Prenatal Caffey Disease Case Report and Review of the Literature
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- Category: Vol. 82, September 2014
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Prenatal Caffey Disease Case Report and Review of the Literature, MOHAMED AL-HARBI, NAHLA BARAKAT, WALEED SHABAAN and HALA YOUSEF
Abstract
Background: Two forms of Caffey disease or cortical hyperostosis were described, a severe prenatal form and a milder infantile one. Both are characterized by massive periosteal bone formation that typically involve the diaphyses of long bones.
Case Report: We report a 26 week +4 days old baby girl whose mother had severe polyhydramnios. The baby was born with hydrops fetalis and some dysmorphic features. During routine chest X-ray, periosteal hyperostosis was observed in the ribs and both humeral diaphyses. Skeletal survey showed symmetrical extensive hyperostotic changes affecting the diaphyses of long bones, ribs, skull base and the mandible. These features were consistent radiologically with Caffey disease. The baby had lung hypoplasia, needed high ventilator parameters throughout her life and expired on day 33 of life.
Conclusion: Prenatal Caffey disease is a rare condition which when presented before the age of 35 weeks of gestation is characterized by extensive periosteal hyperostotic bone involvement, angulations and shortening of long bones, polyhydramnios, and hydrops fetalis with high lethality due to prematurity and lung hypoplasia.