RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164067547 T1 De Morsier Syndrome 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=1164067547 RD 2024/10/09 AB It is a rare genetic medical condition characterized by a triad of optic nerve hypoplasia, structural brain abnormalities, and hypothalamic/pituitary deficiencies. To establish the diagnosis, two of the three features must be present. All three are only present in 30% of affected individuals. Neuroradiologically, this syndrome is defined by agenesis of the interhemispheric septum pellucidum, schizencephaly, hydranencephaly, and holoprosencephaly. Clinically, other important features include mild to severe developmental delay, seizures, visual impairment, sleep disturbance, precocious puberty, microgenitalia (especially in males), obesity, anosmia, sensorineural hearing loss, and cardiac anomalies.