Hereditary multiple diaphyseal sclerosis
Lesions
involving the outer surface of the bone in children: a pictorial review. Ribbing disease. Lateral radiograph of right tibia a shows smooth thickening along the anterior cortex; this radiograph was obtained when the patient was 19 years of age. 7 years later, a follow-up lateral radiograph obtained as a young adult b shows further increased thickening along the anterior tibial cortex with narrowing of the medullary cavity. Axial c and sagittal d PD MR images of the tibia obtained at the same time as the second radiograph reveal smooth cortical thickening anteriorly. No unusual abnormal uptake of radiotracer was noted on the concomitantly performed Tc-99m MDP labeled bone scan e
Hereditary multiple diaphyseal sclerosis, also known as Ribbing disease, is a rare sclerosing dysplasia.
Epidemiology
It is more common in females and typically occurs in middle-aged individuals.
Clinical presentation
It may be painful or asymptomatic.
Pathology
Location
Ribbing disease predominantly involves the diaphysis of long tubular bones, most commonly the tibia and femur. The epiphyses are spared. The skull is generally not involved.
It can be unilateral or bilateral .
Radiographic features
Plain radiograph
- intramedullary sclerosis
- cortical thickening and sclerosis at the periosteal and endosteal surface
CT
- similar to radiograph findings
- no periosteal reaction or soft tissue component
MRI
- areas of sclerosis correspond to low signal intensity on T1WI
- short inversion time inversion recovery (STIR) may show high signal intensity due to marrow edema
Nuclear medicine
- increased uptake on Tc-99 m bone scan
History and etymology
The condition was first reported by Ribbing in 1949.
Differential diagnosis
Possible imaging differential considerations include
Siehe auch:
Assoziationen und Differentialdiagnosen zu Ribbing-Syndrom:
