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Dysplasia epiphysealis hemimelica
Acta Orthopaedica Scandinavica
|April 1, 1976
Summary
Dysplasia epiphysealis hemimelica (DEH) cases showed no further growth after epiphyseal fusion. Surgical resection of enlarged femoral condyles in DEH patients had no harmful consequences, with good long-term outcomes.
Area of Science:
- Orthopedic surgery
- Pediatric orthopedics
- Skeletal dysplasias
Background:
- Dysplasia epiphysealis hemimelica (DEH) is a rare congenital developmental anomaly affecting long bones.
- It is characterized by the progressive, unilateral overgrowth of epiphyseal cartilage.
Observation:
- Four cases of DEH were observed, with initial examination ages ranging from 18 months to 5 years.
- Follow-up periods extended from 3 to 20 years.
- Growth arrest was noted after fusion of accessory ossification centers with the regular epiphysis.
Findings:
- Surgical resection of enlarged medial femoral condyles was performed in three patients.
- In two cases, resection involved bone, leaving a non-cartilaginous knee joint surface.
- No further enlargement of the lesion was observed post-fusion.
Implications:
- Surgical intervention for DEH, specifically femoral condyle resection, appears safe and effective.
- Long-term follow-up suggests minimal adverse effects, with patients reporting no complaints and exhibiting near-normal radiographic knee joint appearance.
- This supports surgical management as a viable option for managing symptomatic DEH.