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| <div class="noeditbox">'''Original Editors ''' - [[User:Gertjan Peeters|Gertjan Peeters]] | | <div class="editorbox">'''Original Editors ''' - [[User:Gertjan Peeters|Gertjan Peeters]] |
| '''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}} | | '''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}} |
| </div> | | </div> |
| == Definition/Description == | | == Introduction == |
| | [[File:Adolescent idiopathic scoliosis (Paria et al., 2015).jpg|right|frameless]] |
| | [[Congenital and Acquired Neuromuscular and Genetic Disorders|Congenital]] spine deformities are disorders of the spine that develop in an individual prior to birth. The vertebrae do not form correctly in early fetal development and in turn cause structural problems within the spine and spinal cord. These deformities can range from mild to severe and may cause other problems if left untreated, such as developmental problems with the [[Anatomy of the Human Heart|heart]], [[Kidney|kidneys]] and urinary tract, problems with breathing or walking, and [[paraplegia]] (paralysis of the lower body and legs). |
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| Congenital deformities of the spine are deformities identified at birth that are the result of anomalous vertebral development in the embryo. Minor bony malformations of all types occur in up to 12% of the general population and are usually not apparent. <ref name=":13">Alexander PG. and Tuan RS. (2010). Role of environmental factors in axial skeletal dysmorphogenesis. Birth Defects Res C Embryo Today, 90 (2), pp 118-132</ref> The spine is a complex and vital structure. Anatomically, a variety of tissue types are represented in the spine, including musculoskeletal and neural elements. Several embryologic steps must occur to result in the proper formation of both the musculoskeletal and neural elements of the spine. Alterations in these embryologic steps can result in congenital abnormalities of the spine. <ref name=":0">AKBARNIZ B. A. et al. (2011). The Growing Spine, Management of Spinal Disorders in Young Children. Springer, p. 247, 263, 318.</ref> Based on the type of malformation, the resulting deformity and the specific region of the spine where the malformation occurs, these congenital malformations of the spine can be classified into three main groups:<ref name=":0" />
| | Medical researchers are still unsure of what actually causes the defects responsible for congenital spine deformities. In these disorders, the vertebrae are often missing, fused together and/or misshapen or partially formed.<ref name=":0">Khavkinckinic Congenital spinal deformities Available:https://khavkinclinic.com/congential-spine-deformities/ (accessed 10.10.2021)</ref> |
| # When the neural tube fails to completely close during the embryonic development, this is a [[neural tube defect]]<nowiki/>. The most common example is [[Spina bifida|spina bifida]]. This term refers to a defective fusion of the posterior spinal bony elements, but is still incorrectly used to refer to spinal dysraphism in general, <ref name=":2">BOOS N. and AEBI M. (2008) Spinal Disorders, Fundamentals of Diagnosis and Treatment, Springer, p. 311, 434, 695-696</ref> Spina bifida aperta is an open spinal dysraphism for example, associated with meningocele or meningomyelocele and Spina Bifida Occulta is anexample of closed spinal dysraphisms. <ref name=":3">SCHWARTZ E.S. and ROSSI A., 2015 “Congential spine anomalies: the closed spinal dysraphisms“, Advances in Pediatric Neuroradiology, vol. 45, supp. 3, p. 413-419, </ref>
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| # When two or more vertebrae fail to fully separate and divide with concomitant partial or complete loss of a growth plate, this is called a [[Failure of segmentation|failure of segmentation]]. Examples of this type of congenital spine deformity are congenital [[Scoliosis|scoliosis]], [[Congenital kyphosis|congenital kyphosis]], [[Congenital lordosis|congenital lordosis]] and [[Klippel-Feil syndrome|Klippel-Feil syndrome]]. <ref name=":1">Kaplan K.M et al. (2005). Embryology of the spine and associated congenital abnormalities. Spine J, 5 (5), pp 564-576. </ref>
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| # Congenital [[Scoliosis|scoliosis]] and [[Congenital kyphosis|congenital kyphosis]], and congenital lordosis can also be examples of a [[Failure of formation|failure of formation]]. This type of congenital spine deformity occurs as a result of an absence of a structural element of a vertebra. Typical observable defects are hemivertebrae or wedge vertebrae. <ref name=":1" /> <ref>Goldstein I. (2005). Hemivertebra: prenatal diagnosis, incidence and characteristics, Fetal Diagn Ther,. 20 (2), pp 121-6</ref>
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| [[Image:Defectsofformation.jpg|center]]<br>
| | == Etiology == |
| | The causes of congenital vertebral anomalies are likely to be |
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| Congenital abnormalities of the spine have a range of clinical presentations. Some congenital abnormalities may be benign, causing no spinal deformity and may remain undetected throughout a lifetime. Others may be associated with severe, progressive spinal deformity leading to cor pulmonale or even paraplegia. Some deformities will result in sagittal plane abnormalities, for example kyphosis or lordosis, whereas others will primarily affect the coronal plane, like scoliosis. The resultant spinal deformity is often a complex, three-dimensional structure with differences in both the coronal and sagittal plane, along with a rotational component along the axis of the spine.<ref>Kawakami N, et al. (2009). Classification of congenital scoliosis and kyphosis: a new approach to the three-dimensional classification for progressive vertebral anomalies requiring operative treatment, Spine (Phila Pa 1976), 34 (17), pp1756-65</ref><br>
| | *[[Genetic Conditions and Inheritance|Genetic]] factors, e.g. defects in the Notch signalling pathways. (Notch 1 gene has been shown to coordinate the process of somitogenesis by regulating the development of vertebral precursors in mice), Chromosome 13 and 17 translocations (associated with the development of hemivertebrae). Genetic theories are supported by molecular, animal, and twin population studies. |
| == Clinically Relevant Anatomy ==
| | * Environmental factors have also been suggested, and these include exposure to toxins including carbon monoxide, the use of antiepileptic medication, and maternal [[diabetes]].<ref name=":20">Musculoskeletalkey 6 Congenital Deformities of the Spine Available: https://musculoskeletalkey.com/6-congenital-deformities-of-the-spine/ (accessed 10.10.2021)</ref> |
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| <u>Neural tube deformities</u>
| | == Types == |
| | [[Image:Defectsofformation.jpg|699x699px|alt=|right|frameless]]The spectrum of congenital deformities of the spine includes a range of conditions that blend gradually from [[scoliosis]] through kyphoscoliosis to pure [[kyphosis]]. These deformities occur when an asymmetric failure of development of one or more vertebrae results in a localized imbalance in the longitudinal growth of the spine and an increasing curvature affecting the [[Cardinal Planes and Axes of Movement|coronal and/or sagittal plane]], with a risk for progression during skeletal growth. |
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| Neural tube deformity is a term for congenital anomalies because of incomplete closure of the neural tube in the utero. <ref>Au K.S. et al. (2010). Epidemiologic and genetics aspects of spina bifida and other neural tube defects. Dev. Disabil. Res Rev, 16 (1), pp 6-15</ref> <ref>Swenson L. (2012). Neural tube defects. Nursing Care of the pediatric neruosurgery partient. 4, 119-139</ref> This causes impaired formation of structures along the craniospinal axis. <ref name=":4">Sewell, M. J. et al. 2015, “Neural Tube Dysraphism: Review of Cutaneous Markers and Imaging. “, Pediatric Dermatology, vol 32, pp 161–170, </ref> Spinal neural tube defects can be subdivided into those that are open versus those that are closed:
| | The consequence of unbalanced growth of the spine can be the: |
| * Open: the failure is primary neuralation
| | # Development of a benign curve with slow or no progression, in which case observation may be the only treatment required. |
| * Closed: the structural deformities are mostly limited to the spinal cord. <ref name=":5">McComb J.G., Spinal and cranial neural tube defects. Neurosurgical Issues for the pediatric patient, 1997;4(3):156-166 </ref> <ref name=":6">Amarante M.A. et al. (2012). Management of urological dysfucntion in pediatric patiens with spinal dysraphism: review of the litarature. Neurosurg. Focus, 33 (4). </ref>
