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Kyphoscoliosis
Kyphoscoliosis Deformity Spine

Kyphoscoliosis refers to concomitant kyphosis, i.e., an abnormal anteroposterior curvature of the spine leading to the formation of a hunchback, and scoliosis, which describes a pathological lateral deviation of the vertebral column. Kyphoscoliosis may be congenital or acquired and may interfere with the function of intrathoracic organs. Treatment and prognosis largely depend on the etiology and severity of the disease.

Images

WIKIDATA, CC BY 2.0
WIKIDATA, CC BY 2.0

Presentation

The patient’s age at symptom onset, as well as complaints related to the presence of kyphoscoliosis (KS), depend on the severity of the condition [1]. If not diagnosed prenatally, severe symptoms of congenital KS may be seen at birth. However, mild deformities of the spine may not clinically manifest until years later [2]. In this context, it has been estimated that about 70% of congenital vertebral anomalies result in progressive deformities [3]. Besides the asymmetry of the thoracic wall and the deviation of the vertebral column – which may increase several degrees per year [4] – KS patients typically claim backaches and the inability to sit or stand for a prolonged period of time. Moreover, they may eventually present with the following comorbidities and sequelae:

  • Respiratory insufficiency due to restrictive lung disease: Reduced tolerance to exercise may affect life quality, respiratory failure may be life-threatening. Lung function tests may reveal a diminished inspiratory capacity, vital capacity, and total lung capacity, and blood gas analyses may confirm decreased arterial oxygen saturation [5] [6]. Respiratory muscle strength may be severely reduced [7], and affected individuals may suffer from pulmonary hypertension. Patients may also suffer from recurrent infections of the respiratory tract.
  • Neurological deficits due to spinal cord compression: This condition is more likely to occur in patients suffering from severe kyphosis than in those presenting with sole scoliosis. Thus, the severity of anteroposterior spine deformity correlates with the likelihood of paraplegia or paralysis in KS patients. Those individuals presenting with sharp-angle kyphosis are at high risks of spinal cord compression [8]. Furthermore, thoracic defects are more likely to lead to neurological deficits than lumbar anomalies [4].

Of note, a considerable share of children diagnosed with congenital spine anomalies suffers from additional developmental defects that may affect the cardiovascular or genitourinary system [3]. Thus, pediatric KS patients may present with symptoms unrelated to deviations of the vertebral spine.

Acquired KS may be related to degenerative disease, infectious disease or trauma, or may occur sporadically. While functional impairment of thoracic organs may cause complications as described above, the course of the disease may differ in the progression of the underlying disease.

Entire Body System

  • Hypoxemia

    Hypoxemia is common. Therapy Milwaukee brace controls moderate deformities. Surgical correction is attempted in some to fix spine and arrest progression of the deformity. Oxygen on long term may be necessary in patients with significant hypoxemia. [meddean.luc.edu]

    Dyspnoea Hypoxemia due to hypoventilation in kyphoscoliosis leads to pulmonary vasoconstriction and pulmonary hypertension which give rise to difficulty in breathing Dyspnoea Hypoxemia due to hypoventilation in kyphoscoliosis leads to pulmonary vasoconstriction [medicaljoyworks.com]

    The patient also has severe hypoxemia, with 75% peripheral oxygen saturation (SpO2). [medpagetoday.com]

    (acute respiratory alkalosis) ABG in Severe Kyphoscoliosis chronic ventilatory failure with hypoxemia (compensated respiratory acidosis) acute on chronic situations seen a lot in patients Hemodynamic Findings in Kyphoscoliosis CVP, PA, PVR are all increased [quizlet.com]

  • Prolonged Immobilization

    In patients experiencing neurological changes, operative management may be warranted.[12] This population is particularly sensitive to peri-operative medication and side effects due to functional limitations and prolonged immobilization.[9] Surgical correction [ncbi.nlm.nih.gov]

  • Increased Energy

    Consequently, the spinal alignment becomes imbalance requiring increase energy expenditure to maintain an upright posture. [nspc.com]

Neurologic

  • Spastic Gait

    He presented with some lower extremity weakness and a spastic gait. Patient was in halo gravity for two months for a severe deformity greater than 180 degrees of kyphoscoliosis. [spinal-deformity-surgeon.com]

