Long-Term Surgical Success in Treating Vohwinkel Syndrome: A Case Report

JBJS Case Connect. 2024 Nov 7;14(4). doi: 10.2106/JBJS.CC.24.00251. eCollection 2024 Oct 1.

Abstract

Case: Vohwinkel syndrome is a rare, inherited condition marked by defective keratinization. The disorder may feature digital constriction bands (pseudoainhum) which can lead to autoamputation. Surgical excision is the only treatment of pseudoainhum; however, it is fraught with recurrence. We present a 10-year-old boy with pseudoainhum of the right long and small fingers at the distal interphalangeal joints. He underwent 180-degree band excision and z-plasty.

Conclusion: Since establishing care 8 years ago, he has undergone treatment of 5 constriction bands with only 1 recurrence. We believe this success can at least be partially attributed to the technique described by Upton.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple
  • Child
  • Fingers* / abnormalities
  • Fingers* / surgery
  • Hand Deformities, Congenital* / surgery
  • Hearing Loss, Sensorineural
  • Humans
  • Keratoderma, Palmoplantar
  • Male

Supplementary concepts

  • Vohwinkel syndrome