Society of Skeletal Radiologythe
Imaging Features of Calciphylaxis
41st Annual Meeting, March 25-28, 2018
Ryan Franke MD Mark Kransdorf MD
Mayo Clinic, Arizona
Society of Skeletal Radiologythe
No Disclosures
41st Annual Meeting, March 25-28, 2018
Ryan Franke MD Mark Kransdorf MD
Mayo Clinic, Arizona
Calciphylaxis, also known calcific uraemic arteriolopathy, is an uncommon condition most commonly found in patients with end-stage renal disease, estimated to affect up to 1%-4% of that population and carrying a 1-year mortality rate of 45–80%
Originally described in uremic patients it effects the small vessels causing a vasculopathy involving mural calcification with intimal fibrosis, proliferation, and thrombosis
Calciphylaxis:Background
The primary lesion is the accumulating of calcium salts in the media of small arteries, causing the artery lumen to narrow
The luminal narrowing causes reduced arteriolar blood flow, which leads to ischemia
Ischemic lesions develop in the subcutaneous fat primarily involving the dermal arterioles leading to secondary subcutaneous tissue and skin infarcts and necrosis
On biopsy, calcium deposits are seen in media of small arteries and arterioles
Calciphylaxis:Pathophysiology
Typical clinical presentation includes ischemia and necrosis of the skin, soft tissues, and organs
Small vessel vascular calcifications visible on imaging and are the hallmark of the disease
Patient risk factors include: a high calcium-phosphate product, end-stage renal disease on renal replacement therapy, body mass index >30 kg/m2, female gender and use of warfarin or systemic corticosteroids
Calciphylaxis:Clinical Presentation
Classically, the diagnosis is based on the clinical presentation in combination with skin biopsy, however definitive histopathologic diagnosis is often challenging
Calciphylaxis:Diagnosis
Imaging evaluation now plays an important complimentary role in the diagnosis of calciphylaxis!
We reviewed our experience with the imaging features of 11 patients with biopsy proven calciphylaxis to identify the characteristics of this uncommon entity
Objective:
We retrospectively reviewed all available records in our institutional electronic pathology report data base for all patients with a diagnosis of “calciphylaxis”
A total of 43 patients were identified All pathology reports and imaging studies for these patients
were reviewed
Patients and Methods:
Study group inclusion criteria required: Definitive pathological diagnosis of calciphylaxis or
findings “strongly suggestive” of calciphylaxis and Imaging studies (radiographs, CT, bone scan and/or
US) of the area of the biopsy
Patients and Methods:
The study group included 11 patients: Four men and seven women Average age of 52 years (range 37-77 years)
Imaging studies reviewed included: Radiographs (11 patients) CT (5 patients) Bone scan (2 patients)
Results:
The dominant imaging feature was fine small vessel vascular calcification
Calcifications were within the subcutaneous adipose most prominent deep to the skin
These were seen well on both radiographic and CT examinations
Results:
Skin thickening and varying amounts of associated panniculitis immediately adjacent to the skin thickening were best seen on CT
These findings were less well-delineated on radiographs
Ill-defined increased tracer accumulation was readily identified on bone scintigraphy in the areas of the calcification
Results:
Clinical photographs were available in 7 patients
These demonstrated varying amount of skin necrosis including ulceration, involving the legs, feet, and abdominal wall
Results:
Typical Imaging Features:Skin Thickening and Inflammation
Radiographs show extensive diabetic vascular calcification
Radiographs show extensive diabetic vascular calcification
…and extensive small arteriole vascular calcification
Radiographs show extensive diabetic vascular calcification
…and extensive small arteriole vascular calcification
…and skin thickening with Inflammatory change
Oblique radiograph of the foot shows the necrotic ulcer over the heel to better advantage (white arrow); note small vessel calcification (black arrows)
Intraoperative image during ulcer debridement
Radiographs from a different patient show similar findingswith fine small vessel calcification (arrows)
Patient developed an area of infarction… which ulcerated
Patient developed an area of infarction… which ulcerated
Patient developed an area of infarction… which ulcerated
Note extensive small vessel calcification (arrows)
Clinical photo shows infarctionand ulceration
Posterior image MDP bone scan with correlating CT
Increased tracer accumulation correlatedto areas of vascular calcification and inflammation
Increased tracer accumulation on the right also correlate toareas of vascular calcification (arrows) and inflammation (arrow head)
CT demonstrates extensive vascular calcifications (arrows) andedema (asterisks) with overlying ulcerations (arrowheads)
**
Evaluation of the ventral abdominal wall revealedulceration and discoloration with histology showing calciphylaxis.
Calciphylaxis:Conclusion
Imaging findings demonstrating extensive fine small vessel vascular calcification within the subcutaneous adipose tissue, typically just deep to the skin, are characteristic of calciphylaxis
These findings are well seen on both radiographs and CT
Calciphylaxis:Conclusion
Scintigraphy will show increased tracer primarily localized to the subcutaneous tissue
Associated inflammatory change with skin thickening is commonly seen
Ischemia, infarction, necrosis, ulceration and infection are well recognized complications and a major cause of morbidity and mortality
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Calciphylaxis: Ryan Franke, M.D. MSK Fellow at Mayo Clinic AZ [email protected] #Calciphylaxis