When does idiopathic scoliosis begin, which sex is more likely affected, and at what age is it generally diagnosed? How does this differ from neuromuscular disease-related scoliosis?

Her curve is 75°. Is this cause for concern? How would you assess her pulmonary function? Do you need pulmonary function tests? What type of pulmonary dysfunction is most common with this level of scoliosis? Does it matter if her scoliosis is due to neuromuscular disease or is idiopathic?

Do you need more information on her cardiac status? What type of cardiac disease would she be at risk for, and how would you diagnose the extent of her cardiovascular compromise (if any)? How would your considerations differ in a patient with non-idiopathic scoliosis such as that associated with Duchene muscular dystrophy?

Her asthma is only a problem when she has a cold. She is maintained on beclomethasone (Qvar®) inhaler bid. She uses an albuterol inhaler when she has active wheezing. Do inhaled steroids require perioperative coverage with “stress steroids?” She was on oral steroids 2 months ago for 2 weeks. Will she need perioperative steroids? How will you prepare her for the operating room?

Should she receive erythropoietin preoperatively? What are the relative advantages/disadvantages to autologous donation of blood vs. acute isovolemic hemodilution? What other methods are available to avoid blood transfusion?

She is needle phobic; she is crying and keeps pulling her hand away as you try to look for an IV. Should you premedicate – with what medications? Would you use nitrous oxide to help start the IV?

Scoliosis is a complicated pathological problem that involves lateral curvature of the spine. Idiopathic adolescent scoliosis (AIS) is the most common form of scoliosis and is found in 1–3 % of children/adolescents between 10 and 16 years of age. This accounts for 70 % of all cases of scoliosis. Females are affected 3.6 times as often as males. Neuromuscular disease-related scoliosis can have many causes including neuropathic disease (cerebral palsy, syringomyelia), myopathic disease (muscular dystrophy, amyotonia congenita), neurofibromatosis, mesenchymal disorders (Marfan’s syndrome, Morquio disease, Still disease), or trauma. Although variable in nature, neuromuscular scoliosis often begins at an earlier age and progresses faster. It is ultimately more likely to require surgical correction.

Yes, there is cause for concern because curvatures of 65° or greater can cause significant restriction of ventilation. The need for pulmonary function testing would depend on her exercise tolerance. If she has excellent exercise tolerance, I would not pursue testing. As scoliosis becomes severe, the rotation of the vertebrae causes the ribs to form a rib hump and restrict the thoracic cage. Vital capacity, forced expiratory volume in 1 s (FEV ), and PaO are all decreased. The FEV-to-FVC ratio is largely unchanged. A preoperative vital capacity of less than 35 % is considered a significant predictor of respiratory compromise in the perioperative time frame. Lung function should be optimized prior to surgery. Any lower respiratory tract symptoms or signs (rales, rhonchi, wheezing) should prompt a thorough evaluation and postponement of surgery. In these cases 4–6 weeks should pass before administering general anesthesia for correction.

Patients with severe scoliosis are at risk for chronic hypoxia, pulmonary hypertension, and cor pulmonale. Hypoxic pulmonary vasoconstriction is an ongoing risk in patients with chronic hypoxia. Right ventricular hypertrophy and (eventually) cardiomyopathy can result. Any patient with known or suspected cardiac disease should have a preoperative cardiac evaluation. An ECG to evaluate for ischemia or axis deviation is indicated as is an echocardiogram to determine overall function and structural anomalies. Compromised patients will require intraoperative cardiac monitoring with consideration for transesophageal echocardiography during the operative case. A central line for trending of central venous pressures and administration of vasoactive medications would also be indicated. Patients with Duchene muscular dystrophy often have a dilated cardiomyopathy with compromised left ventricular function. In these cases, the cardiac compromise would be expected to be more marked than that with idiopathic scoliosis and accompanying cardiac dysfunction.

Inhaled corticosteroids such as beclomethasone, budesonide, fluticasone, and triamcinolone are the cornerstones of therapy for persistent asthma. While the use of these drugs can result in undeniable improvement in overall symptoms, inhaled corticosteroids are suppressive rather than curative. No clinically important adrenal suppression has been shown to occur with the administration of these medications in their recommended doses. Patients who have been taking systemic corticosteroids for more than 2 weeks in the prior 6 months are considered at risk for adrenal suppression in the setting of major surgery. In this case she would need perioperative systemic steroids. If she were recently on systemic steroids, I would obtain a pulmonary consult to check her current pulmonary function and her responsiveness to bronchodilators. I would prepare her by placing her on oral steroids for 4–5 days preoperatively and continuing for the week after surgery. I would also have her take her bronchodilator therapy during the perioperative period.

The use of perioperative erythropoietin therapy has been shown to increase hematocrit levels before and after scoliosis surgery. On the other hand studies have not shown that the administration of the drug decreases the exposure to transfused blood for patients undergoing scoliosis surgery. I would not administer the medication to this patient, but it could be considered in specific cases where preoperative anemia is a major concern. Autologous donation can be performed if the patient is over 50 kg and she is not anemic. The blood can be harvested from the patient and stored for as much as 6 weeks. Donation can occur twice weekly and can be done up to 72 h prior to surgery. There is some evidence that this practice can decrease the exposure to allogenic blood. It is critical to consider the extent of the surgery and the overall likelihood of transfusion. If the correction is going to be large and transfusion is a certainty, then I would offer autologous donation as an option for the patient. Acute normovolemic hemodilution (ANH) is a technique in which blood is removed from the patient around the time of induction of anesthesia. Circulating volume is maintained with colloid or crystalloid. As opposed to autologous transfusion, there is less chance of clerical error and administration of the incorrect blood – since it does not leave the operating room. In addition, the blood that is returned to the patient is fresh and contains all of the factors, 2,3 DPG, and normal electrolyte components of the patient’s blood.