1215 - Calciphylaxis Following Parathyroidectomy: A Novel Case Presentation

Calciphylaxis is a rare, life-threatening vascular calcification disorder most often associated with hyperparathyroidism and end-stage renal disease (ESRD). Parathyroidectomy is considered a treatment for calciphylaxis; paradoxical development following complete gland removal is exceptionally uncommon.

Case Description:

We describe a 74-year-old woman with chronic kidney disease (CKD) stage 4 by cystatin C–based estimated glomerular filtration rate (eGFR) who developed painful necrotic thigh lesions three months after a complete parathyroidectomy. Prior to surgery, she presented with life-threatening hypercalcemia (calcium 18.5 mg/dL), markedly elevated parathyroid hormone (PTH 2126 pg/mL), phosphorus 6.4 mg/dL, and creatinine 3.64 mg/dL. She required three sessions of temporary dialysis for stabilization. Her acute kidney injury resolved after calcium control, with renal function returning to baseline CKD stage 4. Surgical pathology revealed an atypical parathyroid tumor. Despite successful tumor removal and aggressive postoperative management, she developed calciphylaxis. She underwent multiple incision and drainage (I&D) procedures and received intravenous sodium thiosulfate without improvement.

Discussions:

In a case documented in 2007, a 59-year-old female had a partial parathyroidectomy followed by an occurrence of calciphylaxis. After the second episode, she had a total parathyroidectomy. Another documented occurrence of calciphylaxis following a parathyroidectomy was in 2013 in a 62-year-old female. Following the procedure and resuming calcium and vitamin D supplements due to hypocalcemia, she had another episode of calciphylaxis. Lastly, a 60-year-old male in 2015 who had elevated parathyroid hormone (PTH) levels and received a parathyroidectomy, which dropped his PTH levels. Following the procedure, the patient developed calciphylaxis. 

Conclusions:

This case highlights an unusual presentation of calciphylaxis following parathyroidectomy in a patient with CKD stage 4. The presence of an atypical parathyroid tumor explains the extreme biochemical abnormalities. The refractory course despite aggressive management underscores the aggressive nature of calciphylaxis and the importance of timely recognition, supportive care, and early goals-of-care discussions.