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The elbow in syndromic craniosynostosis
P J Anderson1, C M Hall, R D Evans
1Craniofacial Centre, Great Ormond Street Hospital for Children, London, UK.
The Journal of Craniofacial Surgery
|August 7, 1998
Summary
Elbow anomalies are common in Apert's and Pfeiffer's syndromes, but not Saethre-Chotzen syndrome. This study highlights the need for orthopedic review in managing these craniosynostosis-related conditions.
Area of Science:
- Orthopedics
- Genetics
- Pediatric Surgery
Background:
- Craniosynostosis is associated with over 100 syndromes, often including limb anomalies.
- Elbow anomalies are reported in Apert's, Pfeiffer's, Crouzon's, and Saethre-Chotzen syndromes, but their prevalence and severity are not well-documented.
Purpose of the Study:
- To determine the types, incidence, and severity of elbow anomalies in patients with specific craniosynostosis syndromes.
- To compare elbow anomaly findings across Apert's, Pfeiffer's, Crouzon's, and Saethre-Chotzen syndromes.
Main Methods:
- Prospective radiographic study conducted over 12 months.
- Inclusion of patients from Great Ormond Street Hospital's Craniofacial Centre with Apert's, Pfeiffer's, Crouzon's, or Saethre-Chotzen syndrome.
Main Results:
- Elbow anomalies were frequent in Apert's and Pfeiffer's syndromes, less common in Crouzon's syndrome.
- No elbow anomalies were observed in patients with Saethre-Chotzen syndrome.
- Complete synostosis was the most severe anomaly noted in Apert's, Pfeiffer's, and Crouzon's syndromes, potentially requiring surgical intervention.
Conclusions:
- The incidence of elbow anomalies in Apert's, Pfeiffer's, and Crouzon's syndromes appears higher than previously reported.
- Severe elbow synostosis may necessitate orthopedic management, especially as affected individuals increasingly survive into adulthood.