Kyphoscoliosis

What is Kyphoscoliosis?

Kyphoscoliosis is a condition where the normal curve of the spine shifts or twists. Think of it as if your spine is bending sideways and rotating at the same time. There’s a medical way to measure this: if the spine bends to the side more than 10 degrees, it’s termed ‘adult scoliosis’. If it’s less than 10 degrees, it could just be a change in posture. Terms like kyphosis and lordosis describe the curves of the spine when you look at it from the side. The cervical (neck) and lumbar (lower back) spine normally curve a bit to the back, between 35 and 80 degrees. The thoracic spine (mid-back) tends to curve a bit forward, between 30 and 50 degrees, and this curve tends to get larger as we age.

Kyphoscoliosis happens most often in the upper and middle part of your back, but it can also happen where your neck meets the upper part of your back. How serious the condition is, is determined by a measure called the ‘Cobb angle’. Moderate cases may have a Cobb angle between 25 to 100 degrees, and severe cases have a Cobb angle greater than 100 degrees. The effects of these spine changes depend on how severe and where the curve is, how much of the spine is involved, and the degree of rotation. These factors may increase physical effort or even affect lung function.

What Causes Kyphoscoliosis?

Kyphoscoliosis, a condition that causes curvature and hunching of the spine, can have many different causes, and these can vary based on a patient’s age, sex, and other factors. The causes can be grouped into three categories: idiopathic, secondary, or congenital.

Idiopathic causes are when we can’t pinpoint a clear reason for the condition. Even though it’s unclear, these causes represent the vast majority of kyphoscoliosis cases.

Secondary causes are linked to diseases or circumstances that affect the body. These can be age-related changes, long-term inflammation, fractures that have occurred after trauma, changes after surgery, or small repetitive injuries from overuse. As the discs in our spine naturally wear down over time, this can lead to changes in the way the spine moves and cause increasing forward hunching.

Other secondary causes can be due to infections, neuromuscular disorders like cerebral palsy, muscular dystrophy, polio, Friedrich’s ataxia, spinal muscular atrophy, spina bifida, and conditions that affect the body’s connective tissue like Ehlers-Danlos syndrome, chondrodysplasia, Marfan syndrome.

Scheuermann’s disease, which is also known as juvenile kyphosis, is a slowly worsening disease of the spine that most commonly impacts teens. It results when three or more neighbouring spine bones wedge at an angle of more than 5 degrees. In Scheuermann’s disease, hunching is because of a disorder in the growth centre of a bone in the spine. On an X-ray, there might be evidence of narrowing of the edge of a spine bone and Schmorl’s nodes, which are protrusions of the disc into the spine bone.

Congenital causes refer to abnormalities that happen during the development of the baby in the womb, which may be linked to other abnormalities in the brain and urinary system. These defects could include irregular ribs, missing spine bones, or half-formed spine bones.

Functional causes are often temporary and can be due to muscle spasms or inappropriate posture, while structural causes are usually irreversible.

Risk Factors and Frequency for Kyphoscoliosis

Kyphoscoliosis is a disease that results from many different conditions and illnesses. This makes it challenging to know exactly how many people have it. However, we can get a better understanding of how common it is by looking at the main causes. For example, a common cause of kyphoscoliosis in young people is Scheuermann’s disease.

• In the United States, Scheuermann’s disease affects about 0.4% to 8% of the population.
• It is seen more often in boys.
• It generally affects children between the ages of 13 and 16.

Signs and Symptoms of Kyphoscoliosis

Children with a condition called kyphoscoliosis may not initially show symptoms. Instead, they may be brought in for an evaluation by a family member who noticed things like uneven shoulders or hips, a prominent part of the spine or shoulder blade, uneven waistline, or a change in the way the child walks. Chronic pain, weakness, numbness, or loss of control of bowel or bladder are uncommon in children but should they occur, they require thorough investigation.

The child’s health history should include growth trends, when the changes were noticed, any neurological changes, and how this is affecting the child’s day-to-day life. Referrals to a specialist can help calm concerned parents and allow for closer monitoring and treatment if necessary.

Adults with this condition may see a doctor due to back pain, concerns about physical appearance, or worsening respiratory function. Just like in children, a detailed patient history and assessment for changes in neurological or psychosocial health is important.

The physical examination should weigh height, overall body structure and symmetry, spinal alignment, flexibility, and ease of movement. Skin assessment for changes in color, dimples, or unusual hair growth can rule out other related conditions. One test often used is the Adam’s forward bend test. If a curve or irregularity is noted during the examination, an X-ray might be needed to confirm the findings.

A thorough examination should also include checking for muscle strength, reflexes, sensation, movement range in all joints, differences in limb length, and walking pattern analysis. Studies show a connection between worsening kyphoscoliosis and weakened spinal muscles. These patients may also have difficulty standing up from a chair, walking, and completing other functional tests.

