Clinical manifestations

Presentation and course

Back pain presents in a bimodal distribution in children and adolescents. This distribution correlates to adolescent growth spurts. Lower back pain is rarely seen in the youth before they reach school age. Studies show that low back pain continues to be increasingly prevalent until the age of 12. At that time, there is a sharp increase in the prevalence of low back pain until the age of 18, at which point it levels off and is very similar to low back pain reported in adults (45). Researchers have attempted to study the overall prevalence of back pain through metaanalysis studies and report a variable range of 7% to 72% in children. In a retrospective study that reviewed 232 patients from birth to 18 years of age who presented to a pediatric emergency department with a chief complaint of back pain, nonpathologic diagnosis was found in 76.8% of the visits (11). This variability reflects the different etiologies and definitions of back pain in literature, but most importantly refutes the previously accepted notion that back pain is rare and always a serious entity in the pediatric population (26).

The history and physical examination are critical in the assessment of back pain and should be performed in the context of age (50). Attention must be paid to onset of symptoms, characterization and radiation of pain, and exacerbating and alleviating factors. Physicians should ask if the pain is worse in the mornings, accompanied by stiffness, or improved by activity. Early morning stiffness and pain routinely are associated with inflammatory etiologies. Neurologic exam, including checking for deep tendon reflexes in L2, L3, L4, and S1, is invaluable. Gait, coordination, and flexibility can be assessed using the Adams forward bending test, straight leg raise test, FABER (Flexion Abduction External Rotation) test, or Trendelenburg test. A family history is important to identify hereditary conditions. Red flag symptoms can indicate a serious underlying problem like malignancy, neoplasm, or infection. Alerting symptoms include an acute onset of pain with no history of trauma, pain that occurs at rest, or pain that causes awakening from sleep. Back pain with fever, nocturnal pain, and bony tenderness may suggest infectious etiology, whereas recent weight loss and bruising might be indicative of malignancy (50). Red flag symptoms include patient age under 3 years, weight loss, fatigue, bladder or bowel incontinence, and radiculopathy (22). It is imperative to perform a complete review of systems to rule out gastrointestinal or renal etiologies for back pain.

Spondylolysis is due to a defect in the pars interarticularis, which may be either congenital or due to a pathological fracture (50). Spondylolysis usually affects children who are older than 10 years of age (31). Patients with spondylolysis complain of moderate pain in the lumbar region, which is worsened by physical activity and alleviated by rest. The described pain can sometimes radiate to the posterior thigh or buttocks or both (06). Paravertebral spasm, hyperlumbar lordosis, loss of normal lordosis, limited lumbar extension and flexion, and localized tenderness can sometimes be observed on physical examination (16). When oblique views of x-rays are negative, a SPECT scan is performed. If SPECT is positive, a CT scan is performed to assess the lesion in detail (22).

Advanced spondylolisthesis can present with hamstring tightness and a “crab-like” gait with knees and hip flexion. When individuals with advanced spondylolisthesis are observed from the side, they may show flattening of the lumbar lordosis as well as vertically oriented sacrum. Additionally, in very serious cases, a “stair step” can be palpated at the level of slippage (24). The work-up for spondylolisthesis is similar to that of spondylolysis.

Spondylodiscitis is described as the concurrent infection of vertebral disc and vertebral bodies and is considered a very rare but severe disease in children. Delayed treatment can result in death, spinal deformities, chronic pain, or segmental instabilities. In order to reduce negative outcomes, early diagnosis and treatment is essential. In toddlers and children, it may be difficult to diagnose as symptoms can present as subacute or chronic and symptoms have exhibited as solely back pain, difficulty walking, and irritability. A recent infection is routinely present. Children usually report back pain that is worse with movement. Fever is not always present. Treatment includes prolonged broad spectrum antibiotic therapy for several weeks. Length of antibiotic therapy is based on repeat imaging and response to antibiotics. MRI still remains the goal standard for diagnosis and efficacy of treatment (07).

Apophyseal ring fractures, which result in avulsion of bony fragments displaced in the spinal canal, are seen in athletes who lift weights, wrestle, or participate in gymnastics. Apophyseal avulsions are more commonly seen in males because they reach skeletal maturity at a later age than females. Therefore, females are exposed to trauma for a shorter duration while the skeleton is immature. Onset of pain is acute and intermittent, but progressive. This pain may be accompanied by sciatica, paraspinal muscle spasm, positive straight leg test, and limited back movement (68).

