Fig 3 - uploaded by Young-Hwan Hwang
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The microscopic image showed fibromyxoid degeneration (H&E stain; magnification×100,×400 in small box).
Source publication
We describe a case of infrarenal aortic hypoplasia in a 52-yr-old woman who presented with claudication. Computed tomographic angiography revealed an abrupt absence of the infrarenal aorta, with collateral flow reconstituting the iliofemoral systems. After a polytetrafluoroethylene graft was interposed between the aortic stump and the iliac bifurca...
Context in source publication
Context 1
... findings revealed absence of infrarenal aorta repl- aced with fibrous band (Fig. 2). The pathology revealed fibr- omyxoid degeneration (Fig. 3). Polytetrafluoroethylene graft was interposed between the subdiaphragmatic thoracic aorta to the iliac bifurcation. The claudication resolved, and ankle- brachial index became 1.1. For her knee joint arthritis, she was started with methylprednisolon of 8 mg and non-steroid anti- inflammatory drug. After negative results of Pathergy test and human lymphocyte antigen B 51, we ruled out the Behcet's disease and she tapered the medications to stop successfully. She wanted the antihypertensive drug for mild hypertension and was taken amlodipine at 5 mg ...
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Citations
... The external factors that can have a positive impact on the development of small sized aorta are trauma, therapeutic radiation in pregnancy, congenital rubella, hormonal instability, the use of oral contraceptive pills and inflammation. Acquired etiologies were also suggested in fibromuscular dysplasia or Takayasu's arthritis [9]. Small sized aorta has been reported to occur almost exclusively in women and heavy smokers with a relatively early age of onset. ...
... Aortic occlusion or thrombosis occlusion occurs more frequently in SAS more often than in MAS [9]. Because of these facts described case can be presented more as an unusual form of SAS than MAS, despite the involvement in the level of visceral branches (agenesis of RRA). ...
... Usually first appears claudication, especially buttock claudication which later descend below the knee. Trophic changes are the end-stage of leg ischemia, particularly present in chronic abdominal aortic occlusion [9]. Renovascular hypertension is the result of narrowing of renal arteries while digestive symptoms are caused by steno-occlusive changes in splanchnic arteries. ...
A 53-year-old woman presented in vascular emergency department with black change at the top of the right toe and blue staining fingers of the both foots. Patient was examined clinically, with CDS (Color Duplex Scan) and MSCT angiographic examination. It has been confirmed the absence of peripheral pulses of both legs, decreased ABI (right 0.23; left 0.35), thrombosis of the juxta- and infra-renal small sized aorta (diameter 16 mm just above LRA, 11 mm just bellow LRA and only 9 mm just above AB) and the absence of the right renal artery and right kidney. Patient was operated through transperitoneal approach and it has been performed thrombectomy and infrarenal aorta replacement with12 mm Dacron tubular graft.
Objectives
Abdominal aortic coarctation and hypoplasia are uncommon diseases, recognized most often in pediatric-aged individuals. Comprehensive studies regarding the pathologic spectrum of these aortopathies are nonexistent. This investigation was undertaken to better define the histologic and morphologic character of abdominal aortic narrowings affecting children and assess its potential relevance to contemporary clinical practice.
Methods
Aortic specimens obtained during open operations in children being treated for symptomatic, noninflammatory abdominal aortic narrowings at the University of Michigan were subjected to histologic study following hematoxylin-eosin, Movat, Verhoeff Van Gieson, and Masson’s trichrome preparations. Microscopic findings were correlated with the anatomic aortic images. In addition, a detailed review was completed of all prior reports in the English literature that included images depicting the histologic character of noninflammatory abdominal aortic narrowings in children.
Results
Among a series of 67 pediatric-aged individuals undergoing open surgical interventions for abdominal aortic narrowings, eight children ranging in age from 9 months to 18 years, had adequate aortic tissue available for study. The loci of the specimens paralleled the anatomic sites of segmental coarctations observed in the entire series, with involvement of the suprarenal abdominal aorta (3), intrarenal aorta (2), and infrarenal aorta (1). Diffusely hypoplastic abdominal aortas (2) included one case of a de facto aortic duplication, represented by a channel that paralleled the narrow native aorta and gave origin to celiac artery branches, as well as the superior mesenteric and renal arteries. Concentric or eccentric intimal fibroplasia was observed in every aorta, often with internal elastic fragmentation and duplication (4). Media abnormalities included elastic tissue disorganization (3), and focal medial fibrosis (1). Organizing luminal thrombus occurred in two infants. Coexistent ostial stenoses of the celiac, superior mesenteric, or renal arteries were observed in all but the only child who having an infrarenal aortic coarctation. Neurofibromatosis-1 affected one child whose histologic findings were indistinguishable from those of the other children. Review of prior published histologic images of abdominal aortic coarctation and hypoplasia affecting children from other centers revealed a total of 14 separate reports, each limited to single case photomicrographs of which 11 exhibited intimal fibroplasia.
Conclusions
Intimal fibroplasia is a common accompaniment of developmental abdominal aortic coarctation and hypoplasia. It is posited that intimal fibroplasia, that is likely progressive in instances of abnormal shear stresses in these diminutive vessels, may contribute to less salutary outcomes following endovascular and certain open reconstructions of pediatric abdominal aortic narrowings.
Infrarenal aortic hypoplasia is a very rare anomaly of the vascular tree characterised by diffuse stenosis of the infrarenal abdominal aorta. The pathogenesis of this condition seems to be unclear despite the advancements of vascular surgery. In general, the most common presenting symptom is claudication occurring in 3rd to 4th decade of life. Here, a rare and interesting case of infrarenal aortic hypoplasia is reported in a 32 year-old female patient who presented with a history of low backache and bilateral intermittent lower extremity claudication. Using Doppler ultrasonography and computed tomographic angiography, we could demonstrate the abrupt termination of the infrarenal aorta with collateral reformation.
