Sporadic congenital infantile cortical hyperostosis (Caffey's disease)

• Published in: American journal of perinatology Vol. 15; no. 11; p. 629
• Main Authors: Al-Tawil, K I, Ahmed, G S, Al-Hathal, M M, Al-Zuwayed, M A
• Format: Journal Article
• Language: English
• Published: United States 1998
• Subjects: Fatal Outcome; Female; Humans; Hydrops Fetalis - complications; Hyperostosis, Cortical, Congenital - complications; Hyperostosis, Cortical, Congenital - diagnosis; Hyperostosis, Cortical, Congenital - therapy; Infant, Newborn; Male; Pregnancy; Respiration, Artificial; Ultrasonography, Prenatal
• ISSN: 0735-1631
• DOI: 10.1055/s-2007-994081

Prognosis of congenital infantile cortical hyperostosis (Caffey's disease) is poor particularly in premature babies. Two cases are presented of congenital Caffey's disease in premature babies. The first baby was hydropic at birth and had cortical hyperostosis involving the mandible and long bones of right upper limb and both lower limbs. The second baby had cortical hyperostosis of the nasal bones causing severe nasal nonchoanal stenosis that needed surgery, in addition to involvement of long bones of the four extremities. Both babies recovered from the disease and were discharged home well. These cases suggest that the improved outcome of congenital of infantile cortical hyperostosis may reflect improvement of neonatal mechanical ventilation and availability of neonatal total parenteral nutrition.