= CAFFEY DISEASE

= uncommon self-limiting proliferative bone disease of infancy; remission + exacerbations are common

[John Patrick Caffey (1895–1978), pediatrician and professor of radiology at Columbia University, New York]

Cause: ? infectious; ? autosomal dominant with variable expression + incomplete penetrance / sporadic occurrence (rare)

Age:<6 months, reported in utero; M÷F = 1÷1

Histo: inflammation of periosteal membrane, proliferation of osteoblasts + connective tissue cells, deposition of immature bony trabeculae

• sudden, hard, extremely tender soft-tissue swellings over bone
• irritability, fever; leukocytosis, anemia
• ± elevated ESR, increased alkaline phosphatase

Location: mandible (80%) >clavicle >ulna + others (except phalanges + vertebrae + round bones of wrists and ankles)

Site: hyperostosis affects diaphysis of tubular bones asymmetrically, epiphyses spared

• massive periosteal new-bone formation + perifocal soft-tissue swelling
• “double-exposed” ribs
• narrowing of medullary space (= proliferation of endosteum)
• bone expansion with remodeling of old cortex

Prognosis: usually complete recovery by 30 months

Rx: mild analgesics, steroids