Scoliosis is the lateral curvature and three dimensional rotation of the spinal column.
Presentation
Scoliosis has characteristic physical findings. It presents as an abnormal shape and symmetry of the back, sometimes with either a very prominent rib or a bulging shoulder blade. Sometimes there may be a hump at the back. On examination, posture appears to be distorted and limb length may appear unequal. Ramirez and his coworkers from the Texas Scottish Rite Hospital studied more than 2400 patients with scoliosis and found that a full 23% (560 of 2442 patients) had back pain at the time of presentation [7]. Some patients may also suffer from lung hypoplasia and compressed respiratory tree or other thoraco-abdominal organs.
Entire Body System
- Cerebral Palsy
A reassessment of spinal stabilization in severe cerebral palsy. Journal of Pediatric Orthopedics 1994; 14( 6): 731-9. Comstock CP, Leach J, Wenger DR. Scoliosis in total-body-involvement cerebral palsy. [web.archive.org]
However, it can also be present from birth or arise due to related health conditions that affect the individual, such as cerebral palsy and muscular dystrophy. [news-medical.net]
Neuromuscular – In which the scoliosis is symptomatic of a systemic condition, such as cerebral palsy, muscular dystrophy, or paralysis. [hss.edu]
Scoliosis is commonly found in children with cerebral palsy. Many patients with cerebral palsy and scoliosis undergo intrathecal baclofen (ITB) pump placement. [ncbi.nlm.nih.gov]
- Trisomy 21
The fetal karotype was found to be 47, XX,+21 (trisomy 21). In the literature, three scoliosis cases have been reported accompanying the CP along with multiple anomalies and one concomitant pes equinovarus deformity has been reported previously. [ncbi.nlm.nih.gov]
21 Diagnosis A doctor will carry out a physical examination of the spine, ribs, hips, and shoulders. [medicalnewstoday.com]
Respiratoric
- Abnormal Breathing
Respiratory deficiencies may also arise from thoracic deformities and cause abnormal breathing.[64] This directly affects exercise and work capacity, decreasing the overall quality of life.[4] In the health care system of the United States, the average [en.wikipedia.org]
Gastrointestinal
- Abdominal Pain
After an unremarkable immediate postoperative course, on postoperative day 19 the patient presented to the emergency department with abdominal pain, nausea, and vomiting. Unrelenting brown vomitus, abdominal pain, and a 20-lb weight loss were noted. [ncbi.nlm.nih.gov]
For example, a case of appendicitis is first recognized by someone as abdominal pain. In people with Prader-Willi syndrome, they do not recognize the abdominal pain until the bowel inflammation is very advanced. [scoliosisassociates.com]
At that time she had complained of repeated attacks of upper abdominal pain and intolerance to fatty foods. [pubs.rsna.org]
Cardiovascular
- Hypertension
However, notable risk factors including hypertension, strenuous exercise, connective tissue disorders, atherosclerosis, extracorporeal shock wave lithotripsy, and cocaine abuse have been reported. [ncbi.nlm.nih.gov]
Long term, this can lead to breathing and blood pressure problems (pulmonary hypertension) in adulthood. A scoliosis developing over the age of 5 does not significantly impact on heart and lung development. Life expectancy is generally normal. [scoliosis-australia.org]
- Systolic Murmur
The apex beat was palpated on the right side, and a pan-systolic murmur was heard best at the apex. ECG showed depressed ST segment and T waves in leads V1-V6 (Fig. 1A). Chest radiography showed dextrocardia and cardiomegaly (Fig. 1B). [academic.oup.com]
Skin
- Skin Disease
The mission of the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), a part of the U.S. [web.archive.org]
NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases [medlineplus.gov]
National Institute of Arthritis and Musculoskeletal and Skin Diseases. https://www.niams.nih.gov/health-topics/scoliosis. Accessed May 3, 2019. Introduction to scoliosis. [mayoclinic.org]
Photo Credit Gracia Lam Personal Health A Little-Known Skin Disease That Can Disrupt People’s Sex Lives Patients deal with pain and itching and often encounter medical ignorance and mistreatment until affected tissues become irreparably scarred. [nytimes.com]
Bracing According to the National Institute of Arthritis and Musculoskeletal and Skin Diseases, a person with scoliosis may need to use a brace if: They’re still growing and the curvature is more than 25 to 30 degrees. [healthline.com]
Musculoskeletal
- Back Pain
Kyphosis between 50 and 75 degrees, where there is significant growth remaining or persistent back pain, may be managed by a brace. [back.com]
Of 25 patients without back pain in 1968, 11 still are without pain, 11 have occasional pain, 2 have frequent pain, and 1 has daily pain. Both acute back pain scores and current radiographic data were available for 60 patients. [doi.org]