| | # Types of vertebral abnormalities that produce considerable asymmetry in spinal growth and the development of very aggressive deformities with consequent functional, cosmetic, respiratory, and neurological complications. Understanding the anatomical features of the individual vertebral anomalies and their relation to the remainder of the spine makes it possible to predict those abnormalities that are likely to produce a severe curve. Recognizing the natural history of the deformity at an early stage can in turn allow appropriate surgical treatment, with the aim of preventing the development of severe spinal curvature and trunk decompensation.<ref name=":20" /> |
| It is not only the elements of the spine that can be affected. Other bodily organs which formed during the same embryonic stage can also be affected. This is because the paraxial mesoderm is responsible for the formation of the vertebrae, whereas the other two areas of mesoderm (intermediate and lateral) are involved in the development of the urogenital, pulmonary and cardiac systems. Many patients may have difficulty with respiration due to the abnormal curvatures of the spine <ref name=":1" />. It has also been observed that these associated anomalies have an influence on the progression of untreated congenital [[Scoliosis|Scoliosis]].<ref>Marks D.S. et al. (2009). The natural history of congenital scoliosis and kyphosis. Spine (Phila Pa 1976). 34 (17), pp 1751-1755</ref>
| | Examples of a congenital spinal deformities include: |
| | * Congenital [[Thoracic Hyperkyphosis]] |
| | * Congenital [[Scoliosis]] |
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| <u>Failure of segmentation/ formation</u> | | *[[Klippel-Feil syndrome]] <ref>Klemme WR et al. (2001). Hemivertebral excision for congenital scoliosis in very young children. J Pediatr Orthop. 21 (6), pp761-764.</ref> |
| | == Characteristics/Clinical Presentation == |
| | [[File:Scoliosis cobb.gif|right|frameless|228x228px]] |
| | Congenital abnormalities of the spine have a range of clinical presentations. Some congenital abnormalities may be benign, causing no spinal deformity and may remain undetected throughout a lifetime. |
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| The bony structure of the spine is determined in the first 6 weeks of intrauterine life, at the mesenchymal stage of embryonic development. Somites form and undergo a process of segmentation and recombination. This is necessary to give rise to the cartilaginous mould that afterwards will ossify to form the bony spine. Errors during this process can lead to either failure of formation, failure of segmentation or a combination of both. <ref>Fender, D. et al. Jan 2014 “Spinal disorders in childhood II: spinal deformity“, Surgery (Oxford), Vol. 32, Nr1, pp 39–45, </ref>
| | Some deformities will result in sagittal plane abnormalities, for e.g. kyphosis or lordosis, whereas others will primarily affect the coronal plane e.g. scoliosis. The resultant spinal deformity is often a complex, three-dimensional structure with differences in both the coronal and sagittal plane, along with a rotational component along the axis of the spine.<ref>Kawakami N, et al. (2009). [https://scholar.google.com/scholar_url?url=https://journals.lww.com/spinejournal/Fulltext/2009/08010/Classification_of_Congenital_Scoliosis_and.00005.aspx&hl=en&sa=T&oi=gsb&ct=res&cd=0&d=17108192740910527630&ei=MHEGYunMNryO6rQP-PmgiAk&scisig=AAGBfm2jeliVEpx0mxH3vyFgvxszCJ7kAQ Classification of congenital scoliosis and kyphosis]: a new approach to the three-dimensional classification for progressive vertebral anomalies requiring operative treatment, Spine (Phila Pa 1976), 34 (17), pp1756-65</ref> |
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| | == Symptoms of Congenital Spine Deformities == |
| | Doctors often detect any spine deformity at birth if there is any abnormal curvature in the back. However, some spine deformities until later in childhood and/or adolescence when symptoms worsen. Physical signs of congenital spine deformities typically include: |
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| | * Tilted pelvis |
| | * Difficulty walking |
| | * Difficulty breathing |
| | * Abnormal curvature or twisting in the back, left or right, forward or backward |
| | * Uneven shoulders, hips, waist or legs |
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| [[Image:Types_of_congenital_scoliosis.png|center]]<br>Examples of a failure of segmentation are:
| | == Respiratory Implications == |
| * Congenital kyphosis
| | Abnormal development of the spine can cause significant scoliosis, kyphosis, or lordosis, resulting in body deformities that can be distressing to patients and their families. The more serious threat to long-term health is the adverse effect of abnormal development of the spine on [[Pulmonary Function Test|pulmonary function]]. |
| * Congenital [[Scoliosis|Scoliosis]]
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| Examples of a failure of formation are:
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| * [[Klippel-Feil syndrome|Klippel-Feil syndrome]] <ref>Klemme WR et al. (2001). Hemivertebral excision for congenital scoliosis in very young children. J Pediatr Orthop. 21 (6), pp761-764.</ref>
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| * Congenital kyphosis
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| * Congenital [[Scoliosis|Scoliosis]]
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| The anomaly is present at birth, so a curvature is noted much earlier than patients with idiopathic scoliosis. Large deformities can result due to the number of years of growth remaining. <ref>Lonstein J.E. (1999). congenital spine deformaties: scoliosis, kyphosis, and lordosis. orthopedic clinics of north america, 30 (3), pp387-405</ref>
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| == Epidemiology /Etiology ==
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| The etiology of congenital spine deformities varies greatly because there are many different types. It is difficult to identify one factor that may be responsible with prevalence being different for each of them. <ref name=":1" /> They can be divided into 3 groups:
| | * This is well documented for curves exceeding 90 degrees, which cause severe restrictive lung disease, but not well understood for lesser curves. Pulmonary function is an important determinant of long-term survival. |
| # '''''Neural tube deformities''''' such as spina bifida which is not a very common disease. It is believed that spina bifida is caused during pregnancy due to a deficiency of vitamin B and is also dependant on genetic and environmental factors. Obese women with poorly controlled diabetes have a greater risk of having children with spina bifida. In America less than 1 in 1000 children are born with it each year. Having a child with spina bifida increases the chance that further children will also develop the condition by 8 times <ref>Fichter M. A. et al. 2008 “Fetal spina bifida repair–current trends and prospects of intrauterine neurosurgery”, Fetal Diagnosis and Therapy, vol 23, nr 4 , pp 271–286, </ref> <ref>Burke R., Liptak G. (2011) . Providing a Primary Care Medical Home for Children and Youth with Spina Bifida, American Academy of Pediatrics, 128, pp 1645-1657</ref>
| | * Increased rates of mortality, mostly resulting from pulmonary failure, have been seen in patients with untreated infantile scoliosis beginning at the age of 20 years, with a rise in mortality rates to fourfold above normal by the age of 60 years<ref>Musculokeletalkey Respiratory Implications of Abnormal Development of the Spine Available:https://musculoskeletalkey.com/4-respiratory-implications-of-abnormal-development-of-the-spine/ (accessed 10.10.2021)</ref> |
| # Congenital spine deformities due to '''''failure of segmentation'''''. In this case, the vertebrae are fused together and cause congenital kyphosis, congenital lordosis, and [[Scoliosis|Scoliosis]]. <ref>McMaster Mj., singh H. 1999 “Natural History of Congenital Kyphosis and Kyphoscoliosis. A Study of One Hundred and Twelve Patients” ,Journal of Bone no People and Joint Surgery, vol 81 , nr 10, pp 1367 -83, </ref> Congenital [[Scoliosis|Scoliosis ]]is not the most common type as it only accounts for 15% of the total cases <ref>Elizabeth D Agabegi, Agabegi, Steven S. Step-Up to Medicine. 3de edition. Baltimore:Lippincott Williams & Wilkins, 2008 </ref> with thoracic scoliosis being the most common at 64% of the total cases, Thoracicolumabar scoliosis accounts for 20% with 11% located in the lumbar region and 5% in the lumbosacral region. <ref name=":1" /> [[Klippel-Feil_syndrome|Klippel-Feil syndrome]] is also due to a failure of segmentation. There is not much known about the prevalence of this syndrome.
