Ellis–Van Creveld syndrome and its anesthetic implications

Ellis–Van Creveld syndrome and its anesthetic implications

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...

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Bibliographic Details
Main Authors: Rajesh Gupta, Sangeeta Khanna, Meera Luthra, Yatin Mehta
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2018-01-01
Series:The Indian Anaesthetists' Forum
Subjects:
Anesthesia
autosomal recessive
chondroectodermal dysplasia
disorders
dwarfism
Ellis–Van Creveld syndrome
mesoectodermal dysplasia
pediatric
polydactyly
Online Access:http://www.theiaforum.org/article.asp?issn=2589-7934;year=2018;volume=19;issue=2;spage=85;epage=88;aulast=Gupta
Description
Summary: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.
ISSN:2589-7934
0973-0311

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