Lymphangiomatosis is a rare disorder. It is commonly localised in the skeletal system and called Gorham's disease, and in thoracic or abdominal organs. Involvement of the pericardium is rare and has been described in less than 20 patients worldwide. The case of a 14-year-old boy presenting with asymptomatic chylopericardium and interstitial lung disease is presented. After lung biopsy, performed to confirm the diagnosis of pulmonary lymphangiomatosis, he developed chylothorax and massive loss of chyle via chest drains. Thoracic duct ligation did not result in clinical improvement. Treatment with interferon α2b was given and because of clinical deterioration radiotherapy was added to the treatment. This resulted in a rapid decrease of chyle production in the patient.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3047284 | PMC |
| http://dx.doi.org/10.1136/bcr.08.2009.2206 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Lyve1-Driven Nras Causes Edema, Enlarged Lymphatic Vessels, and Hepatic Vascular Defects in Embryonic Mice.
Pediatr Blood Cancer
December 2024
Division of Pulmonary Biology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.
Background: Kaposiform lymphangiomatosis (KLA) is a complex lymphatic anomaly associated with a somatic activating NRAS p.Q61R (NRAS) mutation. KLA is characterized by malformed lymphatic vessels that can lead to effusions and coagulopathy.
View Article and Find Full Text PDFPulmonary lymphangiomatosis: insights into an ultra-rare disease.
Respir Res
November 2024
Center for Pulmonary Medicine, Department of Pneumology, Mainz University Medical Center, Mainz, Germany.
Article Synopsis
- Pulmonary lymphangiomatosis (PL) is an extremely rare lung disease marked by abnormal lymphatic growth, with no established diagnostic or treatment guidelines, prompting a study to gather patient data.
- The study reviewed 12 patients diagnosed from 1996 to 2022, noting that most were younger women, non-smokers, and commonly experienced symptoms like difficulty breathing and coughing up blood or lymphatic fluid, with severe impacts on lung function.
- Treatment with sirolimus resulted in significant improvements for patients, although further research is needed to fully understand the disease and develop comprehensive treatment strategies.
Pericardial fenestration and thoracic duct ligation for treatment of chylopericardium as first symptom of underlying generalized lymphatic anomaly: a case report.
Acta Chir Belg
February 2025
Department of Thoracic Surgery, University Hospitals Leuven, Leuven, Belgium.
Introduction: Chylopericardium represents a rare condition of chyle accumulation within the pericardial sac, caused by abnormal thoracic duct anatomy or prolonged increased pressure. Nothing by mouth (NPO) policy and total parenteral nutrition (TPN), even in combination with pericardial drainage, render only a temporary solution. Surgical intervention with thoracic duct ligation and creation of a pericardial window is believed to be the most effective treatment.
View Article and Find Full Text PDFDiffuse pulmonary lymphangiomatosis as a differential diagnosis of anterior mediastinal mass.
J Surg Case Rep
September 2024
Department of Thoracic Surgery, Fundación Cardioinfantil, Bogotá, 111321, Colombia.
Diffuse pulmonary lymphangiomatosis (DLP) is an extremely rare silent disease, characterized by proliferation and thickening of abnormal pulmonary, pleural, and mediastinal soft tissue lymphatic channels. Its clinical presentation is nonspecific symptoms such as cough, dyspnea, and hemoptysis. Tomographic findings for DLP include thickening of the interlobular septa and peribronchovascular interstitium and ground glass opacities.
View Article and Find Full Text PDFGeneralized lymphatic anomaly: A case report and review of literature.
J Cutan Pathol
October 2024
Dermatology and Venereology Department, Faculty of Medicine, Assiut University, Assiut, Egypt.
Generalized lymphatic anomaly (GLA), previously known as lymphangiomatosis, is a rare developmental disease characterized by abnormal proliferation of lymphatic vascular structures that may involve the dermis, soft tissue, bone, and visceral parenchyma. Being an uncommon condition and the lack of specific symptoms often result in a delayed diagnosis or even misdiagnosis, which, in addition to its progressive nature, can lead to dysfunction of vital organs, and ultimately, a poor prognosis. In this report, we present a unique case of GLA in an upper Egyptian female child.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!