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- Title
Ellis Van Creveld Syndrome: Cardiac Anomalies and Anesthetic Implications.
- Authors
Guha, Amrita; Malhotra, Rohit; Saxena, Rachit; Keshri, Vikas K.
- Abstract
Ellis-Van Creveld syndrome (EVC), also known as mesoectodermal dysplasia, is a rare autosomal recessive disorder with a tetrad of clinical features, comprising dwarfism, polydactyly, ectodermal dysplasia with sparse hair, hypoplastic nails and enamel, hypodontia and conical teeth and congenital heart disease (CHD). We report an 18-year-old girl with short stature and polydactyly, who got admitted to our hospital with shortness of breath on exertion for the last 2 years. On echocardiography, a partial atrioventricular canal (AV canal) defect was diagnosed, which was repaired surgically. The patient had an uneventful perioperative period.
- Subjects
ECTODERMAL dysplasia; CONGENITAL heart disease; NAIL diseases; SHORT stature; NAIL polish; SYNDROMES; HYPODONTIA
- Publication
Annals of Cardiac Anaesthesia, 2023, Vol 26, Issue 3, p346
- ISSN
0971-9784
- Publication type
Article
- DOI
10.4103/aca.aca_166_22