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[Diseases of the pulmonary lymphatic system in children]
1Service de Pneumologie Pédiatrique, INSERM E0213, Hôpital d'Enfants Armand-Trousseau, 26, avenue du Dr. Arnold-Netter, 75571 Paris Cedex 12. ralph.epaud@trs.ap-hop-paris.fr
Insights
Pediatric lymphatic system diseases, like chylothorax, involve complex lung lymphatic anatomy and physiology. Management is challenging, with treatments including diet, medications, and surgery, but efficacy assessment is difficult.
Area of Science:
- Pediatric Pulmonology
- Lymphatic System Physiology
- Thoracic Medicine
Context:
- Diseases of the lymphatic system in children present unique management challenges.
- The pulmonary lymphatic system's complex anatomy and physiology are crucial for regulating interstitial fluid.
- Dysplasia in lymphatic vessels can lead to conditions like chylothorax and pulmonary interstitial fluid overload.
Purpose:
- To review the complexities of pediatric lymphatic system diseases affecting the lungs.
- To outline current and potential therapeutic strategies for these rare conditions.
- To highlight the difficulties in assessing treatment efficacy.
Summary:
- Pediatric lymphatic disorders, particularly those involving the lungs, are rare and complex.
- Dysplastic lymphatics disrupt normal function, causing fluid leakage and conditions such as chylothorax.
- Current management involves dietary changes, medications (cytotoxic agents, somatostatin, interferon-alpha), and surgery, though efficacy is hard to determine.
Impact:
- Improved understanding of pulmonary lymphatic dysfunction in pediatric patients.
- Informed clinical decision-making for managing rare lymphatic diseases.
- Highlights the need for further research into effective and measurable therapeutic interventions.
Abstract:
Diseases of the lymphatic system in children include a group of exceptional conditions difficult to manage. The anatomy of lymphatic system is complex in the lung. Variable from one subject to another, its complex physiology plays an important role in air-blood exchanges occurring in the lung. In the pulmonary interstitium and in the pleura, the lymphatic system acts like an overflow valve capable of regulating variations in interstitial fluid. The presence or development of dysplasic lymphatics causes leakage, dilatation, and reflux of the lymph through incontinent valves leading to chylothorax and/or fluid overload in the pulmonary interstitium. Symptomatic care is usually proposed, based on a fat-free diet supplemented with light-chain triglycerides and liposoluble vitamins. Other therapeutic options can be proposed. Medical options include cytotoxic agents, somatostatin, and interferon-alpha. Surgery may also be useful, but an assessment of therapeutic efficacy is very difficult due to partial effects and the small number of cases studied.