Pulmonary lymphangioleiomyomatosis (LAM) having no extra-pulmonary manifestations with chemical and mechanical pleurodesis: A case report and review of literature

Q4 Medicine

Radiology Case Reports Pub Date : 2025-05-02 DOI:10.1016/j.radcr.2025.02.103

Mustafa Shehzad MD , Beenish Sabir MBBS , Dawood Shehzad MD , Haris Mumtaz Malik MBBS , Anurag Jha MBBS , Muhammad Nabeel Saddique MBBS , Javed Iqbal RN, Ph.D

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引用次数: 0

Abstract

Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease characterized by abnormal smooth muscle proliferation, leading to cystic destruction of the lung parenchyma. We present the case of a female in her early 40s who presented with intermittent chest pain for 1 month. Imaging revealed left hydropneumothorax with multiple bilateral pulmonary parenchymal cysts, and a subsequent lung biopsy confirmed the diagnosis of pulmonary LAM. The patient underwent video-assisted thoracoscopic surgery (VATS) with mechanical and chemical pleurodesis. Given her stable condition, no immediate sirolimus therapy was initiated, and close follow-up with serial imaging was planned. This case highlights the diagnostic challenges of LAM, especially with low VEGF-D levels, and underscores the role of pleurodesis as a management option in select patients. Early recognition and tailored management are essential to optimize patient outcomes.

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无肺外表现的肺淋巴管平滑肌瘤病伴化学性和机械性胸膜萎缩1例报告及文献复习

淋巴管平滑肌瘤病（LAM）是一种罕见的进行性肺部疾病，以异常平滑肌增生为特征，导致肺实质囊性破坏。我们提出的情况下，在她的40岁出头的女性谁提出了间歇性胸痛1个月。影像学显示左侧气胸积液伴多个双侧肺实质囊肿，随后的肺活检证实肺LAM的诊断。患者接受了胸腔镜手术（VATS），并进行了机械和化学胸膜切除术。鉴于患者病情稳定，未立即开始西罗莫司治疗，并计划密切随访并进行系列影像学检查。本病例强调了LAM的诊断挑战，特别是低VEGF-D水平，并强调了胸膜切除术作为治疗选择的作用。早期识别和量身定制的管理对于优化患者预后至关重要。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Radiology Case Reports Medicine-Radiology, Nuclear Medicine and Imaging

CiteScore

1.10

自引率

0.00%

发文量

1074

审稿时长

30 days

期刊介绍： The content of this journal is exclusively case reports that feature diagnostic imaging. Categories in which case reports can be placed include the musculoskeletal system, spine, central nervous system, head and neck, cardiovascular, chest, gastrointestinal, genitourinary, multisystem, pediatric, emergency, women''s imaging, oncologic, normal variants, medical devices, foreign bodies, interventional radiology, nuclear medicine, molecular imaging, ultrasonography, imaging artifacts, forensic, anthropological, and medical-legal. Articles must be well-documented and include a review of the appropriate literature.