine, may occur with scoliosis (Figure 2).

The Cobb angle measurement on the lateral view may quantify the kyphosis or lordosis (Figure 3).

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FEATURE ARTICLE

For patients with neurological issues, the clinician may obtain both supine and/or upright radiographs. For those who have difficulty standing, an upright view in sitting may give additional infor-mation to the supine view (Figure 4 and Figure 5). These additional views may provide useful information. In the supine position, they can show how minimal or improved the curve can be (how flexible is the curve), and in the sitting position

they can show the maximum amount of the curve, with gravity a factor.

RADIOGRAPHIC ASSESSMENT OF MATURITY

The patient’s skeletal maturity, which is an important component regarding natural history and for treatment deci-sions, can be assessed on the spinal ra-diographs by evaluating the graduated ossification of the growth plate of the ili-

ac crest during the adolescent years. The extent of calcification correlates with maturity level using the Risser grading scale (Table 1).7

A bone-age X-ray (an AP view of the hand and wrist,) is another way to deter-mine skeletal age and, therefore, spinal growth remaining.

Thus, spinal radiographs confirming the diagnosis of scoliosis or kyphosis and the skeletal maturity along with the underlying etiology, are the basis for predicting natural history and potential issues, future treatment options, and management plans.

DEFINITIONS OF SCOLIOSIS AND KYPHOSIS

The Pediatric Orthopaedic So-ciety of North America1 and the Scoliosis Research Society2 de-fine scoliosis as a complex three-

Figure 1. A standing posteroanterior radiograph of a patient with adolescent idiopathic scoliosis shows a primary right thoracic curve that mea-sures 60 degrees with a compensatory left lum-bar curve.

Figure 2. A standing lateral view radiograph of a patient (same patient as in Figure 1) with adoles-cent idiopathic scoliosis. The lateral view demon-strates hypo-kyphosis and rib rotation.

Figure 3. Lateral view radiograph of an adolescent shows an exaggerated kyphosis measuring ap-proximately 86 degrees.

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dimensional rotational deformity of the spine characterized by a lateral curva-ture of the spine in the frontal plane with a Cobb angle of more than 10 de-grees, with decreased thoracic spinal kyphosis in the sagittal plane and clini-cally characterized by a posterior rib prominence that is produced by rotation of the vertebrae in the transverse plane.

Kyphosis is defined as an exagger-ated roundback deformity of the tho-racic or thoracolumbar spine that mea-sures greater than 40 degrees by Cobb technique.1,2,4

DIFFERENTIAL DIAGNOSIS OF SCOLIOSIS TYPES Idiopathic

Only 2% to 3% of the general popu-lation has significant scoliosis, and this can be further categorized according to the underlying cause. The most com-mon type is idiopathic scoliosis, which is seen in otherwise healthy children with no associated disease nor identi-fiable source of the curvature. These patients typically present with thoracic curve (90% right), lumbar curve, or S-shaped curve (primary curve and a compensatory curve to maintain bal-ance). These curves can progress during growth. Idiopathic scoliosis is further divided by age (adolescent, juvenile, and infantile). Early onset scoliosis (infantile and juvenile) has the greatest potential for curve progression.3,4

CongenitalCongenital scoliosis occurs due to

abnormal development of the verte-brae during embryogenesis, resulting in conjoined vertebra (failure of seg-mentation or a “bar”) or misshapen segments (failure of formation such as hemi-vertebrae, butterfly-shaped vertebrae, or tripedicular vertebrae). Scoliosis of this nature may be identi-fied at any time throughout life with a

variety of presentations, from mild to severe scoliosis and sometimes with complex curve patterns (Figure 6).

Congenital scoliosis can be associated with other anomalies (ie, cardiac, and renal); therefore, the clinician needs to

Figure 4. Posteroanterior radiograph of a sitting patient with neuromuscular scoliosis shows a long C-shaped sweeping curve (right thoraco-lumbar curvature of 84 degrees from T5 to L3 and a pelvic obliquity).

Figure 5. Lateral view radiograph of a sitting pa-tient (the same patient as in Figure 4) with neuro-muscular scoliosis. This lateral view reveals hyper-kyphosis.

TABLE 1.

