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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2018-01-01
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doaj-01b5ac42679d4c6a8febd4de8a4bd8012020-11-25T02:28:30ZengWolters Kluwer Medknow PublicationsThe Indian Anaesthetists' Forum2589-79340973-03112018-01-01192858810.4103/TheIAForum.TheIAForum_37_18Ellis–Van Creveld syndrome and its anesthetic implicationsRajesh GuptaSangeeta KhannaMeera LuthraYatin MehtaEllis–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.http://www.theiaforum.org/article.asp?issn=2589-7934;year=2018;volume=19;issue=2;spage=85;epage=88;aulast=GuptaAnesthesiaautosomal recessivechondroectodermal dysplasiadisordersdwarfismEllis–Van Creveld syndromemesoectodermal dysplasiapediatricpolydactyly |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Rajesh Gupta Sangeeta Khanna Meera Luthra Yatin Mehta |
spellingShingle |
Rajesh Gupta Sangeeta Khanna Meera Luthra Yatin Mehta Ellis–Van Creveld syndrome and its anesthetic implications The Indian Anaesthetists' Forum Anesthesia autosomal recessive chondroectodermal dysplasia disorders dwarfism Ellis–Van Creveld syndrome mesoectodermal dysplasia pediatric polydactyly |
author_facet |
Rajesh Gupta Sangeeta Khanna Meera Luthra Yatin Mehta |
author_sort |
Rajesh Gupta |
title |
Ellis–Van Creveld syndrome and its anesthetic implications |
title_short |
Ellis–Van Creveld syndrome and its anesthetic implications |
title_full |
Ellis–Van Creveld syndrome and its anesthetic implications |
title_fullStr |
Ellis–Van Creveld syndrome and its anesthetic implications |
title_full_unstemmed |
Ellis–Van Creveld syndrome and its anesthetic implications |
title_sort |
ellis–van creveld syndrome and its anesthetic implications |
publisher |
Wolters Kluwer Medknow Publications |
series |
The Indian Anaesthetists' Forum |
issn |
2589-7934 0973-0311 |
publishDate |
2018-01-01 |
description |
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. |
topic |
Anesthesia autosomal recessive chondroectodermal dysplasia disorders dwarfism Ellis–Van Creveld syndrome mesoectodermal dysplasia pediatric polydactyly |
url |
http://www.theiaforum.org/article.asp?issn=2589-7934;year=2018;volume=19;issue=2;spage=85;epage=88;aulast=Gupta |
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