Discitis presents in toddlers and young children as back pain and persistent nighttime pain. Low-grade fever, abdominal pain, and loss of appetite and weight can accompany this condition. Toddlers may experience increased nighttime crying and may become highly irritable (27). In children less than 3 years of age, the primary presenting symptom is usually refusal to walk, or limpness, whereas back pain is the predominant presenting symptom in older children who can communicate. Discitis often presents in the lumbosacral area, whereas osteomyelitis can affect any region of the spine. Osteomyelitis is more likely to present with a history of fever; however, the absence of fever does not rule out osteomyelitis (20). The work-up for discitis and osteomyelitis involves blood tests, blood culture, and x-rays, which are either nonspecific or normal. MRI is the diagnostic test of choice for discitis (27).

Scheuermann kyphosis presents at the onset of the pubertal growth spurt, between 13 and 17 years of age. The incidence of this disease is 2.8%, and the male to female ratio is 2:1 (14). Between 30% and 60% of the patients complain of pain in the midthoracic interscapular area between T7 and T9, which corresponds with the apex of the deformity (23). Increased facet-joint stresses can cause excessive lordosis in the lumbar area. On physical examination, rigid hyperkyphosis and a rigid spine are observed. When the patient is viewed from the side, the Adam forward-bend test shows the gibbus deformity – an abrupt posterior angulation of the thoracic spine. Mild scoliosis and hamstring tightness is oftentimes seen as well (03). It is imperative to perform a thorough physical and neurologic exam, paying detailed attention to any café-au-lait spots or midline deformities. Abnormal physical exam findings are followed up with anteroposterior and lateral x-ray views of the spine. MRI and CT scan can help further identify the lesions (48).

Disc herniation occurs in 0.4% to 3.8% of the pediatric population, with most cases occurring in the 12- to 16-year-old age group (58). Although trauma is considered to be the most common causative agent for disc herniation, several authors argue that trauma exacerbates previous functional and morphological anomalies and predispositions in the pediatric population (18). For example, Varlotta and colleagues found a significant positive family history in children with disc herniation as compared to a control group, indicating a familial trait at play (75). Disc herniation is likely to present with pain only if it is associated with structural deformities like disc prolapse or Scheuermann disease, which may delay the diagnosis of this process. Patients may present with localized pain and sciatica – a sign of nerve root compression or irritation. One distinctive feature present in the pediatric population with disc herniation is that 90% of patients have a positive straight leg raise test elicited on physical exam. Plain films are not useful in evaluating disc herniation. The gold standard for diagnostics of herniated disc is followed up with MRI (50).

Juvenile idiopathic arthritis rarely presents as back pain because it affects the cervical spine and spares the thoracolumbar region. Juvenile idiopathic arthritis affects more boys than girls and presents in late childhood and adolescence. Patients complain of sacroiliitis, whereas back pain is a later finding. During forward flexion on physical examination, loss of lumbar lordosis and flattening of the lumbar spine are noted in addition to reduced range of motion of the spine. Synovial changes are apparent on MRI scans, and patients are referred to pediatric rheumatology for further management (16).

Primary tumors of the spine are rare in childhood and usually present with vague symptoms of malaise and fatigue. Some patients complain of painful scoliosis as well as consistent pain and nighttime pain, and may have no neurologic abnormalities. Osteoid osteoma, osteoblastoma, and aneurismal bone cyst are the most common benign spine tumors of childhood. Malignancies of the spine include Ewing sarcoma, lymphoma, leukemia, and metastatic lesions. Ependymomas and astrocytomas are rare spinal cord tumors usually found in the cervical region and oftentimes present as pain, torticollis, or sensory disturbances. Suspicion of tumors is worked up by obtaining blood work, which may show nonspecific abnormal findings; however, MRI and CT scans are crucial in diagnosis. Patients are then referred to pediatric oncologists and neurosurgeons for assessment of treatment and possible resection of tumor (16).

Clinical vignette

A 12-year-old boy presented to the clinic with a 6-month history of low back pain with radiation to the left hip and right knee. He also had tightness in his hamstrings, left more than right, which was interfering with his gait. He was unable to extend his legs or touch the ground with his hands without bending his knees. His stiffness was worse in the morning and improved throughout the day. Advil^® and Motrin^® did not alleviate the pain, but heat pads did. He reported no bowel or bladder incontinence. His physical exam was normal for sensation, strength, and muscle bulk, but he had positive straight leg raise test, which was worse on the left leg.

