Abstract
The spectrum of prenatal cortical hyperostosis includes a mild phenotype that typically
presents after 35 weeks of gestation, and a severe form that presents earlier. The
skeletal and systemic manifestations of the severe phenotype remain unexplained. A
review of reported cases indicates that older mothers and firstborn infants are overrepresented.
This combination suggests decreased fertility. Fourteen years after the birth of the
present case, his mother presented with renal failure from multiple myeloma raising
the possibility that a maternal antibody may play a role in the etiology of severe
prenatal Caffey disease. The present case report is also intended to alert clinicians
to potential difficulties with tracheal intubation secondary to micrognathia from
mandibular involvement during a critical growth period.
Keywords
prenatal Caffey disease - maternal age - birth order - difficult intubation