Nonsurgical Management of Early-Onset Scoliosis

Review Article Nonsurgical Management of Early-onset Scoliosis

Abstract Robert J. Thorsness, MD Early-onset scoliosis is potentially fatal if left untreated. Although John R. Faust, MD surgical management with growing instrumentation may be necessary, this is not a panacea and is associated with high Caleb J. Behrend, MD complication rates. Recent evidence has demonstrated that James O. Sanders, MD nonsurgical treatment can be an effective early management strategy in delaying or even precluding the need for surgery, especially surgery with growing instrumentation. The goal of both nonsurgical and surgical management is to control or correct the spinal curve to allow appropriate pulmonary development while delaying definitive fusion until an appropriate skeletal age. Although more commonly used to

From the Department of Orthopedic delay surgery, serial cast correction using the Cotrel and Morel Surgery, Rush University Medical elongation-derotation-flexion technique may result in complete Center, Chicago, IL (Dr. Thorsness), correction in patients with infantile idiopathic scoliosis and smaller the Department of Orthopaedics, University of Texas Health Science curve magnitudes. Center San Antonio, San Antonio, TX (Dr. Faust), the Department of Orthopaedic Surgery, Virginia Tech Carilion School of Medicine and arly-onset scoliosis (EOS) is risks of various treatment options is Research Institute, Roanoke, VA (Dr. Ea potentially fatal disorder. The essential for optimal outcomes. Behrend), and the Department of spinal deformity can result in pulmo- Orthopaedics, University of Rochester, Rochester, NY (Dr. nary restriction caused by the distor- Presentation and Sanders). tion of the chest wall, which decreases Evaluation Dr. Sanders or an immediate family chest volume and causes abnormal member has stock or stock options respiratory mechanics. EOS has less History held in Abbott, AbbVie, GE pulmonary involvement than does Healthcare, and Hospira; serves as 1,2 Identification of the etiology of EOS a board member, owner, officer, or late-onset scoliosis and occurs in should be the initial focus of the committee member of the American children aged ,5 years. However, Academy of Orthopaedic Surgeons, other reports have described the patient evaluation in a child with the Pediatric Orthopaedic Society of a spine deformity. Because examining condition in patients aged ,8to10 North America, and the Scoliosis infants and toddlers for subtle clinical Research Society. None of the years.3 Infantile scoliosis is diagnosed and radiographic changes and mild following authors or any immediate in patients aged #3 years, and juve- congenital deformities can be chal- family member has received anything nile scoliosis is diagnosed in patients of value from or has stock or stock lenging, neuromuscular or syndromic aged 4 to 10 years. Adolescent sco- options held in a commercial company features may be overlooked. Table 1 or institution related directly or liosis is diagnosed in patients aged 10 lists some of the more common indirectly to the subject of this article: to 18 years. The etiology of EOS may Dr. Thorsness, Dr. Faust and Dr. diagnoses that can present with spinal Behrend. be idiopathic, congenital, neuromus- deformity in early childhood. cular, or syndromic.4 Here, we define J Am Acad Orthop Surg 2015;23: A thorough history, physical 519-528 EOS as scoliosis that occurs in young examination, and radiographic eval- patients, particularly those younger http://dx.doi.org/10.5435/ uation are required when assessing JAAOS-D-14-00019 than age 5 years, who are still in the a patient for EOS. The clinician early rapid growth phase. EOS is should determine when the curve Copyright 2015 by the American Academy of Orthopaedic Surgeons. often difficult to treat, and an initially presented, whether it has understanding of the benefits and progressed, and if there is associated

