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A 3 year-old with a history of cleft palate and a seizure during the neonatal
period presents for anesthesia for a brain MRI as part of the ongoing work up of
his delayed speech and motor development. You note a harsh, holosystolic
murmur. Is an echocardiogram prior to anesthesia important for this patient?

a) Yes

b) No
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==Answer==
Answer a. The chromosome 22q11.2 deletion syndrome (velocardiofacial or
DiGeorge syndrome) occurs in 1 in 3,000 children. Associated phenotypes
include abnormal facies, cleft palate, thymic hypoplasia and T-cell
immunodeficiency, hypocalcemia and cardiac defects. Conotruncal anomalies
and outflow tract anomalies are frequent but myriad cardiac defects may occur
and the majority of children with this syndrome have some cardiac defect. T-cell
immunodeficiency requires strict aseptic technique for line placement and use of
irradiated blood for transfusion. Hypocalcemia is common at birth, and may
cause neonatal seizures. Learning and behavioral disorders are common but may
not be recognized until school age.
==Notes==
<references />
:This question originally printed in the [http://www.amazon.com/Justin-L.-Lockman/e/B00FLG5AEE/ref=ntt_dp_epwbk_0 Pediatric Anesthesiology Review Topics] kindle book series, and appears courtesy of Naerthwyn Press, LLC.
==Keywords==
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