A 54-year-old woman just complained about sever back pain without any radiculopathy and neurodeficit of low limb for 2 years, Visual Analogue Scale (VAS) for back pain was 9 points and x-ray showed adult LDS with lordosis angle of 10° from 5th thoracic [ncbi.nlm.nih.gov]
- Osteoporosis
Kyphosis is most commonly seen in older women, as a result of osteoporosis. What Causes Kyphosis? Kyphosis occurs when the vertebrae in the upper back become wedge-shaped. [back.com]
In older patients, particularly women, it is also often related to osteoporosis. Osteoporosis weakens the bone, making it more likely to deteriorate. The combination of these changes causes the spine to lose its ability to maintain a normal shape. [depuysynthes.com]
If it is thought that osteoporosis of your spine is contributing to your symptoms, you may be given medication and supplements to help strengthen your bones. Find out more about treating osteoporosis. [web.archive.org]
If osteoporosis is present, then treatment of the osteoporosis may slow the progression of the scoliosis as well. This can be accomplished in several ways. [umms.org]
With osteoporosis, the spine can usually be left alone, as long as there’s no pain or problems with the nervous system. But the osteoporosis itself will usually need to be treated in order to prevent future fractures. [pennmedicine.org]
- Fracture
Following a T3-L4 correction with apical posterior column releases, an L4 Chance fracture with loss of distal fixation occurred on post-op day 3. [ncbi.nlm.nih.gov]
When kyphosis is caused by a vertebral compression fracture due to osteoporosis or cancer, a minimally invasive procedure called kyphoplasty is a common treatment. [back.com]
Fractures that typically do better with operative treatment include open or compound fractures, displaced growth plate fractures, and fractures that involve the surface of a joint. [my.clevelandclinic.org]
Osteoporosis with fractures — The bones are prone to fracture in people with osteoporosis and the spine is a favored location for fracture. [drugs.com]
Bone density: Because good bone density reduces the risk of fractures in aging adults, you may be asked to undergo a bone-density scan to determine the strength of your bones. [mayfieldclinic.com]
- Muscle Weakness
Scoliosis decreases the chest wall compliance directly and the lung compliance indirectly (due to progressive atelectasis and air-trapping), causing a significant increase in the work of breathing that, because of the associated respiratory muscle weakness [ncbi.nlm.nih.gov]
All these changes in our body are possible because of muscles weakness and imbalance. Weak, overloaded, stretched or shortened muscles lose their supportive function. [bodycareclinic.ie]
Common causes that doctors may identify include: cerebral palsy, a group of nervous system disorders that affect movement, learning, hearing, seeing, and thinking muscular dystrophy, a group of genetic disorders that result in muscle weakness birth defects [healthline.com]
- Leg Length Inequality
length inequality as cause of scoliosis other important findings on physical exam leg length inequality midline skin defects (hairy patches, dimples, nevi) signs of spinal dysraphism shoulder height differences truncal shift rib rotational deformity [orthobullets.com]
This may cause crowding of the ribs on one side. scoliosis S-shaped spinal curvature in frontal plane, causing muscle/soft-tissue compensation, e.g. flexible (soft-tissue contracture) or fixed (bony ankylosis) deformity; causes apparent leg length inequality [medical-dictionary.thefreedictionary.com]
Psychiatrical
- Distractibility
Growth-friendly implants can be classified into 3 distinct subtypes: distraction-based, guided growth, and compression-based strategies. 22 Distraction-Based Implants Distraction-based implants are the most common devices used in EOS. [doi.org]
For treatment of scoliosis secondary to extensive chest resection in the growing children with Askin tumors, distraction-based growth-friendly treatment is an available surgical option. [ncbi.nlm.nih.gov]
Neurologic
- Headache
In patients with Chiari malformation, headache, dysaesthesia and paraparesis disappeared. [ncbi.nlm.nih.gov]
Scoliosis can result in: headaches shortness of breath back pain digestive problems menstrual-cycle disturbances fertility issues leg, hip and knee pain chronic fatigue future spinal kyphosis (widow’s hump) Scoliosis Treatments “Watch and wait” for scoliosis [spinecorrectioncenter.com]
In patients with Chiari malformation, headache, dysaesthesia and paraparesis disappeared. Conclusions. [dx.doi.org]
