What is kyphoscoliosis?
Kyphoscoliosis Kyphoscoliosis is a thoracic cage deformity that causes extrapulmonary restriction of the lungs and gives rise to impairment of pulmonary functions, as described earlier for restrictive lung diseases. The condition may be primary (idiopathic) or secondary to neuromuscular disease,...
What is the medgen UID for kyphoscoliosis?
Restrictive lung disease due to kyphoscoliosis MedGen UID: 744650 •Concept ID: C1997603 Disease or Syndrome SNOMED CT: Restrictive lung disease due to kyphoscoliosis (427908002) Recent clinical studies Etiology
What are respiratory complications of kyphoscoliosis?
Respiratory complications are a common entity for restrictive chest wall diseases such as kyphoscoliosis. When such anatomical distortions are severe, patients have an increased risk of respiratory failure associated with poor clinical outcomes due to resulting malformation of the thoracic cavity.
What are the mortality and morbidity associated with kyphoscoliosis?
Kyphoscoliosis increases the risk of all-cause mortality. In women, the severity of the kyphoscoliosis is correlated with an increased risk of vertebral fractures even in those without prior history of fractures. [3] Given changes in biomechanics, patients are subject to gait changes and are at a higher risk of falls.
What is kyphoscoliosis?
Kyphoscoliosis is defined as a deviation of the normal curvature of the spine in the sagittal and coronal planes and can include a rotation of the spinal axis.[1] Adult scoliosis is defined as a lateral deviation of more than 10 degrees in the coronal plane as measured by the Cobb angle.
What is the difference between scoliosis and kyphoscoliosis?
Though the conditions may be similar, they aren't exactly the same. Scoliosis is a sideways curve of your spine — often taking the shape of the letter 'S' or 'C'. Kyphosis is more of a forward rounding of the back, which leads to a hunchback or slouching posture.
How does kyphoscoliosis cause pulmonary hypertension?
Pulmonary hypertension (PH) is a life-threatening complication of kyphoscoliosis with poor prognosis. It develops due to alveolar hypoventilation similarly as other diseases with alveolar hypoventilation, such as obstructive sleep apnea and obesity hypoventilation syndrome.
What is thoracic kyphoscoliosis?
Kyphoscoliosis is a combination of two spinal conditions: kyphosis and scoliosis. Scoliosis causes an unnatural sideways spinal curve, while kyphosis causes an excessive outward spinal curve. So kyphoscoliosis is the development of an unnatural sideways and forward/backward spinal curve simultaneously.
Is kyphoscoliosis the same as kyphosis?
Kyphosis causes the spine to curve abnormally on the sagittal plane, meaning it twists forward or backward, giving the back a rounded or hunched appearance. People with kyphoscoliosis have a spine that curves both to the side and forward or backward at the same time.
What is idiopathic scoliosis and kyphoscoliosis?
Idiopathic scoliosis has a strong tendency to flatten the normal kyphosis of the thoracic spine. Winter taught that idiopathic scoliosis is a hypokyphotic disease. [6, 7] In most cases, diagnoses of kyphoscoliosis were clinical misinterpretations of the rib hump associated with an otherwise hypokyphotic thoracic spine.
Does kyphoscoliosis cause restrictive lung disease?
Kyphoscoliosis is a thoracic cage deformity that causes extrapulmonary restriction of the lungs and gives rise to impairment of pulmonary functions, as described earlier for restrictive lung diseases. The condition may be primary (idiopathic) or secondary to neuromuscular disease, spondylitis, or Marfan syndrome.
Can kyphoscoliosis cause shortness of breath?
INTRODUCTION: Abnormalities of the thoracic cage are an unusual cause of dyspnea. This is a rare case of extrinsic compression of a proximal bronchus due to vascular infringement from severe kyphoscoliosis.
How does kyphosis affect lung function?
Abstract. Background: Hyperkyphosis reduces the amount of space in the chest, mobility of the rib cage, and expansion of the lungs. Decline in pulmonary function may be greater in persons with more severe kyphosis; however, no prospective studies have assessed this association.
What kind of curvature of the spine is manifested in kyphosis?
Although the medical term for a curve that is greater than normal (more than 50 degrees) is actually hyperkyphosis, the term kyphosis is commonly used by doctors to refer to the clinical condition of excessive curvature in the thoracic spine that leads to a rounded upper back.
What is the difference between lordosis and kyphosis?
Lordosis (also known as swayback) is when the lower back, above the buttocks, curves inward too much, causing the child's abdomen to protrude and buttocks to stick out. Kyphosis is when the upper spine curves too far outward, forming a hump on the upper back.
