TY - CHAP M1 - Book, Section TI - Meckel-Gruber Syndrome (MKS) A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Marciniak, Bruno A1 - Dalens, Bernard J. PY - 2006 T2 - Syndromes: Rapid Recognition and Perioperative Implications 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. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/03/29 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=58075575 ER -