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Mönckeberg’s arteriosclerosis, also called medial calcific sclerosis or Mönckeberg’s sclerosis, is a form of vessel hardening due to increased calcium deposits in the tunica media layer. There is disagreement over its clinical significance and aetiology and its relation to atherosclerosis and vascular calcification. Its clinical presentations and treatment are still debated. More effort should be directed on attempting to distinguish between atherosclerotic lesions and Mönckeberg’s lesions on the basis of age, location and the pattern of calcifications where there is considerable overlap between intimal or medial and involvement of the internal elastic lamina border between those planes. In-depth research is still needed to create consensus guidelines for the diagnosis and management of this condition. This article includes a review of the literature and a case report of a 22-year-old man with the condition.

The aim of this article is to report and highlight on a new fatal presentation for ATS. Arterial tortuosity syndrome (ATS) is a very rare genetic disorder, with connective tissues involvement. All over the body and it affects both sexes equally. Approximately one hundred patients have been registered in the literature. Patients with ATS have facial, musculoskeletal, genitourinary, visceral, skin and abdominal wall defects. It is associated with life-threatening complications in infancy and early childhood and the first few months of life are crucial for the high possibility of lifethreatening events such as cardiac or even respiratory failure. ATS is an autosomal recessive disease due to mutation(s) in the SLC2A10 gene, and up to 23 mutations have been identified in the SLC2A10 gene by highly specific genetic investigations. The chance for a child to be genetically normal for that trait is 25%. The risk is equal for both males and females.

Objective: Mönckeberg arteriosclerosis is called medial calcific sclerosis, which is a form of vessel hardening due to increased calcium deposits in the tunica media layer. Its clinical significance and etiology and its relation to atherosclerosis and vascular calcification are still a matter of disagreement. Its clinical presentations and the treatment are still debatable.Methods: The aim of this article was to focus on a special pathologic entity that the vascular surgeon could encounter and that could be a limbthreatening and consequently life-threatening condition. In our reported case, a 22-year-old, average-build young man reported repeated muscle cramps and fatigue with heavy exercise that were relieved by rest. The condition was then neglected by him but increased later, and bilateral claudication pain with increased duration of pain was noticed with regular exercise.Results: The patient was fully investigated by laboratory and radiologic study. Computed tomography angiography showed extensive calcification affecting the vasculature of both lower limbs bilaterally (mirror images anatomically; Figs 1 and 2). The patient is under medical treatment and follow-up as of the writing of this case Conclusions: More effort should be expended in an attempt to distinguish between atherosclerotic lesions and Mönckeberg lesions on the basis of age predominance and location. The pattern of calcifications had considerable overlap between intima and media and involved the internal elastic lamina border between those planes.

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