Conditions arising as a result of scoliosis include rib deformity, shortness of breath, digestive problems, chronic fatigue, acute or dull back pain, leg, hip, and knee pain, acute headaches, mood swings, and menstrual disturbances. [scoliosissystems.com]
- Quadriplegia
The patients who had the largest curves at the time of skeletal maturity had spastic quadriplegia and either a thoracolumbar or a lumbar curve, and they were bedridden. [ncbi.nlm.nih.gov]
The patients who had the largest curves at the time of skeletal maturity had spastic quadriplegia and either a thoracolumbar or a lumbar curve, and they were bedridden. Related Topics Loading Related Articles [doi.org]
PARALYTIC This is the term applied to the curvature which frequently develops when there is loss of spinal cord function early in life from disease or disorder, particularly injury (quadriplegia and paraplegia). [scoliosis-australia.org]
- Paresis
Flexible nasendoscopy confirmed left vocal cord paresis. Stretch injury to the recurrent laryngeal nerve from the left T5 pedicle screw or intubation may have caused the vocal cord paresis. The pedicle screw was removed during revision surgery. [ncbi.nlm.nih.gov]
PubMed Google Scholar Kluba T, Giehl JP: A surprising case of paresis following scoliosis correction. Eur Spine J. 2001, 26 (6): 1495-1499. [dx.doi.org]
Workup
Laboratory tests
A scoliometer may be used to diagnose the condition. Genetic testing may also be conducted, if needed. Tests may also include hematocrit and haemoglobin levels. Serum alkaline phosphatase levels and vitamin D levels may also be assessed.
Imaging
- Full spine AP/Coronal CT scan
- Lateral/saggital plane X-rays
- MRI
Test results
On the basis of physical examination and imaging studies, scoliosis can be diagnosed and the extent of rotation can be assessed.
Treatment
Conservative management
It mainly constitutes the use of braces, casts and analgesics. In a prospective, multicenter study from the Scoliosis Research Society, Nachemson and his coworkers found brace treatment (an underarm plastic brace worn for at least 16 h/d) to be successful 74% of the time (95% confidence interval [CI], 52-84%) [8]. The results of a study conducted for infantile scoliosis patients suggest that body casting is useful in cases of smaller, flexible spinal curves, and vertical expandable prosthetic titanium rib
(VEPTR) is a viable alternative for larger curves [9].
Surgery
Spinal fusion is the most commonly used surgical treatment. For a thoracic curve with adequate flexibility, posterior spinal fusion with instrumentation is used. If the curve is large and stiff, an anterior release including diskectomy and bone grafting is performed first. Large bone screws, pedicle screws and hooks are used to surgically derotate the spine. Endoscopic spinal instrumentation techniques have also been introduced and continue to evolve [10].
Prognosis
Scoliosis begins insidiously and persists for life unless surgically corrected. It is a chronic progressive disease and may prove to be fatal. Clinical outcomes following treatment of idiopathic scoliosis are strongly linked to curve magnitude [6]. Mortality rate is associated with ‘curve effects’, meaning the distorted posture and curved spine may lead to damage of respiratory and cardiovascular organs. With appropriate treatment, mortality rate can be significantly reduced and quality of life can be substantially improved.
Etiology
Idiopathic scoliosis is the most common type of spinal deformity confronting orthopedic surgeons [2]. It has multifactorial causes, including genetics among which CHD7 gene is known to be involved. Congenital scoliosis is due to spinal malformation of the fetus between the 3rd and 6th week of development. Several neuromuscular diseases like poliomyelitis, cerebral palsy, spinomuscular atrophy, Ehlers Danlos syndrome, Marfan syndrome or neurofibromatosis may be the primary cause or associating disease in secondary or neuromuscular scoliosis.
Epidemiology
Incidence
Infantile scoliosis is a very rare condition with only 1% cases in North America. Idiopathic scoliosis on the other hand is a relatively common condition affecting 2-3% of the United States population, which is equivalent to about 5 to 9 million cases [3].
Age
The prevalence of scoliosis was highest (1.2%) in patients aged 12-14 years [4].
Sex
In early-onset infantile scoliosis, 60% males are affected. In late-onset infantile scoliosis, 90% females are affected. In idiopathic scoliosis, females are at a greater risk.
Pathophysiology
The pathophysiology of scoliosis is still unclear. The spinal curvature that characterizes scoliosis may be due to malformation occurring during intrauterine development, resulting in infantile scoliosis. This type includes scoliosis appearing either at birth or within the first 3 years of life. Infantile scoliosis, along with genetic factors, is also associated with nursing posture and the side of deviation of the curve.
