# Defying the Odds: Survival in Severe Prenatal Caffey's Disease

## TL;DR

A premature infant with severe prenatal Caffey's disease survived and showed full recovery, offering hope for early diagnosis and management of this rare condition.

## Contribution

This case presents one of the earliest documented survivors of severe prenatal Caffey's disease with long-term follow-up.

## Key findings

- The infant showed near-complete bone remodeling by one year and full radiographic resolution by two years.
- The child achieved normal growth and ambulation after a prolonged neonatal intensive care stay.
- The case suggests that skeletal manifestations of prenatal Caffey's disease can resolve spontaneously after managing prematurity complications.

## Abstract

Caffey’s disease, or infantile cortical hyperostosis, is a rare disorder characterized by subperiosteal new bone formation and diffuse soft tissue inflammation in infants younger than six months. A much rarer prenatal form presents before birth with diffuse, symmetric cortical thickening and often results in perinatal death, especially when it develops early in gestation. The severe form may be associated with pulmonary hypoplasia, hepatic dysfunction, and high neonatal mortality. We report the case of a premature infant born at 27 weeks’ gestation to a 36-year-old mother with chronic hypertension. Prenatal ultrasound demonstrated polyhydramnios, fetal hydrops, and abnormal long-bone ossification. Postnatal radiographs showed diffuse “cotton-wool” periosteal reactions involving all long bones and ribs, consistent with prenatal Caffey’s disease. Differential diagnoses such as osteogenesis imperfecta, congenital infection, and skeletal dysplasia were excluded through laboratory and genetic testing. The infant required prolonged ventilatory support but gradually improved and was discharged after a 19-week neonatal intensive care stay. At one-year follow-up, imaging showed near-complete remodeling of affected bones with only mild humeral deformity, and by two years, the child demonstrated normal growth, ambulation, and complete radiographic resolution. With diagnosis at 27 weeks, this case highlights one of the earliest documented survivors of severe prenatal Caffey's disease and suggests that once complications of prematurity are managed, the skeletal manifestations can resolve spontaneously. Awareness of this condition may help avoid misdiagnosis and guide appropriate counseling and follow-up.