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A case report of generalized lymphangiomatosis with chylopericardium: the crucial role of magnetic resonance lymphangiography.

ABSTRACT: Background:Chylopericardium due to generalized lymphangiomatosis is a rare clinical entity. Its aetiology and treatment remain unclear. Case summary:We report one case of a 51-year-old man who was diagnosed with generalized lymphangiomatosis with idiopathic chylopericardium by bilateral inguinal intranodal contrast-enhanced magnetic resonance lymphangiography. Magnetic resonance lymphangiography demonstrated abnormal communications between the pericardial sac and the thoracic duct. The patient with idiopathic chylopericardium was therefore successfully treated by exclusive surgical ligation of the abnormal communications and partial pericardiectomy by thoracotomy. The patient's postoperative recovery was uneventful, and no recurrence of pericardial effusion occurred during the 13-month follow-up. Discussion:Magnetic resonance lymphangiography showed a good capability for evaluating the extent of generalized lymphangiomatosis and therefore is helpful for delineating the anatomy of the thoracic duct and identifying chyle leakage and abnormal communications between the thoracic duct and the pericardial sac. This makes a simple ligature of the abnormal communications, instead of thoracic duct mass ligation possible in the treatment of chylopericardium.

SUBMITTER: Mi H

PROVIDER: S-EPMC7649488 | biostudies-literature | 2020 Oct

REPOSITORIES: biostudies-literature

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A case report of generalized lymphangiomatosis with chylopericardium: the crucial role of magnetic resonance lymphangiography.

Mi Honglan H Chi Jiachang J Zhao Xiaojing X Lu Qing Q

European heart journal. Case reports 20200909 5

<h4>Background</h4>Chylopericardium due to generalized lymphangiomatosis is a rare clinical entity. Its aetiology and treatment remain unclear.<h4>Case summary</h4>We report one case of a 51-year-old man who was diagnosed with generalized lymphangiomatosis with idiopathic chylopericardium by bilateral inguinal intranodal contrast-enhanced magnetic resonance lymphangiography. Magnetic resonance lymphangiography demonstrated abnormal communications between the pericardial sac and the thoracic duct ...[more]

PMID: 33204977

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Project description:BackgroundClinical examination and lymphoscintigraphy are the current standard for investigating lymphatic function. Magnetic resonance imaging (MRI) facilitates three-dimensional (3D), nonionizing imaging of the lymphatic vasculature, including functional assessments of lymphatic flow, and may improve diagnosis and treatment planning in disease states such as lymphedema.PurposeTo summarize the role of MRI as a noninvasive technique to assess lymphatic drainage and highlight areas in need of further study.Study typeSystematic review.PopulationIn October 2019, a systematic literature search (PubMed) was performed to identify articles on magnetic resonance lymphangiography (MRL).Field strength/sequenceNo field strength or sequence restrictions.AssessmentArticle quality assessment was conducted using a bespoke protocol, designed with heavy reliance on the National Institutes of Health quality assessment tool for case series studies and Downs and Blacks quality checklist for health care intervention studies.Statistical testsThe results of the original research articles are summarized.ResultsFrom 612 identified articles, 43 articles were included and their protocols and results summarized. Field strength was 1.5 or 3.0 T in all studies, with 25/43 (58%) employing 3.0 T imaging. Most commonly, imaging of the peripheries, upper and lower limbs including the pelvis (32/43, 74%), and the trunk (10/43, 23%) is performed, including two studies covering both regions. Imaging protocols were heterogenous; however, T2 -weighted and contrast-enhanced T1 -weighted images are routinely acquired and demonstrate the lymphatic vasculature. Edema, vessel, quantity and morphology, and contrast uptake characteristics are commonly reported indicators of lymphatic dysfunction.Data conclusionMRL is uniquely placed to yield large field of view, qualitative and quantitative, 3D imaging of the lymphatic vasculature. Despite study heterogeneity, consensus is emerging regarding MRL protocol design. MRL has the potential to dramatically improve understanding of the lymphatics and detect disease, but further optimization, and research into the influence of study protocol differences, is required before this is fully realized.Level of evidence2 TECHNICAL EFFICACY: Stage 2.

Project description:Background and Objectives: When considering surgery for patients with breast cancer-related lymphedema (BCRL), it is crucial to determine which surgery will be most effective for the patient and establish the indications for each surgery. Our study retrospectively compared the results of preoperative noncontrast MR lymphangiography (NMRL) performed on the lymphedematous limb of patients before surgery, with the aim of analyzing whether preoperative NMRL can be used as a criterion for determining the type of surgery. Materials and Methods: From January 2020 to June 2022, a total of 138 patients with lymphedema underwent surgery at Seoul National University Bundang Hospital. All patients underwent preoperative NMRL imaging and were classified into stages 1-3 based on the MRI severity index using the authors' previous reference. Three types of surgery, LVA, LVA + liposuction, and LVA + VLNT, were conducted on all patients. The effectiveness of the surgery was evaluated one year postoperatively using the interlimb volume difference before and after surgery, the fluid volume of the edematous limb measured by bioimpedance spectroscopy, and the subjective satisfaction of the patients through the Lymph Q questionnaire. Results: In this study, out of a total of 138 patients, 26 (19%) were MRI stage 1, 62 (45%) were stage 2, and 50 (36%) were stage 3. Of the 83 patients who underwent LVA surgery, the greatest decrease in interlimb volume difference was observed in stage 2 patients, and subjective satisfaction was also the most effective in stage 2. In the case of LVA + liposuction patients, a significant volume decrease and a high satisfaction were observed in stage 3 patients. In the case of LVA + VLNT patients, there was no difference in volume decrease according to the stage, but a greater decrease in body fluid volume was observed as the MRI severity index score increased through BIA. Conclusions: In conclusion, this study demonstrates that NMRL imaging is a useful modality for determining the most effective surgical method and predicting the surgical outcome in patients with lymphedema. This highlights the importance of using NMRL in the treatment planning of lymphedema patients.

Dataset Information

A case report of generalized lymphangiomatosis with chylopericardium: the crucial role of magnetic resonance lymphangiography.

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A case report of generalized lymphangiomatosis with chylopericardium: the crucial role of magnetic resonance lymphangiography.

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