Risser Grading Scale

Risser Grade Skeletal Findings and Maturity

0 No sign of calcification correlates with skeletal immaturity

1 Initial appearance of ossification of the iliac apophysis calcified 0%-25%

correlates with prepuberty and maximal rate of spinal growth

2 Ossification 25%-50% across the superior ilium correlates with continuing

spinal growth

3 Ossification 50%-75% across the superior ilium correlates with slowing of

spinal growth

4 Ossification 75%-100% across the superior ilium without full fusion correlates

with near complete spinal growth

5 Ossification fused to iliac crest relates to full maturity

Adapted from the Spinal Deformity Study Group.7

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FEATURE ARTICLE

perform a detailed history and complete physical examination with attention to associated systems.8

NeuromuscularNeuromuscular scoliosis affects pa-

tients with central neurologic dysfunc-tion (ie, brain injury, cerebral palsy, spina bifida, stroke, spinal cord injury) and peripheral nerve or muscular condi-tions (ie, spinal muscular atrophy, mus-cular dystrophy). The muscular imbal-ance, hypotonia, spasticity, decreased mobility, lack of positional balance, and decreased ability to compensate for this can cause the spine to assume a long sweeping C-shaped curve that may ex-tend to the sacrum with pelvic obliquity, negatively affecting functional sitting ability. Curves of this nature are more likely to be progressive, typically wors-ening during growth as the trunk elon-gates, but this may continue to increase

into adulthood as well (regardless of the degree of the curve).9

SyndromicSyndromic scoliosis may be associated

with a plethora of conditions not primari-ly congenital/structural or neuromuscular in etiology. Some fairly common genetic and syndromic diagnoses with increased risk of scoliosis include arthrogryposis, diastrophic dysplasia, Klippel–Feil syn-drome, Marfan syndrome, or neurofibro-matosis type 1; therefore, patients with these conditions should be screened on a consistent basis.10

DIFFERENTIAL DIAGNOSIS OF KYPHOSIS TYPES

Thoracic or thoracolumbar spinal kyphosis should be evaluated for any structural abnormalities (eg, congenital or developmental kyphosis); if none are found then it is considered postural in nature. If vertebral body wedging (>5 degrees in at least three contiguous vertebrae) is noted, the patient may be diagnosed with Scheuermann’s defor-mity, which is often associated with irregularity of the vertebral endplates.4

FUNCTIONAL AND MEDICAL PROBLEMS OF SCOLIOSIS AND KYPHOSIS

Scoliosis usually is not associated with functional difficulties. However, if the magnitude of the curve pro-gresses significantly, altered spinal alignment, decreased spine flexibility, eventual degenerative changes, and symptoms of pain have the potential to occur during the patient’s lifetime. Re-strictive lung disease (with measurable decrease in forced expiratory volumes or forced vital capacity) can occur in patients with thoracic curves greater than 70 degrees.4 Neurological disabil-ity is extremely rare. Kyphosis is as-sociated with a modestly increased risk

of back pain but otherwise no signifi-cant risk of functional issues, although it may have a negative impact on body image.1-3,10

ADVANCED IMAGINGTesting with magnetic resonance imag-

ing (MRI) may be warranted in infantile/ juvenile scoliosis, congenital scolio-sis or kyphosis, patients with atypical curves, significant persistent pain symp-toms, neurologic abnormality or, rarely, other findings on examination (ie, mid-line cutaneous anomalies or sacral dim-ple) or history. MRI may identify anom-alies of the vertebrae, discs, and spinal cord, including potential etiologies (eg, tethered cord). A computed tomography scan may be used to help further char-acterize the deformity.3,4 The results of these imaging tests may affect treatment decisions.

NONSURGICAL ORTHOTIC (BRACE) TREATMENT

Orthotic treatment continues to be recommended as a nonsurgical option for managing adolescent idiopathic sco-liosis (AIS) patients who have curves of 25 to 45 degrees and are of appro-priate skeletal age (immature.) Chil-dren with significant remaining growth potential (Risser grading scale 0-2) have the greatest propensity for curve progression, which may lead to long-term consequences and eventual surgi-cal recommendation. Treatment with a customized brace (thoracolumbar sacral orthosis [TLSO]) to fit the patient pro-vides external forces to the trunk’s spe-cific curve patterns. The goal of bracing is to control or limit the progression of the curve during remaining growth and thus avoid long-term sequela and spi-nal fusion surgery. Multicenter studies have shown that bracing is effective for many children and adolescents if worn for more than 13 hours per day, with ef-

Figure 6. Radiograph showing congenital scolio-sis with moderate to severe curves.

