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Apert Syndrome Type III Hand: Prevalence and Outcomes
Cassio E Raposo-Amaral1,2, Lucas L Medeiros1, Cesar A Raposo-Amaral1
1Institute of Plastic and Craniofacial Surgery, SOBRAPAR Hospital, Campinas.
The Journal of Craniofacial Surgery
|February 2, 2023
Summary
Upton type III Apert hands are the most common, not least prevalent. A three-stage protocol successfully created a 5-digit hand in 87% of patients with type III Apert hands.
Area of Science:
- Plastic Surgery
- Orthopedic Surgery
- Genetics
Background:
- Apert syndrome is a genetic disorder characterized by craniosynostosis and syndactyly.
- The Upton type III hand is the most severe form of syndactyly in Apert syndrome.
- Previously, type III hands were considered the least prevalent among Apert syndrome patients.
Purpose of the Study:
- To determine the prevalence of Upton type III hands in Apert syndrome patients.
- To describe surgical treatment strategies for achieving a 5-digit hand in type III Apert hands.
Main Methods:
- Retrospective review of 15 years of Apert syndrome hand surgeries.
- Data collection included demographics, surgical techniques, and outcomes.
- Exclusion criteria: incomplete records or <6 months follow-up.
Main Results:
- 40 out of 93 Apert patients (43%) presented with Upton type III hands.
- A 5-digit hand was achieved in 35 (87%) of these patients.
- Average of 3.37 operations per patient was required.
Conclusions:
- Upton type III hands are the most prevalent among Apert syndrome patients.
- A three-stage surgical protocol can consistently achieve a 5-digit hand in most type III Apert hands.
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