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| # Congenital spine deformities due to '''''failure of formation''''' are rare. The prevalence of hemivertebrae is 0.33 out of 1000 births and there is an equal number of women and men affected. ''Goldstein'' showed that 23 out of the 26 children with hemivertebrae developed a congenital spine deformity like scoliosis, congenital lordosis or congenital kyfosis. <ref>Goldstein I. (2005). Hemivertebra: prenatal diagnosis, incidence and characteristics, Fetal Diagn Ther,. 20 (2), pp 121-6</ref>
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| Environmental factors, genetic factors, vitamin deficiency, chemicals, and drugs, singly or in combination, have all been implicated in the development of congenital abnormalities during the embryonic period. Whatever the factor is that influences the embyo it must affect the embryo at a very early stage of development, before cartilage bone can develop. These defects can lead to a failure of segmentation or a failure of formation of the spine. <ref>Hensinger RN. (2009). Congenital scoliosis: etiology and associations. Spine (Phila Pa 1976)., 34 (17), pp1745-1750. </ref>
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| Studies carried out confirmed various possible causes that may help in the prevention of these anomalies. showing that during critical stages of development, fetal exposure to thalidomide can lead to severe congenital malformations. ''Ghidini et al.'' suggested that mothers taking lovastatin have an increased risk for congenital abnormalities. Certain progestin/estrogen compounds may also increase the incidence of these anomalies. <ref name=":1" />
| | == Diagnostic Procedures == |
| | There are several different procedures that can be used to carry out the imaging of the spine. <ref name=":9">SORANTIN E. et al., 2008 “[https://scholar.google.com/scholar_url?url=https://www.sciencedirect.com/science/article/pii/S0720048X08004014&hl=en&sa=T&oi=gsb&ct=res&cd=0&d=12593037565671745085&ei=X3EGYoHAHYz2yATZiLfgDQ&scisig=AAGBfm3bup_DMRDb2Rc9kUtl5i7oAnXiSw MRI of the Neonatal and Paediatric Spine and Spinal Canal]“, European Journal of Radiology, vol. 68, nr. 2, p. 227 – 234</ref> |
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| == Characteristics/Clinical Presentation == | | *[[X-Rays]] are useful for showing structural deformities such as hemivertebrae, butterfly vertebra, or incomplete fusion of posterior elements. X-ray is used if no imaging of the spinal cord is required. For scoliosis, erect posterior-anterior frontal and/or lateral views (with breast shielding) are usually obtained. <ref name=":10">Patrick D Barnes. (2009). Pediatric radiology : chapter 9, spine imaging. (3). Mosby</ref> |
| | *[[MRI_Scans|MRI]] is most frequently used for imaging of the spine in adults as the spinal canal and its content can be analysed. |
| | *[[CT Scans]] continue to be the preferred method for the assessment of localised bony abnormalities, or a calcified component, of the spinal canal, foramina, neural arches, and articular structures. <ref name=":10" /> |
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| A defect early in fetal life may have a variety of clinical presentations as several systems develop from a common precursor.<ref name=":1" /> Examples of these clinical presentations are scoliosis, hyperlordosis and/or hyperkyphosis. Congenital abnormalities of the spine also have the potential to affect the spinal cord and associated nerve roots. The neurological symptoms can vary from minor motor or sensory signs to paraplegia, depending on the type and severity of the congenital spine deformity, but congenital spine deformities are not always immediately obvious at birth. <ref>Oskouian R.J. Jr. et al. (2007). Congenital abnormalities of the thoracic and lumbar spine. Neurosurg, Clin. N. Am., 18 (3), pp 479-498, </ref> They can present as a deformity with growth or with clinical signs of neurologic dysfunction during adolescence or adulthood.
| | == Treatment == |
| | [[File:Thor 4.png|right|frameless]] |
| | In most cases, nonoperative treatment options are recommended before surgery is considered. Nonoperative treatment options typically include [[Pain Medications|pain medication]], certain braces and physical therapy (that includes [[Gait training|gait]] and [[posture]] training). |
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| '''Neural tube deformities'''
| | === Surgery === |
| | Surgery Is Considered If: |
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| As described earlier, neural tube deformities can be divided into two groups:
| | # The spinal deformity is progressing |
| * '''Open''': neural elements and/or membranes are exposed to the external environment.
