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Kyphoscoliosis

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.


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.

Splenomegaly
  • We found no serious adverse event, drug-related platelet reduction, or splenomegaly. Leukocyte levels remained below 21,000/µL. CD34 increased significantly at day 5 of G-CSF administration. Low-dose G-CSF was safe and well tolerated by the patient.[ncbi.nlm.nih.gov]
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]
  • (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]
  • Besides hypoventilation and hypoxemia, the reasons for increased pulmonary artery pressure may involve different mechanisms.[link.springer.com]
  • Commonly the other listed options are needed to correct atelectasis or hypoxemia and to help remove retained secretions.[quizlet.com]
Microstomia
  • Here we are presenting a newborn with multiple congenital anomaly like microstomia with pouting lips, H like dimple of the chin, hypoplastic alae nasi, plug like nostrils, short and broad neck.[ncbi.nlm.nih.gov]
Torticollis
  • He showed torticollis, and complained of severe pain in his neck and left upper arm. Radiographic examinations revealed that the C6 vertebra was translated anteriorly and laterally to the C7 vertebra.[ncbi.nlm.nih.gov]
Ulcer
  • Abstract A case of spontaneous gastric perforation is reported in a 75-year-old woman due to massive hemorrhaging from a benign gastric ulcer.[ncbi.nlm.nih.gov]
Incontinence
  • Furthermore, he developed a significant progression of neurological deficits, including weakness of both legs and urinary and bowel incontinence.[ncbi.nlm.nih.gov]

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.

Hypercapnia
  • Firstly, we agree with the contributing factors of hypercapnia [ 7 , 8 ].[link.springer.com]
  • If there was still a demand for mechanical ventilation after weaning due to hypercapnia we preferred to continue with NIV.[mrmjournal.biomedcentral.com]
  • Am J Respir Crit Care Med 170(4):456–465 Google Scholar Gonzalez J, Sharshar T, Hart N et al (2003) Air leaks during mechanical ventilation as a cause of persistent hypercapnia in neuromuscular disorders.[link.springer.com]
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 diseasewhich decreased vital capacity, functional residual capacity, tidal volume, and increased respiratory rate. [1], [5] The severity of pulmonary impairment depends on the degree of the Cobb's angle[joacp.org]

Treatment

  • PATIENTS AND METHODS: We retrospectively reviewed 14 patients who had VEPTR( ) treatment of early-onset kyphoscoliosis.[ncbi.nlm.nih.gov]
  • CONCLUSION: A nonsurgical treatment was a useful intervention for some of the physical and psychosocial aspects that this patient with kyphoscoliosis encountered.[ncbi.nlm.nih.gov]
  • Scoliosis too has similar forms of treatments available as Kyphosis including bracing, physical therapy and various types of surgeries.[en.wikipedia.org]
  • The authors present the first case report for treatment of this condition using a navigation system.[ncbi.nlm.nih.gov]
  • PURPOSE: To present a technical note related with double-segment total vertebrectomy for the surgical treatment of a patient who had neglected congenital kyphoscoliosis in lumbar spine. STUDY DESIGN: Case report.[ncbi.nlm.nih.gov]

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]
  • 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]
  • Treatment and prognosis largely depend on the etiology and severity of the disease. 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.[symptoma.com]
  • We believe that this letter may provide important information regarding the prognosis and efficacy of NIV.[link.springer.com]

Etiology

  • […] are etiologically heterogeneous with genetic, epigenetic, and environmental factors contributing to their occurrence.[ncbi.nlm.nih.gov]
  • 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]
  • Based on the results of this analysis, the authors propose a 2-category risk stratification system for the timing of deformity correction depending on the primary neurogenic etiology.[ncbi.nlm.nih.gov]
  • 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]
  • Treatment and prognosis largely depend on the etiology and severity of the disease. 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.[symptoma.com]

Epidemiology

  • 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]
  • Smith, a professor of epidemiology at Kent State University in Ohio who studies infectious diseases, said that despite warnings from medical experts and the availability of vaccines, the practice of deliberately exposing children to disease continues.[nytimes.com]
  • Epidemiology The prevalence of these conditions varies considerably according to the precise definition that is used. In early childhood about 60% are boys but, in the adolescent variety, girls represent 90%.[patient.info]
Sex distribution
Age distribution

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

  • This in turn had led to mechanical obstruction and prevented egress of blood arising from a bleeding arteriole in the base of a chronic gastric ulcer.[ncbi.nlm.nih.gov]
  • […] of the spine and prevent further curvature.[quizlet.com]
  • The heterogeneous nature of the majority of published studies prevents unequivocal conclusions being drawn.[ncbi.nlm.nih.gov]
  • We emphasize the importance of recognizing potential symptoms early to prevent additional renal injury. Caution should be taken with deformities around the origin of the renal arteries and great vessels, especially in patients with atherosclerosis.[ncbi.nlm.nih.gov]
  • 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]

References

Article

  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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Last updated: 2018-06-22 11:29