Dysplasia Epiphysealis Hemimelica Treatment & Management

  • Author: Khalid A Bakarman, MD, MB, BCh, SBIO; Chief Editor: Harris Gellman, MD   more...
 
Updated: May 6, 2010
 

Medical Therapy

Supportive joint care, consisting of short-term splinting of the joint, may be beneficial.

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Surgical Therapy

Most reported cases of DEH in the literature have been treated surgically, with surgical treatment consisting of excision of the mass, as well as correction of any angular deformity, while preserving the integrity of the affected joint as much as possible.[26, 27]

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Follow-up

After surgery, the affected limb requires a short period of immobilization with a cast or splint, followed by active range of motion of the joint.

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Complications

  • Recurrence of the angular deformity after the corrective osteotomy - May be anticipated if the growth plate at the affected joint is open and active and the lesion itself has not been removed[23, 28]
  • Degenerative osteoarthritis
  • Fixed deformity
  • Leg-length discrepancy[4, 28, 29]
  • Loose bodies
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Outcome and Prognosis

DEH, although an uncommon condition, can result in considerable disability because of direct involvement of the articular surface of the joint. Treatment with surgical excision offers the best results, but corrective osteotomy and reoperation for recurrent lesion may also be required.[30, 31]

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Contributor Information and Disclosures
Author

Khalid A Bakarman, MD, MB, BCh, SBIO  Pediatric Orthopedic Consultant, King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia

Disclosure: Nothing to disclose.

Coauthor(s)

Robert Mervyn Letts, MD, FRCS(C), FACS  Former Chief, Department of Surgery, Division of Pediatric Orthopedics, Children's Hospital of Eastern Ontario, University of Ottawa; Consultant Pediatric Orthopedic Surgeon, Sheikh Khalifa Medical City, UAE

Disclosure: Nothing to disclose.

Specialty Editor Board

Howard A Chansky, MD  Associate Professor, Department of Orthopedics and Sports Medicine, University of Washington Medical Center

Howard A Chansky, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Sean P Scully, MD, PhD  Professor, Department of Orthopedics, University of Miami

Sean P Scully, MD, PhD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, International Society on Thrombosis and Haemostasis, and Society of Surgical Oncology

Disclosure: Nothing to disclose.

Dinesh Patel, MD, FACS  Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital

Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD  Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami School of Medicine

Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, and Arkansas Medical Society

Disclosure: Nothing to disclose.

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Anteroposterior and lateral radiographs of the right ankle of a child aged 3.5 years showing a lesion in the medial dome of the talus and the epiphyseal part of fibula. It is causing ankle pain and deformity.
Anteroposterior radiograph of a child at age 7 years (same patient as in the image above). The radiograph depicts an increase in the size of the lesion where it was excised, but it did recur at age 12 years, at which time the lesion was removed.
Anteroposterior and lateral radiographs of the right ankle of a child aged 3.5 years revealing a lesion in the medial dome of the talus and the epiphyseal part of fibula. The lesion causes ankle pain and deformity.
 
 
 
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