1. Gabriella Bluett-Mills March 8, 2012

2.  Usually right sided  Generally involves 7-10 vertebrae  >100 causes severe cardiac and respiratory dysfuction  <65 respiratory impairment is minimal

3.  Scoliosis causes restrictive lung disease by lateral rotation of the spine.  Can be idiopathic or secondary to neuromuscular disease.  If vital capacity is >70%, respiratory reserve should be adequate postop  If vital capacity is <40%, postop ventilation will probably be necessary

4.  Restrictive lung disease  Causes increased A-a gradient, alveolar hypoventilation, and hypoxemia  PaCo2 is usually normal  ↓ vital capacity, ↓ TLC, ↓ RV, ↓ FRC,↑Vd/Vt  ↓ FEV1, ↓FVC, normal FEV1/FEC

5.  Chronic hypoxemia  PTN and cor pulmonale  EKG changes  RVH  RBBB  Righ axis deviation

6.  Mitral valve prolapse seen in 25% of children affected

7.  Tests  PFTs  ABG- hypoxemia, hypercarbia, acidosis exacerbate PTN  CXR to check for signs of chronic aspiration pneumonia  Treat infection/bronchospasm prior to surgery  Obtain autologous blood

8.  Avoid N20 since it can worsen PTN  CVP monitoring to assess fluid status  Be prepared for pneumothorax

9.  Ventilatory weaning postop should be slow and cautious  If vital capacity is <40%, postop ventilation is necessary

10.  Paralysis  Hemorrhage  Fat and air embolism  Pneumothorax

11.  Propanalol and captoril decrease total dose of SNP  Sodium nitroprusside generally preferable to nitroglycerin for reliable and sustained induction of hypotension in children and adolescents  Labetalol is effective and not associated with tachycardia, intrapulmonary shunt or increased CO

12.  After a narcotic base is established small increments of naloxone are administered until the patient responds to verbal commands and moves lower extremities  Assistant holds the head and ET tube

13.  Minimizes need for donor blood  Begin three weeks before operation, with 4-7 days between collections to allow for adjustment in blood volume