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.

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