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Arch Dis Child 90:711 doi:10.1136/adc.2004.065334
  • Images in paediatrics

Infantile cortical hyperostosis

  1. D Suri,
  2. D Dayal,
  3. M Singh
  1. Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh-160012, India; meenusingh4rediffmail.com

      A 14 week old male infant presented with multiple tender bony swellings involving the legs, forearm, and lower jaw since 1 month of age (fig 1). No history of fever, trauma, or child abuse was forthcoming. He was irritable and had difficulty in feeding since the appearance of the jaw swelling. Bowing of the lower limbs with pseudoparalysis was observed. There was no response to adequate vitamin C supplements received prior to hospitalisation. Investigations showed mild increase of ESR, normal blood biochemistry, sterile cultures, and negative parental serology for syphilis. Bone radiographs revealed periosteal elevation, new bone formation, and cortical thickening involving the diaphyses of bilateral tibia, ulna, and femur (fig 2). The characteristic triad of irritability, swelling, and bone lesions, age at presentation, and mandibular involvement clinched the diagnosis.

      Infantile cortical hyperostosis or Caffey’s disease classically presents in infants less than 5 months of age, though has also been reported to occur in utero. Mandibular involvement is seen in 75–80%. Radiological findings are diagnostic; however, other conditions with similar clinicoradiological features such as scurvy, osteomyleitis, syphilitic osteochondritis, hypervitaminosis A, metastatic neuroblastoma, and battered baby syndrome need to be excluded. Treatment is palliative with NSAIDs, and sometimes steroids. Prognosis is good and disease resolves without any sequelae in 6–9 months. Early recognition helps to avoid unnecessary treatment.


      Graphic
      Figure 1

       Bony swellings of mandible and limbs.


      Graphic
      Figure 2

       Radiographs showing periosteal elevation and hyperostosis.

      Footnotes

      • Parental consent was obtained for publication of figure 1