TY - CHAP M1 - Book, Section TI - Lujan Fryns Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - Inherited syndrome with marfanoid features and X-linked mental retardation. It is characterized by mild-to-moderate mental retardation, marfanoid development, tall and thin stature with long and slender limbs. Other features include arachnodactyly, joint hyperextensibility, narrow face, small chin, large testes, hypotonia, and malformations of the brain and the heart. The association with an agenesis or partial development of the corpus callosum is present in more than 75% of affected individuals. The most common heart defects include spontaneous dilation of the aortic root with acute aortic valve insufficiency, dissection of the ascending aorta, and to a lesser degree ventricular and atrial septal defects. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=1164077258 ER -