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neurofibromatosis type 1 or Marfan appear less severe than is seen with Table 1 syndrome. standing views. No guidelines exist to Common Conditions That Present determine the appropriate age at With Spine Deformity in Early Physical Examination which to change from supine to Childhood standing views; this change depends During the physical examination, the Idiopathic infantile scoliosis on the ability of the child to reliably clinician evaluates the curve, neuro- cooperate for the imaging. The ra- Congenital scoliosis logic status of the patient, and hall- diographs are assessed for the num- Fused ribs with chest wall deformity marks of associated conditions. In ber of rib-bearing and lumbar-type Mesenchymal and connective tissue young children with spinal defor- disorders vertebrae as well as congenital mity, the examination requires care- Marfan syndrome anomalies, such as unpaired pedicles ful observation because manual Beals syndrome and spina bifida. In young children, motor and detailed sensory exami- radiographs may be difficult to Ehlers-Danlos syndrome nations are rarely possible. The cli- interpret because the spine is not yet Neurofibromatosis nician examines the deformity, fully ossified, and the posterior ele- Skeletal dysplasias looking for subtle prominences on ments may not be fully visualized on Spondyloepiphyseal dysplasia one side of the spine compared with the AP view. Assessment of sagittal Storage diseases the other. alignment may not be reliable in Neuromuscular During the neurologic examina- young children, but obtaining a lat- Cerebral palsy tion, gait should be evaluated in eral view is important to visualize Myelodysplasia children who can walk. The clinician congenital vertebral malformations. Syringomyelia also should assess the deep tendon Tethered cord and abdominal reflexes for signs of Spinal cord tumor clonus, Hoffman and Babinski signs, Additional Workup Spinal muscular atrophy and increased or decreased muscle In children who may have syndromic Congenital myopathies tone. The extremities should be scoliosis, a genetic consultation is Poliomyelitis carefully assessed for symmetry necessary. If skeletal dysplasia is Arthrogryposis because neurologic involvement can suspected, radiographs of the cervical Spinal cord injury affect growth, with the affected side spine also should be evaluated. MRI Friedreich ataxia being smaller than the contralateral should be obtained for all patients Syndromes side. Proximal weakness of the upper with spinal curves when neurologic Unspecified syndromes extremities may manifest by the child signs are identified or treatment is Down syndrome slipping through the arms when considered. The goal of MRI is to be picked up by the axilla (ie, Meryon Prune belly syndrome able to evaluate for the presence of sign). Proximal weakness of the lower Prader-Willi syndrome abnormalities (eg, tethered cord, in- extremities may manifest by the Rubinstein-Taybi syndrome traspinal tumor, Chiari malforma- child’s placing his or her hands on the Noonan syndrome tion) that may need to be addressed legs to gradually push up from a sit- Chromosomal anomalies before scoliosis is treated. In children ting position (ie, the Gower sign). with congenital spine deformities, Foot deformities, including equinus, MRI can sometimes demonstrate cavus, or varus, may be the only renal abnormalities, often precluding neurologic signs detected. The child pain. The birth and developmental the need for renal ultrasonography. should be examined for syndromic history should be evaluated, par- features such as low-set ears, a high ticularly with regard to prenatal or arched palate, wide-set eyes, arach- perinatal difficulties (eg, apnea, intra- Natural History nodactyly, and café-au-lait spots. ventricular hemorrhage). Excessive drooling may reflect bulbar dysfunc- Infantile Idiopathic Scoliosis tion. By age 3 or 4 years, most children Imaging Infantile idiopathic scoliosis has two have developed bowel and bladder Initial radiography should include forms: progressive and resolving. control, and difficulties in this regard a PA or AP view and a lateral view of Most cases resolve spontaneously, mayreflectaspinalcordissue.A the spine. In infants, radiographs are but the reported rates vary, ranging careful family history may elucidate obtained with the patient positioned from 12% to 92% based on the potential genetic causes, such as supine, which makes the curve patient population and selection

