Abstract

Calciphylaxis, a disorder of calcium-phosphate metabolism that can result in arterial calcification, skin and solid organ calcium deposits, and nonhealing ulcerations, is associated with significant morbidity and mortality. Although its most common cause is secondary hyperparathyroidism in patients with renal failure, vascular surgeons are frequently called on to evaluate these nonhealing extremity wounds. We reviewed our experience of a multidisciplinary approach in treating patients with calciphylaxis and nonhealing ulcers. Over a 14-month period at a tertiary center, five patients were seen with calciphylaxis and nonhealing leg wounds. Demographics, disease characteristics, surgical treatment, and outcomes were analyzed. All five patients were black women aged 40 +/- 8.9 years with hypertensive renal failure undergoing long-term hemodialysis (80 +/- 43 months). They had large, painful lower extremity wounds or necrotic ulcers (mean size, 135 cm(2)) that had developed over 2 to 4 months. Three patients had palpable pedal pulses, one patient had Doppler pedal signals, and one patient had absent pedal flow. Arteriogram was performed in the latter two patients, and one patient underwent lower extremity revascularization because of superficial femoral artery stenosis with symptomatic improvement. Four patients underwent aggressive debridement by the vascular surgical service, and two needed plastic surgeon-performed skin grafting. All patients had elevated parathyroid hormone levels (mean, 1735 pg/mL; > 25 x normal level); mean preoperative calcium levels were normal (10 mg/dL). After either subtotal (n = 4) or total (n = 1) parathyroidectomy by an experienced endocrine surgeon, a significant reduction in parathyroid hormone and calcium levels was seen (122 pg/mL and 7.9 mg/dL, respectively; P <.05). There were no postoperative complications or amputations; one patient died 12 months after parathyroidectomy of severe preexisting cardiopulmonary disease. Complete wound healing was observed by 4.8 +/- 2 months. During a mean follow-up period of 9 months (range, 1 to 18 months), all wounds remained healed without ulcer recurrence. The diagnosis of calciphylaxis should be considered in patients with end-stage renal disease with atypical tissue necrosis or subcutaneous nodules. Early recognition of calciphylaxis and multidisciplinary treatment, including diligent wound care, frequent debridement, parathyroidectomy, and appropriate skin grafting or revascularization, can result in improved wound healing and limb salvage.

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