and Beth L. Johnson2
(1)
Surgical Pathology and Laboratory Medicine, Keck School of Medicine (Ret), University of Southern California (USC), Chatsworth, CA, USA
(2)
Northwestern Medicine, At Central DuPage Hospital, Winfield, IL, USA
(3)
International College of Surgeons, Cardiothoracic and Vascular Surgery, Glen Ellyn, IL, USA
Keywords
Ischemic pathologyAmputationArteriosclerosisRaynaud’s diseaseBuerger’s disease (TAO)Behcet’s diseaseHeyde’s syndromeGangreneIntroduction
The title ischemic limb disease affects both the upper but, in general, predominately the lower extremities. This disease entity is very multifactorial and in many of its diagnostic subsections can be very challenging. The principal diagnostic classifications include occlusive arteriosclerotic disease , with secondary thrombosis or embolism, effecting about 85 % of the cases in this category—as illustrated in Table. 4.1 [1, 2]. Miscellaneous ischemic etiologies comprise the remainder of the 15 % of clinical cases. The embolic cases, including the thrombus and tumor emboli, represent a variety of unrelated but nevertheless equally destructive clinical outcomes [3, 4]. More uncommonly, in massive trauma and extremity fractures, the rare fat and marrow embolism may be associated with extensive ischemia. Vascular dissection of major arterial channels is definitely associated with ischemic damage to distally normally perfused extremities and organs [2, 5]. Consequences of fluid, specifically amniotic fluid embolism, are significant but rarer.
Table 4.1
Pathologic considerations of critical ischemia of the extremities
Occlusive arteriosclerotic disease |
Majority of patients |
Embolic |
Tumor |
Thrombus |
Fat |
Marrow |
Amniotic |
Foreign body |
Vascular dissection |
Surgery |
Aneurysms with clots |
Raynaud’s |
Primary |
Secondary |
Other associated diseases |
Buerger’s/thromboangiitis obliterans (TOA) |
Behcet’s disease/Marco Polo disease |
Coagulation defects |
Fig. 4.1
Artery with calcified media and thrombus in patient who required a below-knee amputation for ischemia and gangrene
Both central and peripheral ischemic vascular limb disease have been studied extensively. Figures 4.1, 4.2 and 4.3 demonstrate the underlying vascular process seen in many of these ischemic legs. An early radiologically and pathologically correlated study of the disease concepts in the study of the aortic arch and brachiocephalic vessels was presented in Tokyo in 1969 [3]. The vascular lesions were appropriately documented in a graphic design. The study was histopathologically amplified by detailed microphotographs of the classical features of occlusive atherosclerosis, gamut of cholesterol granulomas, old and recent thrombosis, as well as gitter cells (macrophages seen in brain infarcts) in the brain. Further, denoted were white versus hemorrhagic infarcts, a rare but debilitating and sometimes fatal complication of carotid endarterectomy [6]. Also of note, in relation to complications of management of atherosclerotic vessel disease is the rare but nevertheless significant occurrence of atherosclerotic embolization to distal organs, such as the liver, spleen, kidneys, and extremities, especially during vascular surgery. The following paragraphs depict and mention a variety of pathological entities, which amplify the diversity of causes responsible for ischemic limb diseases, as discussed and presented in photographic form by Endlich and Roscher [4].
Fig. 4.2
Right femoral artery, plaque and thrombus
Fig. 4.3
Photomicrograph of luminal thrombus from the right femoral artery
Many times the pathologist is the final physician to see the extremity with severe ischemic concerns. When the pathologist receives the amputated digit or extremity, the patient’s options have been exhausted from the clinical and pathologic aspects in most circumstances. Despite recent advances in both diagnostic and therapeutic approaches, many patients face the consideration of amputation each year. Arteriosclerotic vascular changes with the resultant loss of the leg are illustrated in amputation specimens (Fig. 4.4). Multiple experimental and clinical programs and products have been developed, applied, and studied with improvement but not prevention. Thus, new multicenter programs to involve multi-specialty and various conceptual programs have been initiated with multi-specialty representation to hopefully reduce and minimize the amputation rate. The etiology and variety of ischemic producing diseases are multiple, and a few of the less common such nonatherosclerotic causes of ischemic pathology are herein listed.
Fig. 4.4
Atherosclerotic gangrene necessitating amputation
Raynaud’s Disease
Pathological entities, such as Raynaud’s disease , affecting primarily the upper extremities may lead to poor distal perfusion and remains poorly understood. The disease is clinically characterized by hypersensitivity of the skin to cold items such as cold dinnerware to the affected limb (i.e., hand or arm) and leading to vascular spasm and ischemia. Primary Raynaud’s has a 75 % predilection for female patients with the 15–40 age group predominating. In this entity, there usually is no vascular pathologic damage demonstrable. Primary Raynaud’s disease (white skin disease) due to the release of norepinephrine produces muscular vessel spasm and temporary limb ischemia. However, in secondary Raynaud’s disease, there is an association with other diseases such as mono- and polymyositis, dermatomyositis, scleroderma, lupus, rheumatoid arthritis, and carpal tunnel syndrome. Sjogren’s syndrome has also been reported to occur in patients with secondary Raynaud’s disease. In the secondary form of Raynaud’s, there is a 25 % incidence of a family history predilection. This implies a genetic causation for Raynaud’s disease. Causative factors postulated to elicit Raynaud’s symptoms include a variety of unrelated entities such as stress, cold remedies, beta blockers , and exposure to cancer chemotherapy. A very unusual example of a patient with a long history of the Raynaud’s phenomenon is seen in Figs. 4.5, 4.6, 4.7, and 4.8.