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ficacy increasing with longer wear and peaking at 18 hours per day.11-13 Such brace wear may significantly reduce the risk of curve progression and subsequent surgery. Brace treatment for thoracic ky-phosis typically requires a particularly high-based brace (a TLSO with high cervical or clavicle extension [ie, “Mil-waukee” style]) that is worn for 1 to 2 years. Some correction of the kypho-sis with bracing may occur during use; however, partial loss of this improve-ment may occur over time after discon-tinuation of the brace.

Brace compliance may be a chal-lenge for some children and adolescents due to a variety of reasons (eg, discom-fort, function, sensory, and peer issues). School accommodations can be made for removal of the brace in a private set-ting, if desired, for continued participa-tion in physical education and/or sports. After-school and nighttime bracing may be an effective option to improve com-pliance. Monitoring devices (heat sen-sors) placed in the brace are being used by some providers with the expectation that compliance can be measured more accurately. Compliance has been shown to improve with such documentation and appropriate feedback11 (Table 2).

OTHER NONSURGICAL TREATMENT OPTIONS

Over the past century, many nonsur-gical treatments have been attempted for scoliosis management, including spinal manipulation, chiropractic care, spine exercises, physical therapy, and yoga. Each of these have limited evidence of efficacy.

Physical therapists assess the pos-tural deficits, pelvic imbalance, core and hip stability, weakness, and pain. They may focus treatment to include a variety of techniques such as thoracic and lumbar joint mobilization, lower extremity stretching and flexibility, pos-

tural stabilization and center of gravity training, spinal alignment training and feedback, neuromuscular re-education, core strengthening, soft tissue mobili-zation, and proprioception in the treat-ment sessions along with home exercise programs. Some therapists incorporate a methodical exercise approach to scolio-sis, which is an individualized method adapted for idiopathic scoliosis based on self-correction techniques incorporated in functional exercises.14

The “Schroth” method, developed in Germany nearly 100 years ago, has recently become popular in the United States as a conservative option for sco-liosis patients.15 Schroth exercises pro-mote a customized treatment directed toward spinal rotation, designed to help correct the imbalances of the scoliotic spine. Schroth treatment goals are to in-crease awareness of alignment, to slow or halt curve progression, and improve cardiopulmonary function. The avail-ability of trained and certified Schroth therapists varies with locale. Although this specific program is promising, the evidence of any efficacy of this method is still limited.15

Yoga maneuvers and poses to realign the spine through strengthening muscles on the convex side while stretching liga-ments on the contralateral side requires daily practice for months to potentially benefit posture.16 General fitness, mar-tial arts, sports participation, and overall physical education classes are not con-traindicated, and thus should be contin-ued and even encouraged after diagno-sis of scoliosis. In the end, any and all of these methods do no harm and may improve core strength and posture, but none have been proven to affect the true structural nature or alter the natural his-tory or risk of progression of idiopathic scoliosis.

Serial casting of the trunk is an option typically reserved for infantile scoliosis

patients with significant otherwise un-controllable curves, often in conjunction with eventual bracing.

SURGICAL TREATMENT OPTIONS FOR SPINAL DEFORMITY

Surgery is generally reserved for children who have scoliotic curves greater than 45 to 50 degrees or, more rarely, those with significant imbalance or symptoms. Kyphosis correction sur-gery is less commonly required, yet of-fered for patients who have curves of significant magnitude or, rarely, sharp deformities of a lesser degree (with the potential of neurologic compromise). Surgical technique depends on numer-ous factors including etiology, curve magnitude, spinal balance, and remain-ing growth. Surgical advances have been made in procedural safeguards, surgi-cal techniques, and instrumentation to improve patient safety and outcomes. Although technological advances have evolved over the decades, the goals of the surgery remain largely unchanged. The overall goal of surgical intervention is to prevent further progression of the primary and compensatory curvatures while improving alignment and over-all balance. Spinal fusion intervention includes instrumentation (combination of rods, with hooks, cables, wires, and screw fixation) and bone graft (autograft, allograft, and bone substitutes) to create bone fusion of the vertebrae to achieve long-term stability and alignment. Sur-gical alternatives such as in situ fusion constructs that allow for or encourage growth, or address accompanying chest wall deformity may be indicated for specific pathology (such as congenital or juvenile scoliosis, or rib anomalies). Developing technologies include verte-bral stapling or tethering to manipulate spinal growth. Although the risks of spine surgery include infection, blood loss with need for transfusion, failure of