| | # The condition has caused unbearable physical deformity |
| <u>Myelomeningocele</u> is characterised by being most frequently present in the lumbosacral region, <ref name=":7">Rolton D. et al. 2014, “Scoliosis: a review Paediatrics and Child Health“, Vol 24, nr 5, pp 197–203,</ref> a bulging of a sac like structure covered by a thin membrane with CSF that will drain if the sac is disrupted <ref name=":8">Salih M.A. et al. 2014, “Classification, clinical features, and genetics of neural tube defects“,Saudi Med. J., vol 35, pp5-14, </ref> | | # The patient experiences [[Chronic Pain and the Brain|chronic pain]] that cannot be relieved by nonoperative treatment options |
| | # The condition has caused [[Myelopathy|compression of the nerve roots]] or spinal cord<ref name=":0" /> |
| | Spinal instrumentation for congenital spine deformity cases is safe and effective, <ref>Hedequist D.J. (2009). [https://scholar.google.com/scholar_url?url=https://journals.lww.com/spinejournal/Fulltext/2009/08010/Letters_to_the_Editor.00008.aspx&hl=en&sa=T&oi=gsb&ct=res&cd=0&d=14487695445756530477&ei=r3EGYtmvL4TyyASduqq4Dg&scisig=AAGBfm1gNlnDA_n49gROQP0tUg89Ot8EGw Instrumentation and fusion for congenital spine deformities], Spine (Phila Pa 1976), 1;34 (17), pp1783-90</ref> <ref name=":15">Hedequist D.J. et al. (2004). [https://scholar.google.com/scholar_url?url=https://journals.lww.com/spinejournal/fulltext/2004/09150/The_Safety_and_Efficacy_of_Spinal_Instrumentation.22.aspx&hl=en&sa=T&oi=gsb&ct=res&cd=0&d=6705511578530450649&ei=y3EGYvW6M4K4ygTc_rBI&scisig=AAGBfm1ETJHQCwdKVPXJBKqM9oG-KAJBCQ The safety and efficacy of spinal instrumentation in children with congenital spine deformities], Spine (Phila Pa 1976), 15;29 (18), pp 2081-2086</ref> as is growing rod surgery for selected patients with congenital spinal deformities. <ref name=":16">Elsebai HB et al.,2011 “[https://scholar.google.com/scholar_url?url=https://journals.lww.com/pedorthopaedics/Fulltext/2011/01000/Safety_and_Efficacy_of_Growing_Rod_Technique_for.1.aspx&hl=en&sa=T&oi=gsb&ct=res&cd=0&d=12030621565327864571&ei=7nEGYr2CGceUywTTgoa4Cg&scisig=AAGBfm3UIzLrBxHKjDZ5nelvYadXpHrQAA Safety and Effficacy of Growing Rod Technique for Pediatric Congenital Spinal Deformities]“, J Pediatr Orthop, vol 31, nr 1, pp 1-5. Jan-Feb, </ref> <ref name=":17">Yazici M. and Emans J.2009, “[https://scholar.google.com/scholar_url?url=https://journals.lww.com/spinejournal/Fulltext/2009/08010/Spinal_Instrumentation_Without_Fusion_for.00010.aspx&hl=en&sa=T&oi=gsb&ct=res&cd=0&d=9304556392105449818&ei=CXIGYumpHbuP6rQPgrSAyAI&scisig=AAGBfm0jT5yF5Lh-yHMvb4dMJ1a0BtjGpg Fusionless Instrumentation Systems for Congenital Scoliosis: Expandable Spinal Rods and Vertical Expandable Prosthetic Titanium Rib in the Management of Congenital Spine Deformities in the Growing Child]“, Spine, Vol 34, Nr 17, pp 1800-1807</ref> The size and weight of the patient determines the size of the spinal implants, whereas the surgical fixation anchors are determined by the anatomy of the patient and the anomalies present. <ref name=":15" /> The complications associated with the use of this spinal instrumentation are infrequent and the curve correction, length of immobilisation and fusion rate is improved.<ref name=":15" /> |
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| <u>Myelocele</u> Is associated with similar clinical implications as myelomeningocele but is flat <ref name=":8" /> | | # '''Growing Rod Surgery:''' Growing rod surgery is one of the options for the correction of scoliosis, a modern alternative treatment for young children with early onset scoliosis. The incidence of complication remained relatively low <ref name=":16" /> <ref name=":17" /> and is also recommended for patients where the primary problem is at the vertebral column. |
| | # '''Expansion Thoracostomy and VEPT:''' For severe congenital spine deformations, when a large amount of growth remains, expansion thoracostomy and VEPTR (a curved metal rod designed for many uses), are the most appropriate choice. Used when the primary problems involve the thoracic cage, eg when there are rib fusions and/or with developing Thoracic Insufficient Syndrome, <ref name=":17" /> but the incidence of complications using VEPTR is, however, relatively high.<ref name=":18">Moramarco M and Weiss HR. (2015). [https://scholar.google.com/scholar_url?url=https://europepmc.org/article/med/26573161&hl=en&sa=T&oi=gsb&ct=res&cd=1&d=12345306911325150121&ei=L3IGYu_GN7eN6rQP8ZCWsAI&scisig=AAGBfm2KlJYAcSu0w_3HZ4P92EdVGI_XwA Congenital Scoliosis], Curr Pediatr 2015 Nov 17</ref> |
| | # '''Resection and Fusion:''' For treating congenital scoliosis caused by hemivertebra posterior hemivertebra, resection and monosegmental fusion appears to be effective. This treatment results in an excellent correction in both the frontal and sagittal planes. <ref>Zhu X. et al. 2014, “[https://scholar.google.com/scholar_url?url=https://publishing.rcseng.ac.uk/doi/abs/10.1308/003588414X13824511650173&hl=en&sa=T&oi=gsb&ct=res&cd=0&d=18087741045513419297&ei=WXIGYqmhAYz2yATZiLfgDQ&scisig=AAGBfm21gVoosoDPguDLvFikQ4Kvmop9TA Posterior hemivertebra resection and monosegmental fusion in the treatment of congenital scoliosis.] “, Article from Annals of The Royal College of Surgeons of England, Vol 96, Nr5, pp. 41-44</ref> Early surgery is typically prescribed as a treatment for children with congenital scoliosis, even though there is little evidence for its long term results. <ref name=":18" /> |
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| <u>Hemimyelomeningocele</u> is a rare lesion where the spinal cord has been divided by a bony spur with one segment open and the other closed <ref name=":6" />
| | === Physical Therapy === |
| * '''Closed''': spinal abnormalities covered by skin. Some go undetected or remain asymptomatic, <ref name=":6" /> <ref>McComb J.G., Spinal and cranial neural tube defects. Neurosurgical Issues for the pediatric patient, 1997;4(3):156-166 </ref> but there are variants that can be associated with cord tethering, subcutaneous mass or more severe spine abnormalities. The lumbosacral region is the most common location of these defects <ref name=":4" /> An affected child has an intellectual function and other functions are as well less affected <ref name=":5" /> Lipomyelomeningocele and lipomyelocele are characterised by subcutaneous fatty mass above the gluteal crease <ref name=":8" />
| | [[File:Cp child sit balance.jpg|right|frameless]] |
| <u>Failure of segmentation</u><br>Congenital scoliosis is charaterised by:
| | The [[International Classification of Functioning, Disability and Health (ICF)|ICF]] has underscored the need for therapists to provide a holistic approach to treatment, focusing not only on [[Therapeutic Exercise|exercises]], [[Stretching|stretches]], and what a child is unable to do, but also on the child’s abilities. The approach to therapy is functional and questions whether a child can actively participate with his or her current level of function. |
| * a three-dimensional deformity involving lateral curvature and rotation
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| * a benign prognosis
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| * the fact that worst curves occur with unilateral defects
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| * a curve site dependent on the location of apical vertebra <ref>Rolton D. et al. 2014 “Scoliosis: a review Paediatrics and Child Health“, Vol 24, nr 5, pp 197–203</ref>
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| <u>Congenital kyphosis</u>
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| * Can progress rapidly and may cause spinal cord compression and paralysis <ref>Fichter M. A. et al. 2008 “Fetal spina bifida repair–current trends and prospects of intrauterine neurosurgery”, Fetal Diagnosis and Therapy, vol 23, nr 4 , pp 271–286</ref>
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| <u>Failure of formation</u><br>Congenital scoliosis
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| * Poor prognosis <ref name=":7" />
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| <u>Congenital kyphosis</u>
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| * Absence and asymmetry of the growth plates <ref>Priya K. et al. (2015). congenital kyphosis in thoracic spine secondary to absence of two vertebral bodies, J clin diagn res, 9 (1). </ref>
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| * [[Klippel-Feil_syndrome|Klippel-feil syndrome]]
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|
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| == Differential Diagnosis ==
| | A physiotherapy assessment is required for children with early onset scoliosis to enable them to function to their fullest potential within society. Assessments provide a baseline for future interventions and establish goals that are appropriate and achievable for the child and the family within their environment. |
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|
| Most congenital spine deformities are diagnosed in the uterus, and if not, at birth. as they are clearly present e.g. Spina Bifida, scoliosis. Some disorders, however, might not become symptomatic until childhood or even adulthood <ref name=":3" /> In these cases, MRI scans or ultrasound can be used to diagnose the specific disorder, <ref name=":9">SORANTIN E. et al., 2008 “MRI of the Neonatal and Paediatric Spine and Spinal Canal“, European Journal of Radiology, vol. 68, nr. 2, p. 227 – 234</ref> therefore, for many conditions there is not a differential diagnosis as the deformity/anomaly will be clear on an MRI.