Can kyphoscoliosis cause atelectasis?
In severe kyphoscoliosis the deformity of the thorax compresses the lungs and restricts alveolar expansion, which in turn causes alveolar hypoventilation and atelectasis.
What is kyphoscoliosis associated with?
Kyphoscoliosis is defined as scoliosis associated with kyphosis of 50 degrees or greater. If kyphosis is present, appropriate dynamic roentgenograms (i.e., hyperextension lateral over a bolster to evaluate the flexibility of the curve) should be obtained. Paraplegia is not uncommon in patients with severe kyphosis.
When does kyphoscoliosis occur?
Kyphoscoliosis occurs early, often in the first year of life, in metatropic dysplasia and is rapidly progressive. From: Surgical Management of Spinal Deformities, 2009. Download as PDF. About this page.
What is the condition that causes pulmonary restriction?
Kyphoscoliosis. Kyphoscoliosis is a thoracic cage deformity that causes extrapulmonary restriction of the lungs and gives rise to impairment of pulmonary functions, as described earlier for restrictive lung diseases. The condition may be primary (idiopathic) or secondary to neuromuscular disease, spondylitis, or Marfan syndrome.252.
What is a thoracic kyphoscoliosis?
Thoracic kyphoscoliosis is a disorder of the spine that involves abnormal curvature of the spine in both coronal and sagittal planes. Sometimes described as a combination of kyphosis (the exaggerated, rounded-back curve) and scoliosis (a lateral or sideways curve), this disorder is believed to be potentially more serious than scoliosis alone because its curves can progress rapidly and deformities can lead to spinal cord compression and paraplegia as well as respiratory failure (when curves exceed 70°) and hypoxemia and cor pulmonale (when curves exceed 100°). Respiratory failure and hypoxemia occur when thoracic kyphosis is extreme because it reduces available visceral space, compromising lung capacity and venous return to the heart.
What is the name of the disorder that causes short trunked dwarfism?
Kniest dysplasia is an autosomal dominant disorder characterized by kyphoscoliosis, severe short trunked dwarfism, cleft palate, flat face, hearing defects, and joint contractures.101,102 Patients with Kniest dysplasia have severe congenital myopia and vitreoretinal degeneration. The vitreous cavity contains fibrous, clouded, membranous structures floating in the retrolental space ( Fig. 115.9 ). Other eyes have cortical and posterior subcapsular opacity of the lens, and also veil-like vitreous opacities in the periphery. The common retinal changes include perivascular lattice degeneration and white-without-pressure of various degrees. Bullous retinal detachment with Kniest dysplasia can be treated successfully, especially by vitreous surgery with silicone oil tamponade. 103
What is pyknodysostosis?
Pyknodysostosis is a rare autosomal recessive disorder caused by an inactivating mutation in the lysosomal cathepsin K ( CTSK) gene and characterized clinically by osteosclerosis, fragility fractures, short stature, dysmorphic facial features, dental abnormalities, distal phalangeal acroosteolysis, and delayed closure of cranial sutures. 32 There is a range of age at presentation from childhood to later adult years contributing to an underdiagnosis or misdiagnosis of this form of short-limb dwarfism. The condition is thought to be a form of osteosclerosis as both have progressive generalized osteosclerosis and recurrent fractures. Pyknodysostosis may have been responsible for the short stature and other physical features of the French impressionist painter Henri de Toulouse-Lautrec. 33
What is the cause of abnormal nocturnal ventilation in kyphoscoliosis?
The major mechanism responsible for abnormal nocturnal ventilation in kyphoscoliosis is alveolar hypoventilation. As a result of changes in the thoracic shape, the diaphragm has to work in a mechanically disadvantageous posture. The suppression of accessory muscle activity during REM sleep leads to hypoventilation.
What is kyphoscoliosis thoracolumbar spine?
Continuing Education Activity. Kyphoscoliosis is a disease of the thoracolumbar spine in which normal curvature is excessively deviated in multiple planes. Its complications are varied by multiple factors including the age of onset and the primary disease that contributes towards its presentation.
What is the most common cause of kyphoscoliosis in children?
A common cause of juvenile kyphoscoliosis is Scheuermann's disease which has an incidence of 0.4% to 8% in the USA, occurs more commonly in boys, and typically affects children between the ages of 13 and 16 years old. Pathophysiology.
What is the Cobb angle of kyphoscoliosis?
Moderate kyphoscoliosis is a Cobb angle ranging from 25 to 100 degrees, whereas severe kyphoscoliosis is a Cobb angle greater than 100 degrees.
What is the degree of lordosis?