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5-8 criteria. Spontaneous resolution is Figure 1 more likely in scoliosis that presents during the first few years of life than in scoliosis with a later presentation.9 Left untreated, the prognosis for patients with curves that progress is invariably poor. By age 5 years, 57% of untreated children have a curve .70°.8 In adolescence, thoracic curves .70° are associated with sig- nificantly lower forced expiratory volume and forced vital capacity values than are smaller spinal curves.10 Moreover, these large thoracic curves can cause thoracic insufficiency syndrome, which is characterized by decreased thoracic Illustration demonstrating the measurement of the rib-vertebra angle between growth and lung volume. This in- the apical thoracic vertebra and its rib. The angle is formed by a line drawn from hibits alveolar development and may the mid-point of the neck of the rib to the mid-point of the rib head (a) and a line drawn perpendicular to the upper or lower border of the vertebral body (b). cause restrictive lung disease and (Reproduced from Gillingham BL, Fan RA, Akbarnia BA: Early onset idiopathic abnormal breathing mechanics, scoliosis. J Am Acad Orthop Surg 2006;14 [2]:101-112.) which can lead to pulmonary hyper- tension and cor pulmonale, with respiratory failure and death at an convex side. Mehta8 found that levels of interobserver and intra- early age.5,7,8,11,12 Curves .70° can approximately 80% of curves with observer reliability, whereas the rib ultimately progress and are associ- an initial RVAD $20° and all curves phase demonstrates substantial ated with a higher mortality rate than with ribs in phase 2 had progressed. interobserver reliability.14 However, that associated with smaller curves.11 Other studies have verified these there is poor interobserver agree- results.5,7,12 ment in selecting the apical vertebrae The phase is the relationship and, despite good overall statistical Progressive Versus between the apical rib head and the reliability, 18% of RVAD measure- Nonprogressive Curves corresponding vertebral body. When ments show .10° of variation,14 Mehta8 identified radiographic pre- a rib head is in phase 1, the apical rib which can be particularly problem- dictors that can be used to distin- head on the convex side does not atic with curves that have a RVAD of guish progressive curves from overlap the apical vertebral body on nearly 20°. In borderline cases, resolving curves. These predictors an AP radiograph. In phase 2, the Mehta8 recommended observing the consist of a Cobb angle of .20°, apical rib head on the convex side curve for 3 months; if the RVAD a rib-vertebra angle difference overlaps the upper corner of the apical improved, the curve was likely (RVAD) $20°, and a phase 2 rib- vertebra, indicating a progressive resolving even if the Cobb angle vertebra relationship. The rib- curve8 (Figure 2). Double curves are increased. vertebra angle is measured between more likely to progress than are single Although a modest amount of liter- the apical thoracic vertebra and its curves, despite typically having a low ature exists on the natural history rib (Figure 1). It is the angle between or even negative RVAD at the apex of of infantile idiopathic scoliosis, there a line drawn from the mid-point of the thoracic curve. Mehta8 also found is far less research identifying the the neck of the rib to the mid-point of that double curves demonstrated early natural history of nonidiopathic, non- the rib head and a line drawn per- marked rib asymmetry at T11 or T12, congenital scoliosis. In young children, pendicular to the upper or lower with the concave rib drooped and scoliosis associated with Marfan syn- border of the vertebral body. Once rotation of the two curves in opposite drome is characterized by severe car- the rib-vertebra angle is measured directions. Physically, the RVAD ap- diac and aortic root problems.15 for both ribs, the RVAD can be pears to represent chest wall defor- Typically, the prognosis for syndromic calculated as the difference between mation rather than spinal rotation.13 and neuromuscular scoliosis is worse the RV angle on the concave side of In the setting of infantile scoliosis, than that for idiopathic scoliosis. the curve and the RV angle on the the Cobb angle and RVAD have high Unfortunately, surgical management