| | * Many factors are involved the assessment including the pathology of the child’s condition, family input and expectations, the child’s environment, the equipment needs, and the ability to access services. |
| | * This assessment is necessary to establish what is required for each individual. |
|
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|
| <u>Neural tube deformities</u><br>[[Spina_Bifida_Occulta|Spina bifida]] is an out dated term for spinal dysraphism. <ref>Venkatatamana NK., “Spinal dysraphism“, Journal of Pediatric Neuroscience, vol. 6, supp. 1, p. 31-40, 2011.</ref> This modern term includes many congenital anomalies where the neural tube is defective, which leads to hernia’s of the meninges or other neural tissues.
| | An initial assessment may consist of observation of the child at play. This is most appropriately undertaken within the home environment, where the child will be most at ease and will play with his or her own toys. However, this may not always be possible. Play provides many benefits for both child and therapist. |
| * open: with a non-skin covered back mass, where you have to differentiate between myeolecele and myelomeningocele
| |
| * closed: with a skin-covered back mass, where you have to determine whether there is a subcutanuous mass present or not <ref>Schwartz E.S. and Rossi A., “Congential spine anomalies: the closed spinal dysraphisms“, Advances in Pediatric Neuroradiology, vol. 45, supp. 3, p. 413-419, 2015.</ref>
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| <u>Failure of segmentation</u><br>Congenital scoliosis:
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| In most of the severe cases, scoliosis can be detected without medical imaging. There are different forms that can be present as a result of failure of segmentation:
| | Play is the most likely way in which rapport will be established between the child and the therapist, but it also provides the opportunity to observe a variety of factors when eg In what position does the child play when lying, sitting and/or standing; Does the child move from one position to another?; How does the child move?; What motivates the child in his or her play?; What kind of toys does the child choose? |
| * A block vertebra, which is a bilateral failure with complete fusion of the disc between the involved vertebrae.
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| * A unilateral unsegmented bar has fused discs and facet joints on one side of the vertebral column. <ref name=":2" />
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| Congenital kyphosis:
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| * idiopathic hyperkyphosis
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| * neuromuscular
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| * spinal cord tumor
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| * connective tissue disorders
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| * skeletal dysplasia
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| * infection (tuberculosis, pyogenic, fungal)
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| * tumor
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| * myelomeningocele<ref name=":2" />
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| Congenital spinal canal stenosis
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|
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|
| The sagittal diameter must be <13 mm to be considered a congenital spinal canal stenosis. This disorder itself is not the cause of particular symptoms. It is the Spinal Canal Compression Syndrome that triggers the neural structures in the spinal canal and because of the congenitally narrowed spinal canal, these patients face a greater risk of developing the syndrome. Depending on the disease, there are differential diagnoses for the Spinal Canal Compression Syndrome at each level of the spine.
| | It is important within the role of physiotherapist to consider the following: |
| * Cervical: MS, Myelitis, B12 hypovitaminosis, spinal tumors, polyneuropathy (PNP), ateriovenous malformations
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| * Thoracal: disc herniation (often calcified), OPLL, arteriovenous malformations, spinal tumors
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| * Lumbar: vascular claudication, spinal metastasis, polyneuropathy. <ref name=":2" />
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| <u>Failure of formation</u>
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|
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|
| Congenital scoliosis<br>As mentioned above there are different types of scoliosis. The following are as a failure of formation:
| | * The child’s abilities, needs, hobbies; |
| * A Hemivertebra, which is a complete unilateral failure of the vertebral formation that can be:<br>○ Fully segmented<br>○ Partially segmented<br>○ Non-segmented<br>A hemivertebra can be further described as incarnated or non-incarnated. | | * Needs of the family members and what is important to them; |
| * A wedge vertebra is a partial unilateral failure.<ref>Debnath UK. et al., “Congenital scoliosis - Quo vadis? “, Indian Journal of Orthopaedics, vol. 44, nr. 2, p. 137 -147, 2010</ref>
| | * Role of the child within the family, especially if the child has siblings; |
| Congenital kyphosis
| | * The environments inside and outside the home to which the child has access and the child’s level of activity and participation.<ref>Musculoskeletal Key [https://musculoskeletalkey.com/25-physiotherapy/ 25 Physiotherapy] Available:https://musculoskeletalkey.com/25-physiotherapy/ (accessed 10.10.2021)</ref> |
| * idem failure of segmentation | |
| [[Klippel-Feil_syndrome|Klippel-Feil syndrome]]
| |
| * [[Congenital_torticollis|Congenital muscular torticollis]] <ref>Gray E et al. “Congenital fusion of the cervical vertebrae“, Surg Gynecol Obstet, 1964 vol118, pp373-85, feb .</ref>
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| * Wildervanck syndrome | |
| * MURCS association
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| * Turner or Noonan syndrome
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| * Morquio syndrome
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| * Tuberculosis of the cervical spine
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| * Nevoid basal cell nevus syndrome
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| * Crouzon syndrome
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| * Apert syndrome
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| * Binder syndrome
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| * Oculo-auriculo-vertebral spectrum <ref>Naikmasur V.G. et al., “Type III Klippel-Feil syndrome: case report and review of associated craniofacial anomalies“, Springer, vol. 99, nr. 2, p. 197 – 202, 2011</ref>