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instrumentation or fusion, and rarely respiratory or neurologic compromise, these are relatively uncommon in the pediatric population.17

The decision to undertake surgical intervention in AIS, congenital, and syndromic scoliosis, and in kyphosis is based on the patient and family’s de-termination of the potential long-term benefits to alter the natural history, including prevention of later dysfunc-tion, disfigurement, and pain, weighed against the extensive effort and poten-tial complications related to surgery (Figure 7 and Figure 8).

NEUROMUSCULAR SCOLIOSISFor children, adolescents, and

young adults with neuromuscular sco-liosis or kyphosis, the treatment deci-sions, including surgical options, are complex, as both the natural history and response to treatment are quite varied. The underlying neurological etiology and disease course without such intervention are highly variable; thus, long-term outcomes are less pre-dictable. The risk of curve progression is influenced by the disease itself, the magnitude and rigidity of the curve, and balance of the spine. Furthermore, due to muscular imbalance, scoliosis can progress after the child has reached skeletal maturity. Brace treatment has

limited scientific evidence for preven-tion of curve progression in this popu-lation; however, orthotics are used to help function (ie, improved posture during activities) and for their possible impact on delaying or halting progres-sion. In children who are primarily wheelchair bound, increased support-ive seating components may be incor-porated (chest strap, lateral supports, molded back or specialized seating system) to sustain upright posture, al-leviate discomfort, and to reduce fami-ly concerns of positioning. Due to con-cern that pulmonary function may be affected by the spinal deformity with diaphragmatic displacement elevated within the thoracic cage, monitoring respiratory status may be warranted. Surgical intervention most often en-tails instrumentation to the lower lum-bar spine or to the pelvis to positively affect balance and sitting tolerance (Figure 9 and Figure 10).

Collaboration with the patient and family when making the decision to undergo this surgery is important so they can understand the potential ben-efits and possible risks. In certain cas-es, surgical intervention is determined more by the parent’s perspective on the child’s behalf, with quality-of-life considerations and goals of treatment including the patient’s expression of

discomfort or parent’s belief regard-ing amount of pain and expectations of comfort achieved, assessment of the child’s current level of function and predicted improvements, alleviation of the burden of care, and facility of patient care needs. These benefits may offset the more complex surgery risks (eg, prolonged anesthesia, increased blood loss, higher risk of infection).18

POSTSURGICAL CONSIDERATIONS Postsurgical management in the

hospitalized patient requires a team of professionals. The medical and nurs-ing teams closely monitor the patient’s vital signs, neurological examination, gastrointestinal/genitourinary func-tion, respiratory function, pain, and surgical site for signs of infection, along with other issues. In our hos-pital, pain management is typically guided by the anesthesia pain team through protocols (initially with epi-dural catheter and/or intravenous pain medications, then transitioned to oral medications) with the entire team’s around-the-clock assessments, includ-ing the patient’s family’s input of pain control, with alterations made to op-timize comfort. Physical therapists educate the patient and family regard-ing spinal precautions (limited trunk forward flexion, avoidance of twisting

TABLE 2.

Treatment Options for Adolescent Idiopathic Scoliosis

Observation Observation or Brace Brace Treatment Observation or Surgery Surgery

<25 degrees

Regardless of maturity level

Risser grade 0-5

25-45 degrees

Moderate/near complete

skeletal maturity

Risser grade 3-4

25-45 degrees

Skeletally immature

Risser grade 0-2

45-49 degrees

Skeletally mature

Risser grade 4-5

≥50 degrees

Regardless of maturity

Risser grade 0-5

20-45 degrees

Skeletally mature

Risser grade 5

– –

45-49 degrees

Risser grade 0-3

Surgery considered

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Figure 7. Posteroanterior radiograph of teenager with adolescent idiopathic scoliosis after poste-rior spinal fusion surgery. Note the alignment of rods and screws.