Initially the patient was sent to an orthopedic surgeon by his pediatrician. He was treated with rehab for possible slipped capital femoral epiphysis, which did not help. An MRI pelvis did not support the diagnosis. He was later seen by neurology and referred to a neurosurgery clinic with an MRI of the lumbar spine that revealed a broad-based disc bulge at L5-S1. A microdiscectomy was offered, and the patient underwent the procedure with a minimally invasive approach. The thecal sac was satisfactorily decompressed.

Postoperatively, the patient’s left leg radiculopathy was improved though his hamstring tightness persisted. The hamstring tightness subsequently improved with a course of physical therapy.

Biological basis

Anatomic localization

Sacroiliac joint pain usually occurs in the buttocks and rarely radiates to the legs (71). The majority of patients with sacroiliac joint pain have tenderness at the sacral sulcus, which is the soft-tissue depression medial to the posterior superior iliac spine (17; 71). The apex of classic type I Scheuermann kyphosis is most commonly localized between T7-T9, and the apex of type II is localized between T10-T12 (74). Discitis usually occurs in the lumbosacral region, whereas osteomyelitis can be found throughout the spine (16). Juvenile idiopathic arthritis usually involves the cervical spine, whereas the thoracic and lumbar spine is oftentimes spared (16). Ependymomas can occur anywhere along the spine; however, they are often found between L4-S1. Astrocytomas are usually found in the proximal spine (54). Spondylodiscitis usually occurs in the lumbar vertebrae but can occur on any vertebral disc and adjacent vertebral bodies (47).

Pathophysiology

Sacroiliac joint pain is reported by young athletes with a history of trauma. Young females are susceptible to sacroiliac injury because of the laxity of their developing pelvic girdles. Sacroiliac pain can be differentiated from radiculopathy with electromyelography studies (71).

Spondylolysis, which usually presents in young athletes over the age of 10, is a defect in the pars interarticularis – the weakest part of the vertebral body. This abnormality can be congenital, can follow stress-related injuries, or may manifest in individuals with a genetic predisposition. Gymnastics, ballet, weightlifting, and football are among the activities that cause hyperextension and rotational loading of the spine that lead to repetitive trauma and, consequently, stress-related fractures resulting in spondylolysis (16). Spondylolysis is usually asymptomatic until the onset of puberty, which coincides with the experience of rapid linear growth (22).

Spondylolysis can evolve into spondylolisthesis, which occurs when a defect in the pars interarticularis leads to forward translation of 1 vertebra in relation to the next vertebra. Oftentimes, the slippage takes place in the fifth vertebral body and is classified into 1 of 4 grades depending on a calculated slippage percentage (40).

Disc herniation in children often requires surgery because the epiphyseal cartilage in this population is not fused, and trauma can cause a mass on the herniated disc. Additionally, the nucleus pulposus in these children is well hydrated, which leads to lower resorption, unlike a similar degenerative lesion in adults (76).

The exact pathophysiology of Scheuermann kyphosis is unclear and remains under debate. Twin studies reveal a genetic component to disease heritability as there is stronger association of Scheuermann kyphosis in monozygotic twins when compared to dizygotic ones (14). Scheuermann originally attributed the deformity to aseptic necrosis of the ring vertebral apophyses (64). However, histological evidence shows irregular mineralization, abnormal endplate vertebral cartilage, disordered vertebral ossification, as well as altered collagen-proteoglycan ratios (04). In 1 landmark study, 93% of bone specimen from suspected Scheuermann kyphosis cadavers had Schmorl nodes (65). Schmorl nodes are formed when intravertebral discs herniate into the end plate of a vertebral body (51). Associations with Legg-Calvé-Perthes, dural cysts, hypertonia, growth hormone hypersecretion, and infections are also reported in literature (55). It is uncertain if the abnormal histological findings are a result or a cause of the disease (23). Kyphosis may be the first occurring symptom, which leads to increased anterior force, which in turn causes anterior body wedging and, consequently, the radiologic and histologic changes discussed above (79). In a groundbreaking twin genetic study, Damborg and colleagues found the heritability of Scheuermann disease to be about 74% (14).

Young athletes who participate in sports like weightlifting, gymnastics, and wrestling expose their spines to repetitive microtrauma or single trauma, which can cause apophyseal ring fractures. These fractures occur in vertebral bodies before they have been completely fused. Apophyseal fractures take place at the junction of the vertebral body and cartilaginous ring apophysis (21).

