Abstract

Abstract Introduction Calcific Uremic Arteriolopathy (CUA), commonly known as Calciphylaxis, is a rare disorder characterized by ischemic necrosis of the skin and histologically by arteriolar calcification. CUA is most commonly seen in patients with end-stage renal disease (ESRD), but can be seen in other patients as well. CUA is an extremely morbid condition and carries a high mortality rate, up to 80% in some studies, even in patients with limited disease. In light of this, many surgeons have adopted a “do-not-touch” approach. Over the past five years, our institution has seen an increase in the referrals of patients with large total-body-surface-area (TBSA) CUA. Methods Retrospective review of all patients with biopsy-proven (by dermatopathology) large, >5% TBSA CUA admitted to a Verified Adult and Pediatric Burn Center from 2015 to present. Demographics, laboratory data, treatment modalities, infectious complications and outcomes, including mortality and wound closure were recorded. Secondary observations included exposure to anticoagulants and recent significant weight loss. Results A total of eight patients with large TBSA CUA were admitted after being transferred from outside hospitals. Average TBSA affected was 13.76% (range 5–27%). Six of these patients (75%) were noted to have non-uremic calciphylaxis. One patient (12.5%) had elevated parathyroid hormone (PTH) levels and hyperphosphatemia while one patient each had isolated hypercalcemia and hyperphosphatemia. All patients had positive wound cultures on admission, and one patient (12.5%) developed a bacteremia in hospital. There were no central line associated bloodstream infections, catheter associated urinary tract infections or ventilator associated infections. All patients underwent surgical debridement (average 4.125, range 2–5), and 5 patients (62.5%) underwent grafting, (average 1.6, range 2–5) and subsequently proceeded to wound closure. In-hospital mortality was 25% and another patient was referred to a hospice facility after being readmitted with medical complications related to her calciphylaxis. Secondary findings included 50% of the patients recently experienced significant weight loss (>100 lbs) and 25% of the patients were taking warfarin within 6 months of presentation. Percent TBSA involvement did not correlate with mortality. Conclusions Utilizing a multi-modal management strategy that includes surgical debridement and skin grafting, patients with large total body surface area calciphylaxis can progress to wound closure. Applicability of Research to Practice Findings may influence surgeons to be aggressive surgically with patients with calciphylaxis leading to improved morbidity and mortality. Improved medical multi-modal therapy has been shown to influence outcomes as well.

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