Dysplasia epiphysealis hemimelica of the distal ulna: a case report and review of the literature
- PDF / 1,897,271 Bytes
- 5 Pages / 595.228 x 841.842 pts (A4) Page_size
- 3 Downloads / 217 Views
EUROPEAN JOURNAL OF MEDICAL RESEARCH
Eur J Med Res (2009) 14: 272-276
June 18, 2009 © I. Holzapfel Publishers 2009
DYSPLASIA EPIPHYSEALIS HEMIMELICA OF THE DISTAL ULNA: A CASE REPORT AND REVIEW OF THE LITERATURE T. Vogel 1, T. Skuban 2, C. Kirchhoff 3, A. Baur-Melnyk 4, S. Siegert 5, B. Heimkes 2 1 Department of Orthopedic and Trauma Surgery, Ruhr-University, Bochum, Germany 2 Department of Orthopedics, Ludwig-Maximilians-University, Munich, Germany 3 Depertment of Sports Orthopedics, Technical University, Munich, Germany 4 Department of Radiology, Ludwig-Maximilians-University, Munich, Germany 5 Department of Pathology, Ludwig-Maximilians University, Munich, Germany
Abstract Dysplasia epiphysealis hemimelica (DEH) or Trevor’s Disease is a very rare disease with an estimated incidence of one in 1.000.000. The majority of cases reported affect the lower limb and only 25 case reports of 33 cases with affection of the upper limb have been published. Here we present a case of DEH affecting the distal ulnar epiphysis and the lunate in an eleven-year-old girl, a DEH location described extremely rarely before. We firstly do not only present clinical and radiological findings (plane radiographs, CT, MRI), but also the surgical approach and the histopathological results of DEH in this uncommon location. Although extremely rare, DEH should be considered also in non-typical locations.
INTRODUCTION
Dysplasia epiphysealis hemimelica (DEH) or Trevor’s Disease is an uncommon developmental abnormality in young children, characterized by unilateral eccentric overgrowth of an epiphysis. Although most cases of this rare condition are reported on the lower limb, up to date a total of 25 case reports of 33 cases with affection of the upper limb have been published. Reported here is a case of DEH affecting the distal epiphysis of the ulna. Clinical, radiological (plane radiographs, CT, MRI) and histological signs as well as operative treatment will be discussed.
CASE REPORT
An eleven-year-old girl was seen in November 2006 in our Department of Pediatric Orthopedics with a painful non-traumatic swelling of the ulnar aspect of the left wrist. Clinical examination revealed a bony tumor on the distal end of the left ulna without any signs of inflammation. Active range of motion was significantly reduced due to pain and passive range of motion was limited to five degrees ulna abduction and 20 degrees dorsal extension. Supination and pronation was limited to five and 10 degrees, respectively. Flexion and radial abduction were within normal ranges. There was no family history of bone dysplasia. Plane radiographs of the left wrist (ap and lateral view) showed a
massive enlarged distal ulnar epiphysis, the metaphysis seemed to be unaffected (Fig. 1). Furthermore it showed a dorsal subluxation of the ulna and irregularities of the lunate. The radial epiphysis as well as the other carpometacarpal bones appeared normal. The typical radiological picture, together with our clinical findings, led to the suspicion of DEH, although the appearan
Data Loading...
