RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164078111 T1 Meckel-Gruber Syndrome (MKS) 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=1164078111 RD 2024/03/29 AB It is 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 micrognathism, obesity, and cleft lip/palate. Associated abnormalities include oligohydramnios, oral clefting, and genital anomalies. Pulmonary hypoplasia is the leading cause of death.