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.

• 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]

  (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]

  Dyspnoea [1] Hypoxemia due to hypoventilation in kyphoscoliosis leads to pulmonary vasoconstriction and pulmonary hypertension which give rise to difficulty in breathing [1] Spinal defects [1] Connective tissues disorders [1] Kyphosis is excessive curvature [explainmedicine.com]

• Asymptomatic

  Patients can be asymptomatic. Mobility of chest wall is impaired, the chest wall is stiff and lung volumes are restricted. Hypoventilation can occur due to small tidal volumes and increased dead space ventilation. [meddean.luc.edu]

  At 2.5 years following surgery, the patient was asymptomatic, mobilizing independently, and had achieved a solid spinal fusion. [ncbi.nlm.nih.gov]

  Isolated Asymptomatic Short Sternum in a Healthy Young Girl. Case Reports in Radiology 2014. Capasso L, Caramiello S, D'Anastasio R. The anomaly of Santa Rosa. Lancet 1999;353(9151):504. [msjonline.org]

  His follow-up was asymptomatic and well balanced in the sagittal and coronal planes at 6 months following operation. Systemic involvement was clinically and radiographically assessed. [karger.com]

• Developmental Disorder

  Discovery of four recessive developmental disorders using probabilistic genotype and phenotype matching among 4,125 families. Nat Genet. 2015 Nov;47(11):1363-9. [disorders.eyes.arizona.edu]

  Karger AG, Basel Introduction Goldenhar syndrome, also known as oculo-auriculo-vertebral dysplasia, is a developmental disorder primarily involving structures derived from the first and second pharyngeal arches during embryogenesis. [karger.com]

  […] development and developmental regression in infancy. [ncbi.nlm.nih.gov]

• Plethora

  The etiology of this syndrome is unclear since it varies genetically and is linked to a plethora of reasons. Case Report: A 13-year-old male presented to the Orthopedic Department with complaints of back bulge with bilateral microtia since birth. [karger.com]

• Severe Pain

  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]

• Pharyngitis

  Karger AG, Basel Introduction Goldenhar syndrome, also known as oculo-auriculo-vertebral dysplasia, is a developmental disorder primarily involving structures derived from the first and second pharyngeal arches during embryogenesis. [karger.com]

• Hearing Impairment

  ACROOSTEOLYSIS-KELOID-LIKE LESIONS-PREMATURE AGING SYNDROME Is also known as premature aging syndrome, penttinen type Related symptoms: Hearing impairment Scoliosis Hypertelorism Micrognathia Sensorineural hearing impairment SOURCES: ORPHANET OMIM MESH [mendelian.co]

  Type I Stickler syndrome has multiple systemic features such as cleft palate, hearing impairment, premature arthritis, micrognathia, kyphoscoliosis, and some signs such as arachnodactyly that are found in the Marfan syndrome. [disorders.eyes.arizona.edu]

  […] and weakness (typically improving during childhood), progressive scoliosis, joint hypermobility, hyperelastic skin, gross motor developmental delay, myopathy, and hearing impairment. [ncbi.nlm.nih.gov]

• Low Back Pain

  A 36-year-old female patient presented with a 3-year history of low back pain, and progressive thoracolumbar kyphoscoliosis. [ncbi.nlm.nih.gov]

  Back pain ranged from 7 to 9 on a visual analogue scale (VAS) of 0 to 10. In-depth clinical neurological examination revealed no deficits, excluding the possibility of neuropathic low back pain. [academic.oup.com]

  Description A 38-year-old woman was referred to the rheumatology department due to mechanical low back pain and progressive scoliosis that started in adolescence. [casereports.bmj.com]

  History A 66-year-old female presented with a history of chronic low back pain, left thigh pain, and a progressive left-sided lumbar hump. She was "hunched over" forward and to the right. [spineuniverse.com]

• 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]

  However, torticollis and cervicothoracic scoliosis were the major deformities in another study. Thoracolumbar scoliosis and kyphoscoliosis were of lesser occurrence [11]. [karger.com]

• Ankylosis

  Inflammation compromises the ligamentous structures of the spinal column, the sacroiliac articulations, and the ribs, leading to the fibrosis and ossification of these structures and, thus, to an increase in rig cage stiffness due to ankylosis and fusion [archbronconeumol.org]

  […] is a rare, lethal genodermatosis with characteristic manifestations that are easily recognizable at birth: thin, tightly adherent translucent skin with erosions at flexure sites, superficial vessels, typical facial dysmorphism, and generalized joint ankylosis [ncbi.nlm.nih.gov]

• Steppage Gait

  These difficulties may cause a higher than normal step (steppage gait) and increase the risk of ankle injuries and tripping. [ncbi.nlm.nih.gov]

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

  (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]

  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]

  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]

  Care should be taken to avoid hypoxia, hypercapnia, acidosis, and anesthetic gases such as nitrous oxide as they increase the pulmonary vascular resistance.[5] Neuraxial blockade in our patient has complications such as unpredictable block, risk of high [mjdrdypu.org]

• 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]

PATIENTS AND METHODS: We retrospectively reviewed 14 patients who had VEPTR(®) treatment of early-onset kyphoscoliosis. [ncbi.nlm.nih.gov]

Kyphoscoliosis can be a severe condition requiring specific kyphoscoliosis treatment. [cobackandspine.com]

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. [healthengine.com.au]

Proceedings: Prognosis for life in childhood scoliosis. Arch Dis Child. 1973 Oct;48(10):824–825. [PMC free article] [PubMed] [Google Scholar] Articles from Thorax are provided here courtesy of BMJ Publishing Group [ncbi.nlm.nih.gov]

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]

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]

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

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]

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]

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

Pathophysiology Pathological Findings Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology Bone, vertebra: Kyphoscoliosis: Gross natural [wikidoc.org]

Epidemiology, Risk Factors, and Pathophysiology Spinal curvature is the most common cause of chest wall deformity. The causes of chest wall deformity are shown in Box 63-1. [clinicalgate.com]

Pathophysiology of spinal deformities in neurofibromatosis. J Bone Joint Surg. 1994; 76 (): 692-700 Rezaian S.M. The incidence of scoliosis due to neurofibromatosis. Acta Orthop Scand. 1976; 147: 534-539 Holt R.T. Johnson J.R. [thespinejournalonline.com]

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]

It’s a procedure with metal rods or screws to prevent independent movement. This allows old and an new spinal material to form together. [slideshare.net]

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]

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.