Dysplasia epiphysealis hemimelica (Trevor’s disease): a rare case report with oral manifestations

Asha ML, Nagaraj BN, Arun Kumar G, Ashok ML

Abstract


Dysplasia epiphysealis hemimelica, also known as Trevor’s disease, is an extremely rare skeletal developmental disorder of unknown etiology, characterized by an osteocartilaginous outgrowth of one or more epiphyses or of a tarsal bone during childhood. It is a rare skeletal hemimelica disorder characterized by asymmetric growth of cartilage in one or more epiphyses. Due to the unusual presentation and variability of the picture, there is no standardized treatment and outcome is very different. Also such an unusual, unique case with craniofacial manifestations has not been reported in the literature. We report a case of a 14-year-old male, who complained of irregularly placed teeth in upper and lower front teeth region. On general physical examination we noticed some skeletal abnormalities with the patient and hence we subjected him to radiographic investigations. The images were consistent with epiphyseal dysplasia hemimelica. The prognosis of DEH is variable and depends basically on its location and size. Carriers of this unusual dysplasia should be periodically monitored for the risk of recurrence.


Keywords


Epiphyseal dysplasia hemimelica, Epiphysis, Trevor’s disease

Full Text:

PDF

References


Mouchet A, Belot J. La tarsomegalie. J Radiol Electrol. 1926;10:289-93.

Trevor D. Tarso-epiphysial aclasis: a congenital error of epiphyseal development. J Bone Joint Surg Br. 1950;32.B(2):204-13.

Fairbank TJ. Dysplasia epiphysialis hemimelica (tarso-ephiphysial aclasis). J Bone Joint Surg Br. 1956;38.B(1):237-57.

Masquijo JJ, Willis B. Dysplasia epiphysealis hemimelica (Trevor’s disease). Arch Argent Pediatr. 2010;108(1):e20-3.

Azouz EM, Slomic AM, Marton D, Rigault P, Finidori G. The variable manifestations of dysplasia epiphysealis hemimelica. Pediatr Radiol. 1985;15(1):44-9.

Björk A. Timing of interceptive orthodontic measures based on stages of maturation. Trans Eur Orthod Soc. 1972:61-74.

Keret D, Spatz DK, Caro PA, Mason DE. Hemimelica epiphysealis dysplasia: diagnosis and treatment. J Pediatr Orthop. 1992;12:365-72.

Trevor D. Tarso-epiphysial aclasis. A congenital error of epiphysial development. J Bone Joint Surg. 1950;32:204-13.

Segal LS, Vrahas MS, Schwentker EP. Dysplasia epiphysealis hemimelica of the sacroiliac joint: a case report. Clin Orthop Relat Res. 1996;333:202-7.

Kuo RS, Bellemore MC, Monsell FP, Frawley K, Kozlowski K. Dysplasia epiphysealis hemimelica: clinical features and management. J Pediatr Orthop. 1998;18:543-8.

Baumfeld D, Pires R, Macedo B, Abreu-E-Silva G, Alves T, Raduan F, et al. Trevor Disease (Hemimelic epiphyseal displasia): 12-year follow-up case report and literature review. Ann Med Health Sci Res. 2014;4(Suppl 1):S9-13.

Smith EL, Raney EM, Matzkin EG, Fillman RR, Yandow SM. Trevor’s disease: the clinical manifestations and treatment of dysplasia epiphysealis hemimelica. J Pediatr Orthop B. 2007;16:97-102.

Skaggs DL, Moon CN, Kay RM, Peterson HA. Dysplasia epiphysealis hemimelica of the acetabulum: a report of two cases. J Bone Joint Surg. 2000;82A:409-14.