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Published on: December 9, 2016
Monostotic femoral Caffey disease masquerading as Ewing sarcoma
Madhuri Kumari1, Surya Nandan Prasad2, Anamika Meena1
1Department of Radiodiagnosis, All India Institute of Medical Sciences - Patna, Patna, Bihar, India.
Insights
This case study details infantile cortical hyperostosis (Caffey disease) in an infant
Area of Science:
- Pediatric Radiology
- Pediatric Orthopedics
- Medical Imaging
Background:
- Infantile cortical hyperostosis (Caffey disease) is a rare disorder characterized by bone inflammation.
- Monostotic involvement, affecting a single bone, is less common than polyostotic forms.
Observation:
- A case of monostotic Caffey disease affecting the left femur in an infant is presented.
- Initial radiographs revealed a lamellated periosteal reaction mimicking a bone tumor.
- MRI demonstrated significant muscle edema without signs of infection or malignancy.
Findings:
- Serial radiographs showed progressive new bone formation characteristic of Caffey disease.
- MRI played a crucial role in differentiating Caffey disease from differential diagnoses like infection and neoplasia.
- The infant experienced a favorable outcome with symptomatic treatment and gradual bony remodeling.
Implications:
- This case highlights the importance of recognizing serial radiographic changes in monostotic Caffey disease.
- MRI is valuable for accurate diagnosis and exclusion of mimics in suspected cases.
- Understanding these imaging features aids in appropriate patient management and parental counseling.
Abstract:
We describe a rare case of monostotic infantile cortical hyperostosis (Caffey disease) involving the left femur of an infant, who presented with recent onset left thigh swelling, following vaccination. Radiological workup showed a lamellated periosteal reaction involving the left femoral diaphysis on radiographs masquerading as a bone tumour. The child underwent MRI of the left thigh, which showed extensive muscle oedema without any abnormal soft-tissue proliferation, marrow signal alteration, cortical breach or collection. The follow-up radiograph showed exuberant new bone formation in the second week. The patient was given symptomatic treatment and the parents were counselled. The child recovered well with gradual resolution of symptoms and bony remodelling on a 6-month follow-up radiograph. Here, we describe the serial changes on the radiographs in Caffey disease with monostotic involvement and the role of MRI in difficult cases to differentiate it from other common mimickers, such as infections and neoplasia.

