Neuromuscular Scoliosis: Difference between revisions
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== Etiology == | == Etiology == | ||
According to the Scoliosis Research Society spinal deviation can be: | According to the Scoliosis Research Society spinal deviation can be:<ref name=":3">Vialle R, Thévenin-Lemoine C, Mary P. Neuromuscular scoliosis. Orthopaedics & Traumatology: Surgery & Research. 2013 Feb 1;99(1):S124-39.[https://www.sciencedirect.com/science/article/pii/S1877056812002745]</ref> | ||
# Neuropathic with central or peripheral motor neuron involvement (or both) | # Neuropathic with central or peripheral motor neuron involvement (or both) | ||
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* Muscle fibre disorders, e.g. Duchenne muscular atrophy | * Muscle fibre disorders, e.g. Duchenne muscular atrophy | ||
* Multifocal disorders, e.g. Spina Bifida | * Multifocal disorders, e.g. Spina Bifida | ||
Orthopaedic manifestaions can be similar amongst conditions, but differ between underlying diagnoses.<ref name=":2" /> | |||
In diseases of motor neuron and muscle fibre disease, onset of scoliosis usually occurs once the individual loses ambulation. Age and onset have not been found to be a predictor of progression and severity of the deformity.<ref>Mullender MG, Blom NA, De Kleuver M, Fock JM, Hitters WM, Horemans AM, Kalkman CJ, Pruijs JE, Timmer RR, Titarsolej PJ, Van Haasteren NC. A Dutch guideline for the treatment of scoliosis in neuromuscular disorders. Scoliosis. 2008 Dec;3(1):1-4.[https://scoliosisjournal.biomedcentral.com/articles/10.1186/1748-7161-3-14#citeas]</ref>NMS itself presents at any age. | |||
In diseases of motor neuron and muscle fibre disease, onset of scoliosis usually occurs once the individual loses ambulation. Age and onset have not been found to be a predictor of progression and severity of the deformity.<ref>Mullender MG, Blom NA, De Kleuver M, Fock JM, Hitters WM, Horemans AM, Kalkman CJ, Pruijs JE, Timmer RR, Titarsolej PJ, Van Haasteren NC. A Dutch guideline for the treatment of scoliosis in neuromuscular disorders. Scoliosis. 2008 Dec;3(1):1-4.[https://scoliosisjournal.biomedcentral.com/articles/10.1186/1748-7161-3-14#citeas]</ref> | |||
== Pathogenesis == | == Pathogenesis == | ||
Orthopaedic manifestations are often similar amongst different conditions but differ in their underlying conditions<ref name=":2" /> | |||
Conditions and their own etiology vary widely, however, there are often common factors that contribute to spinal deformity. These include<ref name=":0" />: | |||
* Asymmetrical paraplegia | * Asymmetrical paraplegia | ||
| Line 37: | Line 37: | ||
* Abnormal posture via central pathways | * Abnormal posture via central pathways | ||
NMS is not always simply a result of trunk muscle weakness. Instead, hypertonia is more often found to be the result of hypertonia in conditions of central neurologic conditions. Muscle imbalances around the spinal axis produce a gradually worsening deformity because of a lack of effective muscle compensation. In this way the deformity often resembles idiopathic scoliosis.<ref name=":3" /> | |||
== Impairments == | == Impairments == | ||
| Line 53: | Line 54: | ||
** Sitting | ** Sitting | ||
** General balance capacity | ** General balance capacity | ||
** Trunk | ** Trunk stability | ||
** Bimanual activities | ** Bimanual activities | ||
** Functioning in activities of Daily Living | ** Functioning in activities of Daily Living | ||
| Line 60: | Line 61: | ||
== General management == | == General management == | ||
=== Components of management === | |||
Addressing scoliosis stemming from a neuromuscular disease or disorder requires various levels of intervention these include:<ref name=":2" /> | |||
* '''Observation''' | * '''Observation''' | ||
* '''Conservative treatment''' | * '''Conservative treatment''' | ||
| Line 69: | Line 71: | ||
In a 2019 study by Murphy and Mooney, it was highlighted that there remains a lack of high level evidence that supports the positive effect of conservative treatment of NMS. The evidence is most supportive of improved sitting balance in patients with flexible curves. In general, as deformity progresses, the effectiveness of bracing decreases. The study noted some improvements in Cobb angles and sitting stability with a 3-point molded brace. | In a 2019 study by Murphy and Mooney, it was highlighted that there remains a lack of high level evidence that supports the positive effect of conservative treatment of NMS. The evidence is most supportive of improved sitting balance in patients with flexible curves. In general, as deformity progresses, the effectiveness of bracing decreases. The study noted some improvements in Cobb angles and sitting stability with a 3-point molded brace. | ||
* Seating systems | * '''Seating systems''' | ||
* '''Surgery''' | * '''Surgery''' | ||
| Line 77: | Line 79: | ||
For example steroid administration to those diagnosed with DMD. | For example steroid administration to those diagnosed with DMD. | ||
