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Acrocephalosyndactyly syndromes: a review
C D Prevel1, B L Eppley, M McCarty
1Division of Plastic and Orthopaedic Surgery, A. B. Chandler Medical Center, University of Kentucky, Lexington 40536, USA.
The Journal of Craniofacial Surgery
|July 1, 1997
Summary
Advances in molecular biology have refined the classification of acrocephalosyndactyly syndromes. This review details these syndromes, focusing on hand anomalies and reconstruction, emphasizing coordinated multidisciplinary care.
Area of Science:
- Genetics
- Molecular Biology
- Medical Genetics
Background:
- The classification of acrocephalosyndactyly syndromes has undergone significant revision over the past 25 years.
- Advances in molecular biology and genetic mapping have driven these changes.
Purpose of the Study:
- To provide an overview of major acrocephalosyndactyly syndromes and their current classification.
- To discuss associated anomalies and hand reconstruction techniques.
Main Methods:
- Review of current literature on acrocephalosyndactyly syndromes.
- Synthesis of information on genetic mapping, clinical manifestations, and surgical interventions.
Main Results:
- Updated classification of acrocephalosyndactyly syndromes based on genetic advancements.
- Detailed discussion of craniofacial, neurological, cardiac, pulmonary, visceral, and extremity anomalies, with a focus on hand anomalies.
Conclusions:
- Surgical reconstruction techniques, timing, and postoperative therapy for hand anomalies are crucial.
- Emphasizes the critical need for coordinated multidisciplinary care for patients with these complex disorders, particularly during the perioperative period.