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Focal scleroderma in children: an orthopaedic perspective
S L Buckley1, S Skinner, P James
1Shriners Hospital, San Francisco, California.
Journal of Pediatric Orthopedics
|November 1, 1993
Summary
Focal scleroderma, a fibrotic connective-tissue disorder, can cause significant extremity contractures. Serial casting and surgical interventions like soft-tissue release are effective treatments for these challenging cases.
Area of Science:
- Rheumatology
- Orthopedic Surgery
- Dermatology
Background:
- Focal scleroderma is a fibrotic connective-tissue disorder affecting skin and subcutaneous tissues.
- It can lead to joint contractures, extremity deformity, and functional impairment.
- The condition has a variable clinical course with remissions and recurrences.
Observation:
- A review of seven patients treated between 1960 and 1990 with significant joint contractures due to focal scleroderma was conducted.
- Patients presented with contractures in the wrist, knee, ankle, and hip.
- One patient had a severe knee-flexion contracture requiring disarticulation.
Findings:
- Serial casting demonstrated utility for wrist, knee, and ankle contractures.
- Soft-tissue release proved effective for contractures at the wrist, hip, and ankle.
- Epiphysiodesis and femoral shortening osteotomy were successful for leg-length equalization in hemiatrophy.
Implications:
- Treatment for resistant focal scleroderma extremity involvement may involve physical therapy, medical management, bracing, serial casting, and surgery.
- Specific surgical techniques like soft-tissue release and osteotomies can effectively address contractures and limb length discrepancies.
- Further research into optimal management strategies for focal scleroderma is warranted.