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Dysplasia Epiphysealis Hemimelica Treatment & Management

  • Author: David A Forsh, MD; Chief Editor: Harris Gellman, MD  more...
 
Updated: May 25, 2016
 

Approach Considerations

Dysplasia epiphysealis hemimelica (DEH), or Trevor disease, should be treated if the lesion is causing deformity, pain, or interference with function.

Supportive joint care, consisting of short-term splinting of the joint, may be beneficial in management. Most cases are treated surgically, but recurrence is common.[27, 35, 36] The most common procedures are excision of the mass and corrective osteotomy.[28]  Excision may be arthroscopic or open. Corrective osteotomy and, subsequently, hemiepiphysiodesis, have been described for correction of deformity.[28, 21]  Surgery is contraindicated if no medical symptoms or no mechanical block is present.

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Surgical Therapy

Most reported cases of DEH in the literature have been treated surgically. Surgical treatment has involved excising the mass and correcting any angular deformity while preserving the integrity of the affected joint to the extent possible.[37, 38]

Oberc et al reported on their experience in treating DEH in six children.[39] Of the four patients treated surgically, two experienced late complications. Of the two patients treated conservatively, one had a positive outcome and the other a negative outcome involving hip ankylosis. The authors advised that conservative treatment involving physical therapy be employed initially in the region of pain and that surgical treatment be considered if pain, joint deformation, or limited range of motion persists.

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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.[28, 29]

Other potential complications include the following:

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Long-Term Monitoring

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

Bosch et al conducted a retrospective study of nine patients with DEH who were treated surgically and were followed up clinically with imaging.[41] Magnetic resonance imaging (MRI) was found to be useful for identifying a potential plane of cleavage between the epiphysis and the pathologic tissue. The investigators recommended early removal of ossifications when a cleavage plane is identified and advised against waiting for a possible complication or for an increase in size.

Because of the risk of recurrence, patients should be followed until skeletal maturity.[22]

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Contributor Information and Disclosures
Author

David A Forsh, MD Chief, Orthopedic Trauma Surgery, Assistant Professor, Department of Orthopedic Surgery, Icahn School of Medicine at Mount Sinai

David A Forsh, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, Orthopaedic Trauma Association, AO North America

Disclosure: Nothing to disclose.

Coauthor(s)

Meredith Bartelstein, MD Resident Physician, Department of Orthopedic Surgery, Mount Sinai Hospital

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

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, Leonard M Miller School of Medicine, Clinical Professor, Surgery, Nova Southeastern 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, Arkansas Medical Society

Disclosure: Nothing to disclose.

Additional Contributors

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.

Dennis P Grogan, MD Clinical Professor (Retired), Department of Orthopedic Surgery, University of South Florida College of Medicine; Orthopedic Surgeon, Department of Orthopedic Surgery, Shriners Hospital for Children of Tampa

Dennis P Grogan, MD is a member of the following medical societies: American Medical Association, American Orthopaedic Association, Scoliosis Research Society, Irish American Orthopaedic Society, Pediatric Orthopaedic Society of North America, American Academy of Orthopaedic Surgeons, American Orthopaedic Foot and Ankle Society, Eastern Orthopaedic Association

Disclosure: Nothing to disclose.

Acknowledgements

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

Disclosure: Nothing to disclose.

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.

References
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Anteroposterior view of left foot. Medial ankle and subtalar joints are significantly involved with epiphyseal growth irregularities.
Oblique view of left foot. Medial ankle and subtalar joints are significantly involved with epiphyseal growth irregularities.
Lateral view of left foot. Medial ankle and subtalar joints are significantly involved with epiphyseal growth irregularities.
Anteroposterior view of left ankle of 12-year-old boy. Medial ankle and subtalar joints and first metatarsophalangeal joints have large epiphyseal osteocartilaginous growths causing significant anatomic changes and clinical symptoms. Large lesion at first metatarsophalangeal joint required excision.
Anteroposterior view of left ankle of 12-year-old boy. Medial ankle and subtalar joints and first metatarsophalangeal joints have large epiphyseal osteocartilaginous growths causing significant anatomic changes and clinical symptoms. Large lesion at first metatarsophalangeal joint required excision.
Lateral view of left foot of 12-year-old boy. Medial ankle and subtalar joints and first metatarsophalangeal joints have large epiphyseal osteocartilaginous growths causing significant anatomic changes and clinical symptoms. Large lesion at first metatarsophalangeal joint required excision.
Three-dimensional reconstruction images of left foot (seen in radiographs of 12-year-old boy's foot) better demonstrate numerous epiphyseal abnormalities, especially at lateral tibial-talar and first metatarsal-phalangeal joints. Entire subtalar joint is also involved. Smaller irregularities are seen in nearly all epiphyses of foot.
Three-dimensional reconstruction images of left foot (seen in radiographs of 12-year-old boy's foot) better demonstrate numerous epiphyseal abnormalities, especially at lateral tibial-talar and first metatarsal-phalangeal joints. Entire subtalar joint is also involved. Smaller irregularities are seen in nearly all epiphyses of foot.
 
 
 
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