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.
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 . 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 . In this context, it has been estimated that about 70% of congenital vertebral anomalies result in progressive deformities . Besides the asymmetry of the thoracic wall and the deviation of the vertebral column – which may increase several degrees per year  – 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  . Respiratory muscle strength may be severely reduced , 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 . Furthermore, thoracic defects are more likely to lead to neurological deficits than lumbar anomalies .
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 . 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 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]
(see Hypoxemia, [[Hypoxemia]]) Normocapnia or 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 [mdnxs.com]
The patient also has severe hypoxemia, with 75% peripheral oxygen saturation (SpO2). [medpagetoday.com]
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Liver, Gall & Pancreas
Developmental defects due to disturbed somitogenesis and impaired formation of the axial skeleton may be detected during prenatal ultrasound examinations . 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 . In any case, the presence of single or multiple hemivertebrae and wedge vertebrae, unilateral unsegmented bars, block vertebrae and butterfly vertebrae may be visualized . Magnetic resonance imaging may be indicated to assess the condition of the spinal cord and to identify comorbidities such as diastematomyelia  . Diagnostic imaging may also be employed to depict acquired pathologies of the skeleton, e.g., rickets, osteopenia, and osteoporosis.
(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.  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. Neuraxial blockade in our patient has complications such as unpredictable block, risk of high [mjdrdypu.org]
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.  The severity of the deformity is best determined by measuring Cobb's angle. [joacp.org]
Edema of the airway results in increased potential for bleeding and smaller sized endotracheal tubes should be used for general anesthesia. Patients with scoliosis suffer from restrictive lung disease which involves decreased vital capacity, functional [mjdrdypu.org]
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