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Figure 2

Illustrations demonstrating rib phase, which is the relationship between the apical rib head and the corresponding vertebral body. A, In phase 1, the apical rib head does not overlap the apical vertebral body on an AP radiograph. B, In phase 2, the apical rib head on the convex side overlaps the apical vertebra, which indicates a progressive spinal curve. (Reproduced from Gillingham BL, Fan RA, Akbarnia BA: Early onset idiopathic scoliosis. J Am Acad Orthop Surg 2006;14[2]:101-112.) of these types of scoliosis is also respect to the amount of thoracic terior fusion because the anterior associated with high complication length needed for adequate pulmo- spine continues to grow and rotate rates.16 nary function as an adult. about the fused posterior spine. The Although growing instrumentation VEPTR has similar complications as well as the additional problem of Management plays an important role in the management of EOS, its use can result in creating a stiff, noncompliant chest serious complications. Early instru- wall and causing stretch on the bra- Surgical mentation developed by Harrington23 chial plexus.26,30 The decreasing Treatment of EOS focuses on con- attempted nonfusion correction of effectiveness of growth instrumenta- 28,31 trolling or correcting the deformity of scoliosis. His early experience, how- tion after several lengthenings the thoracic spine and chest wall. On ever, showed that instrumentation means that there is a window of the basis of historic data, the general without fusion regularly failed sec- approximately 4 years for the appli- recommendation is that fusion be ondary to rod or anchor-point fail- cation of growing instrumentation, delayed until children reach the age of ure. Despite the improvements in with wide individual variation. 10 years.17,18 Fusion performed growing instrumentation technology Therefore, this time frame should be before that age results in increased chosen wisely for a successful out- with the advent of the vertical rates of pulmonary compromise.19 come. If surgery can be delayed until expandable prosthetic titanium rib Karol20 and Karol et al21 found that age 6 or 7, then the patient, with (VEPTR), dual growing rods, and patients had insufficient pulmonary judicious use of growing instrumen- growth modification devices, com- function when the thoracic spine tation, has a greater chance of plications such as infection, instru- length was #18 cm at maturity reaching age 10 years before a final mentation or anchor point failure, following early thoracic fusion. fusion is performed. Dimeglio et al22 reported that nor- and spontaneous fusion are common 24-27 mal thoracic spinal height is 11 cm at with all techniques. The VEPTR birth and 18 cm by age 5 years, with is a device that is attached to the chest Nonsurgical growth of 1.2 cm per year between wall to improve chest volume and, Because of the high complication rate the age of 5 and 10 years. Thus, with indirectly, scoliosis by periodic dis- associated with surgical management normal growth, the spine should traction. More frequent lengthenings of EOS and increasing evidence of reach a sufficient length by age 5 appear to be beneficial, but additional successful treatment with early serial years. However, this is earlier than lengthening also can result in more casting, nonsurgical management is predicted in other studies, which complications. Growing rods can becoming more common. Serial estimate that the age is closer to 10 fracture or become loose or infected casting for infantile scoliosis may years.19 The data of Dimeglio et al22 and can cause a form of crankshaft result in complete correction in some suggest that the thoracic length phenomenon with growth.24,28,29 In patients, but it also plays an impor- would be approximately 25 cm by the crankshaft phenomenon, spinal tant role in delaying the need for this time. Uncertainty remains with deformity progresses following pos- surgery in most patients. Nonsurgical

