Ellis–Van Creveld syndrome and its anesthetic implications

Abstract

Ellis–Van-Creveld syndrome (EVC), otherwise known as chondroectodermal or mesoectodermal dysplasia, is an autosomal recessive disorder found in the Amish population of Pennsylvania in the USA, with the incidence of 1:244,000 for the general population. The classical syndrome encompasses a tetrad of clinical manifestations, including di sproportionate dwarfism, postaxial polydactyly, ectodermal dysplasia (mainly affecting the teeth, nails, and hair), 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 girl who underwent surgical correction of supernumerary digits is presented here.

Keywords

• Anesthesia
• autosomal recessive
• chondroectodermal dysplasia
• disorders
• dwarfism
• Ellis–Van Creveld syndrome
• mesoectodermal dysplasia
• pediatric
• polydactyly