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| Osteopetrosis
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| * Leukemia <ref>Taner S. et al., “Diagnosis: Infantile Malign Osteopetrosis”, Turkish Journal of Hematology, vol. 31, nr. 3, p. 335 – 336, 2014</ref>
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| * Dysosteosclerosis <ref>Wilson C.J. and Vellodi A., “Autosomal recessive osteopetrosis: diagnosis, management, and outcome“, Archives of Diseases in Childhood, vol. 83, p. 449 – 452, 2000</ref>
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|
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|
| == Diagnostic Procedures ==
| | '''See physiotherapy sections in these great pages''' |
|
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|
| There are several different procedures that can be used to carry out the imaging of the spine. The choice of imaging depends on what is required to be analysed, such as bone vs spinal canal. In children, it is important to start with less invasive procedures such as US, due to their cartilage and non-ossified bones. <ref name=":9" />
| | [[Scoliosis]] |
|
| |
|
| [[X-Rays|X-Rays]] are useful for showing structural deformities such as hemivertebrae, butterfly vertebra, or incomplete fusion of posterior elements. X-ray is used if no imaging of the spinal cord is required. For scoliosis, erect posterior-anterior frontal and/or lateral views (with breast shielding) are usually obtained. <ref name=":10">Patrick D Barnes. (2009). Pediatric radiology : chapter 9, spine imaging. (3). Mosby</ref> | | [[Kyphosis]] |
|
| |
|
| [[MRI_Scans|MRI]] is most frequently used for imaging of the spine in adults as the spinal canal and its content can be analysed. <br>''Basu et al.'' suggest that MRI and echocardiography should be an essential part in any evaluation of patients with congenital spinal deformity, <ref>Basu S.et al., 2002 “Congenital Spinal Deformity: A Comprehensive Assessment at Presentation“, Spine, Vol 27, nr 2, pp 2255-2259</ref> with numerous other studies demonstrating the high degree of confidence with MRI use. <ref>Fabry G. (2009). Clinical practice: the spine from birth to adolescence, Eur J Pediatr, 168 (12), pp1415-1420</ref> <ref>Durand D.J., Huisman T.A., Carrino J.A. (2010). MR imaging features of common variant spinal anatomy, Magn Reson Imaging Clin N Am, 18 (4), pp717-726</ref> <ref name=":9" /> <ref>. Grimme J.D. (2007). Castillo M. Congenital anomalies of the spine. Neuroimaging Clin N Am, 17 (1), pp 1-16 </ref> <ref>Brophy JD et al. (1989). Magnetic resonance imaging of lipomyelomeningocele and tethered cord, Neurosurgery, 25 (3), pp336-340</ref> | | [[Scheuermann's Kyphosis]] |
|
| |
|
| [[CT_Scans|CT Scans]] continue to be the preferred method for the assessment of localised bony abnormalities, or a calcified component, of the spinal canal, foramina, neural arches, and articular structures. <ref name=":10" /> | | [[Adam's forward bend test]] |
|
| |
|
| == Outcome Measures == | | == Outcome Measures == |
| | | The most commonly used questionnaires with patients who have undergone spinal surgery include |
| These are the most commonly used questionnaires with patients who have undergone spinal surgery, the most common treatment for congenital spine deformities.
| | *[[Oswestry_Disability_Index|Owestry Disability Index]] <ref name=":11">COPAY A.G. et al.2008 “[https://scholar.google.com/scholar_url?url=https://www.sciencedirect.com/science/article/pii/S1529943007010017&hl=en&sa=T&oi=gsb&ct=res&cd=0&d=15513237013168726639&ei=k3IGYuaYJoz2yATZiLfgDQ&scisig=AAGBfm15vc8DxP7OA_AZ1rurrzL-B567nQ Minimum Clinically important difference in lumbar spine surgery patients]: a choice of methods using the Oswestry Disability Index, Medical Outcomes Study Questionnaire Short Form 36, and Pain Scales“, The Spine Journal, vol. 8, nr. 6, p. 968 – 974, . </ref> <ref name=":12">DEVIN C.J. and McGIRT M.J.2015, “[https://scholar.google.com/scholar_url?url=https://www.sciencedirect.com/science/article/pii/S0967586815000156&hl=en&sa=T&oi=gsb&ct=res&cd=0&d=4773698857369320771&ei=s3IGYui8BYf0yAS3lKWYDA&scisig=AAGBfm0nH_IAyJQjW5wicJDJjpgFNERDbw Best evidence in multimodal pain management in spine surgery and means of assessing postoperative pain and functional outcomes]“, Journal of Clinical Neuroscience, vol. 22, nr. 6, p. 930 – 938. </ref> <ref name=":2">BOOS N. and AEBI M. (2008) Spinal Disorders, Fundamentals of Diagnosis and Treatment, Springer, p. 311, 434, 695-696</ref> |
| * The Balanced Inventory for Spinal Disorders <ref>SVENSSON E. et al., 2009 “The Balanced Inventory for Spinal Disorders: The validity of a Disease Specific Questionnaire for Evaluation of Outcomes in Patients With Various Spinal Disorders“, Spine, vol. 34, nr. 18, p. 1976 – 1983</ref>
| | *[[Brief_Pain_Inventory_-_Short_Form|Brief Pain Inventory]] <ref name=":12" /> |
| * [[Oswestry_Disability_Index|Owestry Disability Index]] <ref name=":11">COPAY A.G. et al.2008 “Minimum Clinically important difference in lumbar spine surgery patients: a choice of methods using the Oswestry Disability Index, Medical Outcomes Study Questionnaire Short Form 36, and Pain Scales“, The Spine Journal, vol. 8, nr. 6, p. 968 – 974, . </ref> <ref name=":12">DEVIN C.J. and McGIRT M.J.2015, “Best evidence in multimodal pain management in spine surgery and means of assessing postoperative pain and functional outcomes“, Journal of Clinical Neuroscience, vol. 22, nr. 6, p. 930 – 938, </ref> <ref name=":2" /> | | *[[Roland‐Morris_Disability_Questionnaire|Roland–Morris disability questionnaire]] <ref name=":12" /> <ref name=":2" /> |
| * The physical component summary <ref name=":11" />
| |
| * The Short Form of the Medical Outcomes Study <ref name=":11" /> <ref name=":2" />
| |
| * [[Brief_Pain_Inventory_-_Short_Form|Brief Pain Inventory]] <ref name=":12" /> | |
| * [[Roland‐Morris_Disability_Questionnaire|Roland–Morris disability questionnaire]] <ref name=":12" /> <ref name=":2" /> | |
| * SRS-22 Questionnaire <ref>FARLEY F.A. M.D. et al .2014, “Congenital Scoliosis SRS-22 Outcomes in Children Treated With Observation, Surgery, and VEPTR“, Spine, vol 39., nr. 22. p. 1868 – 1874, </ref>
| |
| | |
| == Examination ==
| |
| | |
| The physical examination of a patient with kyphosis has various components: <ref name=":14">Chaén G., Dormans J.P. (2009). Update on congenital spinal deformities: preoperative evaluation. Spine (Phila Pa 1976). 1;34(17), pp1766-1774</ref>
| |
| * postural analysis, which may reveal a gibbus deformity or a round back.
| |
| * palpation to assess for spinal abnormalities and may identify tenderness of the paraspinal musculature, which is often present.