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Copyright ª the American Academy of Orthopaedic Surgeons. Unauthorized reproduction of this article is prohibited. Robert J. Thorsness, MD, et al treatment of progressive EOS in- force. Moreover, because a brace been successfully used in numerous cludes bracing, casting, and halo- functions predominantly to stabilize centers. Compared with bracing, gravity traction (HGT).6 As noted adeformityratherthan reverse it, casting allows for improved fit and earlier, Mehta8 recommended repeat bracingislesslikelythancastingto a constant corrective force. Patients radiographs after 3 months to eval- permanently correct a deformity in with very large spinal curves may uate the Cobb angle and RVAD in a patient with EOS. Nevertheless, benefit from precast HGT, with the the setting of resolving or question- bracing plays an important role in goal of delaying surgical intervention. able spinal curves. At 3 months after delaying the need for surgery. With The EDF cast is applied with the the initial radiographs, the Cobb improved understanding of EOS child positioned on a table with angle and RVAD of resolving curves pathophysiology and mechanics, more a head halter and pelvic traction. The had decreased in size.8 effective braces may be developed. table must provide support for the child while ensuring that the thorax, Bracing shoulders, and pelvis are free for cast Casting Although bracing is efficacious for application and manipulation. Suffi- Sayer34 initially described casting for adolescent idiopathic scoliosis,32 no cient traction is applied to narrow the correction of scoliosis in 1877, but studies have examined its efficacy for thorax and allow the spine to be the early history of casting to treat EOS. Nevertheless, it remains the manipulated. The table must allow scoliosis was really confined to cor- most common nonsurgical treatment secure positioning of the patient with recting curves preoperatively and for EOS. However, successful man- head and pelvic traction, allowing maintaining the correction post- agement with this method has been full access to the torso, shoulder gir- operatively using the turnbuckle cast variable.33 Brace fit is often difficult dle, and pelvis39 (Figure 3). for uninstrumented fusions devel- in young patients. Infants and tod- Manipulation rather than traction is oped by Hibbs et al.35 Turnbuckle dlers typically have large abdomens, the primary means of correction casting did not permit walking, and making a proper pelvic mold diffi- because traction will be removed after Risser36 later developed “localizer cult, and their inability to hold still casting, and the patient’s spine will casting,” which used a frame with can make the lumbar and thoracic recoil unless the cast includes the a head halter and pelvic traction and corrective molding challenging. occiput and mandible. This recoil “localizers” that pushed on the curve Young children also have more pli- must be anticipated or the cast may for correction. Cotrel and Morel37 able ribs than do adolescents, and ride up. A silver-impregnated under- further developed Risser’s technique braces using a three-point bend on garment can be helpful in preventing by identifying the key factors of the apical ribs can deform the chest cast irritation. Silver nanoparticles traction, derotation, and bending wall by pushing the ribs toward the do, however, have the capacity to with an ambulatory cast. They called spine. Furthermore, the habitus in penetrate the skin, particularly when the technique EDF casting for young children is typically more it is damaged, although the clinical “elongation (traction), derotation, cylindrical in shape than in adoles- effects of this are unknown.40 Pa- and flexion (bending)” and sug- cents. This is compounded by the tients must be intubated during the need to make the brace sufficiently gested that serial EDF casting could cast molding because thoracic pres- flexible for donning and doffing. An correct infantile scoliosis. However, sure can make ventilation temporar- experienced orthotist and a dedi- with the introduction of effective ily difficult. cated family that is educated in the spinal instrumentation by Harring- Peak airway pressures can double 23 principals of brace wear are required ton, casting became less popular during the procedure, and the anes- for proper bracing in young patients for management of adolescent sco- thesiologist should be prepared for because each time the brace is worn, liosis, and knowledge of the EDF this possibility. Peak airway pres- it must be put on in an ideal position. casting technique persisted in only sures return to baseline after win- Proper bracing should focus on the a few centers. Mehta38 and Sanders dows are cut in the cast.41 For curves rotation and three-dimensional et al39 reported on their experience with an apex superior to T8, the deformity to maximize chest wall using serial EDF casting for infantile shoulders are included in the cast, corrections rather than simply the scoliosis. Since the publication of these and high thoracic curves may require Cobb angle. Although bracing is encouraging reports, there has been an occipital-mandibular extension. convenient because the brace can be resurgent interest in serial EDF cast- When the apex of the curve is at or removed for bathing and other ing, particularly because of the chal- inferior to T8, casting below the activities, being removable precludes lenges associated with growing shoulders is an option. A mirror it from being a continuous corrective instrumentation. EDF casting has now slanted under the table is useful for

