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Annals of Surgical Oncology
Published in: Annals of Surgical Oncology 1/2006

01-01-2006 | Original Article

Long-Term Outcomes in Patients With Calciphylaxis From Hyperparathyroidism

Authors: Allison Duffy, MD, Michael Schurr, MD, FACS, Thomas Warner, MD, Herbert Chen, MD, FACS

Published in: Annals of Surgical Oncology | Issue 1/2006

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Abstract

Background

Calciphylaxis is a rare condition associated with chronic renal failure and entails a very poor prognosis. Pathogenesis is poorly understood but involves abnormalities in calcium and phosphorus metabolism that lead to vascular and extravascular calcification. Patients present with painful ulcerating plaques that progress to gangrenous wounds. Parathyroidectomy has been advanced as a life-saving intervention in these patients, but long-term results with wound healing and survival after parathyroidectomy are not well described.

Methods

Between January 1987 and October 2003, 15 patients with biopsy-confirmed calciphylaxis were treated at the University of Wisconsin. Of these 15 patients, 9 were treated with medical therapy (bisphosphonates and phosphate binders), whereas 6 underwent parathyroidectomy. The medical records were reviewed, and patients or relatives were interviewed. Survival was determined by Kaplan-Meier analysis.

Results

Four patients underwent subtotal parathyroidectomy, and two patients underwent total parathyroidectomy. All had reductions in the intact parathyroid hormone level (mean ± SD, 25.2 ± 4.5 pg/mL). Whereas all six patients treated with parathyroidectomy had partial/complete wound healing, only two of nine in the medical group had any improvements in the skin lesions (P = .006). With up to 80 months of follow-up, patients who underwent parathyroidectomy had a longer median survival compared with those who did not have surgery (39 vs. 3 months; P = .017).

