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Orthop Rev (Pavia)
2021 Feb 01;124:8868. doi: 10.4081/or.2020.8868.
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What's the resolutive surgery for pseudo-ainhum in Vohwinkel syndrome? A case report and review of the literature.
Rovere G
,
Stramazzo L
,
Cioffi A
,
Galvano N
,
Pavan D
,
Restuccia G
,
D'Arienzo A
,
Capanna R
,
Maccauro G
,
D'Arienzo M
,
Camarda L
.
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Vohwinkel Syndrome, also known as Keratoderma Hereditarium Mutilans, is an extremely rare dominant autosomal keratosis. It typically presents with "starfish" keratoses on the knuckles, palmoplantar keratoderma (PPK), hearing impairment and mutilating digital constriction bands (pseudoainhum) that cause strangulation, often leading to autoamputation of the affected digit. Both medical and surgical treatment haven't shown to date consistent results, in the treatment of pseudoainhum. In this study we present the case of a woman with Vohwinkel syndrome who showed constriction bands causing ischemic changes of the 5th digit of the right hand for which she was treated with surgery. We also present a review of the literature for the management of this disease.
Figure 1. a) Palmar keratosis, starfish keratosis and digital constriction bands on the PIP joint; b) radiographs show periosteal thinning of the proximal phalanx; c) MRI shows periosteal thinning and a lack of visualization of the extensor tendon at the proximal PIP.
Figure 2. a) Incision; b) disarticulation of the head of the proximal phalanx, followed by proper hemostasis, tendon plasty procedure, and c) suturing.
Figure 3. a) Orthokeratotic hyperkeratosis, acanthosis and hypergranulosis of the finger and constriction of the proximal PIP; b) Higher magnification of circumferential hyperkeratotic constriction band in the proximal interphalangeal joint. a,b) ematoxylin and eosin stain. Original magnifications: a) 10×; b) 20×.