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, ° Figure 3 with supine curves of 20 or those with casts that are maintaining correction, we often use fiberglass with a waterproof liner and padding that allows patients to bathe and get into a swimming pool. Casting is considered complete when the curve has resolved or the cast is obviously failing. We advise families that 1 year of casting is gen- erally considered the minimum period of treatment. We consider a curve resolved when it measures # ° Illustration (A) and photograph (B) demonstrating patient positioning for 10 on standing radiographs after elongation-derotation-flexion casting. The patient is positioned on a table with the cast is removed. In children with a head halter and pelvic traction. A mirror placed under the table can be used to curves that have resolved, we use visualize rib prominence as well as the posterior cast and the molds. bracing for 1 year after the cast has (Reproduced with permission from Sanders JO, D’Astous J, Fitzgerald M, Khoury JG, Kishan S, Sturm PF: Derotational casting for progressive infantile been discontinued. We recommend scoliosis. J Pediatr Orthop 2009;29:581-587.) continuation of casting for unresolved curves until the growth velocity has decreased, typically at visualizing rib prominence, the pos- over the chest and abdomen to relieve age 4 or 5 years, and then using terior cast, and the molds. As the the pressure on the chest and allow for a brace for unresolved curves. If the plaster is applied, it is important to abdominal distention and breathing curve is progressing, particularly if it obtain a good mold over the iliac because younger children are dia- is approaching 70°, we discuss the crests because the pelvis is the phragmatic (ie, belly) breathers (Fig- use of growing instrumentation with foundation of the cast. A well- ure 5). A posterior window is made on the family. Further reasons to dis- molded and snug cast is less likely the concave side of the cast, allowing continue casting include the ribs to rub and cause pressure sores than the depressed, concave ribs and spine being pushed toward the spine on the is a cast that is excessively padded, to move posteriorly (Figure 6). convexity or continued curve pro- poorly molded, or loose. Plaster is For casts under the shoulders, the gression despite casting. Other preferred because it is very moldable superior trim line is at the manu- medical conditions, such as severe and expands slightly when setting, brium. This is not as important for asthma, can make cast wear intol- unlike fiberglass, which contracts. casts that include the shoulders, as erable for patients and families. The cast must not push the ribs long as the upper thorax is casted. toward the spine because that would The lower trim should hold the pelvis Complications narrow the space available for the securely while allowing the hips to Casting can cause skin breakdown lungs. Rather, the posteriorly rotated flex .90°. Lack of hip flexion can although, in our experience, only minor ribs are rotated anteriorly to create cause the cast to ride up when the skin irritation has occurred, and it a more normal chest configuration, patient is sitting, particularly in car has healed without further compli- with counter rotation applied through seats that require significant hip cations. The cast may need to be the pelvic mold and upper torso39 flexion. removed to manage other medical (Figure 4). For a typical left lower Proper casting corrects the curve by issues, such as viral respiratory ill- thoracic curve, the pelvis is carefully rotating and shifting it toward the ness, asthma exacerbation, and molded and stabilized while the left midline without pushing the ribs abdominal surgery, or if the cast be- posterior thorax is rotated anteriorly, toward the spine. If the cast is pushing comes wet or soiled. The repeated use and the right anterior thorax is the ribs toward the spine and nar- of anesthetics for cast application rotated posteriorly and stabilized rowing the convex side of the chest, may carry a risk of complications. In against the left pectoral girdle. we recommend removing the cast and animal studies, neuron cell death Two windows are made to improve either reapplying or abandoning it. associated with the repeated use of the patient’s respiratory capacity Casts are changed every 2 to 4 anesthetics has been reported, but while preventing the lower ribs from months based on the child’s growth. this has not been demonstrated in rotating. An anterior window is made In select patients, typically those humans.42-44 However, another

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Figure 4 Figure 5

Photograph of a child wearing an elongation-derotation-flexion cast, with an anterior window made to improve respiratory capacity.

