A Case Report on Ellis–van Creveld Syndrome: Clinical, Embryological, Anesthetic, and Surgical Implications

Abstract: Postaxial polydactyly (PAP) in the form of rudimentary soft tissue masses is quite common. Management involves ligation or surgical excision. Rarely do literature discussions cover complex variants in which the extra finger is fully developed. Ellis–van Creveld syndrome (EVC) or chondroectodermal dysplasia is a rare disorder characterized by PAP. When chondral dysplasia encompasses PAP, dwarfism, and genu valgum, ectodermal dysplasia involves nails and teeth. We describe two EVC cases. When one had cardiac malformations, the other had dental anomalies. One of them underwent genu valgum correction and removal of the additional finger. The hand surgery proved complex due to fusion of its metacarpal with the fifth metacarpal. Pediatric Outcomes Data Collection Instrument (PODCI) scores at 1.5 and 2.5 years after hand and knee surgeries were excellent. A multidisciplinary approach is essential to achieve comprehensive care. Additional embryological research is necessary to elucidate the clinical manifestations described in this report.

Standort
Deutsche Nationalbibliothek Frankfurt am Main
Umfang
Online-Ressource
Sprache
Englisch

Erschienen in
A Case Report on Ellis–van Creveld Syndrome: Clinical, Embryological, Anesthetic, and Surgical Implications ; volume:57 ; number:S 01 ; year:2024 ; pages:S106-S110 ; 01
Indian journal of plastic surgery ; 57, Heft S 01 (2024), S106-S110, 01

Beteiligte Personen und Organisationen
Mohan, Monusha
Sabapathy, S. Raja

DOI
10.1055/s-0044-1793845
URN
urn:nbn:de:101:1-2502201023020.106933984656
Rechteinformation
Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
Letzte Aktualisierung
15.08.2025, 07:24 MESZ

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Beteiligte

  • Mohan, Monusha
  • Sabapathy, S. Raja

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