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 Table of Contents  
REVIEW ARTICLE
Year : 2017  |  Volume : 1  |  Issue : 3  |  Page : 45-49

Strategies in early-onset scoliosis treatment


Department of Pediatric Orthopaedics, Medical University of Lublin Ul. Gębali 6, 20-093 Lublin, Poland

Date of Web Publication18-Dec-2017

Correspondence Address:
Dr. Michal Latalski
Ul. Gebali 6, 20-093 Lublin
Poland
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/EJSS.EJSS_14_17

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  Abstract 


Background: Early-onset spinal deformities are still challenging to pediatric orthopedic surgeons. One still looks for the ideal treatment of these deformities. Preservation of thoracic motion, spine growth, and protection of cardiac and lung function are crucial to the success of the treatment. Purpose: The purpose of this review is to present the reader's brief description of available methods of treatment of early- onset scoliosis (EOS). Methods: Authors searched the PubMed for locating and selecting the data. Extracting and synthesizing the data were done by orthopedic surgeons based on their knowledge and experience in the treatment of EOS patients. Results: The current treatment techniques include nonsurgical strategies, such as body cast or brace in younger patients with a smaller curve (<50°). Surgical treatment of spinal deformity should be considered when progression increases. Definitive fusion is rarely indicated in young patients. Strategies based on compression (staples or tethers), distraction (growing rods, vertical expandable prosthetic titanium rib), and growth guiding (Shilla, growth guidance system) are presented with their advantages and disadvantages. Repeated surgeries – planned and unplanned are burdensome for patients. Although technology improves, the complication rates in the treatment are still high. Conclusion: Treatment of EOS is challenging. Although much is already known about the treatment of children with EOS, there is still no gold standard in proceedings.

Keywords: Early-onset scoliosis, growing rods, infantile scoliosis, pediatrics, spine surgery


How to cite this article:
Latalski M, Fatyga M, Starobrat G, Danielewicz A. Strategies in early-onset scoliosis treatment. J Spinal Stud Surg 2017;1:45-9

How to cite this URL:
Latalski M, Fatyga M, Starobrat G, Danielewicz A. Strategies in early-onset scoliosis treatment. J Spinal Stud Surg [serial online] 2017 [cited 2018 Oct 21];1:45-9. Available from: http://www.jsss-journal.com/text.asp?2017/1/3/45/221105




  Introduction Top


According to the consensus statement on early-onset scoliosis (EOS) by The Scoliosis Research Society (SRS),[1] EOS refers to spine deformity that is present before 10 years of age. This term suggested Dickson in 1994[2] and includes not only idiopathic scoliosis but also those children with neuromuscular, congenital, or syndromic scoliosis. This classification is based not on the degree of the deformity, but on the functional abilities of the child connected with its lung and thorax growth. During growth, complex phenomena occur quickly one after another. They are well synchronized so that the length of the extremities and the spine are proportional. However, they do not grow at the same pace. The proportions change because each segment of the body grows at its own pace. The smallest mistake in development can lead to deformities that have a negative impact not only on the growth but also on the shape of the chest and the volume and the development of the lungs.[3],[4],[5],[6],[7],[8],[9] The resulting deformation shows a domino effect. Curved spine subject to rotation and torsion causes deformity of the chest. It reduced its volume which effects on lung development. Over time, scoliosis becomes a severe general pediatric disease with a reduced body mass index, the presence of cor pulmonale and thoracic insufficiency syndrome (TIS).[10],[11] In the most severe cases, these changes may be fatal.[12],[13]

The purpose of this review is to present the reader's brief description of available methods of EOS treatment.


  Methods Top


In this systematic review, we searched the databases of PubMed, Medline, ProQuest, and Google Scholar from 1982 to 2017 and a partial gray literature search was also conducted.

Authors entered into databases engine keywords: early onset, juvenile, scoliosis, strategies conservative, fusionless, 184 results were found. Only English language publications were included in this study.

The treatment goal of EOS is to minimize spinal deformity, with maximizing lung function over the life of the patient. The next goal is to delay and to minimize the extent of any final spinal fusion with maximizing motion of chest and spine. Treatment should minimize complications, procedures, hospitalizations, and burden for the family. Considering the overall development of the child is necessary.[1]

Observation is mentioned as one of the strategies, but it cannot be regarded as a treatment. It can be used as a monitoring of the curve behavior. Repeated examination and radiographic evaluation during development give a chance to look for the progression of the deformity. While the curve progress, the appropriate treatment should be used.


