{"title":"弥漫性食管子宫内膜异位症及其手术挑战。","authors":"Tanvi Luthra, Sachit Anand, Anjan Kumar Dhua, Devendra Yadav, Vishesh Jain, Prabudh Goel, Devasenathipathy Kandasamy, Sandeep Agarwala, Rohan Malik","doi":"10.4103/jiaps.jiaps_47_24","DOIUrl":null,"url":null,"abstract":"<p><p>Diffuse esophageal leiomyomatosis (DEL) is a rare condition characterized by the growth of multiple benign smooth muscle tumors (leiomyomas) in the esophagus. These tumors can cause obstruction and functional impairment of the esophagus, leading to symptoms such as difficulty swallowing, chest pain, and weight loss. The disease can present with leiomyomas or glomerular nephropathy in other body parts, hearing defects, astigmatism, or myopia (Alport's syndrome). However, isolated cases are also reported in the literature. Surgical management of DEL presents several challenges due to the location and extent of the tumors. Herein, we describe a girl afflicted with DEL and required multiple surgeries for management, highlighting the unique challenges faced by the surgical team in restoring esophageal function and improving the patient's quality of life.</p>","PeriodicalId":16069,"journal":{"name":"Journal of Indian Association of Pediatric Surgeons","volume":"29 5","pages":"549-553"},"PeriodicalIF":0.0000,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521230/pdf/","citationCount":"0","resultStr":"{\"title\":\"Diffuse Esophageal Leiomyomatosis and the Surgical Challenges Encountered.\",\"authors\":\"Tanvi Luthra, Sachit Anand, Anjan Kumar Dhua, Devendra Yadav, Vishesh Jain, Prabudh Goel, Devasenathipathy Kandasamy, Sandeep Agarwala, Rohan Malik\",\"doi\":\"10.4103/jiaps.jiaps_47_24\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Diffuse esophageal leiomyomatosis (DEL) is a rare condition characterized by the growth of multiple benign smooth muscle tumors (leiomyomas) in the esophagus. These tumors can cause obstruction and functional impairment of the esophagus, leading to symptoms such as difficulty swallowing, chest pain, and weight loss. The disease can present with leiomyomas or glomerular nephropathy in other body parts, hearing defects, astigmatism, or myopia (Alport's syndrome). However, isolated cases are also reported in the literature. Surgical management of DEL presents several challenges due to the location and extent of the tumors. Herein, we describe a girl afflicted with DEL and required multiple surgeries for management, highlighting the unique challenges faced by the surgical team in restoring esophageal function and improving the patient's quality of life.</p>\",\"PeriodicalId\":16069,\"journal\":{\"name\":\"Journal of Indian Association of Pediatric Surgeons\",\"volume\":\"29 5\",\"pages\":\"549-553\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-09-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521230/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Indian Association of Pediatric Surgeons\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/jiaps.jiaps_47_24\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/8/23 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Indian Association of Pediatric Surgeons","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/jiaps.jiaps_47_24","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/8/23 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
摘要
弥漫性食管平滑肌瘤病(DEL)是一种罕见的疾病,其特点是食管内生长多个良性平滑肌瘤(平滑肌瘤)。这些肿瘤可造成食道阻塞和功能障碍,导致吞咽困难、胸痛和体重减轻等症状。这种疾病还可能伴有身体其他部位的肾小球肾病、听力缺陷、散光或近视(阿尔波特综合征)。不过,文献中也有个别病例的报道。由于肿瘤的位置和范围,DEL 的手术治疗面临着一些挑战。在此,我们描述了一名患有 DEL 并需要多次手术治疗的女孩的情况,突出强调了手术团队在恢复食管功能和改善患者生活质量方面所面临的独特挑战。
Diffuse Esophageal Leiomyomatosis and the Surgical Challenges Encountered.
Diffuse esophageal leiomyomatosis (DEL) is a rare condition characterized by the growth of multiple benign smooth muscle tumors (leiomyomas) in the esophagus. These tumors can cause obstruction and functional impairment of the esophagus, leading to symptoms such as difficulty swallowing, chest pain, and weight loss. The disease can present with leiomyomas or glomerular nephropathy in other body parts, hearing defects, astigmatism, or myopia (Alport's syndrome). However, isolated cases are also reported in the literature. Surgical management of DEL presents several challenges due to the location and extent of the tumors. Herein, we describe a girl afflicted with DEL and required multiple surgeries for management, highlighting the unique challenges faced by the surgical team in restoring esophageal function and improving the patient's quality of life.
期刊介绍:
Journal of Indian Association of Pediatric Surgeons is the official organ of Indian Association of Pediatric Surgeons. The journal started its journey in October 1995 under the Editor-in-Chief Prof. Subir K Chatterjee. An advisory board was formed with well-versed internationally reputed senior members of our society like Late Prof. R K Gandhi, Prof. I C Pathak, Prof. P Upadhyay, Prof. T Dorairajan and many more. since then the journal is published quarterly uninterrupted. The journal publishes original articles, case reports, review articles and technical innovations. Special issues on different subjects are published every year. There have been several contributions from overseas experts.