| |
| * range of motion during flexion, extension, side bending and spinal rotation of the back. Asymmetry is be noted. Adam’s forward bend test may reveal a thoracolumbar kyphosis, although ''Karachalios et al'' suggested that this test is not a sure diagnostic criterion for the early detection of scoliosis due to the unacceptable number of false-negative findings. <ref>Karachalios Theofilos et al.,1999 “Ten-Year Follow-Up Evaluation of a School Screening Program for Scoliosis: Is the Forward-Bending Test an Accurate Diagnostic Criterion for the Screening of Scoliosis? “, Spine, Vol 24, nr22, pp 2318</ref> Rather, the combined back-shape analysis methods is recommended.
| |
| ''Côté Pierre et al''. however, suggested that the Adam’s forward band test is more sensitive compared to the scoliometer and consider it to be the best non-invasive clinical test to evaluate scoleosis. <ref>. Côté Pierre et al., “A Study of the Diagnostic Accuracy and Reliability of the Scoliometer and Adam's Forward Bend Test“, Spine, Vol. 23, nr. 7, pp 796–802, 1 April 1998 </ref>
| |
| | |
| A complete neurologic evaluation should also be carried out to rule out in the presence of intraspinal anomalies <ref name=":14" />. Patients with congenital spondylolisthesis show deficits in their neurological examinations. <ref name=":1" /> A neurological evaluation includes an evaluation of pain, numbness, paresthesia, tingling, extremity sensation and motor function, muscle spasm, weakness and bowel/bladder changes. <ref name=":2" />
| |
| | |
| For the examination of spina bifida oculta, X-ray examination is the only valid test to confirm this type of neural tube defect. <ref name=":0" />
| |
| | |
| == Medical Management ==
| |
| | |
| The natural history, the character and location of the deformity ultimately influence the choice of treatment.<ref name=":13" /> Spinal instrumentation for congenital spine deformity cases is safe and effective, <ref>Hedequist D.J. (2009). Instrumentation and fusion for congenital spine deformities, Spine (Phila Pa 1976), 1;34 (17), pp1783-90</ref> <ref name=":15">Hedequist D.J. et al. (2004). The safety and efficacy of spinal instrumentation in children with congenital spine deformities, Spine (Phila Pa 1976), 15;29 (18), pp 2081-2086</ref> as is growing rod surgery for selected patients with congenital spinal deformities. <ref name=":16">Elsebai HB et al.,2011 “Safety and Effficacy of Growing Rod Technique for Pediatric Congenital Spinal Deformities“, J Pediatr Orthop, vol 31, nr 1, pp 1-5. Jan-Feb, </ref> <ref name=":17">Yazici M. and Emans J.2009, “Fusionless Instrumentation Systems for Congenital Scoliosis: Expandable Spinal Rods and Vertical Expandable Prosthetic Titanium Rib in the Management of Congenital Spine Deformities in the Growing Child“, Spine, Vol 34, Nr 17, pp 1800-1807</ref> The size and weight of the patient determines the size of the spinal implants, whereas the surgical fixation anchors are determined by the anatomy of the patient and the anomalies present. <ref name=":15" /> The complications associated with the use of this spinal instrumentation are infrequent and the curve correction, length of immobilisation and fusion rate is improved.<ref name=":15" />
| |
| | |
| <u>Growing rod surgery</u><br>Growing rod surgery is one of the options for the correction of scoliosis, a modern alternative treatment for young children with early onset scoliosis. ''Elsebai HB et al''. focused on its use in progressive congenital spinal deformities. The incidence of complication remained relatively low <ref name=":16" /> <ref name=":17" /> and is also recommended for patients where the primary problem is at the vertebral column.
| |
| | |
| <u>Expansion thoracostomy and VEPT</u><br>For severe congenital spine deformations, when a large amount of growth remains, expansion thoracostomy and VEPTR (a curved metal rod designed for many uses), are the most appropriate choice. These methods are used when the primary problems involve the thoracic cage, for example when there are rib fusions and/or with developing Thoracic Insufficient Syndrome, <ref name=":17" /> but the incidence of complications using VEPTR is, however, relatively high.<ref name=":18">Moramarco M and Weiss HR. (2015). Congenital Scoliosis, Curr Pediatr 2015 Nov 17</ref>
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| | |
| <u>Resection and fusion</u><br>For treating congenital scoliosis caused by hemivertebra posterior hemivertebra, resection and monosegmental fusion appears to be effective. This treatment results in an excellent correction in both the frontal and sagittal planes. <ref>Zhu X. et al. 2014, “Posterior hemivertebra resection and monosegmental fusion in the treatment of congenital scoliosis. “, Article from Annals of The Royal College of Surgeons of England, Vol 96, Nr5, pp. 41-44</ref> Early surgery is typically prescribed as a treatment for children with congenital scoliosis, even though there is little evidence for its long term results. Evidence base is also lacking to confirm the hypothesis that spinal fusion surgery for children with congenital scoliosis is effective Additionally, there are conflicting data about the safety of hemivertebra resection and segmental fusion which uses pedicle screw fixation. <ref name=":18" />
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| | |
| == Physical Therapy Management ==
| |
| | |
| Physical therapy helps patients to continue with daily activities. There are various interventions for congenital spine deformities, such as bracing and postural training and surgical, as described under medical management.
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| For '''''Scoliosis''''':<br>Various approaches for conservative management can be considered, although opinions are divided for the its early treatment. Braces cannot correct a spinal curve, but they can be used for preserving the spine's shape and delaying early surgery. <ref>Götze HG. (1978). Prognosis and therapy of the congenital scoliosis, Z Orthop Ihre Grenzgeb., 116 (2), pp258-266</ref> <ref name=":7" /> It is important, therefore, that conservative management is considered before surgery. External support with casts or braces is considered to be successful in only a small percentage of cases and that surgery is the definitive treatment <ref>Leatherman K. et al. Two-stage corrective surgery for congenital deformities of the spine. journal of bone and joint surgery, pp 324-328</ref>, although this is refuted by ''Fender et al.'' who claim that surgical intervention during infancy is the aim of the treatment before compensatory curves can develop. <ref>Fender, D. et al. 2014 “Spinal disorders in childhood II: spinal deformity“, Surgery (Oxford), Vol. 32, Nr1, pp 39–45, </ref>
| |
| | |
| Few cases have been reported on the influence of conservative treatment. ''Kaspiris et al'' claim that segmentation failure should be treated with early surgery before growth in puberty. For scoliosis due to failure of formation, further investigation is needed to determine whether a conservative approach would be necessary.<ref>Kaspiris A., Theodoros B. Grivas, Weiss H-R. and Turnbull D.2011, “Surgical and conservative treatment of patients with congenital scoliosis: a search for long-term results“, Scoliosis, Vol. 6, nr. 12, pp. 1-17, . </ref> More research is needed to prove the effectiveness of conservative treatment in congenital scoliosis.