Neuromuscular scoliosis may be a result of other preexisting neuromuscular diseases such as Ehlers-Danlos syndrome, Marfan syndrome, neurofibromatosis, etc. The location is defined by the vertebra that is most deviated and rotated from midline, called the apical vertebra [5].
Whatever the type of scoliosis it may be, it is known that there are segments of the spinal column that are misaligned and these fusion defects result in the development of curvature of the spine to one side, which then progresses to three dimensional rotation and the resultant ‘S’ shaped spinal column.
Prevention
Scoliosis may be prevented by maintaining correct posture and engaging in daily exercise and a healthy diet. Congenital scoliosis, however, has no known prevention but its progress can be halted or at least somewhat managed with appropriate treatment. Neuromuscular scoliosis can be prevented from occurring by adequately managing the underlying cause or associating disease with medication and physiotherapy.
Summary
Scoliosis is defined as a lateral curvature of the spine, most often than not accompanied by rotation. By convention, 10 degree of curvature (as measured by the Cobb angle) defines a scoliosis [1]. Spinal curvatures that are of less than 10 degree are simply referred to as spinal asymmetry. On imaging, the spinal deformity appears to be of an ‘S’ shape. Scoliosis may be broadly classified into 3 types: Congenital, idiopathic and neuromuscular. All three are described below.
Patient Information
Definition
Scoliosis is defined as lateral or sideways curving and rotation of the spine. The curvature needs to be equal to or more than 10 degrees for the deformity to be classified as scoliosis. It may be present since birth, called infantile scoliosis or it may develop later on in life. The late-onset scoliosis is often a result of predisposing conditions.
Cause
The most common type of scoliosis is idiopathic, meaning it has no known cause. Neuromuscular scoliosis may be due to other diseases affecting the body’s structure. Infantile scoliosis is due to genetic factors and in some rare cases, it may be due to trauma during pregnancy.
Signs and symptoms
Scoliosis presents as abnormal shape and symmetry of the spine which may be accompanied with a hump and distorted posture. There may be protrusion of a rib or shoulder blade and unequal length of arms. Patients experience back pain which is exacerbated with movement and their physical activity becomes somewhat restricted.
Treatment
Scoliosis can be conservatively managed with the help of casts and braces and painkillers. In severe cases, the curved spine may have to be corrected with the help of surgery which includes insertion of screws and rods to straighten the spine. Surgery is followed with physiotherapy to help relax and strengthen the muscles.
References
- Kane WJ. Scoliosis prevalence: a call for a statement of terms. Clin Orthop Relat Res. 1977;126:43-46.
- Lonstein JE. Idiopathic scoliosis. In: Lonstein JE, Bradfordn DS, Winter RB, Ogilvie J, eds. Moe's Textbook of Scoliosis and Other Spinal Deformities. 3rd ed. Philadelphia, PA: WB Saunders Co; 1995:219-256.
- Negrini S, Aulisa AG, Aulisa L, et al. 2011 SOSORT guidelines: Orthopaedic and Rehabilitation treatment of idiopathic scoliosis during growth. Scoliosis. 2012;7(3):1-35.
- Stirling AJ, Howel D, Millner PA, et al. Late-onset idiopathic scoliosis in children six to fourteen years old. A cross-sectional prevalence study. J Bone Joint Surg Am. 1996;78(9):1330-1336.
- Tachdijan MO. The Spine. In: Clinical Pediatric Orthopedics: The Art of Diagnosis and Principles of Management. Stanford, CA: Appleton & Lange; 1997:p325.
- Tsutsui S, Pawelek J, Bastrom T, et al. Dissecting the effects of spinal fusion and deformity magnitude on quality of life in patients with adolescent idiopathic scoliosis. Spine. 2009;34(18):E653-E658.
- Ramirez N, Johnston CE, Browne RH. The prevalence of back pain in children who have idiopathic scoliosis. J Bone Joint Surg Am. 1997;79(3):364-368.
- Nachemson AL, Peterson LE. Effectiveness of treatment with a brace in girls who have adolescent idiopathic scoliosis. A prospective, controlled study based on data from the Brace Study of the Scoliosis Research Society. J Bone Joint Surg Am. 1995;77(6):815-822.
- Smith JR, Samdani AF, Pahys J, et al. The role of bracing, casting, and vertical expandable prosthetic titanium rib for the treatment of infantile idiopathic scoliosis: a single-institution experience with 31 consecutive patients. J Neurosurg Spine. 2009;11(1):3-8.
- Picetti G 3rd, Blackman RG, O'Neal K, Luque E. Anterior endoscopic correction and fusion of scoliosis. Orthopedics. 1998;21(12):1285-1287.