Testing for Kyphoscoliosis

Along with taking your detailed medical history and physical examination, a practical test is crucial to understand your condition fully. Research suggests that the “6-minute walk test” can reveal more about the severity of spinal deformities than other conventional tests, such as checking lung functionality or measuring the level of gases in the blood.

Images produced using X-rays can help to identify the degree of spinal bend and are a vital part of the overall assessment. To get the most accurate images of the spine, the medical staff aim to ensure your limbs are even in length (as unevenness can lead to an overestimation of the curve). They will take upright images from both the front (‘PA view’) and the side of the neck, chest, and lower back.

A measure called the “Cobb angle” is then applied to these images to understand the severity of any curvature (‘kyphoscoliosis’), to identify any risk of the condition getting worse, and to determine the best treatment approach. If the spine angle in the thoracolumbar region (the part of the spine including the lower chest and the lower back) exceeds 40 degrees, the condition is referred to as kyphosis.

Measurements are taken on the front view radiograph by drawing a parallel line to the uppermost part of the curve, and a parallel line to the lowermost part of the curve. Then, perpendicular lines are used to calculate the curve angle.

Skilled professionals are able to use an ‘anatomically efficient’ model, which helps them to understand how the spine is positioned. Here, a vertical line, or a ‘plumb line’, is drawn from the seventh bone of the neck down to the upper back corner of the first bone of the sacrum, which is situated between your hip bones. If this line falls in front of the femoral head (the ball-like part of the thigh bone which fits into the hip socket), the spine is considered to be standing in a ‘positive sagittal balance,’ whereas if it falls behind the femoral head, it is in a ‘negative sagittal balance.’

Adolescent idiopathic scoliosis is a condition where the spine has structural deformities. Since 1983, various models have been developed to describe and classify the types of curves in the spine and recommend surgery where needed. As medical techniques have advanced, these classification systems have evolved. The most recent is the ‘Lenke Classification’ established in 2001, which makes use of three main categories to better describe the condition.

Further investigations such as a bone mineral density scan or an MRI may be performed if it will help decide on if and when surgical intervention may be necessary. Research has shown a link between having a larger Cobb angle and a poorer result on lung functionality tests.

Frequently, structural deformities can lead to issues with lung function, which may lead to reduced exercise tolerance and difficulties with breathing. Tests may show uneven chest expansion and reduced lung capacity due to the restrictions imposed by these deformities. Various measures including functional residual capacity (FRC), forced vital capacity (FVC), and FEV1 (the amount of air you can forcefully exhale in one second) can show a reduction in lung functionality. If the heart and lungs aren’t working properly (a condition known as cor pulmonale), it can indicate a higher chance of severe health issues in the future. An echocardiogram (an ultrasound of the heart) can help assess this by measuring pressures in the pulmonary arteries.

Treatment Options for Kyphoscoliosis

The best course of action to treat kyphoscoliosis, or abnormal curvature of the spine, will depend on several factors. These include what’s causing the condition, the age of the patient, how severe the condition is and whether there are any related neurological issues. For many patients, especially younger ones, the condition might not affect their ability to function in any significant way. But, when the condition is causing pain, aesthetic concerns or fears about getting worse, doctors might recommend more tests and specialist care.

If surgery isn’t necessary, doctors might recommend one of several treatments:

• Observation: Sometimes, the best course of action is to monitor the condition over time, especially if it’s not causing major issues. In this case, patients would have regular medical imaging and assessments to check how the curvature and symptoms are progressing.
• Pain management: Pain can often be managed with NSAIDs, a type of over-the-counter pain reliever. For more severe cases, stronger medication might be prescribed, but this must be done carefully due to certain risks. Medications like cyclobenzaprine, a muscle relaxant, can also be prescribed, but do come with side effects like lethargy.
• Respiratory management: Chest abnormalities like decreased lung volume can disrupt proper lung function, leading to issues like chronic respiratory failure. To manage this, doctors might recommend treatments like chest physiotherapy, bronchodilators, and diuretics. Other strategies might involve getting vaccinated against the flu and pneumonia.
• Cardiac management: Because the condition can also put strain on the heart, doctors may use echocardiography — a type of sonogram of the heart — to check for right ventricular overload.
• Bracing: Especially in younger patients with developing skeletons, braces can help prevent the curvature from getting worse. These braces range in structure and rigidity and can provide support to weak muscles and help correct abnormal curvature.
• Physical therapy and exercise: Special exercise programs designed to stretch and strengthen the body can significantly improve the curvature of the spine. These may include things like Pilates or other targeted exercises. Especially for older patients, these programs can be quite effective.
• Spinal cord stimulation: For patients with chronic back pain that hasn’t improved with other treatments, spinal cord stimulation may be an option. This involves interrupting pain signals to the brain using electrical impulses.
• Yoga: Yoga can help reduce pain for some patients. However, people with weak bones should avoid extreme positions.
• Lifestyle: Making simple changes, such as quitting smoking or losing weight, can also improve symptoms and quality of life.

