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Two case reports with literature review of the EEC syndrome: Clinical presentation and management

Marcello Augello, Britt-Isabelle Berg, Andreas Albert Müller1

  • 1Department of Oral and Cranio-Maxillofacial Surgery, University Hospital Basel, Basel, Switzerland.

Case Reports in Plastic Surgery & Hand Surgery
|June 3, 2016
PubMed

Insights

This study details the "Basel concept" for cleft lip/palate repair in EEC syndrome siblings. Early, one-stage closure at 3-4 months aims to restore normal anatomy and muscle function.

Area of Science:

  • Craniofacial surgery
  • Pediatric plastic surgery
  • Genetics and rare diseases

Background:

  • Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome presents complex craniofacial anomalies.
  • Cleft lip and palate repair requires careful consideration of anatomical and functional outcomes.
  • Early intervention is crucial for optimal development in children with EEC syndrome.

Observation:

  • The study focuses on siblings diagnosed with EEC syndrome.
  • The
  • Basel concept
  • for surgical repair was applied.
  • This approach involves early, one-stage closure of all cleft components.

Findings:

  • Surgical repair was performed at 3-4 months of age.
  • The
  • Basel concept
  • aims to establish normal anatomical and muscular conditions early.
  • This strategy facilitates improved functional outcomes.

Implications:

  • Early, one-stage repair may optimize functional results for EEC syndrome patients.
  • The
  • Basel concept
  • offers a standardized approach for complex cleft lip/palate cases.
  • Further research can validate long-term benefits of this early intervention strategy.

Keywords:
EEC syndromelip adhesion“all-in-one”-concept

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