Abstract
Casting is being utilized as a therapeutic strategy in some mild to moderate cases obviating surgical intervention for management of early-onset scoliosis (EOS). Bracing, another conservative modality, applies comparable correcting forces on chest wall and axial skeleton. But cast application carries additional advantage of sustained restorative force which bypasses issue of compliance seen with brace wear. There is no specific blanket treatment, conservative or surgical, for the early-onset spinal deformities. Serial cast application provides near total correction of less severe curves (less than 500 to 600) if treatment is initiated before age of 2 yrs. In this review article, we will assess the evolution of plaster cast application in management of EOS and also describe technique of EDF (Elongation- Derotation- Flexion) casting.
Keywords: EOS, Body cast, Plaster of Paris, Risser, Mehta, Elongation -Derotation -Flexion (EDF)
1. Introduction
Casting is being utilized as a therapeutic strategy in some mild to moderate cases obviating surgical intervention for management of early-onset scoliosis (EOS). These conditions may be associated with syndromal or neuromuscular etiologies in few. Spine deformities may affect chest wall and cause a reduction in lung function which warrants prompt intervention.1 In casting for EOS, traction-derotation force to chest wall and axial skeleton can be applied, which can be performed with or without general anesthesia (GA). These forces, when transferred to the axial skeleton, achieve substantial deformity correction. With time, plaster-cast uses growing capability of immature skeleton to guide development of chest wall and vertebrae.2,3 Theoretically, other conservative treatment modalities including bracing apply corrective forces comparable to plaster cast on chest wall including the axial skeleton. However, plaster-cast provides a sustained restorative force and bypasses issue of compliance seen with brace wear.1
There is no specific management strategy for EOS. Surgical treatment options for EOS include distractive, compressive and guided growth based modalities. These procedures need repeated operative interventions and are fraught with many complications.4 Above mentioned techniques can lead to unintended fusion, not allowing dorsal spine from reaching its maximal growing ability and restricting area accessible for development of alveoli. Therefore, delaying surgical intervention is advantageous in young children.5,6
An exclusive phenomenon observed from growth rod technique is the “law of diminishing returns” which may be seen with even early surgical interventions. This could be due to increasing rigidity of growing spine with sustained implants in-situ or automatic fusion due to damaged posterior elements following large distractive force at sporadic interval. It may also be related to variability in radiographic measurements of spine height on coronal plane radiographs7
Serial cast application provides near total correction of idiopathic curves which are lesser than 600 in magnitude if treatment is begun before age of 2 yrs8,9 (Fig. 1, Fig. 2) For higher magnitude deformities in juvenile age group, literature shows successive plaster-cast delays index growth-rod intervention in non-congenital deformity; thereby reducing complication rate.10, 11, 12 The current article discusses the evolution of plaster cast application in management of EOS and also describes technique of EDF (Elongation- Derotation- Flexion) casting.
Fig. 1.
(A) Whole spine X-ray of an 8-month old child with Cobb angle of 520 and RVAD of 390. (B) Corrective casting showing significant decrease in the magnitude of the curve. (C) Anteroposterior X-ray at 5 years 3 months showing near complete correction of the scoliosis.
Fig. 2.
(A)Anteroposterior X-ray of whole spine of an 11-month old child with poor weight gain and developmental delays. (B) Observing for 5 months after diagnosis of scoliosis, after five serial corrective casts at age 3 showing correction of scoliotic deformity and improvement in lung shadow.
