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[Infantile cortical hyperostosis (the Caffey-Silvermann syndrome)]
K Ciharová1, J Horák, K Bláhová
1I. dĕtská klinika fakultní nemocnice, Praha-Motol.
Ceskoslovenska Pediatrie
|February 1, 1991
Summary
Infantile cortical hyperostosis (Caffey-Silvermann syndrome) affected six infants, primarily before four months old. This study details clinical and pathological findings, including elevated inflammatory markers and bone involvement, highlighting diagnostic challenges.
Area of Science:
- Pediatrics
- Medical Genetics
- Radiology
Background:
- Infantile cortical hyperostosis, also known as Caffey-Silvermann syndrome, is a rare disorder characterized by bone overgrowth.
- Early diagnosis and understanding of its clinical presentation are crucial for appropriate management.
Observation:
- A retrospective review of six pediatric patients diagnosed with infantile cortical hyperostosis between 1984 and 1989.
- All affected infants developed symptoms before 4.5 months of age, with a notable male predominance (5 out of 6).
- The study documented non-familial occurrences of the condition.
Findings:
- Pathological findings included elevated erythrocyte sedimentation rate (ESR) in 5/6 patients, leukocytosis in 5/6, thrombocytosis in 3/4, eosinophilia in 3/6, and elevated alkaline phosphatase in 2/6.
- Skeletal involvement was most frequently observed in the mandible (four cases), with one case each of hip joint and multifocal involvement.
- One detailed case presented with significant clavicular involvement, initially misdiagnosed as a bone tumor.
Implications:
- The findings underscore the importance of considering infantile cortical hyperostosis in the differential diagnosis of bone abnormalities in infants.
- Understanding the spectrum of clinical and laboratory findings aids in accurate diagnosis and management of Caffey-Silvermann syndrome.
- This case series contributes to the literature on the presentation and diagnostic considerations of this rare pediatric condition.