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Severe Multifocal Noninfectious Wound Dehiscence After Cavovarus Reconstruction in TRPV4-Associated
Moamen Elhaddad1, Jacob Stibelman1, Alexander Carrillo-Kashani1
1Department of Podiatric Foot and Ankle Surgery, Cedars-Sinai Medical Center, Los Angeles, California.
Abstract:
BackgroundCharcot-Marie-Tooth disease (CMT) commonly produces progressive cavovarus/equinovarus deformity, and severe pediatric deformity may require complex reconstruction when bracing and therapy fail. Postoperative wound complications can occur after extensive hindfoot/midfoot surgery, but genotype-specific factors influencing wound healing remain poorly defined. Charcot-Marie-Tooth disease type 2C (CMT2C) is associated with transient receptor potential vanilloid 4 (TRPV4) variants, and experimental data suggest that TRPV4 signaling may influence fibroblast behavior and extracellular matrix depositionCaseA 10-year-old girl with genetically confirmed CMT2C due to TRPV4 c.806G>A (p.Arg269His) presented with progressive ambulatory difficulty, severe bilateral stiff equinovarus (left worse), and a left knee flexion contracture (~25°). Weightbearing cone-beam computed tomography confirmed severe left cavovarus deformity. She underwent left anterior distal femoral hemiepiphysiodesis followed by complex left foot/ankle reconstruction, including Achilles Z-lengthening with posterior capsulotomy, multiple soft-tissue releases, posterior tibial tendon transfer to the lateral cuneiform, talonavicular release, peroneus longus-to-brevis transfer, and calcaneocuboid fusion. Within weeks, she developed severe multifocal wound dehiscence across multiple incision sites without clinical or microbiologic evidence of infection. She required readmission, serial debridements, and negative-pressure wound therapy. Adjunct dermal punch-biopsy-derived fibroblast explant culture showed delayed early outgrowth and reduced adherence/spread versus control, with preserved proliferation after passage. Plastic surgery performed operative debridement; perfusion imaging confirmed viable tissue, and biodegradable temporizing matrix was applied to key wounds with bridged vacuum-assisted closure, resulting in wound healing with long-term follow-up.ConclusionThis case highlights unusually severe, noninfectious, multifocal wound dehiscence following technically appropriate, consensus-guided reconstruction in a child with TRPV4-associated CMT2C. Although causality cannot be established, the complication pattern raises the possibility of genotype-associated wound-healing vulnerability and supports heightened postoperative surveillance, early multidisciplinary involvement, and consideration of staged reconstruction in similarly high-risk patients.Level of EvidenceTherapeutic, Level V (Case Report).
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