When the spine is viewed laterally, a normal degree of lordosis (posterior curvature) can be seen in both the cervical and lumbar spine ranging between 35 and 80 degrees; whereas the thoracic spine has a natural degree of kyphosis (forward curvature) generally between 30 and 50 degrees.[2] .
What causes the spinal cord to flatten?
Curvature may also cause flattening of the spinal cord that can result in demyelination of anterior funiculus and atrophy of the anterior horn. [28] Further, surgical complications may also lead to neurological compromise secondary to the challenge of restoring spinal stability to three-dimensional deformities.[24] .
Does kyphoscoliosis increase the risk of all-cause mortality?
Kyphoscoliosis increases the risk of all-cause mortality. In women, the severity of the kyphoscoliosis is correlated with an increased risk of vertebral fractures even in those without prior history of fractures. [3] Given changes in biomechanics, patients are subject to gait changes and are at a higher risk of falls.
Does yoga help with kyphoscoliosis?
Research on the use of SCS in kyphoscoliosis is lacking, therefore success rates, optimal frequency, and complications remain uncertain. [24] Yoga may alleviate pain in some, however, care should be taken to avoid extreme hyperflexion and hyperextension, especially in patients with osteopenia or osteoporosis.[25] .
How to treat kyphoscoliosis?
5. Surgery. If you have severe kyphoscoliosis, doctors may recommend surgical correction.
What are the best ways to treat kyphoscoliosis?
severity of the spine curvature. ability to maintain a healthy posture. age. other medical conditions. impact on day-to-day routines. There are many noninvasive treatment methods for kyphoscoliosis. If these don’t work, however, your doctor may recommend surgery. 1. Checkups.
What is the name of the condition that causes the spine to curve?
Understanding Kyphoscoliosis. Kyphoscoliosis is an abnormal curve of the spine on two planes: the coronal plane, or side to side, and the sagittal plane, or back to front. It’s a combined spinal abnormality of two other conditions: kyphosis and scoliosis. Scoliosis causes the spine to curve abnormally on the coronal plane, ...
Why does my spine curve?
Kyphosis causes the spine to curve abnormally on the sagittal plane, meaning it twists forward or backward, giving the back a rounded or hunched appearance. People with kyphoscoliosis have a spine that curves both to the side and forward or backward at the same time. This condition can occur at any age, including at birth.
What is the procedure that connects bones in the spine together with metal rods or screws to prevent independent movement?
A common surgical option is a spinal fusion. It’s a procedure that connects bones in the spine together with metal rods or screws to prevent independent movement. This allows old and new spinal material to form together. Doctors can also install an adjustable rod.
What is the condition that impairs the growth of spinal bones, cartilage, and connective tissue?
Poor posture over time may result in postural kyphoscoliosis. It can be treated with extensive physical therapy. Osteochondrodysplasia. This is a type of skeletal dysplasia, a condition that impairs the growth of spinal bones, cartilage, and connective tissue. Degenerative diseases.
What are the symptoms of kyphoscoliosis?
In more severe cases, kyphoscoliosis can affect the lungs, nerves, and other organs. More severe symptoms include: disfigurement. back pain. trouble breathing. weakness or paralysis. stiffness.
Is LTOT effective for kyphoscoliosis?
While this approach is associated with improved long-term survival and exercise capacity, it remains unable to prevent the cardiopulmonary deterioration of the kyphoscoliosis patient.
Is vasoactive medication safe for kyphoscoliosis?
Considering the progressive nature of PH in kyphoscoliosis, despite ventilatory and oxygen treatment, they propose that adding a vasoactive medication offers a safe means of combating cardiopulmonary decline, in a long-term setting as well as an acute setting.
Is pulmonary hypertension associated with kyphoscoliosis?
Pulmonary hypertension in kyphoscoliosis is associated with a high mortality rate , which persists despite use of current management options. To date, the use of PAH-approved drugs for kyphoscoliosis-related PH have been associated with positive outcomes and may limit progression of disease.
Is kyphoscoliosis a clinical condition?
Given that kyphoscoliosis is encountered relatively rarely in clinical practice, and PH is often not diagnosed in a timely manner, in the absence of guidelines, they urge clinicians to direct their attention to identifying patients who might benefit from vasoactive agents in addition to ventilation/oxygenation therapy.
What is spirometry?
Spirometry is a method of assessing lung function by measuring the volume of air that the patient is able to expel from the lungs after a maximal inspiration. It is a reliable method of differentiating between obstructive airways disorders (e.g. chronic obstructive pulmonary disease, asthma) and restrictive diseases (e.g. fibrotic lung disease).
Assess the quality of results
Three consistent volume-time curves are required, of which the best two curves should be within 5% of each other.