Figure 8. Lateral view radiograph of teenager (the same patient as in Figure 7) with adolescent idio-pathic scoliosis after posterior spinal fusion sur-gery. Note the lateral view of rods and screws in pedicles.

and prone positions, limited upper ex-tremity weight lifting, limited shoul-der flexion past 90 degrees, and limit-ed lower extremity hip flexion past 90 degrees), and the treatment team rein-forces these with functional activities. The first 2 days after surgery, ambula-tory patients are typically walking in the room or hallway, and patients with neuromuscular disorders are sitting in an appropriate wheelchair. A postop-erative brace is occasionally fabricat-ed to maintain postural alignment and follow through with precautions.

After discharge home, effective communication, including key infor-mation provided regarding postop-erative plans for individualized care, may help to facilitate coordination of care for such things as pain control at home and guidance for weaning medi-cations, strategies for constipation relief, and assessment for wound/inci-sion infection through the family and primary care provider. We recommend that the spinal precautions be used for 3 months; however, limitation of ac-tivities (such as no physical education and sports participation) may be rec-ommended for at least 6 months while the bone fusion matures. Specific ac-tivity restrictions may be individual-ized based on the patient’s underlying diagnosis, medical history, age, and healing outlook.

SURGICAL OUTCOMES REPORTEDThe Scoliosis Research Society

(SRS) studies complication rates for all deformity operations and tracks statistics such as neurological defi-cit and wound infection rates. In the reported study of operative treatment in pediatric scoliosis by Reames et al.18 the overall reported complica-tion rates varied by type: idiopathic with the lowest rate (6.3%), neuro-muscular population with the highest

rate (17.9%), and congenital (10.6%). Other differences in the incidence of complication rates occurred based on scoliosis etiology19 (Table 3).

CONCLUSION For children and adolescents with

significant scoliosis or kyphosis, pa-tients and families are encouraged to

Figure 9. Posteroanterior radiograph of a patient with neuromuscular scoliosis after posterior spi-nal fusion surgery. Note the instrumentation from T2 extended to the ileum for pelvic balance.

Figure 10. Lateral view radiograph of a patient (the same patient as in Figure 9) with neuro-muscular scoliosis after posterior spinal fusion surgery.

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FEATURE ARTICLE

consider the long-term issues involved in the complex decision-making about intervention with bracing or surgery. The use of a brace has the most proven efficacy to prevent the progression of adolescent idiopathic scoliosis com-pared to other nonsurgical options,13 but it may be less effective for kypho-sis management. Long-term outcome data support spine fusion treatment in children and adolescents who are sur-gical candidates,19 with excellent heal-ing rates (bone fusion) and minimal complication rates. For patients with neuromuscular scoliosis, families have a more challenging decision regard-ing the options, as this group often has more limited goals and higher compli-cation rates.

REFERENCES 1. Pediatric Orthopaedic Society of North Amer-

ica. Position statement. Screening for the early detection of idiopathic scoliosis in ad-olescents. https://posna.org/POSNA/media/Documents/Position%20Statements/1122-Screening-for-the-Early-Detection-of-Idiopathic-Scoliosis-in-Adolescents.pdf. Accessed December 1, 2017.

2. Scoliosis Research Society. Three-dimen-sional terminology of spinal deformity. http://www.srs.org/professionals/online-education-and-resources/glossary/three-dimensional-terminology-of-spinal-defor-mity#40. Accessed December 1, 2017.

3. Hresko TM. Idiopathic scoliosis in adoles-cents. N Engl J Med. 2013;368:834-841. doi:10.1056/NEJMcp1209063.

4. Spinal deformities: idiopathic scoliosis. In:

Sarwark J, LaBella C, eds. Pediatric Or-thopaedics and Sports Injuries: A Quick Reference Guide. 2nd ed. Elk Grove Vil-lage, IL: American Academy of Pediatrics; 2014:123-136.

5. Brooks, JT, Sponseller, PD. What’s new in the management of neuromuscular scolio-sis? J Pediatr Orthop. 2016;36(6):627-633. doi:10.1097/BPO.000000000000497.

6. Spinal Deformity Study Group. Coronal cobb measurements: proximal thoracic (PT), main thoracic (MT), and thoracolum-bar/lumbar (TL/L) curves. In: Radiographic Measurement Manual. http://www.oref.org/docs/default-source/default-document-li-brary/sdsg-radiographic-measuremnt-man-ual.pdf?sfvrsn=2. Accessed November 15, 2017.

