- Email: [email protected]
Vanishing leg ulcers
ARTICLE IN PRESS
Vanishing leg ulcers Demetrios Moris, MD, MSc, PhD, MACS, Vasileios Zavvos, MD, PhD, and Georgios Zavos, MD, PhD, Athens, Greece
From the Transplantation Unit, Laikon General Hospital, Athens, Greece
CALCIFIC UREMIC ARTERIOLOPATHY, also known as calciphylaxis, is a rare, often fatal complication usually associated with end-stage renal disease (ESRD).1 It is characterized by skin ulceration and necrosis, leading to significant pain. The incidence of calciphylaxis has risen in the last decade, with recent estimates as high as 5% of dialysis-dependent patients, but the true prevalence is likely unknown.1 The relevant literature regarding its management is primarily derived from case reports and small case series that frequently describe treatment decisions based on manipulating “risk factors,” such as female sex, obesity, diabetes, use of warfarin, low albumin levels, calciumbased phosphate binders, vitamin D analogs, and long-term use of high-dose iron salts.1 The main focus of the current literature is on the role of parathyroidectomy as a treatment for calciphylaxis in patients with persistent ESRD, as evidenced by contemporary publications.2-4 Here, we present an impressive case of a patient with ESRD and on hemodialysis who presented with leg ulcers due to calciphylaxis that vanished after total parathyroidectomy. A 53-year-old man with ESRD who was undergoing hemodialysis presented with painful, indurated, subcutaneous plaques with overlying livedo racemose and nonhealing, stellate-shaped ulcers covered by black eschar (Fig, A). Clinical examination revealed present pulses, and lower extremity Doppler ultrasonography identified biphasic arterial blood flow bilaterally. According to his medical history, the patient was also experiencing secondary hyperparathyroidism (parathyroid hormone level, 1,456 pg/mL) and an elevated calciumphosphorus product (84 mg2/d2) despite Accepted for publication December 9, 2016. Reprint requests: Demetrios Moris, MD, MSc, PhD, MACS, Transplantation Unit, Laikon General Hospital, Agiou Thoma 17 Street, Athens, Greece. E-mail: [email protected]. Surgery 2017;j:j-j. 0039-6060/$ - see front matter Ó 2017 Published by Elsevier Inc. http://dx.doi.org/10.1016/j.surg.2016.12.006
receiving cinacalcet 90 mg/d and phosphate binders. He had also been undergoing regular hemodialysis 3 times per week, with 4-hour sessions, for 10 years. The etiology of his renal failure was unknown, and the patient had no systemic disease when hemodialysis was started. The clinical diagnosis of calciphylaxis was set, and the decision for parathyroidectomy was taken. Preoperative scintigraphy indicated the presence of 4 hyperplastic parathyroid glands. Intraoperatively, all glands were identified, removed, and sent for frozen section that certified the presence of the parathyroid tissue. The patient had rapid and significant improvement of postoperative levels of parathyroid hormone, calcium, and phosphorus, as well as improvement of skin necrosis and clinical status (Fig, B). The final histology analysis reported the presence of hyperplastic parathyroid glands due to secondary hyperparathyroidism. The patient is symptom free at 1-year follow-up. 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.1 The literature remains equivocal about the cost-effectiveness of parathyroidectomy as a treatment option in these patients.2-4 As in our case, the literature supports the idea that subtotal or total parathyroidectomy is associated with long-term survival and is more likely to promote healing if performed earlier in the course of disease.2-4 Other authors highlight that operative risks and complications from parathyroidectomy do not outweigh its benefits in patients with calciphylaxis.3 In a large cohort study using data from the United States Renal Data System, operative parathyroidectomy was associated with a 2% short-term death rate, a 39% increase in the rate of subsequent hospitalization, and a remarkable 20-fold increase in severe hypocalcemia in the postparathyroidectomy period compared with the preparathyroidectomy period.5 In addition, rates for cardiac events were significantly higher in the SURGERY 1
ARTICLE IN PRESS 2 Moris, Zavvos, and Zavos
Surgery j 2017
Fig. Bilateral leg ulcers covered by black eschar (A); healing leg ulcers after parathyroidectomy (B). (Color version of this figure is available online.)
postparathyroidectomy period compared with the preparathyroidectomy period.5 Thus, further prospective studies and clinical trials are indicated to clarify the role of parathyroidectomy in patients with calciphylaxis.
REFERENCES 1. Nigwekar SU, Kroshinsky D, Nazarian RM, Goverman J, Malhotra R, Jackson VA, et al. Calciphylaxis: risk factors, diagnosis, and treatment. Am J Kidney Dis 2015;66:133-46.
2. Duffy A, Schurr M, Warner T, Chen H. Long-term outcomes in patients with calciphylaxis from hyperparathyroidism. Ann Surg Oncol 2006;13:96-102. 3. Weenig RH, Sewell LD, Davis MD, McCarthy JT, Pittelkow MR. Calciphylaxis: natural history, risk factor analysis, and outcome. J Am Acad Dermatol 2007;56:569-79. 4. Lal G, Nowell AG, Liao J, Sugg SL, Weigel RJ, Howe JR. Determinants of survival in patients with calciphylaxis: a multivariate analysis. Surgery 2009;146:1028-34. 5. Ishani A, Liu J, Wetmore JB, Lowe KA, Do T, Bradbury BD, et al. Clinical outcomes after parathyroidectomy in a nationwide cohort of patients on hemodialysis. Clin J Am Soc Nephrol 2015;10:90-7.
