RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164074272
T1 Johanson-Blizzard Syndrome (JBS)
T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9781259861789
LK accessanesthesiology.mhmedical.com/content.aspx?aid=1164074272
RD 2024/04/24
AB A  polymalformative  syndrome  characterized  by  nasal  alar  hypoplasia  (beak  shaped),  scalp  defects,  hypothyroidism,  pancreatic  achylia,  congenital  heart  defects,  and  congenital  sensorineural  deafness.  Usually  detected  at  birth  when  beak-shaped  nasal  defect  is  noticed  in  association  with  pancreatic  insufficiency  and  the  presence  of  an  imperforate  anus.  Important  features  in  anesthesia  include  in  these  infants  distinctive  craniofacial  abnormalities  with  aplasia  or  hypoplasia  of  the  nasal  alae,  maxillary  hypoplasia,  a  small  pointed  chin,  and  small  opening  of  the  mouth  and  microcephaly.