Pediatric AnesthesiaA Brief Review of Common Disorders

By: Christine Mazza, T4

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, aplastic zygomatic arches, micrognathia, choanal atresia– 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

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

Review in Pictures

Cardiac Abnormalities• Cyanotic Defects (R L) – defect between arterial and venous circulation with outflow

obstruction on venous side– Tetralogy of Fallot, truncus arteriosis, 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

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 subglottic stenosis– Difficult airway –consider ETT 1 size down– Decreased MAC, exaggerated response to muscle relaxants– Pre-op cardiac work up, c-spine films?

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