Vanishing leg ulcers Demetrios Moris, MD, MSc, PhD, MACS, Vasileios Zavvos, MD, PhD, and Georgios Zavos, MD, PhD, Athens, Greece
From the Transplantation Unit, Laikon General Hospital, Athens, Greece
CALCIFIC UREMIC ARTERIOLOPATHY, also known as calciphylaxis, is a rare, often fatal complication usually associated with end-stage renal disease (ESRD).1 It is characterized by skin ulceration and necrosis, leading to significant pain. The incidence of calciphylaxis has risen in the last decade, with recent estimates as high as 5% of dialysis-dependent patients, but the true prevalence is likely unknown.1 The relevant literature regarding its management is primarily derived from case reports and small case series that frequently describe treatment decisions based on manipulating “risk factors,” such as female sex, obesity, diabetes, use of warfarin, low albumin levels, calciumbased phosphate binders, vitamin D analogs, and long-term use of high-dose iron salts.1 The main focus of the current literature is on the role of parathyroidectomy as a treatment for calciphylaxis in patients with persistent ESRD, as evidenced by contemporary publications.2-4 Here, we present an impressive case of a patient with ESRD and on hemodialysis who presented with leg ulcers due to calciphylaxis that vanished after total parathyroidectomy. A 53-year-old man with ESRD who was undergoing hemodialysis presented with painful, indurated, subcutaneous plaques with overlying livedo racemose and nonhealing, stellate-shaped ulcers covered by black eschar (Fig, A). Clinical examination revealed present pulses, and lower extremity Doppler ultrasonography identified biphasic arterial blood flow bilaterally. According to his medical history, the patient was also experiencing secondary hyperparathyroidism (parathyroid hormone level, 1,456 pg/mL) and an elevated calciumphosphorus product (84 mg2/d2) despite Accepted for publication December 9, 2016. Reprint requests: Demetrios Moris, MD, MSc, PhD, MACS, Transplantation Unit, Laikon General Hospital, Agiou Thoma 17 Street, Athens, Greece. E-mail: [email protected]. Surgery 2017;j:j-j. 0039-6060/$ - see front matter Ó 2017 Published by Elsevier Inc. http://dx.doi.org/10.1016/j.surg.2016.12.006
receiving cinacalcet 90 mg/d and phosphate binders. He had also been undergoing regular hemodialysis 3 times per week, with 4-hour sessions, for 10 years. The etiology of his renal failure was unknown, and the patient had no systemic disease when hemodialysis was started. The clinical diagnosis of calciphylaxis was set, and the decision for parathyroidectomy was taken. Preoperative scintigraphy indicated the presence of 4 hyperplastic parathyroid glands. Intraoperatively, all glands were identified, removed, and sent for frozen section that certified the presence of the parathyroid tissue. The patient had rapid and significant improvement of postoperative levels of parathyroid hormone, calcium, and phosphorus, as well as improvement of skin necrosis and clinical status (Fig, B). The final histology analysis reported the presence of hyperplastic parathyroid glands due to secondary hyperparathyroidism. The patient is symptom free at 1-year follow-up. 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.1 The literature remains equivocal about the cost-effectiveness of parathyroidectomy as a treatment option in these patients.2-4 As in our case, the literature supports the idea that subtotal or total parathyroidectomy is associated with long-term survival and is more likely to promote healing if performed earlier in the course of disease.2-4 Other authors highlight that operative risks and complications from parathyroidectomy do not outweigh its benefits in patients with calciphylaxis.3 In a large cohort study using data from the United States Renal Data System, operative parathyroidectomy was associated with a 2% short-term death rate, a 39% increase in the rate of subsequent hospitalization, and a remarkable 20-fold increase in severe hypocalcemia in the postparathyroidectomy period compared with the preparathyroidectomy period.5 In addition, rates for cardiac events were significantly higher in the SURGERY 1
ARTICLE IN PRESS 2 Moris, Zavvos, and Zavos
Surgery j 2017
Fig. Bilateral leg ulcers covered by black eschar (A); healing leg ulcers after parathyroidectomy (B). (Color version of this figure is available online.)
postparathyroidectomy period compared with the preparathyroidectomy period.5 Thus, further prospective studies and clinical trials are indicated to clarify the role of parathyroidectomy in patients with calciphylaxis.
REFERENCES 1. Nigwekar SU, Kroshinsky D, Nazarian RM, Goverman J, Malhotra R, Jackson VA, et al. Calciphylaxis: risk factors, diagnosis, and treatment. Am J Kidney Dis 2015;66:133-46.
2. Duffy A, Schurr M, Warner T, Chen H. Long-term outcomes in patients with calciphylaxis from hyperparathyroidism. Ann Surg Oncol 2006;13:96-102. 3. Weenig RH, Sewell LD, Davis MD, McCarthy JT, Pittelkow MR. Calciphylaxis: natural history, risk factor analysis, and outcome. J Am Acad Dermatol 2007;56:569-79. 4. Lal G, Nowell AG, Liao J, Sugg SL, Weigel RJ, Howe JR. Determinants of survival in patients with calciphylaxis: a multivariate analysis. Surgery 2009;146:1028-34. 5. Ishani A, Liu J, Wetmore JB, Lowe KA, Do T, Bradbury BD, et al. Clinical outcomes after parathyroidectomy in a nationwide cohort of patients on hemodialysis. Clin J Am Soc Nephrol 2015;10:90-7.