7. Spinal Deformity Study Group. Adoles-cent idiopathic scoliosis. In: Radiographic Measurement Manual. http://www.oref.org/docs/default-source/default-document-library/sdsg-radiographic-measuremnt-manual.pdf?sfvrsn=2. Accessed November 15, 2017.

8. Pahys JM, Guille JT. What’s new in con-genital scoliosis? J Pediatr Orthop. 2016. doi:10.1097/BPO.0000000000000922.

9. Whitaker AT, Sharkey M, Diab M. Spinal fu-sion in patients with globally involved cere-bral palsy. J Bone Joint Surg. 2015;97:782-787. doi:10.2106/JBJS.N.00468.

10. McPhail GL, Howells SA, Boesch RP, et al. Obstructive lung disease is common in children with syndromic and congenital scoliosis: a preliminary study. J Pediatr Orthop. 2013;33(8):781-785. doi:10.1097/BPO.0000000000000078.

11. Karol LA, Virostek D, Felton K, Jo CH, Butler L. The effect of the Risser stage on bracing outcome in adolescent idiopathic scoliosis. J Bone Joint Surg. 2016;98:1253-1259. doi:10.2106/JBJS.15.01313.

12. Weinstein SL, Dolan LA, Wright JG, Dobbs MB. Effects of bracing in adoles-cents with idiopathic scoliosis. N Engl J

Med. 2013;369:1512-1521. doi:10.1056/ NEJMoa1307337.

13. Negrini S, Minozzi S, Bettany-Saltikov J, et al. Braces for idiopathic sco-liosis in adolescents. Cochrane Data-base Syst Rev. 2015;(6):CD006850. doi:10:1002/14651858.CD006850.pub3.

14. Romano M, Negrini A, Parzini S, et al. SEAS (Scientific Exercises Approach to Scoliosis): a modern and effective evidence based approach to physiotherapic specific scoliosis exercises. Scoliosis. 2015;10:3. doi:10.1186/s13013-014-0027-2.

15. Kuru1 T, Yeldan I, Dereli EE, Ozdincler AR, Dikici, F, Colak I. The efficacy of three-di-mensional Schroth exercises in adolescent idiopathic scoliosis: a randomised controlled clinical trial. Clin Rehabil. 2016,30(2):181-190. doi:10.1177/0269215515575745.

16. Fishman, LM, Groessl EJ, Sherman KJ. Serial case reporting yoga for idiopath-ic and degenerative scoliosis. Glob Adv Health Med. 2014;3(5):16-21. doi:10.7453/ gahmj.2013.064.

17. Farley FA, Ying L, Jong N, et al. Con-genital scoliosis SRS-22 outcomes in chil-dren treated with observation, surgery and VEPTR. Spine. 2014;39(22):1868-1874. doi:10.1097/BRS.0000000000000546.

18. Reames DL, Smith JS, Fu K-MG, et al.; Scoliosis Research Society Morbidity and Mortality Committee. Complications in the surgical treatment of 19,360 cases of pe-diatric scoliosis. a review of the Scoliosis Research Society morbidity and mortality database. Spine. 2011;36(18):1484-1491. doi:10.1097/BRS.0b013e3181f3a326.

19. Smith JS, Kasliwal MK, Crawford A, Shaffrey CI. Outcomes, expectations, and complications overview for the surgical treatment of adult and pediatric spinal de-formity. Spine Deformity. 2012, in press. doi:10.1016/j.jspd.2012.04.011. http://www.spine-deformity.org/article/S2212-134X(12)00015-9/pdf. ccessed December 1, 2017.

TABLE 3.

Complications Rates of Pediatric Scoliosis Surgery

Complications (n =19,360) Idiopathic scoliosis (n = 11,227) Neuromuscular (n = 4,657) Congenital (n = 2,012)

Neurologic deficit, % 0.8 (86) 1.1 (49) 2 (41)

Pulmonary issues, % 0.6 (63) 1.9 (90) 1.1 (23)

Superficial wound infection, % 0.5 (61) 1.7 (79) 1.3 (27)

Deep wound infection, % 0.8 (95) 3.8 (177) 0.9 (18)

Death, % 0.02 (2/10,000) 0.3 (3/1,000) 0.3 (3/1,000)

Adapted from Reames et al.18