| |
| | |
| Numerous brace designs have been developed for the spine. The type of brace chosen depends on different factors such as: location of the curve, flexibility of the curve, number of curves and position and rotation of some of the vertebrae. Braces have to be worn until the patient stops growing, then surgery can take place. <ref name=":19">Creswell E.J.1969, “The conservtaive managment of scoliosis in children and adolescents, and the use of the milwaukee brace “, Australian Journal of Physiotherapy, Vol. 15, Nr.4, pp 149–152, </ref>
| |
| | |
| [[Milwaukee_brace|Milwaukee brace]]: used for scoliosis. The brace is initially worn for a limited number of hours until the patient is can comfortably wear it all day and night to eventually lead a normal life. The brace may only be removed once a day. ''Creswell'' suggested 2 exercises that the patient should do while wearing this brace:
| |
| # Standing, displace the trunk away from the primary lateral pressure pad.
| |
| # Standing, breathe in and expand chest posteriorly on side opposite to primary pressure pad. <ref name=":19" />
| |
| When the brace can be discarded, the patient should gradually reduce its use, so long as their posture remains unchanged. During periods where the brace is not worn, it is recommended that the patient is as active as possible. <ref name=":19" />
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| | |
| Until surgery takes place exercise has to be done twice daily to maintain and improve mobility in multiple directions, especially for spinal extension and strength of the trunk muscles. There are different sorts of exercises for every age. Creswell suggests the Klapps' protocol: <ref name=":19" />
| |
| * Teach correct pelvic tilting in supine lying, prone lying, standing, and kneestanding.
| |
| * Teach the patient to correct their posture infront of a mirror, so that the shoulders are directly above the pelvis.
| |
| *If the primary curve is deteriorating or measures 25° or more, patients aged 2 1/2 years upwards are fitted with a Milwaukee Brace. Once this is fitted, it is worn day and night, and is removed only once daily for bathing and exercises.
| |
| *If at any time the curve continues to deteriorate rapidly even with a Milwaukee Brace, the patient may have a corrective localised plaster jacket applied for a period of 3 to 4 months. This is put on with the patient lying on a frame, with traction. Jacket use usually considerably improves the scoliosis. When the jacket is removed, Milwaukee Brace use is resumed immediately, and exercises are again done regularly.
| |
| *The patient stays in the Milwaukee Brace for a number of years, until surgery is undertaken, or until all the following criteria are satisfied:
| |
| # They are no longer growing
| |
| # They are able to easily maintain the same posture out of the brace as in it
| |
| # X-rays of the pelvis show that the iliac apophyses have closed posteriorly. <ref name=":19" />
| |
| Post surgery rehabilitation is essential. Children who undergo anterior and posterior hemivertebral excision have to wear a plaster for 3 months to maintain the spinal correction. After 3 months of immobilisation, retraining of the postural muscles is crucial to maintain the stability of the spine. <ref>Klemme WR et al. (2001). Hemivertebral excision for congenital scoliosis in very young children. J Pediatr Orthop. 21 (6), pp761-764</ref>
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| <br>For '''''Neural Tube Defects:'''''<br>As with spina bifida, therapy depends on the type of condition. Spina bifida occulta does not require any treatment. It can be discovered at a later age and then surgery can be done. In spina bifida aperta, surgery is done immediately after birth. [40 [LOE:5]]Afterwards, physiotherapy is needed to help the patient gain strength and mobility to allow the child to walk when he’s older. Also wearing a brace can help to improve control and function. [41 [LOE:5]]<br>According to Strohkendl et al, children with spina bifida also have shortage of environmental experience. This can not only be helped by individual physical therapy. Specifically selected and organised physical education makes it possible to acquire the necessary mobility and social experiences. The physical education consist of giving the children exercise suitable for their age. In choosing the exercises it is first of all necessary to go back to the so-called fundamental activities like climbing, hanging by one's hands, sliding, pushing oneself up off the ground, swinging or throwing and catching, before going on to wheel-chair sports. Wheel-chair sport promises a varied selection for group exercises (games) and for everyday use. Using the wheel-chair as sports equipment, it is possible for persons with other types of locomotive handicaps to be integrated into the group. The most important teaching criteria are in this case: the creation of a happy atmosphere, a high degree of clarity, the fulfilment of individual inclinations, the encouragement of independence, the development of community life and the fulfilment of everyday tasks. [68 [LOE:3A]]<br>Teaching how to swim to children with spina bifida you use the same didactic procedures as in normal children but you must be aware that spina bifida children have a greater initial fear and in the existence of contractions, a scoliosis, hyperlordosis or -kyphosis due to the resultant instability of the water. Because of this, specifically oriented swim- and work-aids must be used. [68 [LOE:3A]]
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| <br>For '''''Kyphosis''''': <br>If no treatment is applied in kyphosis the curve will continue to progress with 7 degrees each year. During the adolescent growth spurt, it will reach its maximum. [64 [LOE:2B]]<br>When an operative treatment is too expensive or denied, patients with myelomeningocele and congenital kyphosis can be managed by modified wheelchairs and orthoses. Patients are able to function with reasonable comfort with these wheelchair modifications.[43 [LOE:2A]] According to Winter et al., brace treatment was ineffective for adolescents during their growth spurt. [64 [LOE:2B]]<br>
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| == Key Research ==
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| add links and reviews of high quality evidence here (case studies should be added on new pages using the [[Template:Case Study|case study template]])<br>
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| == Resources <br> ==
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| add appropriate resources here <br>
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| == Clinical Bottom Line ==
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| Congenital spine deformities are spinal deformities identified in the uterus or at birth which are the result of anomalous vertebral development in the embryo. We can classify the deformities into three groups: 1) due to neural tube deformities/defects, 2) due to failure of segmentation, and 3) due to failure of formation. A lot of factors such as environmental factors, genetic factors, vitamin deficiency, chemicals, and drugs, singly or in combination, have been implicated in the development of congenital abnormalities during the embryonic period. There are various techniques to diagnose a deformity, such as ultrasound, x-rays, MRI and CT scans. In children, it is important to start with the less invasive procedure because of their cartilage and non-ossified bones. So imaging in children is done with ultrasound. To complete the diagnose it is important to inspect, palpate, test the range of motion of the spine of the patient, and to do a neurological evaluation. If the patient has an open deformity due to neural tube defect, surgery is done directly after birth. If the patient has a closed deformity due to neural tube defect, surgery can be delayed. There is some controversy about the treatment of congenital spine deformities due to a failure of segmentation/formation. Some recommend surgery during infancy, whereas others would first use conservative management techniques, such as braces and exercise. If this does not work, they would then also suggest surgery. <br>Congenital scoliosis presents a major challenge to the physician as a result of the possibility of a wide variety of primary and secondary abnormalities. These abnormalities develop during fetal life, and thus treatment of these patients often necessitates numerous tests and thorough repetitive examination by the physician.” was stated in an article in the spine journal, 2005. [31 [LOE:1A]]<div class="researchbox">
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| </div>
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| == References == | | == References == |
| | | [[Category:Vrije Universiteit Brussel Project|Vrije_Universiteit_Brussel_Project]] |
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| | | [[Category:Conditions]] |
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| | [[Category:Paediatrics]] |
| | [[Category:Paediatrics - Conditions]] |
| | [[Category:Lumbar Spine]] |
| | [[Category:Lumbar Spine - Conditions]] |
| | [[Category:Thoracic Spine]] |
| | [[Category:Thoracic Spine - Conditions]] |
| | [[Category:Congenital Conditions]] |
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