Trevor disease

Trevor

disease 9 yr old girl Talus

School ager

with chronic ankle pain. AP and lateral radiographs of the ankle (above) and 3D CT without contrast of the ankle (below) show multiple intraarticular epiphyseal osteochondromas in the tibiotalar joint.The diagnosis was Trevor disease.

pediatricimaging.org • CC-by-nc-sa 4.0

Lesions

involving the outer surface of the bone in children: a pictorial review. Trevor’s disease (dysplasia epiphysealis hemimelica) in 3-year-old boy. Antero-posterior radiograph of the left foot a shows asymmetric osteochondral overgrowth arising from the medial epiphysis of the tibia (arrow) and from multiple ossification centers within the tarsal bones of the foot. An axial STIR image b and axial and coronal T1 images c and d demonstrate cortico-medullary coalescence and cartilaginous signal (arrow)

insightsimaging.springeropen.com • Open Access

Radiopaedia • CC-by-nc-sa 3.0 • de

Dysplasia epiphysealis hemimelica (DEH), also known as Trevor disease, is an extremely rare, non-hereditary disease that is characterized by osteochondromas arising from the epiphyses.

Epidemiology

The incidence is estimated at ~1:1,000,000 . There is a recognized male predilection (M:F = 3:1). It usually presents in young children.

Pathology

Classification

Azouz et al. has proposed the following classification:

classic form: characteristic hemimelic distribution involving more than one bone or epiphysis within a single lower extremity
localized form: single bone affection unilateral or bilateral
generalized form: involving the whole limb from pelvis to foot

Etiology

The etiology is uncertain but is thought to be congenital.

Location

lower extremity is more common than upper extremity
distal ends are more affected
medial aspects are affected twice as commonly as lateral
the distal femur is one of the most frequent sites
other sites are around the ankle joint

Radiographic features

Radiograph

Typically shows an irregular mass with focal ossification arising from one side of the affected epiphysis . This can result in widening of the affected joint.

CT

Useful for assessing cortical continuity with the epiphysis.

MRI

MRI is useful for evaluation of underlying ligaments and cartilages. Osteochondral overgrowth signal intensity will be similar to normal epiphyseal cartilage and bone.

Treatment and prognosis

Surgical excision of mass with preservation of joint.

Complications

fragmentation and detachment leading to intra-articular bony fragments
deformation of the ligaments and tendons affecting the joint mechanism
chronic tendonitis and tenosynovitis due to chronic irritation of tendons by mass or bony fragments
development of secondary osteoarthritis of affected joint

History and etymology

After French surgeon Albert Mouchet (1869-1963) and Joseph Belot (1876-1953) were the first to describe the condition in 1926 , it was elaborated on by David Trevor (1906-1988), an English orthopedic surgeon from London . Later on, in 1956, Thomas John Fairbank (1912-1998) coined the name "dysplasia epiphysealis hemimelica" because of the medial or lateral half predisposition within a limb .