2. The history and evolution of casting in scoliotic deformity
The management strategies in EOS have evolved from casting to instrumentation to growth friendly modalities with renewed interest in casting at present. Thus the treatment of EOS has come “full circle” back to where it started. Application of plaster-cast for management of scoliotic deformities dates back to 18th century. ‘Anthonius Mathijsen’, the Dutch military surgeon, had discovered that water-soaked plaster of Paris (POP) bandage hardens in a short time giving adequate support for fractured extremities. POP was soon adapted for managing spine deformity.13
Lewis Sayre in his book on correction of spinal deformities with traction (partial suspension) and application of POP cast, described 10 patients who were mostly adolescent females who appeared to have moderate-sized idiopathic curves, in the section on rotary lateral curvature of the spine.14 Later, Bradford and Brackett (1893) reported their method of lateral pressure and plaster-cast application. They reported 5 patients treated using traction-force device and plaster-cast. They managed full correction of deformity in 4-year old boy. One major limitation of these initial reports was a small sample size.15
Hibbs defined the use of plaster casts as a corrective method to get preliminary stabilization prior to operative intervention and attain added correction, hold it till the bones fuse after surgery.16 Joseph Risser discovered frame that is named after him to treat deformity with plaster cast. His method utilizes principle of elongation-derotation to correct spine deformity.17 This was modified by Cotrel Morel et al., in 1960s by adding flexion as the third corrective force. They applied the corrective force through straps instead of a localizer and specifically called it de-rotation. Lateral flexion was achieved by straps pulling laterally as well by adjusting the pelvic and halter traction in a differential manner. This became popular as EDF (Elongation- Derotation- Flexion) plaster-cast method which is the most commonly used maneuver now.18 In the early 1950s Scott and Morgan reported that some EOS curves resolve with time, however they were not able to identify absolute criteria for distinguishing between resolving and progressive curves.19 Later in 1960s, Conner showed that syndromic EOS have higher chances of progression.20 However, no studies could define criteria to differentiate these two curve types.21,22 Mehta, in 1970s demonstrated children treated with plaster-cast before completing 20 months of age for curve around 300 had no progression. They showed 100 decrease in deformity on completing skeletal development. Mehta also described the Rib vertebral angle distance (RVAD) and demonstrated value of more than 20 is associated with curve progression. She further described four phenotypes, “sturdy idiopathic with well-developed muscular tone, slim idiopathic with delicate features and ligamentous laxity, and progressive curves in those with known syndromes and those with unknown syndromes”.23 Later, 136 children with Idiopathic Scoliosis were reviewed by Mehta and despite the same treatment to all children, the results were different. In the 94 children who completed treatment within 2-yrs deformity was completely corrected. However, in 42 children treated after 30 months of age, curve was partially corrected. Surgical intervention was carried out in 35.7% of this sub-group. She concluded early management was a critical factor impacting the prognosis.8 These findings were further confirmed by many studies.
Ceballos et al. corroborated Mehta’s prognostic criteria in a study of 113 patients with infantile scoliosis. The authors showed relation between position of rib head and adjoining vertebra at apex of deformity was best indicator of prognosis. In Sanders et al.’s review of 55 children with progressive infantile scoliosis managed with EDF plaster-cast, 83% didn’t need operative intervention. They also confirmed that plaster-cast correction at young age, smaller magnitude of deformity and idiopathic curves have improved prognosis24 Waldron et al., assessed 20 children with EOS managed using Risser cast with GA and showed 65% of children didn’t need operative intervention. The authors concluded EDF plaster-cast is an efficacious strategy to delay surgery even in severe deformity.25 Fletcher et al., assessed a cohort of 29 children having IS treated using EDF plaster-cast. In their study, 21 children did not require surgery. Baulch et al. showed sequential plaster-cast application secures lengthening of dorsal spine in patients with EOS, and maintains normal lung function. They evaluated 36 children with EOS. In their series, surgery was delayed by 25-months in 69% cases26
Morin and Kulkarni, studied sequential plaster-cast in non-Syndromic infantile scoliosis. They concluded it is effective for managing Mehta’s benign-type deformity. It obviates need for fusion in 66% patients.27 Johnston et al., in their study of 27 patients, showed sequential EDF casting is a useful tool to buy time in patients of EOS. They found the average Cobb angle didn’t alter after 28 months of management.28 Smith et al., assessed 31 children with infantile scoliosis. Of thirty one, seventeen children were managed by sequential EDF cast. Out of them, five children needed surgical intervention. They concluded, serial cast application has better results in children with small flexible deformities.29
3. Epidemiology
Present estimates reveal approximately 1 out of 1000 people are affected by congenital scoliosis. Most of these deformities are small in magnitude and pass off undetected. The familial incidence of congenital scoliosis (CS) is 1–5%, thus majority of the cases are sporadic. The male to female ratio is 1: 1.4. Infantile scoliosis (IS) and juvenile scoliosis (JS) are less common as compared to AIS. Typically, in infantile idiopathic scoliosis (IS) these are left-side thoracic curves, often occurring among boys. In contrast, juvenile cases are typically diagnosed at age 5-yrs in males and 7-yrs in females, accounting for about 10%–20% of idiopathic scoliosis cases. Juvenile cases occur predominantly in girls. The deformity pattern is similar to AIS. AIS is the most prevalent type. Among adolescents, the prevalence of 100curves is <3%, with 5% of curves showing a progression > 300. The prevalence of curves greater than 10° is higher among girls, with a 4: 1 ratio of males to females, increasing to 9:1 when curves reach an operative range of about 40–50°.
