RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Marciniak, Bruno
A1 Dalens, Bernard J.
SR Print(0)
ID 58075575
T1 Meckel-Gruber Syndrome (MKS)
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=58075575
RD 2024/04/25
AB Characterized  by  postaxial  polydactyly  and  central  nervous  system  malformation  (encephalocele,  severe  hydrocephalus),  large  polycystic  kidneys,  and  liver  failure  (fibrosis).  Other  clinical  features  include  microcephaly,  abnormality  of  the  larynx  and  tongue,  severe  mandibular  micrognatism,  obesity,  and  cleft  lip/  palate.  Associated  abnormalities  include  oral  clefting  and  genital  anomalies.  Pulmonary  hypoplasia  is  the  leading  cause  of  death.