Workup

Developmental defects due to disturbed somitogenesis and impaired formation of the axial skeleton may be detected during prenatal ultrasound examinations [9]. At a later age, vertebral anomalies causing KS may be visualized by means of plain radiography and computed tomography. The single most important advantage of computed tomography scans is the possibility of three-dimensional reconstructions of the vertebral spine [10]. In any case, the presence of single or multiple hemivertebrae and wedge vertebrae, unilateral unsegmented bars, block vertebrae and butterfly vertebrae may be visualized [11]. Magnetic resonance imaging may be indicated to assess the condition of the spinal cord and to identify comorbidities such as diastematomyelia [3] [12]. Diagnostic imaging may also be employed to depict acquired pathologies of the skeleton, e.g., rickets, osteopenia, and osteoporosis.

Serum

  • Hypercapnia

    Start of NIV in patients with kyphoscoliosis is indicated in combination of daytime hypercapnia and symptoms reflecting hypoventilation or the development of its associated complications. [portal.lf.upjs.sk]

    (see Chronic Hypoventilation, [[Chronic Hypoventilation]]) Hypercapnia occurs when VC is <30% of predicted (hypercapnia may also be related to patient age and inspiratory muscle weakness) Pulmonary Function Tests (PFT’s) (see Pulmonary Function Tests [mdnxs.com]

    Firstly, we agree with the contributing factors of hypercapnia [ 7, 8 ]. [link.springer.com]

    Lilius also described associated malformations in 21.8% of cleft lip/palate children. [6] Hypoxia, hypercapnia, acidosis and any increase in pulmonary vascular resistance should be avoided. [joacp.org]

    This suggests that PS 20 delayed the development of exercise-induced hypercapnia compared with unassisted exercise, consistent with the findings of Vila et al. 22. [erj.ersjournals.com]

Pulmonary Function Test

  • Decreased Vital Capacity

    Sue displayed a restrictive lung pattern, evidenced by a decreased vital capacity and tidal volume with severe nocturnal dyspnoea, resulting in raised carbon dioxide levels in arterial blood and decreased oxygenation. [ncbi.nlm.nih.gov]

    Patients with scoliosis suffer from restrictive lung disease which decreases vital capacity, functional residual capacity, tidal volume, and increased respiratory rate. [4] The severity of the deformity is best determined by measuring Cobb's angle. [joacp.org]

    An angle more than 60° results in a restrictive type of pulmonary impairment with a decrease in forced expiratory volume in 1 s, forced vital capacity, and chest wall compliance.[1],[6] General anesthesia is indicated in scoliosis because of maternal [mjdrdypu.org]

Treatment

An older 2011 study suggested that chiropractic treatment may aid in alleviating pain and increase flexibility while living with scoliosis. Note that this kind of treatment isn’t a cure for the curvature of scoliosis. [healthline.com]

Prognosis

The prognosis depends more on why the scoliosis occurred. If the Scoliosis as occured as a result of an infection by another disease, then the prognosis would be based upon this infection, not the scoliosis. [myvmc.com]

If surgery is required, a balance must be drawn between improving prognosis as the child gets older and bigger and worsening prognosis as the condition deteriorates. [patient.info]

Causes - Ehlers-Danlos syndrome - type VIa - kyphoscoliosis Prevention - Ehlers-Danlos syndrome - type VIa - kyphoscoliosis Diagnosis - Ehlers-Danlos syndrome - type VIa - kyphoscoliosis Prognosis - Ehlers-Danlos syndrome - type VIa - kyphoscoliosis EDS [checkorphan.org]

Etiology

The etiology of congenital scoliosis and its development remains unclear and has not yet been fully identified, even there are theories that congenital scoliosis could be derived from the failure of formation or failure of segmentation, which are etiologically [ncbi.nlm.nih.gov]

Kyphoscoliosis Chapter Outline Etiology and Epidemiology Kyphoscoliosis affects approximately 2% of the people in the United States—mostly young children who are going through a growing spurt. [thoracickey.com]

Etiology of this condition is not yet fully established. [karger.com]

