Tag: early onset scoliosis

Scoliosis awareness month – Early-onset Scoliosis

Posted on June 7, 2021 by - Blog

Early-onset Scoliosis is an umbrella term used by many organisations (including the scoliosis research society) to include scoliosis cases that present under the age of 10. Within this bracket, there are really two further categories of scoliosis we need to understand.

The first is Infantile scoliosis – which is the name given to scoliosis cases that are diagnosed in children between the ages of 0 to 3 years. Infantile Scoliosis is at least as common in boys as girls, which is worth bearing in mind since adolescent cases (which comprise the majority of overall cases) are predominantly female cases[1].

Juvenile scoliosis is therefore diagnosed when scoliosis of the spine is apparent between the ages of 4 and 10. It is less common than adolescent scoliosis and comprises about 10-15% of total idiopathic scoliosis cases.  It is found more often in boys between the ages of 4-6 and curves tend to be left-sided, while in older children it is more common in girls and curves are right-sided and similar to adolescent scoliosis.[2]

 

What causes early-onset Scoliosis?

There are several main categories that comprise early-onset scoliosis cases – these are:

  • Idiopathic – Curves for which there is no apparent cause – this is probably the kind of scoliosis you are most familiar with, as it forms the bulk of scoliosis cases, especially in teens.
  • Congenital – Here the cause is incorrect development of the Vertebrae in-utero. It is sometimes associated with cardiac and renal abnormalities.
  • Neuromuscular – In children with neuromuscular disorders including spinal muscular atrophy, cerebral palsy, spina bifida and brain or spinal cord injury.
  • Syndromic – Certain syndromes, such as Marfan’s, Ehlers-Danlos and other connective tissue disorders, as well as neurofibromatosis, Prader-Willi, and many bone dysplasias may be associated with EOS.

At the UK Scoliosis clinic, we mainly focus on the treatment of the idiopathic variety – which, as the name implies, is currently without defined cause. There are two main theories that explain the development of idiopathic infantile scoliosis – the first postulates that some children are simply born with a spine that is already curved, while the second suggests that the curvature occurs after birth and may be linked to the way a baby is handled. Much more research is required to clarify this, however.

 

What is the prognosis for early-onset Scoliosis?

The Scoliosis research society notes especially for early-onset cases, that early Scoliosis carries a risk of heart and lung problems in childhood which may become increasingly problematic in adult years[3] – but it’s worth noting that other research has shown that scoliosis can negatively impact the heart and lungs as the deformity increases in other age categories[4]. When untreated, severe EOS may be associated with an increased risk of early death due to heart and lung disease – the term Thoracic Insufficiency Syndrome (TIS) is commonly used to describe the potential combined spine and lung problems in EOS.

Idiopathic scoliosis has a number of possible treatment pathways, both non-surgical and surgical, whereas congenital and syndromic cases are more complex, and require in-depth evaluation to determine the best pathway. In all instances, it is important that suspected cases in infants should be investigated with a complete neurological examination and MRI or CT scan. This will serve to rule out any underlying neurological condition or disease process and allow the best treatment to be given as soon as possible.

 

How can we treat early-onset scoliosis?

Bracing may be an effective approach in idiopathic cases with good flexibility in the curve – however, rigid curves are less likely to benefit from this approach. Casting (which is a similar approach, using a plaster cast rather than a brace) is also a possible approach here.

Early-onset scoliosis is, however, the only broad category of scoliosis where the “wait and see” approach may have some value. The Scoliosis research society guidelines suggest that Idiopathic early onset scoliosis with curves greater than 30-35 degrees are most likely to progress and some studies have suggested the progression to surgical threshold for this group may be as high as 100%[5] – however, children younger than age 2 with infantile idiopathic curves less than 35 degrees stand a chance of the condition resolving without further treatment.

 

What does early-onset Scoliosis look like?

