CLINICAL CASES Free access

Minerva Anestesiologica 2003 May;69(3):169-75

Copyright © 2003 EDIZIONI MINERVA MEDICA

language: English, Italian

Sedation for Magnetic Resonance Imaging in a child affected with Williams syndrome

Baldinelli F., De Carli A., Accinelli G.

1st Service of Anesthesia and Resuscitation, “San Maurizio” Regional General Hospital, Bolzano, Italy

Williams syn­drome (WS) is a ­rare genet­i­cal dis­or­der ­with an inci­dence of 20-50 000 ­live ­births. It is ­caused by a dela­tion of 1 elas­tin ­allele locat­ed with­in chro­mo­some sub­unit 7q11.23 (long arm). It is char­ac­ter­ized by: supra­val­vu­lar aor­tic sten­o­sis, mul­ti­ple periph­er­al pul­mo­nary ­artery sten­o­sis, a typ­i­cal ­face (elf­ine ­face), men­tal and stat­ural defi­cien­cy, char­ac­ter­is­tic den­tal mal­for­ma­tion, tran­sient hyper­cal­ce­mia ­that ­occurs dur­ing the 1st ­year of ­life. We ­present the ­case of a 7-­month-old ­infant affect­ed ­with WS. In ­order to clar­i­fy the car­diac find­ings, the ­baby ­under-­went a MRI inves­ti­ga­tion, requir­ing an anes­the­sio­log­i­cal assis­tance. In ­this ­case a ­deep seda­tion ­approach was car­ried out by giv­ing chlor­al ­hydrate 10% per os (80 mg/kg). We did not per­form a gen­er­al anes­the­sia in ­order to ­avoid the ­risk relat­ed to it. No oth­er ­drugs ­were ­used. During the pro­ce­dure SpO2, HR, RR, and ­ETCO2 ­were in nor­mal ­range; ST ­tract anal­y­sis did not ­reveal any path­o­log­i­cal ­change The exam­ina­tion last­ed 95 minutes; at the end the ­baby was ­kept ­under sur­veil­lance in the recov­ery ­room for 30 minutes, he ­could be pre­co­cious­ly fed. All the pro­ce­dure was unevent­ful.