Clinical Picture In majority it is of idiopathic etiology. Deformity results in shortening of height. Patients can be asymptomatic. Mobility of chest wall is impaired, the chest wall is stiff and lung volumes are restricted. [meddean.luc.edu]

Epidemiology

Kyphoscoliosis Chapter Outline Etiology and Epidemiology Kyphoscoliosis affects approximately 2% of the people in the United States—mostly young children who are going through a growing spurt. [thoracickey.com]

Chapter Outline Etiology and Epidemiology Kyphoscoliosis affects approximately 2% of the people in the United States—mostly young children who are going through a growing spurt. [clinicalgate.com]

Epidemiology of neurofibromatosis type 1. Am J Med Genet. 1999;89:1–6. Article CAS Google Scholar Akbarnia BA, Gabriel KR, Beckman E, Chalk D. Prevalence of scoliosis in neurofibromatosis. Spine (Phila Pa 1976). 1992;17:S244–8. [link.springer.com]

Carbapenem-resistant Enterobacteriaceae: epidemiology and prevention. Clin Infect Dis. 2011 Jul 1. 53(1):60-7. [Medline]. van Duin D, Bonomo RA. [emedicine.medscape.com]

Pathophysiology

The aim was to conduct a systematic review of available data concerning the pathophysiology and treatment of kyphoscoliosis. [ncbi.nlm.nih.gov]

The incidence of kyphoscoliosis reaching an angle of 35 0 is 1 in 1000 and that more than 70 0 is 1 in 10,000. [5] Severe scoliosis is rare in parturients, which varies from 1 in 1500 to 1 in 12,000 pregnancies. [3],[6] The major pathophysiological effects [joacc.com]

Following a brief examination of the force these muscles are required to exert in normal conditions, we review the pathophysiology of some characteristic alterations brought about by a) chest wall diseases such as kyphoscoliosis; b) other diseases involving [archbronconeumol.org]

Prevention

It can help prevent any future damage, though. Braces are typically worn throughout the day. They become more effective as they’re worn more frequently. 3. [healthline.com]

References

  1. Campos MA, Weinstein SL. Pediatric scoliosis and kyphosis. Neurosurg Clin N Am. 2007; 18(3):515-529.
  2. Jog S, Patole S, Whitehall J. Congenital scoliosis in a neonate: can a neonatologist ignore it? Postgrad Med J. 2002; 78(922):469-472.
  3. Debnath UK, Goel V, Harshavardhana N, Webb JK. Congenital scoliosis - Quo vadis? Indian J Orthop. 2010; 44(2):137-147.
  4. Marks DS, Qaimkhani SA. The natural history of congenital scoliosis and kyphosis. Spine (Phila Pa 1976). 2009; 34(17):1751-1755.
  5. Huang SW, Wu CL, Lin CC, et al. Effect of long term intermittent nocturnal non-invasive positive pressure ventilation on patient with severe kyphoscoliosis and hypoxaemia. BMJ Case Rep. 2009; 2009.
  6. Fuschillo S, De Felice A, Martucci M, et al. Pulmonary rehabilitation improves exercise capacity in subjects with kyphoscoliosis and severe respiratory impairment. Respir Care. 2015; 60(1):96-101.
  7. Casas A, Pavia J, Maldonado D. [Respiratory muscle disorders in chest wall diseases]. Arch Bronconeumol. 2003; 39(8):361-366.
  8. Masini M, Maranhao V. Experimental determination of the effect of progressive sharp-angle spinal deformity on the spinal cord. Eur Spine J. 1997; 6(2):89-92.
  9. Goldstein I, Makhoul IR, Weissman A, Drugan A. Hemivertebra: prenatal diagnosis, incidence and characteristics. Fetal Diagn Ther. 2005; 20(2):121-126.
  10. Kawakami N, Tsuji T, Imagama S, Lenke LG, Puno RM, Kuklo TR. 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). 2009; 34(17):1756-1765.
  11. Kusumi K, Turnpenny PD. Formation errors of the vertebral column. J Bone Joint Surg Am. 2007; 89 Suppl 1:64-71.
  12. Sucato DJ. Management of severe spinal deformity: scoliosis and kyphosis. Spine (Phila Pa 1976). 2010; 35(25):2186-2192.
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