The below X-ray shows an example early onset Scoliosis case. It’s usually not possible to tell how severe scoliosis is without taking an X-ray, although external signs can suggest that the condition may be present. This is why regular screening is so important!

 

 

 

[1] https://www.srs.org/patients-and-families/conditions-and-treatments/parents/scoliosis/early-onset-scoliosis/infantile-idiopathic-scoliosis

[2] https://www.srs.org/patients-and-families/conditions-and-treatments/parents/scoliosis/early-onset-scoliosis/juvenile-idiopathic-scoliosis

[3] https://www.srs.org/patients-and-families/conditions-and-treatments/parents/scoliosis/early-onset-scoliosis

[4] Sperandio EF, Alexandre AS, Yi LC, et al. Functional aerobic exercise capacity limitation in adolescent idio- pathic scoliosis. Spine J. 2014;14(10):2366–72. PubMed doi:10.1016/j.spinee.2014.01.041

Sperandio EF, Vidotto MC, Alexandre AS, Yi LC, Gotfryd AO, Dourado VZ. Exercise capacity, lung function and chest wall shape in patients with adolescent idiopathic scoliosis. Fisioter Mov. 2015;28(3):563–72. doi:10.1590/0103-5150.028.003.AO15

Barrios C, Pérez-Encinas C, Maruenda JI, Laguía M. Significant ventilatory functional restriction in adoles- cents with mild or moderate scoliosis during maximal exercise tolerance test. Spine. 2005;30(14):1610–5. doi:10.1097/01.brs.0000169447.55556.01

 

[5] Progression risk of idiopathic juvenile scoliosis during pubertal growth, Charles YP, Daures JP, de Rosa V, Diméglio A. Spine 2006 Aug 1;31(17):1933-42.

Study suggests bracing is also effective in early-onset scoliosis patients

Posted on October 24, 2019 by - Blog

While the majority of studies on scoliosis bracing focus primarily on adolescent scoliosis sufferers, there are many other groups who do suffer from scoliosis in significant numbers. Over the last few weeks, we have looked at scoliosis treatment in older individuals -this week we’re examining the best options for very young (infantile or juvenile) patients.

Today, scoliosis in infants and juveniles is treated either with serial casting or with a bracing approach (bracing usually in children at the older end of the age range.)  Serial casting – where a child is placed in a series of casts, with the goal of correcting scoliosis has often been the preferred approach, since early-onset of scoliosis (EOS) patients are skeletally immature and have the largest potential for fast recovery through non-operative treatments[1]. As bracing technology has improved however, it has also become common practice for bracing to be prescribed after casting to maintain the initial correction. Bracing is now also prescribed to patients who are not able to tolerate casting[2] – but new research is now beginning to explore bracing as a “first choice” option for younger patients.

Such studies are welcome since overall, bracing studies are usually done on AIS patients, which means that while there is a strong case to be made for bracing in other groups, it has been slow to assemble the scientific proof of concept. A recent study from 2019 has now added significantly to our understanding of bracing in younger patients and is (so far as we are aware) the first study to explore the effectiveness of CAD/CAM bracing approaches in very young patients.

 

Bracing in young children – new research

The study[3], conducted at Children’s Hospital of Wisconsin sought to understand how effective a customised over-corrective brace (like ScoliBrace) was in treating scoliosis in young patients with Infantile scoliosis (IS) and Juvenile scoliosis (JS).

Thirty-eight patients (22 males, 16 females; 17 IS, 21 JS) were recruited for this study. 9 children were diagnosed with neuromuscular scoliosis, 1 congenital scoliosis, and 28 with IS or JS. The average age was 6.2 years old (ranging from 4 months to 10-years-old). Criteria for inclusion included:
1) All subjects are diagnosed with IS or JS (idiopathic, neuromuscular, or congenital);
2) Subjects must have not had any type of spinal surgery prior to bracing treatment;
3) Must be under 10 years old during the time of their first scan;
4) Must have had at least one follow up visit after their baseline scan before the 12-month mark.