Patients experiences with other symptoms like depression and anxiety should also be monitored regularly. For those who are experiencing neurological changes, conservative treatments may be the best first step. If such treatments fail, or the patient experiences persistent pain, neurological changes or conditions that worsen despite bracing, surgical intervention may be the best course of action. However, surgery isn’t usually recommended for elderly patients due to the increased risks and poor bone health.

Surgery for kyphoscoliosis can come in several forms and can involve spine fusion or non-fusion surgeries. Similarly, surgery for kyphosis, the forward rounding of the back, can involve many types of correction surgery. After surgery, it’s critical to manage pain and check for any changes in neurological function. Surgery isn’t generally recommended for older patients due to the increased risk and poor bone health.

What else can Kyphoscoliosis be?

Kyphoscoliosis is a condition in which the spine curves abnormally both sideways and backwards. It can occur for no known reason, even in otherwise healthy individuals. In children, it can start off mildly but it’s important to track it over time to see if the curvature of the spine gets worse. Instead of trying to figure out other conditions that this could be, it might be more helpful to look for the root cause.

Changes in the skin could be a clue that the person has other conditions at the same time, like neurofibromatosis or a type of spinal cord defect called myelomeningocele. Here are some other conditions that you also might want to consider:

• Osteoporosis (a condition that weakens bones)
• Scheuermann disease (a form of spinal curvature)
• Idiopathic kyphoscoliosis (kyphoscoliosis without a known cause)
• Adolescent idiopathic scoliosis (sideways curvature of the spine that starts in adolescence)
• Infantile idiopathic scoliosis with kyphosis (spinal curvature that starts in infancy)
• Age-related hyperkyphosis (dowager’s hump)
• Vertebral fracture (a broken bone in the spine)
• Infections (caused by a type of parasite called Echinococcus granulosus)
• Tumors (abnormal growth of tissue in the body)
• Kniest dysplasia (a type of dwarfism)
• Friedreich ataxia (a genetic condition that affects the nervous system)

What to expect with Kyphoscoliosis

The outlook for kyphoscoliosis, a condition that causes abnormal curvature of the spine, can vary greatly and generally depends on what is causing the condition to get worse. If kyphoscoliosis is made more complicated by pulmonary hypertension, which is high blood pressure in the lungs, it’s linked to a high death risk.

Possible Complications When Diagnosed with Kyphoscoliosis

Kyphoscoliosis, which is a combination of sideways and outward curving of the spine, increases the risk of death from all causes.

• In women, the risk of spine fractures goes up with the severity of the kyphoscoliosis, even if they haven’t had fractures before.
• The changes to body mechanics can alter the way people walk and make them more likely to fall.

Other effects of kyphoscoliosis are most often seen in the lungs. It can lead to decreased ability to exercise and breathe. It can cause conditions where the airways are blocked, the lungs can’t expand as much as they should or there are sleep-related issues with breathing. It can also cause a condition where oxygen levels in the blood are lower than they should be. Tests of lung function might show decreases in the amount of air you can expel forcibly after taking the deepest breath possible (FVC), the amount of air you can exhale with force in one second (FEV1) and in FRC, the amount of air left in your lungs at the end of a breath out. The best treatment for severe chronic respiratory failure caused by kyphoscoliosis is to use a non-invasive ventilator that pushes air into the lungs. If this leads to a condition where the right side of the heart is enlarged due to lung disease (cor pulmonale), the outlook can be poor and the risk of death higher. Skin problems may occur due to pressure from the abnormal spine curvature.

• If the condition progresses, the spinal cord can be compressed, leading to severe problems like partial paralysis of the legs or complete paralysis due to damage to the nervous system.
• As the condition worsens, muscle wasting and tightness in the arms and legs can occur.
• Pressure in the spinal canal may go up as the front part of the spine curves more.
• The curve can lead to decreased blood supply to the spinal cord and a worsening of neurological conditions.
• The spinal cord might flatten and lose its protective covering, leading to nerve damage.

In addition, surgery to correct the curvature may cause complications that damage the nervous system. This is because it can be tricky to restore a stable spine when dealing with three-dimensional deformities. The sudden onset of paralysis after surgery to correct kyphoscoliosis is a known risk.

Preventing Kyphoscoliosis

Patients should be informed about how changes in their body’s structure might affect them. This can lead to difficulties in physical activities, an increased chance of falling, a decrease in strength, and reduced lung function. These changes could potentially increase the risk of death.