4. Impact of scoliosis during the growing phase of spine
Initial decade is a critical time for development of chest wall and spinal column. It is associated with many intricate events occurring within a small time-frame. These phenomena when properly matched, result in balanced appendicular and axial skeleton.30,31 Changes in this phase of growth in spine lead to malformations. This results in disproportionate growth of axial and appendicular skeleton including chest wall hampering development of alveoli. Extreme deformities result in abnormal epiphyseal plate configuration and deformed vertebrae. This eventually leads to abnormal chest wall configuration with reduced mobility and impaired pulmonary development as a “cascading effect”.32 This can result in pulmonary hypertension, Thoracic insufficiency syndrome (TIS) and, may even lead to death.3,30 Literature shows reduction of lung function in children with unaddressed EOS is 15% higher in comparison with adolescent idiopathic scoliosis (AIS).33 Growth of the thoracic cavity is directly related to thoracic growth. Restrictive pulmonary dysfunction may not correct even after surgical intervention.34 Early intervention to optimize lung growth is very important in first 3 years of life when lung and thoracic growth are most rapid. If not addressed at early stage it leads to TIS. As chest wall stiffens from ankylosis of chest wall joints from chronic restriction of range of motion, inspiration becomes increasingly dependent on the diaphragm. The diaphragm and abdominal musculature have an abnormal configuration in EOS, which compromises their ability to generate force. This results in reduction of Maximum inspiration and expiration pressures and Peak Cough Flow.35 In a study by Pehrsson et al., the cause of mortality in 115 unaddressed deformity patients was evaluated. They found that fatality was substantially more in EOS but not adolescent idiopathic scoliosis according to Sweden database.36 Also, Karol et al. found that dorsal spine length of minimum 180–220 mm is essential to decrease chance of TIS.3 These reports highlight the importance of reducing the magnitude of deformity in this crucial growth period to ensure normal pulmonary function.
5. Serial casting in congenital scoliosis: does it have any role?
Congenital scoliosis warrants operative intervention to halt progression as brace/plaster-cast doesn’t prevent progression in majority patients.38 However, early spine fusion is associated with plethora of complications. Hence growth-friendly treatments are being preferred in most types of syndromic EOS. However, as these techniques are associated with multiple complications, it is prudent in delaying the index surgery in children. Traditionally, plaster-casts have been considered ineffective in congenital deformity. They are sometimes applied to manage the secondary curve. However, there is scarce literature related to the role of plaster-cast application in CS.39,40
Demirkiran et al. demonstrated 22% correction in eleven children with congenital scoliosis. They showed a curve correction of >5° in ten patients.38 Cao et al., reported that the mean correction was 20.5% for correction rate. In their series, plaster cast delayed index operative intervention by mean period 1.25 years in 2 children.41
The main aim of non-operative management in CS is to buy time thereby reducing need for sequential operations. Progressive deformities can be managed by EDF plaster cast allowing spine to achieve optimum growth.
6. Technique of EDF (Elongation- Derotation- Flexion) casting
EDF is a maneuver for deformity correction using Cotrel frame. Longitudinal tractional force, postero-lateral moulding with rotational forces are applied. Plaster-cast encompassing dorsal-lumbar-sacral spine is applied followed by lateral manual compression. In the Cotrel/Mehta frame, traction is applied at 2 points; chin/occiput and iliac crest to provide axial correction force. Harness-strap assembly controls magnitude of traction.23 (Fig. 3, Fig. 4).
Fig. 3.
Positioning in the casting frame with the extremities supported but the body left free for cast application. Traction applied through the head halter and through the pelvis.
Fig. 4.
Scale connected to head harness utilized to measure the amount of traction applied.
EDF plaster-cast may be applied effectively with the patient awake or under GA. Canavese et al. assessed 44 children with JS. They showed plaster-casting under GA with addition of muscle relaxant controls deformity progression better when compared to awake casting or under only GA without addition of a muscle relaxant. Addition of a muscle relaxant may help treating doctor better derotate vertebrae while applying axial traction. Casting under GA enables easier monitoring and regulating ventilatory functions in an intubated patient.37 In another study, they found the parameters of curve magnitude, RVAD and apical vertebral degree showed significant improvement when casting was performed under GA.16
In our institute, patient is administered GA and intubated while supine on a stretcher. Tubular stockinet is fastened above the level of the iliac crests. Using the Risser technique child is laid on 2 horizontal metallic rods. They take the weight of shoulders and pelvis. A strap is applied on the convexity of the scoliotic curve. It is tensioned to reduce the deformity by simultaneously applying postern-lateral reduction force. In children with “S” shaped curvature, 2nd strap is applied on contra-lateral convexity. Supporting rods are now taken off to apply POP bandages over straps with moulding over bony prominences with special attention to the iliac crests. Fluoroscopy guidance is taken to assess the hand position before performing the de-rotation manoeuvre. While POP bandages are malleable, lateral pressure is applied by one hand at convexity along with two handed counter-pressure application at concavity of the curve near end vertebrae. This is maintained till POP becomes hard. A mirror at the base of the table is utilized to evaluate and adjust the hand position during derotation. This is reinforced using synthetic fiberglass. A thoraco-abdominal window is cut allowing space for chest wall and abdomen to expand. A posterolateral window made at site of the concavity near apical rib to promote de-rotational forces to be applied. Trimming and padding of plaster cast edges for soft tissue protection is done (Fig. 5). Plaster-cast is changed every 2–3 months as per age of patient and magnitude of deformity.16
Fig. 5.
