Lymphangiomatosis with pulmonary, mediastinal, and spinal involvement in a pediatric patient: a case report
Abstract
BACKGROUND: Lymphangiomatosis is a rare disorder characterized by abnormal proliferation and dilation of lymphatic vessels, resulting in non-specific clinical manifestations. This report describes the case of an 8-year-old male patient with lymphangiomatosis involving the lungs, mediastinum, and spine, with the objective of promoting awareness and understanding of this condition. CASE PRESENTATION: An 8-year-old male patient presented with chest tightness. Two years ago, the patient underwent decortication of the pleural fibrous plate due to empyema. Within two months after the operation, the patient successively developed chylothorax and chylopericardium. Chest CT revealed thickening of the interlobular septa and peribronchovascular interstitial tissue in both lungs. Spinal MRI showed scoliosis in the cervicothoracic region, flattening of several vertebral bodies, and multiple osteolytic lesions. Pathological examination of mucosal tissue obtained from the basal segment of the left lower lobe of the lung via bronchoscopy confirmed a diagnosis of lymphangiomatosis. The patient’s condition improved significantly after three weeks of treatment with sirolimus and bevacizumab, with marked alleviation of the chest tightness symptoms during the 23-month follow-up period. CONCLUSION: Lymphangiomatosis is associated with indolent progression and non-specific clinical/radiological features, frequently manifesting as multiorgan involvement. Diagnostic suspicion should arise when…
How strong is the evidence?
Evidence Hub reads this as a case report, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
Read the paper
Related studies
- Drug repositioning: a brief overview
- Cyclic nucleotide phosphodiesterases in Drosophila melanogaster
- Time-resolved in silico modeling of fine-tuned cAMP signaling in platelets: feedback loops, titrated phosphorylations and pharmacological modulation
- Current View on Green Tea Catechins Formulations, Their Interactions with Selected Drugs, and Prospective Applications for Various Health Conditions
- In silico Structure-based Identification of Novel Acetylcholinesterase Inhibitors Against Alzheimer’s Disease
- Development and In Vitro Evaluation of Controlled Release Viagra® Containing Poloxamer-188 Using Gastroplus™ PBPK Modeling Software for In Vivo Predictions and Pharmacokinetic Assessments
Go deeper in Evidence Hub
Evidence Hub adds a study data sheet (design, sample size, evidence level, key numbers), an AI summary and a chat grounded in the paper's text.