Discitis affects young children because the blood supply to the intervertebral disc and the cartilaginous vertebral end plate is different than it is in adults. There are multiple anastomotic channels that communicate between the vertebral end plate and disc in children that provide a hematogenous route of delivery for bacteria to the disc. These channels involute by adolescence, leaving behind end arteries (80).

Spondylodiscitis can occur either by hematogenous or non-hematogenous spread, with hematogenous spread being the most frequent route. Hematogenous spread involves allowing bacteria from distant sites to contaminate the spine in the setting of bacteremia. Infection origin can include respiratory, skin, oral, urinary, or gastrointestinal tract or any implanted device (47).

Epidemiology

It is evident that the yearly incidence of back pain is associated with growth and pubertal changes. Back pain seeking medical attention during growth periods increases by 12% at the age of 11 years to 22% by the age of 15. In regards to lower back pain, research suggests pediatric patients are less likely to seek medical treatment, with only 24% of patients seeking medical attention for their symptoms. There is an association between back pain in boys and an increase in physical activity such as playing sports (50). One study of American children and adolescents determined that treatment of back pain was sought in 40.9% of participants, of which physical therapy was the most common. The prevalence of back pain was associated with increasing age and was more common in females (19).

Pediatric nonpathologic spinal pain is associated with older age (> 12 years), time spent with learning or watching TV, uncomfortable school desks, sleeping problems, general discomfort, and positive family history (01). One study of 11,619 children and youth in Poland found a self-reported prevalence of lower back pain of 31%, 51.9%, and 71.2% among children aged 10 to 13, 14 to 16, and 17 to 19 years, respectively (29). However, true prevalence of back pain in children or young adolescents remains undecided, with 1 review citing a wide range of 7% to 78% (05).

Differential diagnosis

Listed below are the common differential diagnoses of the various etiologies of back pain (16; 22):

Etiology	Differential diagnosis
Trauma	• Intervertebral disc herniation • Spondylolysis, spondylolisthesis
Infection	• Discitis • Vertebral osteomyelitis
Congenital deformities	• Arachnoid diverticulum • Scheuermann disease • Scoliosis • Spinal fusion • Spinal stenosis
Neoplasm	• Aneurysmal bone cyst • Benign osteoblastoma • Ependymomas • Ewing sarcoma • Lymphoma • Neurofibroma • Neurofibrosarcoma • Osteoid osteoma • Schwannoma
Systemic	• Juvenile idiopathic arthritis • Back pain • Spinal injury • Lumbago

Diagnostic workup

The history and physical examination are important in guiding the diagnostic workup for a child with back pain as there is no standard laboratory workup. For example, signs of infection, like fever, malaise, and weight loss, must be investigated by blood culture, chest radiographs, and PPD testing, if indicated. Additionally, a complete blood count with differential acute phase reactants and lactate dehydrogenase are indicated if malignancy is suspected (16).

Although spinal radiography may be the first step to rule out bony tumors, magnetic resonance imaging (MRI) is quite often the golden standard for diagnosis. MRIs can have limitations in the pediatric population as they are expensive and may require the patient to be sedated, which often requires an anesthesia team at a tertiary pediatric hospital (16).

Sacroiliac joint pain can be diagnosed with a proper physical exam; 89% of patients with sacroiliac joint pain in a study had tenderness in the sacral sulcus. Although this physical finding has low specificity, it has the highest positive predictive value of any other physical examination test when combined with maximal pain below L-5 (17). However, the definitive diagnostic test for sacroiliac joint pain is sacroiliac joint block (71).

According to 1 study, spondylolytic lesions are seen on x-ray 30% to 38% of the time (56). The diagnostic test of choice in a young athlete with back pain is lumbar single photon emission computed tomography (SPECT) scan. If the SPECT scan is positive, a CT scan is performed to localize and identify the defect. If the SPECT scan is negative for spondylolysis, then an MRI may be performed to look for other possible etiologies for back pain (78).

AP and lateral x-ray views are the initial diagnostic tests of choice for Scheuermann kyphosis. Schmorl nodes, disc space narrowing, loss of disc space height, kyphotic deformity, and increased anterior-posterior diameter of the apical thoracic vertebrae may be identified on radiographs (16).

Patients who present with discitis often have abnormal x-ray findings. X-ray images show decreased disc space height as well as erosion of adjacent vertebral endplates. Technetium-99m bone scans also show increased uptake in affected areas. Further investigation by MRI can also show the precise nature of these abnormalities (16).

