TY - CHAP M1 - Book, Section TI - Meckel-Gruber Syndrome (MKS) A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e 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. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164078111 ER -