Illustration demonstrating the elongation-derotation-flexion casting technique. Figure 6 The posteriorly rotated ribs are rotated anteriorly (arrows) to create a more normal chest configuration, with counter rotation applied through the pelvic mold and upper torso. Inset, Illustration demonstrating the location of force applied to the ribs. The goal is to derotate the spine toward normal. (Reproduced with permission from Sanders JO, D’Astous J, Fitzgerald M, Khoury JG, Kishan S, Sturm PF: Derotational casting for progressive infantile scoliosis. J Pediatr Orthop 2009;29:581-587.)

study found that repeated use of against the risks of early surgical anesthetics may be associated with management. Currently, many cen- Photograph demonstrating an learning delays in young children, ters do not have the necessary equip- elongation-derotation-flexion cast although it is difficult to determine ment or education to successfully with a posterior window. whether the delays are related to the manage EOS with serial casting. etiologies that necessitate the repeti- Thus, it is rarely performed outside tive use of anesthetics.45 major academic centers.46 more likely to be successful when There are several variable factors in started at a younger age in patients casting, and the risk-benefit ratio for Results with smaller Cobb angles. Mehta38 casting in certain patients is unclear. In 2005, Mehta38 reported on the classified outcomes based on the In patients with paralytic or neuro- results of 136 patients with pro- patient’s phenotype, but other in- muscular scoliosis, such as those with gressive infantile scoliosis (100 idi- vestigators39 have not found this spinal muscular atrophy or quadri- opathic, 36 syndromic) treated with phenotypic classification easy to plegic cerebral palsy, it is unclear serial EDF casting. Casting stopped apply. whether the benefits of casting out- when radiographs showed a sym- In a study of 55 children with pro- weigh the risks of chest restriction in metric rib cage, derotation of the gressive infantile scoliosis (both idi- this population. Some patients with apical vertebra, and complete or opathic and syndromic diagnoses) EOS have sleep apnea, gastrostomy near complete correction of the who underwent casting, Sanders tubes, silent aspiration, and gastro- curve. A brace was subsequently et al39 reported that the curve esophageal reflux, which may be worn for a few hours each day and decreased in 49 patients (89%). Six compounded by a cast. Furthermore, was discontinued after 6 months if patients (11%) had curve pro- the psychological effect of casting in the correction was maintained. Full gression and underwent surgery. young children is unknown. Because correction was noted in 94 patients Patients who began casting at an the clinical presentation of these pa- (69%) by a mean age of 3.5 years, average age of 1.1 years had near full tients is highly variable, the goals and and partial correction was noted in curve correction. However, full risks of casting must be balanced 42 patients (31%). Casting was curve correction was rare in those

