Trevor’s Disease: A Literature Review regarding Classification, Treatment, and Prognosis apropos of a Case

Abstract

Background. Dysplasia epiphysealis hemimelica (DEH) is rare and its main characteristic is osteochondromas of the epiphysis of long bones. Methods. We report a case of DEH of the ankle in an 8-year-old boy that was resected in 2005. Additionally we collect all the reported cases of DEH. The literature is reviewed regarding the treatment, prognosis, long term function, and patterns and areas affected by DEH. Results. In our case no complications were noted and our patient remains asymptomatic. Reviewing the literature we found that 73 authors have reported 144 cases from 1926 to 2013. We propose and describe a new classification that correlates with prognosis. According to our classification DEH is classified as types 1 with single lower limb involvement, 2 with multiple lower limb, 3 with single upper limb, 4 with multiple upper limb, 5 with upper and lower limb, and 6 with spine. Conclusions. All single lesions should be followed up and if indicated a whole body nuclear bone scan can be useful in identifying the existence of multiple affected joints. Type 1 lesions have better prognosis than 2 and have less chances of developing OA even if not resected. Resection, even if partial, can be a successful treatment for DEH.

Trevor's disease of the distal radioulnar joint in two children with achondroplasia

“…We report two cases of DEH affecting the DRUJ in two female children with familial achondroplasia. Both cases presented with range of motion losses, but only one had noticeable functional (Arealis et al, 2014). For our patient with pain and functional changes (Patient 1), surgery provided significant relief and return of function.…”

“…It is progressive, but there have been no documented cases of malignant transformation (Arealis et al, 2014;Azouz, Slomic, Marton, Rigault, & Finidori, 1985;Lo et al, 2018). The bulk of current understanding for DEH comes from roughly 150 sporadic cases, most involving the lower extremities (knees and ankles) (Arealis et al, 2014;Azouz et al, 1985;Gökkuş et al, 2017;Lo et al, 2018), with fewer than 40 documented cases in the upper extremities (Lo et al, 2018). To our knowledge, no instances of DEH have been reported in achondroplasia.…”

“…This pediatric condition is nonhereditary and thought to have an incidence of one per one million, affecting males to females 3:1 (Gökkuş et al, 2017;Lo, Wong, & Chan, 2018;Wheeldon & Altiok, 2015). It is progressive, but there have been no documented cases of malignant transformation (Arealis et al, 2014;Azouz, Slomic, Marton, Rigault, & Finidori, 1985;Lo et al, 2018). The bulk of current understanding for DEH comes from roughly 150 sporadic cases, most involving the lower extremities (knees and ankles) (Arealis et al, 2014;Azouz et al, 1985;Gökkuş et al, 2017;Lo et al, 2018), with fewer than 40 documented cases in the upper extremities (Lo et al, 2018).…”

Case Report: Trevor Disease in a Child’s Ankle: 3 Years of Postoperative Follow-up

“…Surgery is indicated when the joint blockage leads the patient to have pain and functional impairment. Even after the removal of bone mass, recurrence is frequent, and further resections may be required [2][3][4].…”

Dysplasia epiphysealis hemimelica of the ankle: a fracture-like rare developmental disorder

“…It results from an abnormal control of cell proliferation in the epiphysis leading to bone overgrowth with a cartilage cap that projects into the adjacent joint 5 6. The most commonly affected lower limb joint is the ankle (43.2%) 7. According to Azouz et al ,8 DEH is classified into 3 groups: localised, that affects only one epiphysis; classic, that affects more than one epiphysis in the same limb; and generalised, that involves the entire lower limb.…”