General:
• Term for a variety of lesions associated with deletion of the long arm of chromosome 22
• C Cardiac anomalies
• A Abnormal facies
• T T-cell deficit from hypoplastic thymus
• C Cleft palate
• H Hypocalcemia due to hypoparathyroidism
Airway:
• Abnormal facies
• Cleft palate
• Choanal atresia
• Micrognathia
• Short trachea
Cardiovascular:
• Conotruncal abnormalities
o Truncus arteriosus
o TOF
o Interrupted aortic arch
Gastrointestinal:
• Esophageal atresia
• Imperforate anus
• Diaphragmatic hernia
Neurological:
• Hypocalcemic tetany
• Seizures
Other
• Cell-mediated immune deficiency
• Abnormal catecholamine metabolism
Considerations:
• Difficult intubation
• Irradiate blood products - avoid GVH disease
• SBE prophylaxis
• Beware of epinephrine injections
Links:
• DiGeorge syndrome
• Shprintzen syndrome(velocardiofacial syndrome)
• Conotruncal anomaly face syndrome(Takao syndrome)
References:
Thursday, July 8, 2010
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