Shepherd’s crook deformity: Do you recognize this sign?

Seabra Rato, Maria; Bernardo, Alexandra; Oliveira Pinheiro, Filipe; Bernardes, Miguel; Costa, Lúcia

doi:10.46497/archrheumatol.2023.9869

Cited by 1 publication

(1 citation statement)

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“…Normal bone matrix and marrow is replaced by disorganised fibro-osseus tissue, which can involve single (Monostotic FD) to multiple bones (polyostotic FD). 1 It accounts for approximately 7% of all benign bone tumours. McCune–Albright syndrome and Mazabraud syndrome (MS) are rare entities within the polyostotic FD spectrum with MAS accounting for less than 5% of polyostotic FD.…”

Section: Introductionmentioning

confidence: 99%

Fibrous dysplasia: A tale of two syndromes

Fourie,

Suleman,

Lockhat

et al. 2024

South African Journal of Radiology

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Fibrous dysplasia (FD) is a rare, non-inherited, congenital bone disorder which may be monostotic or polyostotic. The polyostotic form may rarely present in syndromic forms when associated with extra-skeletal manifestations. Mazabraud syndrome is a rare syndrome consisting of polyostotic FD presenting with intramuscular myxomas. McCune–Albright syndrome is recognised by polyostotic FD, precocious puberty and ‘café au lait’ spots. This report describes an adult patient with Mazabraud syndrome and a child with McCune–Albright syndrome.Contribution: Radiographic findings are typical with bowing deformities, sclerotic, lucent or mixed lesions and bony expansion, often with endosteal scalloping. MRI is often non-contributory and may actually mimic a more aggressive process. Early detection and correct diagnosis allow for early preventative treatment and rehabilitation to prevent devastating neurological sequelae and disability.

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Section: Introductionmentioning

confidence: 99%