The synovial and inflammatory changes in juvenile idiopathic arthritis are best viewed by MRI scans with contrast. Plain radiographs have the lowest sensitivity for detection of early arthritic changes, and bone scintography and CT scan can provide a diagnosis; however, they are associated with high radiation exposure (16).

Tumors of the spine and spinal cord can be visualized on plain radiographs; however, MRI and CT scans provide a better view of the lesions. MRIs are necessary to localize meningeal, cord, or nerve sheath tumors. Additionally, bone scintigraphy is required to stage malignant bone tumors (16).

A diagnostic algorithm for a child presenting with back pain exists and includes: complete blood count, C-reactive protein, computed tomography (FX), erythrocyte sedimentation rate, magnetic resonance imaging, nonsteroidal antiinflammatory drugs, and single-photon emission computed tomography (50).

Diagnostic workup for spondylodiscitis includes an MRI as the detection method of choice for evaluation and identification. In addition to radiologic imaging, laboratory studies such as complete blood count, ESR, CRP, blood cultures, and PCR are obtained. Biopsy may be considered if suspecting spondylodiscitis and blood cultures are negative (47).

Management

Conservative management is usually the first line of therapy for back pain in children; this includes NSAIDs, rest, and physical therapy (16). The most effective management of spinal cord tumors in children is gross total resection by decompressive laminectomy, followed by radiation (54).

There are multiple ways to manage spondylolisthesis, which range from no treatment and observation, to bracing, and finally to surgical management. Wiltse and Jackson created a system to help decide which therapy is most effective (81). Asymptomatic children under 10 years of age with a slippage of 25% or less should be monitored with radiographs every 4 to 6 months. Limitation of activity, thoracolumbar orthosis, and Williams flexion exercises are the most effective options in managing slippages less than 50% (61). Frequency of radiographs can be reduced as the patient ages and eliminated once the patient’s growth spurt has ended. The treatment for a child with slippage greater than 50% is oftentimes surgery, regardless of the presence of symptoms (81). The amount of slippage and the degree of lumbar lordosis are the 2 main factors that are considered when making a decision to operate (82). The most common and effective procedure is bilateral posterolateral fusion whereby an autologous iliac bone graft is also utilized (42). Slippages less than 50% only require single-level fusion at L5-S1; however, more severe slippages require L4-S1 fusion (62). Studies show that patients who had reduction casts a few days after surgical correction of severe disc slippage and kyphosis had greater outcomes in terms of sagittal translation and kyphosis than those patients who did not have reduction casts (13).

Conservative therapy for disc herniation includes NSAIDs, rest, and physical therapy. However, conservative management is not as effective in managing disc herniation in children as it is in adults (66). Surgery is the recommended treatment for herniated disc when the pain is refractory to 4 to 6 weeks of conservative management, pain affects a patient’s daily activities, if there is presence of cauda equina syndrome or other neurologic deficits, and presence of spinal deformities. Posterior laminectomy with subtotal hemilaminectomy is oftentimes the procedure of choice (58). Percutaneous endoscopic discectomy is also routinely performed and is advantageous because it reduces postoperative soft tissue swelling and pain (46; 15). However, there is limited evidence in the literature discussing whether or not minimally invasive decompression is superior to open discectomies.

Discitis is usually treated with a variable range of antimicrobial therapy; however, some authors believe that discitis in children is a self-limiting condition that does not require antibiotics (69; 28). Recommended therapy can include 10 to 14 days of intravenous antibiotics followed by 4 weeks of oral antibiotic medication (12). The key to successful rehabilitation is spinal immobilization, which can vary from weeks to months (28).

Juvenile idiopathic arthritis is initially managed with nonsteroidal antiinflammatory (NSAID) therapy. Children are often given ibuprofen, indomethacin, or naproxen. In monoarticular juvenile idiopathic arthritis, NSAID therapy can be replaced or added to intraarticular steroid injections of triamcinolone hexacetonide. If a patient’s pain and stiffness are refractory to the therapies mentioned above, low-dose corticosteroids and possibly low-dose methotrexate are used in treatment (77; 63).

In patients with spondylodiscitis, treatment goals are to eradicate infection or neurologic deficits, preserve spinal structure, and relieve pain. Broad spectrum antibiotics in addition to immobilization and physical therapy are the most effective treatment options in most cases. Surgical treatment is only indicated in those patients with spinal cord compression or cauda equina, progressive neurologic deficits, or failure to respond to conservative treatment options (47).