  Results Top


Conservative treatment

Nonoperative treatment should be taken as the first-choice method.

Although manipulation, physical therapy and exercise have not been shown to influence spinal deformity in EOS by early-onset scoliosis consensus statement, SRS Growing Spine Committee.[1] Physiotherapy scoliosis specific exercises (PSSE) are therapeutic interventions accepted by the 2011 International society on scoliosis orthopedic and rehabilitation treatment. PSSE is recommended for patients with adolescent scoliosis, but it seems to be a good alternative for parents and children with EOS against so-called “wait and see” approach. There are four randomized controlled trials, which have provided strong proof that PSSE is effective for treating Adolescent Idiopathic Scoliosis (AIS) patients with mild and moderate curves.[14],[15],[16],[17]

It is impossible to fully eradicate idiopathic scoliosis with conservative treatment techniques available at present. However, it is possible to prevent further progression, which is usually sufficient according to the SRS criteria.

Casting

While scoliosis progress, other solution is indicated. Body casts have a long history in the treatment of spinal deformity. The elongation-derotation-flexion technic in the correction of scoliosis described Cotrel and Morel in 1964.[18] In 2005, Mehta published her prospective study on the cast treatment of infantile progressive scoliosis on 136 patients.[19] She reported that the spinal deformity was completely corrected if the treatment with casting started early in children with moderate curves. Cast treatment did not resolve the curves in older children with severe curves; however, the curve magnitude was effectively reduced. Following this, currently, the use of body casts is limited to early-onset scoliosis. It provides a nonremovable and well-molded jacket thus imparting a consistent corrective force on the young spine.[20] A small child grows very fast, that is why the frequent need for adjustments to these changes makes brace use impractical. Cast has to be changed every 2–3 months; it adds the disadvantage of repeated general anesthesia. Detailed cast application techniques are also described.[21],[22],[23]

Serial casting can be considered as a definitive treatment for most idiopathic curves and as a delay surgical procedures in more severe idiopathic, syndromic, and even congenital curves.[24],[25]

Bracing

Although a very fast grow makes brace use impractical in very young children, it can be considered in an older one. In the literature, there are very few publications that have evaluated the effects of conservative treatment in EOS patients. The success rate of bracing in the scoliosis treatment varies. Authors mainly describe slowing or stopping the progression of the curve and avoiding or delaying spine fusion. An excellent prognosis with part-time bracing for smaller curves and a poor prognosis in patients with greater Cobb angles reported Kahanovitz et al.[26] One-third of the patients of Tolo and Gillespie [27] treated with the brace-needed surgery. Dabney and Browen reported similar results.[28] Figueiredo and James [29] Mannherz et al.,[30] and McMaster [31] reported up to 87% of the necessity of spinal fusion.

In 2014, Aulisa et al. reported much more promising results of brace treatment in juvenile idiopathic scoliosis.[32] In this group of patients, children with curves under 30° obtained a correction in 83% of cases (incidence of surgery: 1.6%) while curves over 30° reached a correction in 72.2% of cases, with surgery recommended in 5.5% of patients. About the patients who abandoned the treatment, the results showed a progression of the curve, at the time of discontinuation, only in the 11% of cases.

Of course, the early diagnosis of scoliosis is crucial to facilitate the early administration of conservative treatments. For that purpose, school screening that is a predictive and sensitive tool with a low-referral rate can be used.[33],[34]

Surgery

Various growth-friendly are designed for EOS treatment which allows spine and chest to grow with spine and chest deformity control. Surgery is generally indicated when the cast and brace treatment failed with controlling the deformity or if curve pattern does not appear amenable to brace or cast treatment.[1] Extensive thoracic spinal fusion in the very young child is not indicated as is associated with pulmonary compromise. The inhibition of thoracic growth results in a smaller chest with decreased vital capacity and restricted pulmonary disease. The literature does demonstrate that traditional early spinal fusion of the thoracic spine does not allow proper pulmonary development in young children.[11],[35],[36],[37],[38],[39]

Although early surgery may result in success in limited congenital deformities such as single hemivertebra,[40] definitive spinal fusion in early childhood may not successfully prevent future spinal deformity as the child grows and matures. Dubousset et al. described the crankshaft phenomenon.[41] Authors noticed that the more immature the patient, the greater the resultant progression. Goldberg et al.[36] and Karol et al.[38] in their papers suggested, that early fusion, even with both anterior and posterior procedures, cannot be considered definitive in a significant number of cases. The continued progression in 45% of the patients of Goldberg et al. fused before age 10 were observed and revision rate after fusion in the 39% series was reported by Karol et al.).

