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Diagnosis and treatment of diffuse pulmonary lymphangioma in children: A case report
Xiaopu Sun1, Chengyu Lu1, Zhanhang Huang1
1Department of Pediatrics, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong 510120, P.R. China.
Insights
Diffuse pulmonary lymphangioma (DPL) in children presents with cough, shortness of breath, and chylothorax. Diagnosis relies on biopsy, while combined therapies show promise for improving outcomes.
Area of Science:
- Pediatric Pulmonology
- Thoracic Pathology
- Medical Imaging
Background:
- Diffuse pulmonary lymphangioma (DPL) is a rare condition characterized by lymphatic vessel proliferation in the lungs.
- Accurate diagnosis and effective treatment strategies for pediatric DPL remain challenging.
- Understanding the clinical, imaging, and pathological features is crucial for patient management.
Observation:
- A pediatric case of DPL presented with cough, dyspnea, hemoptysis, bloody chylothorax, and pericardial effusion.
- Chest CT revealed characteristic reticular opacities and thickened interlobular septa.
- Lung biopsy confirmed lymphatic vessel hyperplasia, with immunohistochemistry positive for CD31 and D2-40.
Findings:
- DPL exhibits nonspecific clinical and imaging findings, necessitating pathological confirmation.
- Combined treatment with methylprednisolone, propranolol, sirolimus, and somatostatin showed improvement.
- Conservative management of chylothorax and pleural effusion yielded positive therapeutic effects.
Implications:
- B-ultrasound-guided biopsy is a safe and effective diagnostic tool for DPL.
- Propranolol-sirolimus combination therapy shows potential, though individual responses may vary.
- Further research is needed to optimize treatment protocols for pediatric DPL.
Abstract:
The present study aimed to investigate the clinical characteristics of diffuse pulmonary lymphangioma (DPL) in children to improve the diagnosis and treatment of this disease. A case of pediatric DPL was observed for its clinical symptoms, imaging features, lung biopsy pathological characteristics and immunohistochemical phenotypes, and relevant literature was also reviewed. The main clinical manifestations of this pediatric patient were a cough, shortness of breath, hemoptysis, bloody chylothorax and pericardial effusion. Chest computed tomography showed a grid-like shadow and markedly thickened interlobular septa. Pathological examination revealed lymphatic vessel hyperplasia and expansion. Immunohistochemistry showed positive staining of lymphatic endothelial cells CD31 and D2-40. The patient's condition improved after combined treatment with methylprednisone, propranolol, sirolimus and somatostatin, whose bloody chylothorax also achieved good therapeutic effect after conservative treatment. Overall, the clinical and imaging appearances of DPL are lack of characterization, and its clinical manifestations include cough, shortness of breath and chylothorax. Computed tomography may show mesh-like shadows of both lungs and thickened interlobular septa. The definite diagnosis of DPL depends on biopsy pathology. In addition to this case, B-ultrasound-guided puncture biopsy is effective and safe, and propranolol-sirolimus treatment has a certain effect, but the clinical effect may be different. Conservative treatment of pleural effusion can result in better curative effect.
