Department of Radiology, The Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.
Department of Surgery, St. Christopher's Hospital for Children, Drexel University School of Medicine, Philadelphia, PA, USA.
Clin Imaging. 2019 Nov-Dec;58:161-165. doi: 10.1016/j.clinimag.2019.07.008. Epub 2019 Jul 23.
A 15 year old female with a history only significant for long standing dysphagia and intermittent chest pain underwent a screening chest radiograph. Findings of a large mass prompted cross sectional imaging where extensive and marked diffuse esophageal thickening was demonstrated. The patient ultimately underwent biopsy and surgical resection for diffuse esophageal leiomyomatosis. Diffuse leiomyomatosis of the esophagus has been described in the literature for about 100 years with increasing recognition in more recent times. There is a known association with inherited syndromes such as Alport and Esophageal-Vulvar syndrome, though some cases are sporadic. This case report will demonstrate the imaging features of diffuse esophageal leiomyomatosis utilizing multiple modalities including radiography, fluoroscopy, CT, and MRI, with surgical and pathologic correlation.
一位 15 岁的女性,既往仅表现为长期吞咽困难和间歇性胸痛,进行了筛查性胸部 X 线检查。大片肿块的发现促使进行了横断面成像,显示广泛且明显弥漫性食管增厚。患者最终接受了活检和手术切除以治疗弥漫性食管平滑肌瘤病。弥漫性食管平滑肌瘤病在文献中已有约 100 年的描述,近年来越来越受到重视。已知其与遗传综合征有关,如 Alport 和食管-外阴综合征,但有些病例是散发性的。本病例报告将展示利用多种模态(包括放射摄影、荧光透视、CT 和 MRI)进行的弥漫性食管平滑肌瘤病的影像学特征,并与手术和病理相关联。