In addition, definitive spinal fusion in youngest children with early-onset scoliosis has been shown to inhibit thoracic growth, as evidenced by a decreased T1 to T12 height.[35],[38],[42] More than 60% of these patients with short thoracic height already had <50% of normal forced vital capacity, indicating they have restrictive lung disease.

The aim of treatment children with EOS thus becomes a control (slowing down) progression of deformation. However, the precise timing of successive stages of operating the child grows is difficult and always be reckon with the need of additional operating intervention. Therefore, one still looking for a method of scoliosis correction allowing undisturbed growth of the spine.[43],[44],[45],[46],[47],[48]

Growth-friendly techniques

Compression-based

The concept of placing vertebral staples on the convex side of scoliosis in an immature patient seemed should arrest convex growth while allowing normal concave growth to continue and gradually correct scoliosis. The idea was described by Smith et al. in 1954.[49] Unfortunately, the early clinical experience with stapling was disappointing with limited correction, broken staples, and staple dislodgement. That is why a staple using nitinol was designed, a shape memory alloy, where the prongs are straight when cooled, but clamp down into the bone in a C-shape when subjected to body temperature. Betz et al. reported, that none of the patients with nitinol staples with preoperative curves with Cobb angles <30° progressed whereas 18% of preoperative curves ≥30° progressed.[50] Anterolateral flexible tethering may have an advantage compared with staples because the continuous tethering may allow for continued correction through vertebral remodeling. Crawford and Lenke described a case of a 5-year-old boy with 40° juvenile idiopathic scoliosis. After flexible tethering, the correction to 6° was obtained in 4 years follow-up.[51] The success of vertebral stapling and anterolateral flexible tethering is thought to be secondary to asymmetric growth created by the instrumentation. One of the problems is that the stapling or tethering spans the flexible discs, and the immobilized discs may be subject to degeneration. Aronsson and Stokes suggest that the corrective forces can then be focused in the flexible disc, rather than creating the desired effect of modulating vertebral growth and remodeling.[52] Another problem is the concave vertebrae already have decreased growth because of prior loading, and the stapling or tethering operation is usually performed only after documented the progression of scoliosis. The ideal time to use a growth tethering procedure seems to be before peak growth velocity, before menses. Further, follow-up of the patient cohort and further research into efficacy and indications for stapling and tethering are warranted.

Distraction-based

In the early 1950s by Harrington,[53] who used a stainless steel rod-and-hook system to correct spinal deformities. The system dramatically improved the scoliosis surgery, but it had several disadvantages like a requirement for long-term bed rest and the extended use of a plaster jacket after surgery, hook dislodgement, and other instrumentation failures, pseudarthrosis, or flat-back syndrome. Distraction-based surgery using a single subcutaneous distraction rod is still widely used.

Akbarnia et al.[54] popularized a dual growing rod (GR) technique. It enhanced the stability of the construct and reduced the risk of implant failure.[55] In this techniques, pedicle screws and hooks are used for cephal and caudal foundation of the construction with connected rods with special connectors.

Xu et al. showed in the meta-analysis that the dual GR technique can achieve a better coronal correction rate and lengthening with fewer implant-related complications and more wound complications.[56]

In neuromuscular patients, the surgeon should consider pelvic fixation for the distal anchor. Sponseller et al.[57] demonstrated significantly better correction of pelvic obliquity and coronal deformity using dual rods and iliac screws compared with other types of pelvic fixation.

