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Published on: February 5, 2019
Indomethacin treatment of infantile cortical periostosis in twins
R T Couper1, A McPhee, L Morris
1University of Adelaide, Department of Paediatrics, Women's & Children's Hospital, North Adelaide, South Australia, Australia. rcouper@medicine.adelaide.edu.au
Insights
Infantile cortical periostosis (Caffey's disease) in twins presented with limb swelling, fever, and irritability. Indomethacin effectively managed relapses, allowing cessation of prednisolone therapy.
Area of Science:
- Pediatrics
- Genetics
- Rheumatology
Background:
- Infantile cortical periostosis (Caffey's disease) is a rare disorder characterized by bone inflammation.
- Early diagnosis and management are crucial for affected infants.
Observation:
- Twin infants presented with symptoms of Caffey's disease at 2 and 3 weeks of age.
- Initial symptoms included limb swelling, fever, irritability, and bone tenderness.
- Radiographic evidence revealed extensive periosteal new bone formation.
Findings:
- The infants experienced multiple relapses within the first year of life.
- Mandibular and clavicular involvement were observed during some relapses.
- Prednisolone treatment was initiated but later replaced by indomethacin.
- Indomethacin therapy at 3 mg/kg/day proved effective in controlling disease flares and allowed discontinuation of prednisolone.
Implications:
- This case highlights the successful use of indomethacin in managing recurrent infantile cortical periostosis.
- Indomethacin offers a viable alternative for controlling disease activity and reducing reliance on corticosteroids.
- Further research into optimal treatment strategies for Caffey's disease is warranted.
Abstract:
Twin girls presented with infantile cortical periostosis (Caffey's disease) at 2 and 3 weeks of age, respectively. This disorder initially involved their upper and lower limbs and resulted in fever, irritability and tenderness. X-rays showed extensive periosteal new bone formation. Multiple relapses occurred in the first year of life and during some of these relapses mandibular and clavicular involvement was noted. Prednisolone, 1 mg/kg per day, was used to treat relapses until 9 months of age. Indomethacin therapy at this age at a dose of 3 mg/kg per day allowed the cessation of prednisolone therapy and disease flares were thereafter infrequent and responsive to indomethacin.
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