Anesthetic implications of Ellis-van Creveld syndrome.
Ellis-van Creveld syndrome (chondroectodermal or mesoectodermal dysplasia) is an autosomal recessive disorder. Initial reports of the syndrome identified a tetrad of clinical manifestations, including chondrodysplasia, postaxial polydactyly, ectodermal dysplasia, and congenital heart disease (CHD). Additional involvement may occur in organs of endodermal origin, such as the pulmonary, renal, gastrointestinal (hepatic and pancreatic), hematologic, and central nervous systems. The perioperative care of a 2-year, 8-month-old girl who underwent surgical correction of CHD is presented.
Abeles
Andrew I
AI
University of Missouri School of Medicine, Columbia, MO 65212, USA.
Tobias
Joseph D
JD
D000758:Anesthesia / Q000379:methods*; D002675:Child, Preschool; D004613:Ellis-Van Creveld Syndrome / Q000503:physiopathology*; D005260:Female; D006330:Heart Defects, Congenital / Q000209:etiology / Q000601:surgery*; D006801:Humans; D019990:Perioperative Care / Q000379:methods