Conclusions

On the basis of our long-term follow-up of this patient population, subtotal or total parathyroidectomy was associated with long-term survival and was more likely to promote healing if performed earlier in the course of disease. Therefore, patients with calciphylaxis from secondary hyperparathyroidism should be referred promptly for potential parathyroidectomy.
Literature
1.
Angelis M, Wong LL, Myers SA, Wong LM. Calciphylaxis in patients on hemodialysis: a prevalence study. Surgery 1997;122:1083–9CrossRef
2.
Fine A, Zacharias J. Calciphylaxis is usually non-ulcerating: risk factors, outcome, therapy. Kidney Int 2002;61:2210–7CrossRef
3.
Lim SP, Batta K, Tan BB. Calciphylaxis in a patient with alcoholic liver disease in the absence of renal failure. Clin Exp Dermatol 2003;28:34–6CrossRef
4.
Goyal S, Huhn KM, Provost TT. Calciphylaxis in a patient without renal failure or elevated parathyroid hormone: possible etiological role of chemotherapy. Br J Dermatol 2000;143:1087–93CrossRef
5.
Almirall J, Pobo A, Luelmo J, Berna L. Post-infectious acute renal failure due to calciphylaxis—when processes go the wrong way around. J Nephrol 2004;17:575–9PubMed
6.
Payasli C, Hosnuter M, Babuccu O, Kargi E, Kocak E, Isikdemir A. Spotting the signs: a case of late diagnosed painful calciphylaxis. J Wound Care 2005;14:12–3CrossRef
7.
Naik V, Lynch D, Slavcheva E, Beissner R. Calciphylaxis: medical and surgical management of chronic extensive wounds in a renal dialysis population. Plast Reconstr Surg 2004;113:304–12CrossRef
8.
Coates T, Kirkland GS, Dymock RB, et al. Cutaneous necrosis from calcific uremic arteriolopathy. Am J Kidney Dis 1998;32:384–91CrossRef
9.
Essary LR, Wick MR. Cutaneous calciphylaxis: an underrecognized clinicopathologic entity. Am J Clin Pathol 2000;113:280–7CrossRef
10.
Selye H, Gabbiani G, Strebel R. Sensitization to calciphylaxis by endogenous parathyroid hormone. Endocrinology 1962;71:554–8CrossRef
11.
Gipstein RM, Coburn JW, Adams DA, et al. Calciphylaxis in man: a syndrome of tissue necrosis and vascular calcification in 11 patients with chronic renal failure. Arch Intern Med 1976;136:1273–80CrossRef
12.
Giachelli CM. Vascular calcification mechanisms. J Am Soc Nephrol 2004;15:959–64CrossRef
13.
Ahmed S, O’Neill KD, Hood AF, Evan AP, Moe SM. Calciphylaxis is associated with hyperphosphatemia and increased osteopontin expression by vascular smooth muscle cells. Am J Kidney Dis 2001;37:1267–1276CrossRef
14.
Mathur RV, Shortland JR, el-Nahas AM. Calciphylaxis. Postgrad Med J 2001;77:557–61CrossRef
15.
Bleyer AJ, Choi M, Igwemezie B, et al. A case control study of proximal calciphylaxis. Am J Kidney Dis 1998;32:376–83CrossRef
16.
Oh DH, Eulau D, Tokugawa DA, McGuire JS, Kohler S. Five cases of calciphylaxis and a review of the literature. J Am Acad Dermatol 1999;40:979–87CrossRef
17.
Bahar G, Mimouni D, Feinmesser M, David M, Popovzer A, Einmesser R. Subtotal parathyroidectomy: a possible treatment for calciphylaxis. Ear Nose Throat J 2003;82:390–3CrossRef
18.
Arch-Ferrer JE, Beenken SW, Rue LW, Bland KI, Diethelm AG. Therapy for calciphylaxis: an outcome analysis. Surgery 2003;134:941–4; 944–5CrossRef
19.
Girotto JA, Harmon JW, Ratner LE, Nicol TL, Wong L, Chen H. Parathyroidectomy promotes wound healing and prolongs survival in patients with calciphylaxis from secondary hyperparathyroidism. Surgery 2001;130:645–51CrossRef
20.
Kang AS, McCarthy JT, Rowland C, Farley DR, van Heerden JA. Is calciphylaxis best treated surgically or medically? Surgery 2000;128:967–72CrossRef
21.
Chen H. Surgery for primary hyperparathyroidism: what is the best approach? Ann Surg 2002;236:552–3CrossRef
22.
Chen H, Sokoll L, Udelsman R. Outpatient minimally invasive parathyroidectomy: a combination of sestamibi-SPECT localization, cervical block anesthesia, and intraoperative PTH assay. Surgery 1999;126:14–8
23.
Chen H, Mack E, Starling JR. Radioguided parathyroidectomy is equally effective for both adenomatous and hyperplastic glands. Ann Surg 2003;238:332–8CrossRef
24.
Chen H, Pruhs ZM, Starling JR, Mack E. Intraoperative parathyroid hormone testing improves cure rates in patients undergoing minimally invasive parathyroidectomy. Surgery 2005;138:583–90CrossRef
25.
Chen H, Mack E, Starling JR. A comprehensive evaluation of peri-operative adjuncts during minimally invasive parathyroidectomy: which is most reliable? Ann Surg 2005;242:375–83PubMedPubMedCentral
26.
Milas M, Weber CF. Near-total parathyroidectomy is beneficial for patients with secondary and tertiary hyperparathyroidism. Surgery 2004;136:1252–60CrossRef
27.
Sippel RS, Bianco J, Chen H. Radioguided parathyroidectomy for recurrent hyperparathyroidism due to forearm graft hyperplasia. J Bone Miner Res 2003;18:939–42CrossRef
28.
Nichol PR, Mack E, Bianco J, Hayman A, Starling JR, Chen H. Radioguided parathyroidectomy in patients with secondary and tertiary hyperparathyroidism. Surgery 2003;134:713–7CrossRef
29.
Howe SC, Murray JD, Reeves RT, Hemp JR, Carlisle JH. Calciphylaxis, a poorly understood clinical syndrome: three case reports and a review of the literature. Ann Vasc Surg 2001;15:470–3CrossRef
30.
Don BR, Chin AI. A strategy for the treatment of calcific uremic arteriolopathy (calciphylaxis) employing a combination of therapies. Nephrology 2003;59:463–70
31.
Russell R, Brookshire MA, Zekonis M, Moe SM. Distal calcific uremic arteriolopathy in a hemodialysis patient responds to lowering of Ca × P product and aggressive wound care. Clin Nephrol 2002;58:238–43CrossRef
32.
Tittelbach J, Graefe T, Wollina U. Painful ulcers in calciphylaxis—combined treatment with maggot therapy and oral pentoxyfillin. J Dermatolog Treat 2001;12:211–4CrossRef
33.
Milas M, Bush RL, Lin K, et al. Calciphylaxis and nonhealing wounds: the role of the vascular surgeon in a multidisciplinary treatment. J Vasc Surg 2003;37:501–7CrossRef
Metadata
Title
Long-Term Outcomes in Patients With Calciphylaxis From Hyperparathyroidism
Authors
Allison Duffy, MD
Michael Schurr, MD, FACS
Thomas Warner, MD
Herbert Chen, MD, FACS
Publication date
01-01-2006
Publisher
Springer New York
Published in
Annals of Surgical Oncology / Issue 1/2006
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI
https://doi.org/10.1245/ASO.2006.03.042

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