Pediatric Anesthesia A Brief Review of Common Disorders

1. Pediatric AnesthesiaA Brief Review of Common Disorders By: Christine Mazza, T4

2. Diseases with Difficult Airways • Pierre Robin – micrognathia, glossoptosis, cleft palate • Nasal airway, tongue suture, LMA • Pre-op cardiac eval required • Treacher-Collins – facial and pharyngeal hypoplasia, aplasticzygomatic arches, micrognathia, choanalatresia • Narrowing of airway above larynx, LMA useful • Pre-op cardiac eval required • Hurler’s – short thick neck, course facies, macroglossia, large tonsils and adenoids • Post-op subglottic edema common • Cardiac eval pre-op

3. Diseases with Difficult Airways • Goldenhar Syndrome – unilateral (usually) disruption in formation of craniofacial structures and vertebral abnormalities • Difficult airway • Pre-op eval of heart and c-spine • TE fistula – part of VATER syndrome • Awake intubation preferred • Insert tube deeply and pull back until fistula is occluded but both lungs are ventilated • CDH – dyspnea, cyanosis, scaphoid abdomen, BS in chest • Use minimal pressure ventilation to avoid pneumothorax; if sudden cyanosis, assume pneumothorax

4. Review in Pictures

5. Cardiac Abnormalities • Cyanotic Defects (R  L) – defect between arterial and venous circulation with outflow obstruction on venous side • Tetralogy of Fallot, truncusarteriosis, transposition of the great vessels • Pre-op treatment of dz, endocarditis prophylaxis, remove air bubbles from IV tubing • OXYGEN! Hydration, gentle ventilation • Acyanotic Defects (L  R) – single defect between arterial and venous circulation so that blood flows from high to low pressure • ASD, VSD, PDA, cushion defects • Pre-op treatment of dz, endocarditis prophylaxis, remove air bubbles from IV tubing • PPV, low FiO2 as tolerated • PPHN – persistence or return of fetal circulation after birth • Basically same treatment as Cyanotic defects above • Increase pulm blood flow, hyperventilate

6. Genetic/Hematological Diseases • Hemophilia A – most common inherited coagulation disorder • Oral medications, general anesthesia • Intubation acceptable, but be wary of hemorrhage • Sickle Cell • Pre-op: HYDRATION, transfuse to increase Hct to 30 • Intra-op: HYDRATION, oxygenation, replace blood loss when necessary • Porphyria – demyelination, flaccid paralysis, weakness, autonomic dysfunction • NO barbituates, steroids, etomidate, enflurane, ketamine • Ok to use opiates, local, N2O, isoflurane, helothane, atropine • Exaggerated response to muscle relaxants • Trisomy 21 – microcephaly, macroglossia, CHD, duodenal atresia, atlanto-axial instability, congenital subglotticstenosis • Difficult airway –consider ETT 1 size down • Decreased MAC, exaggerated response to muscle relaxants • Pre-op cardiac work up, c-spine films?

7. Resources • Bell, C.; Kain, Z. The Pediatric Anesthesia Handbook Mosby-Year Book, Inc, St. Louis: 1997. • Infosino, Andrew. “Pediatric Upper Airways and Congenital Anomalies”. Anesthesiology Clinicals of North America. 20 (2002): 747-766. • Macksey, Lynn Fitzgerald. Pediatric Anesthetic and Emergency Drug Guide. Jones and Bartlett Publishers, Ontario Canada: 2009. • Stoelting, R; Dierdorf, S. Handbook for Anesthesia and Co-Existing Disease. Churchill Livingstone Publishing, Philadelphia: 2002. • http://www.theaba.org/Home/anesthesiology_initial_certification