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|Kyphoscoliosis in a 10-year-old girl with HSANIII.|
Kyphoscoliosis describes an abnormal curvature of the spine in both a coronal and sagittal plane. It is a combination of kyphosis and scoliosis. This musculoskeletal disorder often leads to other issues in patients, such as under-ventilation of lungs, pulmonary hypertension, difficulty in performing day-to-day activities, psychological issues emanating from anxiety about acceptance among peers, especially in young patients. It can also be seen in syringomyelia, Friedreich's ataxia, spina bifida, Kyphoscoliotic Ehlers-Danlos Syndrome (kEDS), and Duchenne muscular dystrophy due to asymmetric weakening of the paraspinal muscles.
Signs and symptoms
The following are clear signs of Kyphoscoliosis:
- Abnormal hunch along with a presence of S or C-like shape.
- Presence of associated disorders likes hypertension, neurological disorders
- Abnormal gait
Kyphosis by itself refers to an excessive convex curvature of spine occurring in the thoracic and sacral regions. A normal thoracic curvature from the 1st to the 12th spine has a naturally occurring convex shape with angles ranging from 20 degrees to 45 degrees. When the curvature goes past 45 degrees, the condition is termed as Kyphosis.
Kyphosis, depending on the extent of curvature can result in many discomforts including breathing and digestion difficulties, cardiovascular issues, and even neurological deformities. The greater the extent of deformity, the more pressing the need becomes for a surgery.
Kyphosis by itself is of several types, including postural kyphosis caused by slouching. Such forms of kyphosis can be reversed to a certain extent by correcting muscular imbalances. The more debilitating version of kyphosis is Scheuermann’s kyphosis, found mostly in teenagers. There are few other causes of Kyphosis as well, such as congenital, nutritional or osteoporosis induced and post-traumatic.
Depending on the type of Kyphosis, the extent of curvature, the age of the patient, various treatment modalities can be suggested including the provision for posture correction, braces, physiotherapy for correcting the muscular imbalance, and finally the surgery. Surgery can be most useful in patients who have an extensive deformity. The rate of post-surgery complications may be higher in elderly patients.
Scoliosis refers to yet another form of abnormal curvature in which the person’s spine takes an “S” or “C” shape. Scoliosis too has similar forms of treatments available as Kyphosis including bracing, physical therapy and various types of surgeries. Typically, a human spine is straight but in Scoliosis patients; there may be a curve of ten degrees in either direction, left or right.
Kyphoscoliosis may manifest in an individual at different stages of life and for various causes. When present at a young age ranging from childhood to teenage, Kyphoscoliosis may be present from birth due to congenital abnormalities including Spina bifida.
Certain infections can also lead to the development of Kyphoscoliosis such as vertebral tuberculosis or general tuberculosis. Osteochondrodysplasia, a disorder related to the development of bone and cartilage, can also cause this disease.
In later ages, Kyphoscoliosis can occur in patients suffering from chronic degenerative diseases like osteoporosis and Osteoarthritis. This type of incidence is usually seen in patients above 50+ years of age and is mainly attributed to structural changes in the spine and adjoining tissues. Sometimes, a traumatic injury can also lead to its development.
Further, there are many idiopathic occurrences of Kyphoscoliosis where the exact cause is not very well known but is suspected to be caused by genetic factors.
Kyphoscoliosis is one of the main criteria in Kyphoscoliotic Ehlers-Danlos Syndrome. It is caused by mutation in the PLOD1 gene and/or FKBP14 gene. The diagnosis is confirmed by molecular testing and suggested when a patient meets criteria 1 and criteria 2: Congenital muscle hypotonia and congenital or early onset kyphoscoliosis (progressive or non-progressive), respectively.
- Physical Therapy - When the extent of curvature is mild, and the underlying cause is bad posture, and then physical therapy becomes the first line of treatment. Physical therapy may also be advised for other causes as well to delay the development of abnormal curvatures, such as in the case of elderly patients suffering from the degenerative disease of bones and cartilage. Physical Therapy’s primary objective is to strengthen the spinal tissues for either correcting the curvature to the maximum extent possible or contain further damage.
- Back braces- In the cases when the underlying reason for deformity is postural and is detected before the growing age of bones, braces can be used. Braces provide support to the muscles and bones, as well as apply corrective pressure to reduce the curvature. The braces for Kyphoscoliosis have to be designed such that it becomes effective with both abnormal curvatures in the coronal and sagittal plan. The latest in brace design is through the use of CAD/CAM, which is available only in few developed countries such as Germany. The efficacy of braces for correcting curvature issues has been analyzed by a few studies. In one such study, it was found that bracing led to successful outcomes in 72% of patients and correlated strongly with hours of braces worn. [1, 2] However, compliance with wearing braces for a period up to 18 hours or more can be physiologically and psychologically limiting, especially when one considers the teenage period.
- Surgery - Surgery is usually indicated when the extent of deformity is large, causes issues in physiological functions such as breathing, interferes with the daily activities or cosmetically unacceptable to the adult. It is also advised when the patient has passed the age where other therapies such as braces are no longer effective. Kyphoscoliosis implies that the patient has both types of curvature deformities. The decision to choose a particular treatment approach very much depends on multiple factors such as the age of the patient, understanding the root cause of the condition i.e. postural, congenital, etc., risks involved in surgery for the patient, etc.
