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弥漫性食管平滑肌瘤病及所面临的手术挑战

Diffuse Esophageal Leiomyomatosis and the Surgical Challenges Encountered.

作者信息

Luthra Tanvi, Anand Sachit, Dhua Anjan Kumar, Yadav Devendra, Jain Vishesh, Goel Prabudh, Kandasamy Devasenathipathy, Agarwala Sandeep, Malik Rohan

机构信息

Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.

Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India.

出版信息

J Indian Assoc Pediatr Surg. 2024 Sep-Oct;29(5):549-553. doi: 10.4103/jiaps.jiaps_47_24. Epub 2024 Aug 23.

DOI:10.4103/jiaps.jiaps_47_24
PMID:39479412
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11521230/
Abstract

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.

摘要

弥漫性食管平滑肌瘤病（DEL）是一种罕见疾病，其特征为食管内多个良性平滑肌肿瘤（平滑肌瘤）生长。这些肿瘤可导致食管梗阻和功能障碍，引发吞咽困难、胸痛和体重减轻等症状。该疾病可伴有其他身体部位的平滑肌瘤或肾小球肾病、听力缺陷、散光或近视（阿尔波特综合征）。然而，文献中也有孤立病例的报道。由于肿瘤的位置和范围，DEL的手术治疗存在诸多挑战。在此，我们描述了一名患有DEL且需要多次手术治疗的女孩，突出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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/04d9/11521230/1813ebe5fbee/JIAPS-29-549-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/04d9/11521230/3fc123ef0ca0/JIAPS-29-549-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/04d9/11521230/84aeecb25b08/JIAPS-29-549-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/04d9/11521230/2636e6ae40fd/JIAPS-29-549-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/04d9/11521230/65425f649715/JIAPS-29-549-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/04d9/11521230/a56687be549b/JIAPS-29-549-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/04d9/11521230/1813ebe5fbee/JIAPS-29-549-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/04d9/11521230/3fc123ef0ca0/JIAPS-29-549-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/04d9/11521230/84aeecb25b08/JIAPS-29-549-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/04d9/11521230/2636e6ae40fd/JIAPS-29-549-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/04d9/11521230/65425f649715/JIAPS-29-549-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/04d9/11521230/a56687be549b/JIAPS-29-549-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/04d9/11521230/1813ebe5fbee/JIAPS-29-549-g006.jpg

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