== Assessment == | |||
Assessment of the individual includes both the clinical and radiological assessment. | |||
Clinical assessment | |||
The clinical assessment incorporates the following:<ref name=":3" /> | |||
* Tone | |||
* Joint stiffness | |||
* Hip range of motion | |||
* Skin integrity | |||
* Walking capacity | |||
* Static examination of the deformity | |||
* Dynamic examination of the deformity | |||
* Respiratory assessment | |||
* Cardiac assessment | |||
Radiological assessment | |||
When imaging is performed with individuals with NMS it is important to consider the following<ref name=":3" />: | |||
* With non walking patients, images are done in a seated posture, but supine is preferable. | |||
* Specific bending views can be done to assess the stiffening of different spinal levels. | |||
* An AP view is done under asymmetric traction (manual pulling down on one side through the leg) to asses frontal reducibility of pelvic obliquity. Alignment, and therefore no pelvic obliquity is bserved when the line passing through the base of the two sacroiliac interlines are parallel to the shoulder line. | |||
* In terms of pre-operation management, an MRI is important in suspected medullary pathology. | |||
* Thoracic CT scans are performed in cases of thoracic hyperkyphosis or lordosis. This is important to asses how the lungs are affected in regards to the deformity. If in the presence of severe deformity, bronchial stretching following scoliosis correction may induce [[atelectasis]] and decrease lung volume. | |||
Goals of management | |||
In dealing with NMS the goals of management include<ref name=":1">Roberts SB, Tsirikos AI. Factors influencing the evaluation and management of neuromuscular scoliosis: a review of the literature. Journal of back and musculoskeletal rehabilitation. 2016 Jan 1;29(4):613-23.[https://content.iospress.com/articles/journal-of-back-and-musculoskeletal-rehabilitation/bmr675]</ref>: | In dealing with NMS the goals of management include<ref name=":1">Roberts SB, Tsirikos AI. Factors influencing the evaluation and management of neuromuscular scoliosis: a review of the literature. Journal of back and musculoskeletal rehabilitation. 2016 Jan 1;29(4):613-23.[https://content.iospress.com/articles/journal-of-back-and-musculoskeletal-rehabilitation/bmr675]</ref>: | ||
| Line 84: | Line 116: | ||
* Alleviation of pain | * Alleviation of pain | ||
Management is best performed within a multidisciplinary team. While conservative management comprises of physiotherapy and | Management is best performed within a multidisciplinary team. While conservative management comprises of physiotherapy and braciAssessmentry often plays an essential role in the rehabilitative process.<ref>Kotwicki T, Jozwiak M. Conservative management of neuromuscular scoliosis: personal experience and review of literature. Disability and rehabilitation. 2008 Jan 1;30(10):792-8.[https://www.tandfonline.com/doi/abs/10.1080/09638280801889584]</ref> | ||
== Surgical management == | == Surgical management == | ||
Surgical intervention is usually prescribed when the measured Cobb's angle exceeds 40° (usually lying between 40-50°) but may be performed earlier in those diagnosed with conditions such as DMD.<ref name=":1" /> | Surgical intervention is usually prescribed when the measured Cobb's angle exceeds 40° (usually lying between 40-50°) but may be performed earlier in those diagnosed with conditions such as DMD.<ref name=":1" /> | ||
| Line 96: | Line 126: | ||
Intraoperative and postoperative complications tend to be quite high. In a study by Weissmann et al (2021) complication rates were between 33.1% and 40.1% with similar percentages being reported in other reviews. Despite this, the same study found a significant decrease in urinary tract infections, lower respiratory tract infections and even epilepsy after surgery. | Intraoperative and postoperative complications tend to be quite high. In a study by Weissmann et al (2021) complication rates were between 33.1% and 40.1% with similar percentages being reported in other reviews. Despite this, the same study found a significant decrease in urinary tract infections, lower respiratory tract infections and even epilepsy after surgery. | ||
An important component to reducing these complications is to do a thorough patient history (including complications/ conditions present before birth). This would be done to pick up possible co-morbidities. | An important component to reducing these complications is to do a thorough patient history (including complications/ conditions present before birth). This would be done to pick up possible co-morbidities..<ref>Weissmann KA, Lafage V, Pitaque CB, Lafage R, Huaiquilaf CM, Ang B, Schulz RG. Neuromuscular Scoliosis: Comorbidities and Complications. Asian Spine Journal. 2021 Dec;15(6):778.[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8696062/]</ref> | ||
== Physiotherapy management == | == Physiotherapy management == | ||