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Figure 7

A through D, PA radiographs of the spine demonstrating correction of a 56° curve in a girl aged 4 years 4 months. B, The patient underwent serial elongation-derotation-flexion casting for 1.5 years. C, Following casting, the patient used a thoracolumbar sacral orthosis brace. D, PA radiograph of the spine obtained when the patient was aged 8 years 6 months demonstrating curve correction to 24°. The casting allowed the patient to avoid surgical intervention with guided-growth instrumentation. If a fusion is required, it will not be performed until adolescence. who began casting later than age 2 15 of 27 patients with growing rods). benefit from preoperative traction to years. For full curve correction, $1 Fifteen of the 27 casted patients improve pulmonary function. Trac- year of casting was required. Curves eventually underwent surgery at an tion must be applied and monitored resolved in patients with idiopathic average of 1.7 years after casting. carefully.51,52 In select patients with scoliosis who began casting at Casting appears to be helpful in af- compliant and resourceful care- a younger age (,20 months old) and fording complete correction in some givers, traction can be done at home, had curves ,60°. Correction was patients with idiopathic scoliosis and with proper monitoring. We have also achieved in curves with smaller smaller curves but also allows an found that appetite often improves rotation and RVAD at the initiation important delay in surgery without during traction in patients with of casting, except in the setting of increasing complications in patients large curves, and we suspect it is double curves. Although full cor- with curves that do not fully correct because of decreased chest pressure rection was not achieved with cast- (Figure 7). on the abdomen; therefore, HGT ing in older patients, in patients with can be used to improve nutrition larger curves or a nonidiopathic preoperatively. diagnosis, casting resulted in curve Traction Multiple pins (typically eight) are improvement and allowed for a sub- HGT can be an important adjunctive used with traction. The weight is ini- stantial delay in surgery. Other treatment. It can be applied to tem- tially light and is gradually increased recent studies have reported similar porarily decrease a curve for casting over 1 to 2 weeks, with a maximum results, including a 2- to 3-year delay or before surgery. It can be used to weight of half the patient’s body in the need for surgery without any improve preoperative pulmonary weight. It is imperative to regularly additional problems in patients who function and may decrease neuro- check the patient’s cranial nerve required growing instrumenta- logic risk by gradually accustoming function, particularly the function of tion.47,48 Johnston et al49 recently the spinal cord to correction. HGT is cranial nerve VI, because this is reported on a case-matched series indicated in patients with very large among the earliest complications that compared patients diagnosed curves, scoliosis associated with associated with HGT, with a re- with idiopathic or syndromic scoli- kyphosis, and decreased pulmonary ported incidence of 0.07%.53 This osis treated with casting with similar or nutritional status who could ben- complication can result in diplopia. patients treated with growing rods. efit from a temporary curve correc- Other signs of excessive traction are Both groups had similar increases in tion and in-hospital monitoring changes in neurologic status, hyper- spine length, but far fewer compli- before further treatment.50 Patients tension, or overdistraction seen on cations were reported in the cast with severe spinal deformities and lateral radiographic views of the group (1 of 27 casted patients versus associated cor pulmonale may also cervical spine.

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5. Ceballos T, Ferrer-Torrelles M, Castillo F, maturity in infantile-onset scoliosis. Spine Summary Fernandez-Paredes E: Prognosis in infantile (Phila Pa 1976) 2003;28(20):2397-2406. idiopathic scoliosis. J Bone Joint Surg Am 1980;62(6):863-875. 20. Karol LA: Early definitive spinal fusion in EOS is potentially fatal if left young children: What we have learned. untreated, and prudent treatment is 6. Diedrich O, von Strempel A, Schloz M, Clin Orthop Relat Res 2011;469(5): Schmitt O, Kraft CN: Long-term 1323-1329. imperative for a successful outcome. observation and management of resolving Early fusion for EOS is fraught with infantile idiopathic scoliosis a 25-year 21. Karol LA, Johnston C, Mladenov K, Schochet P, Walters P, Browne RH: complications. Nonsurgical treat- follow-up. J Bone Joint Surg Br 2002;84(7): 1030-1035. Pulmonary function following early thoracic ment, including bracing, casting, and fusion in non-neuromuscular scoliosis. J traction, can successfully allow 7. Ferreira JH, de Janeiro R, James JI: Bone Joint Surg Am 2008;90(6):1272-1281. Progressive and resolving infantile appropriate pulmonary development idiopathic scoliosis: The differential 22. Dimeglio A, Bonnel F, Canavese F: Normal while delaying definitive fusion until diagnosis. J Bone Joint Surg Br 1972;54(4): growth of the spine and thorax, in 648-655. Akbarnia BA, Yazici M, Thompson GH, the patient reaches an appropriate eds: The Growing Spine. New York, New skeletal age. Serial cast correction for 8. Mehta MH: The rib-vertebra angle in the York, Springer, 2011, pp 13-42. early diagnosis between resolving and infantile scoliosis with the EDF tech- progressive infantile scoliosis. J Bone Joint 23. Harrington PR: Treatment of scoliosis: nique can be used effectively to delay Surg Br 1972;54(2):230-243. Correction and internal fixation by spine instrumentation. J Bone Joint Surg Am surgery and, in certain instances, it 9. 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