Post-cast application windowing. Large windows created in areas not necessary for deformity correction.
Initially, the cast may appear to be restricting chest wall mobility. However, well-moulded casts do not apply pressure on chest wall and have adequate space for respiratory functions. A study on 37 patients with infantile showed that casting caused temporary pulmonary restriction i.e. increase in maximal inspiratory pressures by 106% at the time of procedure. It came back to baseline value after cutting straps and releasing harnesses.16
7. Complications of sequential plaster-casting
The commonest complaints of serial plaster-cast application are inconsequential dermatological complications like dry skin, rashes or blister formation which usually do not require any formal treatment. Other potential side-effects include muscle weakness, stiff joints after plaster-cast removal and sleeping disorders (plaster-cast intolerance).42
As reported by Sanders et al., most morbid complication is temporary chest pressure applied during procedure which makes ventilation problematic while plaster-cast hardens. However, once anterolateral window is opened, inspiratory air-flow normalizes. Sanders et al. suggested intubating children who undergo plaster-casting under GA for solving this issue43 Dhawale et al. showed similar results and recommended intubation for better airway management44 They recommended adequate post-procedure supervision especially in children with pre-existing pulmonary dysfunction.
Badlani et al. reported a rare case of subclavian vein thrombosis post plaster-cast application. They stressed that one must be alert about diagnosing and treating this potentially serious complication. Judicious trimming of plaster cast in groin and axilla can prevent this.45
Early onset scoliosis questionnaire (EOSQ-24) showed reduced Quality of life (QOL) in patients treated by sequential casting. Repeated anesthesia and radiation exposure are also matter of concern. Multiple exposure to fluoroscopy increases chance of radiation-induced malignancies. The necessity of general anesthesia is under ongoing discussion. Repeated GA at a young age may affect neurocognitive development as per previously published animal experiments.46, 47, 48 The risk benefit ratio must be addressed as these deformities if untreated may prove fatal.49 Effect of GA on neuronal development needs more research. To put children at ease while applying plaster-cast, newer developments like casting while children are distracted with their iPad or their parent’s cell phone, mother’s presence precluding GA are being explored.50
8. Does bracing play any role during cast treatment?
Compliance with Braces is a major problem. No reports are addressing this in EOS patients. Presently brace is applied after adequate correction is attained with plaster-cast, when on ‘cast holidays’ especially in summers as a supplement. We lack adequate literature to encourage use of brace as a solo treatment modality. Usually it is utilized as supplement to plaster-cast. Various authors reported 27–40% maximum up to 100% rate of operative intervention.51, 52, 53, 54, 55 and concluded that brace wear didn’t stop curve progression. Literature shows that children with lesser RVAD and Cobb angle showed effective control of curve progression with bracing due to the benign nature of the curve.36
9. When to convert from casting to growth friendly procedure?
Indications for casting include progressive EOS>30°, Idiopathic: RVAD>20°, Pedicles in phase 2, Idiopathic scoliosis, Congenital scoliosis (CS), syndromic or neuromuscular (NMS). Surgery is advised when the Cobbs angle >50°, when non-surgical treatment fails and curve progresses. EOS with lesser magnitude may be fully corrected with plaster-cast. Patients who achieve satisfactory correction with serial cast treatment, (Cobb < 100) may be converted to bracing. Rapidly progressive deformities in patient’s > 5-yrs usually require surgery depending on the severity. The goal of casting would be to delay the surgical procedure for as long as possible, taking into consideration, in addition to age and progression, pulmonary physiology, nutrition, patient compliance and access to care, as well as cost.
10. Future directions
Efforts are required to propagate use of casting as a method of deformity correction and technique to delay surgical intervention in EOS at earliest possible age. Reducing GA for multiple plaster cast application is to be encouraged to avoid complications in pediatric population and also its effect on brain development and techniques utilizing iPad/mobile and mothers presence to obviate the need for GA.
11. Conclusion
Sequential plaster-cast is proven as an efficient management strategy in EOS. For most idiopathic curves, it may be definitive and in other cases, it is meant to buy time in severe Syndromic/non-syndromic deformities. Doctors treating patients with EOS must be acquainted with sequential casting techniques to tackle small yet significant problem of EOS. A large multi-centric prospective study is the need of the hour to lay down guidelines and protocols for cast application.
Declaration of competing interest
The authors disclose NO conflict of interest.
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