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Rev. esp. patol ; 54(1): 17-21, ene.-mar. 2021. ilus
Article in English | IBECS (Spain) | ID: ibc-202486

ABSTRACT

Mesenteric arteriovenous vasculopathy (MAVD/V) is an extremely rare and poorly understood disease and its incidence is probably underestimated. It is an uncommon, non-inflammatory and non-atherosclerotic form of mesenteric vascular injury, first reported in 2016, with characteristic histopathologic evidence of fibromuscular dysplasia-like vascular changes. We present the case of a chronically ill 84-year-old female with a 5 year history of recurrent small bowel obstruction, who underwent segmental resection of the small bowel. Intraoperative examination showed bowel stricture with fibrosis, intraluminal pill fragments and creeping mesenteric adipose tissue clinically compatible with Crohn's disease. Histological examination showed acute and chronic mucosal injury characterized by crypt distortion, ulcerations with granulation tissue, pseudo-pyloric metaplasia, areas of fibrosis and serosal adhesions. Multiple blood vessels (including both veins and arteries) demonstrated wall hyalinization, elastic degeneration and non-atherosclerotic luminal occlusion. The pattern of the mucosal injury is, in this case, potentially a consequence of acute and chronic ischemic processes secondary to mesenteric arteriovenous vasculopathy


La vasculopatía arteriovenosa mesentérica (MAVD/V) es una enfermedad extremadamente rara y poco conocida, con una incidencia probablemente subestimada. Se trata de una forma infrecuente, no inflamatoria y no aterosclerótica de lesión vascular mesentérica, reportada por primera vez en 2016, con evidencia histopatológica característica de cambios vasculares de tipo displasia fibromuscular. Presentamos el caso de una paciente crónica de 84 años de edad, con historia de cinco años de obstrucción recurrente de intestino delgado a quien se le practicó resección segmental del mismo. El examen intraoperatorio reveló estenosis intestinal con fibrosis, fragmentos intraluminales de píldoras, y tejido adiposo mesentérico serpiginoso clínicamente compatible con enfermedad de Crohn. El examen histológico reveló lesión mucosa aguda y crónica, evidenciada por distorsión de la cripta, ulceraciones con tejido granuloso, metaplasia pseudopilórica, áreas de fibrosis y adherencias serosas. Los múltiples vasos sanguíneos (incluyendo venas y arterias) reflejaron hialinización de la pared, degeneración elástica y oclusión luminal no aterosclerótica. El patrón de la lesión mucosa es, en este caso, una consecuencia potencial de un proceso isquémico crónico secundario a vasculopatía arteriovenosa mesentérica


Subject(s)
Humans , Female , Aged, 80 and over , Fibromuscular Dysplasia/diagnosis , Fibromuscular Dysplasia/pathology , Mesenteric Vascular Occlusion/pathology , Mesenteric Arteries/pathology , Mesenteric Veins/pathology , Crohn Disease/diagnosis , Diagnosis, Differential , Mesenteric Vascular Occlusion/diagnosis , Constriction, Pathologic/pathology , Tomography, X-Ray Computed , Intestinal Obstruction/diagnostic imaging
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