Trevors syndrome

Amy Henkel, M.D.

LUMC

Cindy Rigsby, M.D.

Abstract

2 year old male with limp and left ankle swelling

Keywords

1502 Trevor's dysplasia epiphysealis hemimelica SPR unknown case 4


Publication Date: 2009-02-25

History

2 year old male with limp and left ankle swelling.

Findings

Radiographs of the left ankle demonstrate irregular ossific fragments medial and inferior to the medial distal tibia. There is also sclerosis and irregularity of the adjacent bone as well as the lateral distal tibial epiphysis and dome of the talus.

Radiographs of the left knee demonstrate an ossific mass extending from the distal medial femoral epiphysis into the medial joint space.

Coronal SPGR weighted image with axial and sagittal reformatted images demonstrate a cartilaginous mass with multiple internal ossific foci arising from the medial aspect of the distal femoral epiphysis.

Sagittal and Coronal T2 weighted images also demonstrating the mass arising from the medial aspect of the distal femoral epiphysis. The mass mirrors the signal characteristics of cartilage. It narrows the medial joint space and flattens the medial meniscus.

Similar cartilaginous lesions were found on MR of the ankle corresponding to the radiographic abnormalities.

Diagnosis

Dysplasia Epiphysialis Hemimelica (Trevor's Disease or Trevor Fairbank Disease)

Discussion

Trevor's disease is a rare disorder affecting the epiphyses in children. The lesion consists of osteochondral overgrowth of one or more epiphyses, usually in a lower extremity. The lesion typically involves either the medial or lateral apspect of the epiphysis, hence the term hemimelic. The medial aspect is more commonly involved.

The incidence of DEH has been estimated at 1 case per million population. Boys are affected up to 3 times more frequently than girls. The etiology is unknown but is thought to be congenital.

Trevor's disease typically presents in childhood with painless swelling. The lesions continue to grow until skeletal maturity. The condition is benign, and there have been no reported cases of malignant transformation. However, if left untreated there will be progression to pain and arthrosis of the affected joint. Treatment is usually surgical.

The classic radiographic appearance is of asymmetric epiphyseal growth with multiple ossific centers. CT can be used to more accurately define the anatomy. MR is used as an adjuvant to radiographs for depiction of the lesions as well as the effects on the surrounding joint, including ligments, tendons, menisci and articular surfaces. It is particularly useful in pre-operative planning.

References

  1. Peduto, AJ, et al. MR imaging of dysplasia epiphysealis hemimelica: bony and soft-tissue abnormalities. AJR. 1999;Vol 172, 819-823.
  2. Kuo RS, et al. Dysplasia epiphysealis hemimelica: clinical features and management. J Pediatr Orthop. 1998; 18(4):543-548.
  3. Keret, D., et al. Dysplasia epiphysealis hemimelica: diagnosis and treatment. J Pediatr Orthop. 1992; 12:365-372.

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