| Line 102: | Line 132: | ||
* '''Respiratory function:''' In this aspect it is important to address the respiration | * '''Respiratory function:''' In this aspect it is important to address the respiration | ||
* Pain: A randomized control trial (RCT) published in 2017 showed that core | * '''Pain:''' A randomized control trial (RCT) published in 2017 showed that core stabilization exercises were effective in decreasing pain and also decreasing rotational deformity.<ref>Gür G, Ayhan C, Yakut Y. The effectiveness of core stabilization exercise in adolescent idiopathic scoliosis: A randomized controlled trial. Prosthetics and orthotics international. 2017 Jun;41(3):303-10.[https://journals.lww.com/poijournal/Abstract/2017/41030/The_effectiveness_of_core_stabilization_exercise.11.aspx]</ref> While the participants were diagnosed with idiopathic scoliosis, the principles and outcome should be considered. | ||
== Resources == | == Resources == | ||
*[https://scoliosisjournal.biomedcentral.com/track/pdf/10.1186/1748-7161-3-14.pdf A Dutch guideline for the treatment of scoliosis in neuromuscular disorders] | *[https://scoliosisjournal.biomedcentral.com/track/pdf/10.1186/1748-7161-3-14.pdf A Dutch guideline for the treatment of scoliosis in neuromuscular disorders] | ||
== References == | == References == | ||
<references /> | <references /> | ||
Revision as of 20:38, 19 May 2022
Original Editor - User Name
Top Contributors - Lauren Heydenrych, Wanda van Niekerk and Aminat Abolade
Introduction[edit | edit source]
Neuromuscular scoliosis (NMS) is defined as a non-congenital spinal deformity present in conjunction with any type of pre-existing neuromuscular diagnosis.[1]NMS is generally more severe than adolescent idiopathic scoliosis (AIS) and more progressive, typically continuing after the growth of the individual and spine has ceased.[2]Its progressive nature is more often seen in conditions of neurologic and systemic involvement[1].
Etiology[edit | edit source]
According to the Scoliosis Research Society spinal deviation can be:[3]
- Neuropathic with central or peripheral motor neuron involvement (or both)
- Myopathic
Neuromuscular scoliosis is a common consequence of neuromotor disease. Examples of these include[2]:
- Cerebral Palsy
- Spinal cord injury
- Motor neuron disease, e.g. Spinal muscular atrophy
- Muscle fibre disorders, e.g. Duchenne muscular atrophy
- Multifocal disorders, e.g. Spina Bifida
Orthopaedic manifestaions can be similar amongst conditions, but differ between underlying diagnoses.[1]
In diseases of motor neuron and muscle fibre disease, onset of scoliosis usually occurs once the individual loses ambulation. Age and onset have not been found to be a predictor of progression and severity of the deformity.[4]NMS itself presents at any age.
Pathogenesis[edit | edit source]
Orthopaedic manifestations are often similar amongst different conditions but differ in their underlying conditions[1]
Conditions and their own etiology vary widely, however, there are often common factors that contribute to spinal deformity. These include[2]:
- Asymmetrical paraplegia
- imbalance of mechanical forces
- Intraspinal and congenital abnormalities of the spine
- Altered sensory feedback
- Abnormal posture via central pathways
NMS is not always simply a result of trunk muscle weakness. Instead, hypertonia is more often found to be the result of hypertonia in conditions of central neurologic conditions. Muscle imbalances around the spinal axis produce a gradually worsening deformity because of a lack of effective muscle compensation. In this way the deformity often resembles idiopathic scoliosis.[3]
Impairments[edit | edit source]
The result of a NMS is often limitations or impairments to the individual involved.
These include[2]:
- Pain
- Compromised cardiopulmonary function (secondary to mechanical effects on thoracic volume and compliance.
- Alterations in skin integrity
- Limited or decreased....
- Limb movement
- Gait
- Standing
- Sitting
- General balance capacity
- Trunk stability
- Bimanual activities
- Functioning in activities of Daily Living
- Complications in self image and social interactions
General management[edit | edit source]
Components of management[edit | edit source]
Addressing scoliosis stemming from a neuromuscular disease or disorder requires various levels of intervention these include:[1]
- Observation
- Conservative treatment
- Physiotherapy
- Bracing
In a 2019 study by Murphy and Mooney, it was highlighted that there remains a lack of high level evidence that supports the positive effect of conservative treatment of NMS. The evidence is most supportive of improved sitting balance in patients with flexible curves. In general, as deformity progresses, the effectiveness of bracing decreases. The study noted some improvements in Cobb angles and sitting stability with a 3-point molded brace.
- Seating systems
- Surgery
- Treatment of the underlying pathology.
For example steroid administration to those diagnosed with DMD.
