Indian Journal of Vascular and Endovascular Surgery

CASE REPORT
Year
: 2020  |  Volume : 7  |  Issue : 1  |  Page : 102--104

Revascularization in Mönckeberg medial calcific sclerosis


Jithin Jagan Sebastian, Pranay Pawar, MK Ayyappan, Kapil Mathur, Naveen Rajendra, Radhakrishnan Raju 
 Department of Vascular Surgery and Endovascular Surgery, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Correspondence Address:
Dr. Jithin Jagan Sebastian
Department of Vascular Surgery and Endovascular Surgery, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu
India

Abstract

Mönckeberg medial calcific sclerosis (MMS) is a rare disorder of medial calcification for which open repair has always been the standard. We present two unusual cases of MMS affecting different arterial segments, for which bypass was done. The first patient presented with extensive aortoiliac medial calcification. She had a near normal ankle brachial index on one side and critical limb ischemia on the other. A femorofemoral crossover bypass was carried out. The patient improved symptomatically. The second case involved a femoropopliteal segment Mönckeberg sclerosis for which a femoro-distal bypass was carried out. Both patients continue to remain asymptomatic after 2 years of follow-up. Open surgical repair remains the ideal method of repair in this rare condition of medial calcific sclerosis. The disease progression is very slow in these patients, and usually one segment of artery is involved.



How to cite this article:
Sebastian JJ, Pawar P, Ayyappan M K, Mathur K, Rajendra N, Raju R. Revascularization in Mönckeberg medial calcific sclerosis.Indian J Vasc Endovasc Surg 2020;7:102-104


How to cite this URL:
Sebastian JJ, Pawar P, Ayyappan M K, Mathur K, Rajendra N, Raju R. Revascularization in Mönckeberg medial calcific sclerosis. Indian J Vasc Endovasc Surg [serial online] 2020 [cited 2020 Jun 3 ];7:102-104
Available from: http://www.indjvascsurg.org/text.asp?2020/7/1/102/280677


Full Text



 Introduction



Mönckeberg medial calcific sclerosis (MMS) is a disease of unknown etiology first described by Johann Georg Mönckeberg in 1903.[1] It affects the small- and medium-sized arteries. MMS develops close to the internal elastic lamina or media in muscular arteries in the absence of changes in calcium metabolism. This progressive disease later leads to arterial occlusion. There are very few cases in literature of attempts to revascularize these patients presenting with symptomatic peripheral vascular disease. Open surgery is preferred to endovascular techniques in these patients with extensive calcification.[2] Patency of bypasses in patients with medial calcification is almost unknown. We present two such patients with segmental MMS, who underwent surgical revascularization.

 Case Reports



Case 1

A 52-year-old female presented to our vascular clinic with pain at rest in the right leg for a month. She had an ankle brachial index (ABI) of 0.1. Her medical history included hypertension, and coronary artery bypass grafting done 2 years ago. On examination, she had an ischemic ulcer on the tip of her right second toe. Her right femoral and distal pulses were absent. The rest of her peripheral pulses were normal. The ABI was 0.1 for the right leg and 0.9 on the left. A clinical diagnosis of right iliac occlusion was made. Computed tomography (CT) angiography showed extensive calcification of the aortoiliac segment extending from the renal arteries up to the internal iliac arteries [Figure 1]. Calcification of the internal iliac artery had predisposed to occlusion of the external iliac artery at the bifurcation [Figure 2]. Calcium, phosphorus, and parathyroid hormone (PTH) levels were within normal limits. Doppler scans of the upper limb arteries and CT screening of the lower limb arteries were normal. Since her left femoral pulse was present and showed good volume and absent bruit, a femorofemoral bypass with polytetrafluoroethylene was performed. Intraoperatively, both common femoral vessels were found to be normal and healthy, with no evidence of calcification in the wall. Postoperatively, her ABI improved to 0.9 in the right leg. She was discharged on dual antiplatelet therapy. At 2 years of follow-up, she remained asymptomatic and the ABI was 0.9 in both the legs.{Figure 1}{Figure 2}

Case 2

A 53-year-old female presented with pain and ulceration over the left foot for 10 days. She was known to have hypertension that was well controlled with medication. On examination, only the femoral pulses were palpable in both lower limbs. The ABI was 0.1 on the left, and 0.7 on the right leg. CT angiography demonstrated bilateral extensive femoropopliteal arterial calcification, typical of MMS [Figure 3]. On the left, the distal anterior tibial and peroneal arteries showed reconstitution. Plain X-ray shows a characteristic tram-tracking appearance [Figure 4]. Her calcium, phosphate, and PTH levels were normal. A common femoral to anterior tibial bypass was carried out with a reversed long saphenous vein graft. She was discharged on a single antiplatelet and kept on follow-up. At 2 years follow-up, her left leg ABI was 0.8 and the ulcer had healed. Her right leg ABI remained the same at 2 years.{Figure 3}{Figure 4}

 Discussion



Vascular calcification is usually seen in advanced atherosclerotic disease. MMS is shrouded in a lot of misconception regarding its definition. It is now used to define a nonatheromatous vascular lesion.[3] It most commonly develops in the media of muscular arteries in the absence of calcium metabolism.[4] Both our patients had segmental medial calcific disease without any derangement in calcium homeostasis. Peripheral arterial involvement is the most common site of arterial involvement in MMS. Abdominal aorta, iliac, and visceral artery have also been shown to be involved in rare cases.[5],[6] The other vascular beds were completely normal.

The real incidence of MMS is unknown and only a few case reports exist. The incidence of MMS in banked vascular segments is 25% among chronic kidney disease patients.[7] MMS was not found to be associated with the Framingham risk factors of age, sex, diabetes, hypertension, dyslipidemia, smoking, and chronic kidney disease.[8] There is a strong association between peripheral arterial calcium and heart disease.[9] Both our patients were hypertensive, and our first patient had a history of heart disease.

There are not many case reports of bypass carried out in MMS. These patients usually are asymptomatic as the calcium in the media causes the vessels to remain patent for a long time. They rarely present with critical limb ischemia. Intimal and medial calcification has to be differentiated before planning intervention in these patients. The above-described cases are histologically suggestive of osseous metaplasia and hence suitable for bypass rather than angioplasty as described by Janzen and Vuong.[10] These cases have circumferential calcification of arteries with periadventitial extension.

The role of endovascular surgery in these patients is limited due to the circumferential calcification. The open procedure remains the mainstay of treatment. The first case had extensive aortoiliac segment calcification. Extra-anatomic bypass was carried out in view of the poor cardiac status of the patient and a near normal ABI on the left leg. The disease process progresses very slowly, as evidenced by an unchanged ABI at 2 years of follow-up in both patients.

The patency in these segmental MMS patients is good on account of their slow disease progression. There is usually multi-segmental involvement of arteries, but in our case proximal and distal arteries to the calcified segment were normal; hence, bypass was possible.[11] The patency in bypass to severely calcified infra-popliteal outflow remains at 60%, which is slightly less than 74% in noncalcified arteries.[12] This is, however, better than the 56.7% patency obtained with endovascular procedures.[2]

 Conclusion



MMS is a rare condition found to affect the media of muscular arteries. The disease progresses very slowly and is segmental in these patients. Chronic limb-threatening ischemia in these patients will require a long bypass or extra-anatomic methods to revascularize the limb.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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