imaging features of calciphylaxis - skeletal radiology · 3. lehto s, niskanen l, suhonen m, et al...

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 franke.ryan@mayo.edu #Calciphylaxis