Outcomes

Conservative management of spondylolysis and spondylolisthesis includes cessation of sporting activities and using a spinal brace with lumbar orthosis for at least 3 to 6 months. Previous research suggests bracing has positive effects on healing; however, newer studies suggest bracing has limited effects on healing and approximately 80% of patients heal within a year with rest and physical therapy with emphasis on core training (73). With physical therapy and rehabilitation that includes stretching and strengthening, most patients resume full activity within 6 months of intervention (38). Patients with high-grade slippage who are unresponsive to 6 months of conservative management, or those who have neurologic deficits, may undergo surgery for repair (16). These surgical patients also resume activity within 5 and a half to 6 months postintervention with adequate rehabilitation (60). A multicenter retrospective study of 50 adolescents determined that posterior spinal fusion of spondylolisthesis is associated with a 40% reoperation rate and high rate of postoperative radiculopathy (52). Patients with favorable factors of early detection, unilateral involvement, and location at the fourth lumbar vertebra had the highest likelihood of bony healing (05).

The majority of patients with Scheuermann kyphosis experience resolution of symptoms with over-the-counter NSAID therapy and spinal maturity (16). Most cases are treated with physical therapy and hamstring stretching. Additional interventions may include bracing, but no research supports significant long-term benefits (73). Indications for surgical intervention include severe progressive kyphosis over 70 degrees, back pain refractory to conservative therapy, progression of kyphosis despite bracing, and dissatisfaction with cosmetic deformity (41). Surgical intervention provides significant improvement in deformity. One large literature review on various surgical approaches in Scheuermann disease revealed an average improvement of 30 degrees in kyphosis in postoperative patients (43). Minor complications of surgery include hemothorax, pneumothorax, and wound infection. One of the major complications of surgery is junctional kyphosis, which consequently requires revision surgery (41).

Conservative management of lumbar disc herniation with no neurologic deficits includes NSAIDs, physical therapy, and limitation of activity. Although there is much room for debate on the efficacy of conservative versus surgical management, most authors in the literature state that surgical intervention is superior for children with herniated discs due to the unique nature of the pediatric spine. Additionally, trauma is the most common precipitating factor of disc herniation in the pediatric population. According to one literature review, positive short-term outcome for surgical management was between 93% and 100%. Long-term positive outcome was lower, ranging between 67% and 88%. Minor postoperative complications include wound hematoma and wound infection. Major complications include narrowing of disc space, foraminal stenosis, disc degeneration, and recurrent herniation of the disc at the same level as the initial operation (25; 37).

Discitis in children is a self-limiting condition. Management of discitis is oftentimes conservative and includes immobilization of the spine in order for the lesion to heal as well as antibiotic therapy for 4 to 6 weeks. Sixty-five percent of children in a study by Kayser and colleagues had bony/fibrosis ankylosis of the affected segment in the spine. Interestingly, ankylosis was observed at a lower rate in younger patients. In addition, follow-up at least 10 years of discharge revealed that 80% of patients who returned had no symptoms, and 20% of patients reported back pain (28). All of the patients in the follow-up study had radiograph abnormalities consistent with kyphosis. Some patients also had high-grade intervertebral disc space narrowing as well as fusion of the vertebrae.

Treatment of spinal cord neoplasms requires surgical resection followed by radiation. In a long-term landmark study by O’Sullivan and colleagues, 20-year survival for patients with primary spinal cord tumors was 67% following resection and irradiation (54). However, this condition and treatment is associated with high neurogenic and spinal morbidity. Patients often experience progressive spinal deformities, recurrent tumors, and neurogenic disabilities either due to the primary tumor, the nature of resection, or by the harmful effects of radiation (72). Radiation therapy is reserved for malignant tumors, postoperative regrowth or recurrence, or when there is substantial residual tumor that has been deemed unresectable (36).

Juvenile idiopathic arthritis is believed to have a good prognostic outcome, with 40% to 60% of patients in one study being in remission at the time of follow-up (53). Indicators of poor prognostic outcome include early radiographic involvement, early hip involvement, and presence of rheumatoid factor. Chronic arthritis can affect bone and joint development, which can lead to leg-length equality and developmental problems with the hip (39). NSAIDs are used to reduce structural damage and biologics including antitumor necrosis factor agents are used to improve symptoms of early morning stiffness and function. Disease-modifying antirheumatic drugs such as methotrexate are used when NSAIDs alone do not relieve joint pain and swelling (73).