title: Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases.
abstract: BACKGROUND
  Dysplasia epiphysealis hemimelica (DEH) is a rare developmental bone disorder with hemimelic involvement of one or more epiphysis. We report on nine new cases and discuss the clinical manifestations, the value of MRI, and the results of complete and early surgical resection of these lesions.
  
  
  METHODS
  In this retrospective study, nine patients with a diagnosis of DEH were evaluated. Age at presentation ranged from 1 year to 12 years. The main complaint at diagnosis was a swelling bony mass. Angular deformities were recorded in two patients. All patients were surgically treated and followed up clinically and by imaging. Eight patients underwent excision only.
  
  
  RESULTS
  The average follow-up was 5.6 years (range, 2-10.5 years). All patients had a good outcome without related symptoms. No epiphysiodesis, angular deformity or recurrence was observed. One patient with femoral lesion involving the distal medial part of the epiphysis developed, four months after surgical excision, a calcification outside the area of total excision. This calcification did not increase in size at two years follow-up. Another patient with lateral involvement of the proximal tibial epiphysis presented a postoperative nervous complication. Spontaneous nervous recovery occurred three months after surgery.
  
  
  CONCLUSIONS
  MRI was useful to find a potential plane of cleavage between the epiphysis and the pathological tissue. We recommend early removing ossifications when a cleavage plane is identified. Waiting a possible complication or increasing of size does not seem logical. Of course, the treatment will be not the same if no cleavage plane is found on MRI.
  
  
  METHODS
  IV.
authors.affiliation: Department of Pediatric Orthopaedics Surgery, University hospital of Montpellier, 34295 Montpellier, France.
authors.forename: C
authors.initials: C
authors.lastname: Bosch
authors.affiliation: Department of Pediatric Orthopedics Surgery, University hospital of Beirut, Beirut, Lebanon.
authors.forename: C
authors.initials: C
authors.lastname: Assi
authors.affiliation: Department of Pediatric Orthopaedics Surgery, University hospital of Montpellier, 34295 Montpellier, France.
authors.forename: D
authors.initials: D
authors.lastname: Louahem
authors.affiliation: Department of Pediatric Orthopaedics Surgery, University hospital of Montpellier, 34295 Montpellier, France.
authors.forename: F
authors.initials: F
authors.lastname: Alkar
authors.affiliation: Department of Pediatric Orthopaedics Surgery, University hospital of Montpellier, 34295 Montpellier, France.
authors.forename: P
authors.initials: P
authors.lastname: Mazeau
authors.affiliation: Department of Histopathology, University hospital of Montpellier, 34295 Montpellier, France.
authors.forename: C
authors.initials: C
authors.lastname: Delfour
authors.affiliation: Department of Pediatric Surgery, University hospital of Clermont-Ferrand, 69003 Clermont-Ferrand, France.
authors.forename: F
authors.initials: F
authors.lastname: Canavese
authors.affiliation: Department of Pediatric Radiology, University hospital of Montpellier, 34295 Montpellier, France.
authors.forename: O
authors.initials: O
authors.lastname: Prodhomme
authors.affiliation: Department of Pediatric Orthopaedics Surgery, University hospital of Montpellier, 34295 Montpellier, France. Electronic address: jerome.cottalorda@gmail.com.
authors.forename: J
authors.initials: J
authors.lastname: Cottalorda
mesh_terms: D001848:Bone Diseases, Developmental / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D005260:Female; D005269:Femur / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D011183:Postoperative Complications; D011859:Radiography; D019651:Plastic Surgery Procedures; D012008:Recurrence; D012189:Retrospective Studies; D013977:Tibia / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery
keywords: Bone tumor; Children; Dysplasia epiphysealis hemimelica; Trevor's disease