- Decision for Spine Surgery - The decision to undergo surgery or not is always a complex one, but sometimes, it becomes inevitable such as when the deformity begins to interface with crucial physiological functions like breathing or makes every day normal activities extremely painful. Sometimes, patients in their early adulthood may choose to have surgery because the presence of such deformity causes social issues such as acceptability among their peers, a disability that comes in the way of working, etc. In the case of elderly patients who suffer from such deformity triggered at a later age, other factors are to be considered such as the presence of underlying disease that caused it, whether a progressive decline is expected, and if conservative treatments using physiotherapy or drugs have failed to give relief from debilitating pain.
- Surgical correction is attempted in some to fix the spine and arrest progression of the deformity. Surgical operations may include the use of spinal implants like the Harrington Rod, or the VEPTR.
- Oxygen on long term may be necessary in patients with significant hypoxemia
Risks of corrective spine surgery
A study measured outcome from surgery of 49 cases of scoliosis and kyphoscoliosis. Of this sample, 36 patients were monitored for a period of 8 years.
- 23% - excellent condition
- 29% - good condition
- 34% - satisfactory
- 14% - bad
Bad refers to cases where the surgery failed to address the disease and the patient either had to undergo a revision surgery or continues to suffer from a poor quality of life as before surgery.
Typically post-surgery complications range up to 5% involving all major and minor complications when measured within one year of surgery. However, there may be a progressive decline in patient’s condition after a few years.
In another study that evaluated surgical treatment of kyphoscoliosis and scoliosis due to congenital reasons, 91% of surgeries were found to be successful and met their intended objectives for the two-year follow-up period after surgery. The sample consisted of 23 patients of whom 17 were male and 6 were female, with an average age of 27 years, ranging from 13 to 61 years. The most popular type of surgeries for spinal correction includes pedicle subtraction osteotomy (PSO) and posterior vertebral column resection (pVCR).
Another study which focused on elderly patients found that the rate of complications was much higher for a sample population of 72 cases with mean age of 60.7 years. The rate of complications was as high as 22% in the entire sample. The study points that in the case of elderly patients, surgery should only be considered when there is no other option left; the disease is in progression stage, and the quality of life has degraded to an extent where conservative treatments can no longer help with pain.
While there are many surgical approaches for spinal deformity correction including anterior only, posterior only, anterior-posterior, the techniques that are most popular nowadays include the posterior only VCR or pVCR. One of the studies which analyze pVCR technique also noted the benefit of using a technique called NMEP monitoring in assisting the surgeon avoid any neurological complications while performing a spine surgery.
In conclusion, the decision to undergo a corrective spine surgery is a complex one but sometimes becomes necessary when the quality of life has degraded to such an extent that potential benefits outweigh the risks. No surgery is devoid of risks but by carefully assessing factors such as the skills and experience of the surgical team, previous record or history of outcomes, and the techniques that are used for spine surgery, a patient along with his or her doctor can certainly help in achieving a successful outcome.
As studies are repeatedly pointing out, the success rates for spinal surgeries have improved so much so that the risks rates can now be comparable to other types of surgeries. These success rates also tend to be higher at a younger age when compared to the elderly age.
- "EDS Types". The Ehlers-Danlos Society. The Ehlers-Danlos Society. Retrieved 22 May 2018.
- e: Brady AF, Demirdas S, Fournel-Gigleux S, Ghali N, Giunta C, Kapferer-Seebacher I, Kosho T, Mendoza-Londono R, Pope MF, Rohrbach M, Van Damme T, Vandersteen A, van Mourik C, Voermans N, Zschocke J, Malfait F. 2017. The Ehlers–Danlos syndromes, rare types. Am J Med Genet Part C Semin Med Genet 175C:70–115.
- MedEd at Loyola medicine/pulmonar/diseases/pul22.htm
- Bracing adolescents with idiopathic scoliosis; Journal of Paediatrics and Child Health, Volume 50 (5) – May 1, 2014
- Effects of bracing in adolescents with idiopathic scoliosis by Weinstein S.L., Dolan L.A., Wright J.G. & Dobbs M.B. (2013) The New England Journal of Medicine, 369, 1512 – 1521.
- Surgical treatment of adult degenerative scoliosis; Matteo, Palmisano; Eugen, Dema; Stefano, Cervellati; European Spine Journal, Volume 22 (6) – Nov 1, 2013
- The posterior surgical correction of congenital kyphosis and kyphoscoliosis: 23 cases with minimum 2 years follow-up; Yan, Zeng; Chongqing, Chen; Qiang, Qi; Zhaoqing, Guo; Weishi, Li; European Spine Journal, Volume 22 (2) – Feb 1, 2013
- Complication rate in adult deformity surgical treatment: safety of the posterior osteotomies; Giovanni, Maida; Francesco, Lucero; Francesco, Gallozzi; Marcello, Ferraro; Maggi, Bernardo; European Spine Journal, Volume 24 (7) – Nov 1, 2015
- Vertebral Column Resection for the Treatment of Severe Spinal Deformity; Lawrence, Lenke; Brenda, Sides; Linda, Koester; Marsha, Hensley; Kathy, Blanke; Clinical Orthopaedics and Related Research, Volume 468 (3) – Mar 1, 2010
- Péréon Y, Bernard JM, Fayet G, Delécrin J, Passuti N, Guihéneuc P (1998). "Usefulness of neurogenic motor evoked potentials for spinal cord monitoring: findings in 112 consecutive patients undergoing surgery for spinal deformity". Electroencephalogr Clin Neurophysiol. 108: 17–23. doi:10.1016/s0168-5597(97)00056-7. PMID 9474058.
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