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Chronic Caffey's disease: an uncommon entity in children
S A Rahman1, Y J Chowdhury, M Rahman
1Paediatrics, Bangabandhu Sheikh Mujib Medical University.
Insights
Chronic Caffey's disease, a rare pediatric condition, involves periosteal inflammation and systemic issues. This case highlights key diagnostic features in a young child, aiding future recognition of this uncommon disorder.
Area of Science:
- Pediatric Rheumatology
- Pediatric Endocrinology
Background:
- Chronic Caffey's disease is a rare pediatric condition characterized by periosteal inflammation and systemic disturbances.
- Early diagnosis and management are crucial for optimal outcomes in affected children.
Observation:
- A 30-month-old boy presented with congenital painful soft tissue swellings and developmental delay.
- Clinical examination revealed fever, tender mandibular swellings, limb deformities, and bowing of lower limbs.
Findings:
- Laboratory tests showed normal serum calcium and phosphate with mildly elevated alkaline phosphatase.
- Radiological imaging demonstrated periosteal new bone formation, diaphyseal expansion of long bones, and anterior rib expansion.
Implications:
- This case underscores the importance of considering chronic Caffey's disease in the differential diagnosis of pediatric inflammatory bone disorders.
- Accurate diagnosis based on clinical presentation and imaging is essential for appropriate management and to prevent long-term complications.
Abstract:
Chronic Caffey's disease in an uncommon condition in children is characterized by an acute inflammatory reaction in the periosteum along with systemic disturbances. A 30 months old boy was reported in the pediatric unit of BSMMU, Dhaka about two and half years back with the complaints of multiple painful soft tissue swelling in different parts of the body since birth and delay in growth and development. The child was found well and alert, moderately pale, febrile with hard, tender swelling of mandible on both sides. There were multiple swellings over the right arm, forearm, both thighs and bowing of the lower limbes. Investigations revealed normal serum calcium and phosphate level with mild elevation of alkaline phosphatase. Radiological findings showed periosteal new bone formation in mandible and long bones. There was diaphyseal expansion of the long bones with expansion of the ribs anteriorly. He was diagnosed as a case of chronic caffey's disease on the basis of history, clinical examination and investigation.
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