Celis Pinto Juan Carlos, Hernández Peláez Lucía, Mendoza Pacas Guillermo, Mayordomo Colunga Juan, Balbín Milagros, Pitiot Ana, Torres-Rivas Héctor-Enrique, Blanco Lorenzo Verónica
Hospital Universitario Central de Asturias (HUCA), Pathology Department, Oviedo, Asturias, Spain.
Hospital Universitario Central de Asturias (HUCA), Pediatric Department, Oviedo, Asturias, Spain.
Autops Case Rep. 2023 May 31;13:e2023434. doi: 10.4322/acr.2023.434. eCollection 2023.
Sclerosing Mesenteritis (SM) is a rare diagnosis, particularly in pediatric patients, and is typically non-fatal when appropriately treated. Although molecular and immunohistochemical alterations have been described, no pathognomonic signature has been identified for this entity. This report presents a case of a seven-year-old boy who suffered sudden cardiorespiratory arrest. Upon autopsy, he was found to have multicentric SM on the upper mesentery, which led to bowel wall thinning and abdominal bleeding with bacterial translocation. We performed comprehensive morphological, immunohistochemical, and molecular analyses. SM is an atypical disorder with diverse clinical manifestations, including a rare but potentially fatal course. Early diagnosis is critical, given its potential severity. To our knowledge, this is the first case report of pediatric mortality linked to SM. Our findings emphasize the importance of increased awareness and early detection of SM in pediatric patients.
硬化性肠系膜脂膜炎（SM）是一种罕见的诊断，尤其是在儿科患者中，并且在得到适当治疗时通常不会致命。尽管已经描述了分子和免疫组化改变，但尚未确定该实体的特征性标志。本报告介绍了一名七岁男孩突然发生心肺骤停的病例。尸检时发现他在上肠系膜有多中心性SM，导致肠壁变薄、腹腔出血并伴有细菌移位。我们进行了全面的形态学、免疫组化和分子分析。SM是一种具有多种临床表现的非典型疾病，包括罕见但可能致命的病程。鉴于其潜在的严重性，早期诊断至关重要。据我们所知，这是第一例与SM相关的儿科死亡病例报告。我们的研究结果强调了提高对儿科患者SM的认识和早期检测的重要性。
