Ribbing-Syndrom

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

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