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Long-Term Surgical Success in Treating Vohwinkel Syndrome: A Case Report.
Kirby W Bonvillain1, Andrew B Rees1, Katherine D Drexelius1
1Department of Orthopaedic Surgery, Atrium Health Musculoskeletal Institute-Carolinas Medical Center, Charlotte, North Carolina.
JBJS Case Connector
|November 7, 2024
Summary
Vohwinkel syndrome, a rare keratinization disorder, can cause pseudoainhum leading to autoamputation. Surgical treatment using a specific technique shows success in preventing recurrence of these digital constrictions.
Area of Science:
- Dermatology
- Genetics
- Surgical Pathology
Background:
- Vohwinkel syndrome is a rare autosomal dominant disorder characterized by progressive bilateral symmetric keratoderma and pseudoainhum.
- Pseudoainhum, a constricting band around digits, can lead to autoamputation if left untreated.
- Surgical excision is the primary treatment for pseudoainhum, but recurrence is common.
Observation:
- A 10-year-old boy presented with pseudoainhum affecting the long and small fingers of his right hand at the distal interphalangeal joints.
- The patient underwent surgical treatment involving 180-degree band excision and z-plasty.
Findings:
- Over an 8-year period, the patient received treatment for 5 distinct constriction bands.
- A single recurrence was observed among the treated bands, indicating a high success rate.
- The surgical technique, attributed to Upton, appears to contribute significantly to the low recurrence rate.
Implications:
- The described surgical approach may offer a more effective solution for managing pseudoainhum in Vohwinkel syndrome.
- Minimizing recurrence of digital constriction bands can prevent autoamputation and preserve hand function.
- Further research into surgical techniques for Vohwinkel syndrome is warranted to improve patient outcomes.