During the trial, investigators utilised 3D scanning technology (similar to BraceScan) to map the exact requirements for the scoliosis brace for each patient – this was then manufactured using a CAD/CAM approach, facilitating a very high degree of accuracy. At an initial fitting, a scoliosis specialist checked that the brace was functioning as required and made any small adjustments necessary.

Overall, amongst the patients as a group the starting Cobb angle was 38 ± 14° in the thoracic curve (ranging from 19° to 68°), 30 ± 9.6° in the thoracolumbar (ranging from 19° to 42°), and 36 ± 10.3° in the lumbar sections (ranging from 22° to 53°).

 

Results in younger patients

After brace fitting, the investigators followed the patients for 12 months, with a view to assessing change in Cobb angle.  Firstly, no patients required surgery within the 12-month span, whereas without bracing surgery may have been necessary at least in a few cases.

When compared to the baseline measurements, the in-brace correction reduced the Cobb angle in the patients from 38° to 24.2° in the thoracic region (a 36.3% reduction), 30° to 10.3° in the thoracolumbar region (a 65.7% reduction), and from 36° to 18.5° in the lumbar (a 48.3% reduction). The juvenile group had 23% correction, 47% stabilization, and 30% progression of curves. The infantile group had 50% correction, 32% stabilization, and 18% progression of curves. The following table shows the progress over a series of three-month evaluations.

 

Levels of Curve Month Cobb Angle (°) Curve change (°) % Change
Thoracic 0 38.0 ± 14.0 NA NA
3 30.1 ± 19.7 −5.6 −15.6%
6 30.2 ± 21.5 −5.5 − 15.5%
9 31.5 ± 24.2 −4.2 −11.6%
12 29.4 ± 24.3 −6.2 −17.5%
Thoracolumbar 0 30.0 ± 9.6 NA NA
3 25.2 ± 11.2 0.2 0.6%
6 24.8 ± 11.6 −0.2 −0.9%
9 24.3 ± 10.3 −0.7 −2.7%
12 23.9 ± 10.0 −1.1 −4.5%
Lumbar 0 36.0 ± 10.3 NA NA
3 25.4 ± 14.3 −3.5 −12.2%
6 27.9 ± 14.5 −1 −3.5%
9 30.2 ± 14.2 1.3 4.5%
12 29.9 ± 14.2 1 3.6%

 

 

Is Bracing effective in young patients?

While (as we mentioned at the outset) there have been few in-depth studies considering the effectiveness of bracing in younger patients, the research presented here certainly suggests that the positive results which are typically seen in adolescents can be replicated in younger children.

Overall, the bracing approach used was shown to be effective in correcting nearly half of the thoracic curves and one-third of the other curves, over a period of 12 months. When combining all data, 75% of curves were corrected or stabilized.

As well as being effective, a bracing approach also has significant benefits in terms of quality of life, and cost-effectiveness. Since younger children with scoliosis experience such rapid spinal growth and development, traditional casting needs to be repeated every couple of months – This may be less cost-effective and less patient-friendly because visits are more frequent and may require plaster casting to be done with the patient under general anaesthesia. Bracing, by contrast, requires only a single fitting & fewer follow up visits The brace can also be removed for daily washing which is better for the infants skin and hygiene. As the child grows and changes shape, further braces may be required to treat the scoliosis effectively.

If you would like to know more about bracing in younger children, please contact us.

 

 

[1] Mehta MH. Growth as a corrective force in the early treatment of progressive infantile scoliosis. J Bone Joint Surg Br. 2005;87:1237–47.

[2] Weinstein SL, et al. Effects of bracing in adolescents with idiopathic scoliosis. N Engl J Med. 2013;369:1512–21.

[3] John Thometz, XueCheng Liu, Robert Rizza, Ian English and Sergery Tarima, Effect of an elongation bending derotation brace on the infantile or juvenile scoliosis, Scoliosis and Spinal Disorders 2018 13:13