RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Marciniak, Bruno
A1 Dalens, Bernard J.
SR Print(0)
ID 58061232
T1 Achondrogenesis
T2 Syndromes: Rapid Recognition and Perioperative Implications
YR 2006
FD 2006
PB The McGraw-Hill Companies
PP New York, NY
SN 9780071354554
LK accessanesthesiology.mhmedical.com/content.aspx?aid=58061232
RD 2024/04/23
AB Very  rare  disorder  characterized  by  extreme  short-limbed  dwarfism,  lack  of  development  of  ribs,  and  other  major  bone  formation.  There  are  several  subtypes  of  achondrogenesis.  In  general  these  forms  are  characterized  by  premature  birth,  fetal  hydrops,  normocephaly  but  an  unusually  soft  cranial  vault,  and  a  short  neck  and  trunk.  Affected  individuals  have  extremely  short  limbs,  ribs,  and  vertebra.  The  disorder  often  is  life-threatening  either  in  utero  or  shortly  after  birth.