In general, the rods are lengthened at 6-month intervals, making this modality problematic in children with high comorbidities that can complicate the numerous anesthetics, surgical procedures, and hospitalizations. Hosseini et al. described in their research that lengthening intervals were not statistically different in rod fracture and no rod fracture groups and there was no association between lengthening interval and rod fracture in traditional GR cases. It was shown that patients who had rod fracture were younger and were more likely to have stainless steel rods with smaller than 4 mm diameter.[58] Finally, there appears to be a “law of diminishing returns” for repeated lengthening of GRs. Sankar et al.,[59] reporting on 38 patients, noted the mean T1-S1 length gain from the first lengthening was 1.04 cm and progressively less with subsequent procedures. Authors suggest that this phenomenon may be due to autofusion of the spine from prolonged immobilization by a rigid device.”

The vertical expandable prosthetic titanium rib (VEPTR) is a distraction device which was designed for treatment of skeletally immature patients with TIS (Emans JB, Caubet JF, Ordonez CL, Lee EY, Ciarlo M). The treatment of spine and chest wall deformities with fused ribs by expansion thoracotomy and insertion of VEPTR: a growth of thoracic spine and improvement of lung volumes.[44],[60],[61]

As with growth rods, repeated lengthening are required. Originally intended to treat children with TIS secondary to fused ribs and congenital scoliosis, the indications have expanded to include all etiologies of progressive EOS (congenital, neuromuscular, and syndromic with or without fused ribs) in which the surgeon wishes to avoid or minimize direct exposure of the spine.

The system, however, has its limitations. The contraindications include an insufficient strength of the bone of the ribs and/or the spine in the implant placement sites, congenital absence of the cephal and caudal ribs to which the construct is to be attached. Other specific contraindications are age below 6 months, skeletal maturity or nonfunctional diaphragm. Although [62] both the phenix (France) and MAGEC ([Magnetic Expansion Control System] Ellipse Technologies, Inc., Irvine, CA, USA) are distraction-based systems that can allow growth with remote control theoretically diminishing the complications of infection and lack of soft-tissue coverage. It may be especially helpful in children with comorbidities that make the repeated surgeries necessary with the usual distraction methods especially difficult.[63] Magnetic control GRs are not free from disadvantages. Although it has a lower rate of both deep and superficial infections when compared with traditional GR, it does not completely avoid repeated invasive surgical procedures as previously suggested. It does have a significantly increased risk of metalwork problems and unplanned return to the operation theater.[64] The alternative option is to use spinal growth force to lengthen the assembly; these techniques (Luque trolley, Shilla), using a sliding assembly, are known as growth guidance. Guided growth systems correct spinal deformity by anchoring multiple vertebrae (usually including the apical vertebrae) to rods similar to one stage surgery like in adolescent scoliosis. The difference is, that majority of the anchors are not rigidly attached to the rods. It permits longitudinal growth over time as the anchors slide over the rods. The idea is old. In 1977, Luqué and Cardoso described a construct consisting of two L-or U-shaped rods fixed to the spine using sublaminar wires.[65] The method was burdened with a poor spinal growth maintenance (32%–49%), high spontaneous fusion (4%–100%), and high implant failure (32%).[46],[66]

In 2011, Ouellet described modified construct which consists of inserting apical gliding spinal anchors using muscle-sparing techniques to the proximal and distal fixed anchors found in the dual GR construct.

McCarthy in 2008 described principles of the Shilla instrumentation. The construct uses two rods fixed to pedicular screws inserted in the apical vertebrae, with a fusion of the summit of the curve. Additional pedicular screws are inserted extra-periosteally in the proximal and distal extremities of the rods, enabling the rods to slide.[67] The obvious advantage of the Shilla technique, is that it avoids a number of reoperations.[68] However, it is much more aggressive than the classic GR methods.


  Conclusion Top


Treatment of EOS is challenging. Bracing and casting as nonoperative methods can postpone or sometimes even avoid the need for surgery. Unfortunately, there are still a group of patients where conservatory treatment is not effective. Modern surgical techniques that allow spinal growth are effective in controlling early deformation but show a high rate of complications. The approach to ESO patients requires multidisciplinary teams consisting of orthopedic surgeons, anesthesiologists, pediatricians, and physiotherapists with specific experience, given all the associated abnormalities and comorbidities. Although much is already known about the treatment of children with EOS, there is still no gold standard in proceedings. That is why more studies and development of new solutions are needed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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