Fact-sheet: Hereditary Multiple Osteochondromas - Exostoses
  • Multiple exostoses disease
  • Bessel-Hagen disease


Updated on 03/28/2019 at 9:28 AM

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Definition

Hereditary Multiple Osteochondromas - Exostoses = Bessel-Hagen disease

Autosomal dominant hereditary bone dysplasia

Prevalence 1/20,000

Clinical features

Bilateral involvement, +/- symmetric, predominantly affecting the appendicular skeleton.

Possibly asymptomatic.

Discovered in childhood/adolescence: hard growth(s), short stature

Possible degeneration into chondrosarcoma

Laboratory findings

Normal

Radiography

- Multiple sessile or pedunculated osteochondromas, mainly on long bones (identical semiology to a solitary osteochondroma). - Metaphyseal remodeling disorders (femoral and/or humeral metaphyseal widening).

- Associated Madelung deformity / shortening of the distal ulna. - Bone deformities.

CT

Same as radiographs.

- Better analysis of the cortico-medullary continuity of exostoses, neurovascular relationships, and distant calcifications of a thick cartilaginous cap (poor prognostic sign)

Management

MRI if degeneration of an osteochondroma into chondrosarcoma is suspected (in 5 to 30% of cases, suspected if pain/increase in size/cartilaginous cap > 2cm).

Differential diagnosis

- Solitary osteochondroma.