Assessment[edit | edit source]
Assessment of the individual includes both the clinical and radiological assessment.
Clinical assessment
The clinical assessment incorporates the following:[3]
- Tone
- Joint stiffness
- Hip range of motion
- Skin integrity
- Walking capacity
- Static examination of the deformity
- Dynamic examination of the deformity
- Respiratory assessment
- Cardiac assessment
Radiological assessment
When imaging is performed with individuals with NMS it is important to consider the following[3]:
- With non walking patients, images are done in a seated posture, but supine is preferable.
- Specific bending views can be done to assess the stiffening of different spinal levels.
- An AP view is done under asymmetric traction (manual pulling down on one side through the leg) to asses frontal reducibility of pelvic obliquity. Alignment, and therefore no pelvic obliquity is bserved when the line passing through the base of the two sacroiliac interlines are parallel to the shoulder line.
- In terms of pre-operation management, an MRI is important in suspected medullary pathology.
- Thoracic CT scans are performed in cases of thoracic hyperkyphosis or lordosis. This is important to asses how the lungs are affected in regards to the deformity. If in the presence of severe deformity, bronchial stretching following scoliosis correction may induce atelectasis and decrease lung volume.
Goals of management
In dealing with NMS the goals of management include[5]:
- Preservation of function
- Facilitation of daily care
- Alleviation of pain
Management is best performed within a multidisciplinary team. While conservative management comprises of physiotherapy and braciAssessmentry often plays an essential role in the rehabilitative process.[6]
Surgical management[edit | edit source]
Surgical intervention is usually prescribed when the measured Cobb's angle exceeds 40° (usually lying between 40-50°) but may be performed earlier in those diagnosed with conditions such as DMD.[5]
Indications for surgery are that of progressive deformity with an unacceptable truncal shift or pelvic obliquity that affects standing or sitting balance and/or positioning.[1]
The goal of surgical intervention is to obtain a solid bony arthrodesis of the spine and pelvis in order to restore seated or standing balance.[1]
Intraoperative and postoperative complications tend to be quite high. In a study by Weissmann et al (2021) complication rates were between 33.1% and 40.1% with similar percentages being reported in other reviews. Despite this, the same study found a significant decrease in urinary tract infections, lower respiratory tract infections and even epilepsy after surgery.
An important component to reducing these complications is to do a thorough patient history (including complications/ conditions present before birth). This would be done to pick up possible co-morbidities..[7]
Physiotherapy management[edit | edit source]
Components to address include
- Respiratory function: In this aspect it is important to address the respiration
- Pain: A randomized control trial (RCT) published in 2017 showed that core stabilization exercises were effective in decreasing pain and also decreasing rotational deformity.[8] While the participants were diagnosed with idiopathic scoliosis, the principles and outcome should be considered.
Resources[edit | edit source]
References[edit | edit source]
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Murphy RF, Mooney JF. Current concepts in neuromuscular scoliosis. Current Reviews in Musculoskeletal Medicine. 2019 Jun;12(2):220-7.[1]
- ↑ 2.0 2.1 2.2 2.3 Allam AM, Schwabe AL. Neuromuscular scoliosis. PM&R. 2013 Nov 1;5(11):957-63.
- ↑ 3.0 3.1 3.2 3.3 Vialle R, Thévenin-Lemoine C, Mary P. Neuromuscular scoliosis. Orthopaedics & Traumatology: Surgery & Research. 2013 Feb 1;99(1):S124-39.[2]
- ↑ Mullender MG, Blom NA, De Kleuver M, Fock JM, Hitters WM, Horemans AM, Kalkman CJ, Pruijs JE, Timmer RR, Titarsolej PJ, Van Haasteren NC. A Dutch guideline for the treatment of scoliosis in neuromuscular disorders. Scoliosis. 2008 Dec;3(1):1-4.[3]
- ↑ 5.0 5.1 Roberts SB, Tsirikos AI. Factors influencing the evaluation and management of neuromuscular scoliosis: a review of the literature. Journal of back and musculoskeletal rehabilitation. 2016 Jan 1;29(4):613-23.[4]
- ↑ Kotwicki T, Jozwiak M. Conservative management of neuromuscular scoliosis: personal experience and review of literature. Disability and rehabilitation. 2008 Jan 1;30(10):792-8.[5]
- ↑ Weissmann KA, Lafage V, Pitaque CB, Lafage R, Huaiquilaf CM, Ang B, Schulz RG. Neuromuscular Scoliosis: Comorbidities and Complications. Asian Spine Journal. 2021 Dec;15(6):778.[6]
- ↑ Gür G, Ayhan C, Yakut Y. The effectiveness of core stabilization exercise in adolescent idiopathic scoliosis: A randomized controlled trial. Prosthetics and orthotics international. 2017 Jun;41(3):303-10.[7]
