Mediastinal lymphangioma and intestinal lymphangiomatosis presenting with chylothorax: a systematic review of therapeutic modalities
Abstract Introduction Chylothorax is defined by the accumulation of chyle in the pleural space, characterized by triglyceride levels exceeding 110 milligrams per deciliter. The clinical presentation of chylothorax varies depending on its acuity and underlying etiology. Mediastinal lymphangiomas are...
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2025-04-01
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| Online Access: | https://doi.org/10.1186/s12890-025-03664-3 |
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| author | Marc Assaad Roshan Acharya Wasif Shamsi Elspeth Springsted Mahtab Foroozesh Maria del Mar Cirino-Marcano |
| author_facet | Marc Assaad Roshan Acharya Wasif Shamsi Elspeth Springsted Mahtab Foroozesh Maria del Mar Cirino-Marcano |
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| description | Abstract Introduction Chylothorax is defined by the accumulation of chyle in the pleural space, characterized by triglyceride levels exceeding 110 milligrams per deciliter. The clinical presentation of chylothorax varies depending on its acuity and underlying etiology. Mediastinal lymphangiomas are extremely rare and benign lesions that can affect both infants and adults. They may occur independently or in association with other lymphatic disorders and can lead to complications such as chylothorax. Case report A 60-year-old male patient presented with shortness of breath and was diagnosed with left-sided chylothorax secondary to mediastinal lymphangioma, accompanied by intestinal lymphangiomatosis. Conservative approach was unsuccessful, and surgical therapy was needed. Materials and methods We conducted a thorough search of the PubMed/MEDLINE, PubMed Central, and Google Scholar databases. The search parameters we used included the following Boolean terms: [(“lymphangioma” OR “mediastinal lymphangioma” OR “cavernous lymphangiomas” OR “cystic hygromas” OR “capillary lymphangioma” OR “lymphangioma simplex”) AND (“chylothorax” OR “chylous pleural effusion” OR “chylous effusion” OR “chylous lung”)]. Our search yielded 166 articles in total, out of which we selected 17 articles for inclusion. We included patients who presented to the hospital with chylothorax secondary to mediastinal lymphangioma and those who developed chylothorax after the removal of mediastinal lymphangioma. The primary outcome was the total number of reported cases of chylothorax secondary to mediastinal lymphangioma. The secondary outcomes included patient characteristics, fluid characteristics, clinical manifestations, and therapeutic modalities. Results The systematic review encompassed seventeen case reports. Most patients were male, spanning ages from six weeks to 82 years, with an average age of 28.35 years. Most pleural effusions were on the left side. Few cases were asymptomatic, while the most reported symptom was shortness of breath. The mean pleural triglyceride level was 708 mg/dl, with cystic hygroma being the most common subtype. The anterior mediastinum was the most frequent location. The primary treatment involved surgical removal of the lymphangioma along with thoracic duct ligation. Conclusion Mediastinal lymphangioma is an infrequent etiology of chylothorax and is scarcely documented in the literature. It may be present in isolation or may be associated with extra mediastinal lymphatic anomalies. The management of chylothorax in such cases is challenging with conservative measures often being ineffective, necessitating surgical intervention. The rarity of these conditions complicates the study of potential risk factors and genetic predispositions. Furthermore, there is no established consensus on the therapeutic modalities for patients with similar diagnoses which vary based on patient characteristics. |
| format | Article |
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| institution | Kabale University |
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| spelling | doaj-art-cae2edd90a7c45deaff6399ea9a03ec82025-08-20T03:52:20ZengBMCBMC Pulmonary Medicine1471-24662025-04-0125111210.1186/s12890-025-03664-3Mediastinal lymphangioma and intestinal lymphangiomatosis presenting with chylothorax: a systematic review of therapeutic modalitiesMarc Assaad0Roshan Acharya1Wasif Shamsi2Elspeth Springsted3Mahtab Foroozesh4Maria del Mar Cirino-Marcano5Division of Pulmonary, Sleep and Critical Care Medicine, Carilion Clinic Virginia Tech School of Medicine, Roanoke Memorial HospitalDivision of Pulmonary, Sleep and Critical Care Medicine, Carilion Clinic Virginia Tech School of Medicine, Roanoke Memorial HospitalDivision of Pulmonary, Sleep and Critical Care Medicine, Carilion Clinic Virginia Tech School of Medicine, Roanoke Memorial HospitalDivision of Pulmonary, Sleep and Critical Care Medicine, Carilion Clinic Virginia Tech School of Medicine, Roanoke Memorial HospitalDivision of Pulmonary, Sleep and Critical Care Medicine, Carilion Clinic Virginia Tech School of Medicine, Roanoke Memorial HospitalDivision of Pulmonary, Sleep and Critical Care Medicine, Carilion Clinic Virginia Tech School of Medicine, Roanoke Memorial HospitalAbstract Introduction Chylothorax is defined by the accumulation of chyle in the pleural space, characterized by triglyceride levels exceeding 110 milligrams per deciliter. The clinical presentation of chylothorax varies depending on its acuity and underlying etiology. Mediastinal lymphangiomas are extremely rare and benign lesions that can affect both infants and adults. They may occur independently or in association with other lymphatic disorders and can lead to complications such as chylothorax. Case report A 60-year-old male patient presented with shortness of breath and was diagnosed with left-sided chylothorax secondary to mediastinal lymphangioma, accompanied by intestinal lymphangiomatosis. Conservative approach was unsuccessful, and surgical therapy was needed. Materials and methods We conducted a thorough search of the PubMed/MEDLINE, PubMed Central, and Google Scholar databases. The search parameters we used included the following Boolean terms: [(“lymphangioma” OR “mediastinal lymphangioma” OR “cavernous lymphangiomas” OR “cystic hygromas” OR “capillary lymphangioma” OR “lymphangioma simplex”) AND (“chylothorax” OR “chylous pleural effusion” OR “chylous effusion” OR “chylous lung”)]. Our search yielded 166 articles in total, out of which we selected 17 articles for inclusion. We included patients who presented to the hospital with chylothorax secondary to mediastinal lymphangioma and those who developed chylothorax after the removal of mediastinal lymphangioma. The primary outcome was the total number of reported cases of chylothorax secondary to mediastinal lymphangioma. The secondary outcomes included patient characteristics, fluid characteristics, clinical manifestations, and therapeutic modalities. Results The systematic review encompassed seventeen case reports. Most patients were male, spanning ages from six weeks to 82 years, with an average age of 28.35 years. Most pleural effusions were on the left side. Few cases were asymptomatic, while the most reported symptom was shortness of breath. The mean pleural triglyceride level was 708 mg/dl, with cystic hygroma being the most common subtype. The anterior mediastinum was the most frequent location. The primary treatment involved surgical removal of the lymphangioma along with thoracic duct ligation. Conclusion Mediastinal lymphangioma is an infrequent etiology of chylothorax and is scarcely documented in the literature. It may be present in isolation or may be associated with extra mediastinal lymphatic anomalies. The management of chylothorax in such cases is challenging with conservative measures often being ineffective, necessitating surgical intervention. The rarity of these conditions complicates the study of potential risk factors and genetic predispositions. Furthermore, there is no established consensus on the therapeutic modalities for patients with similar diagnoses which vary based on patient characteristics.https://doi.org/10.1186/s12890-025-03664-3LymphangiomaChylothoraxChylous effusionCystic hygromaCavernous lymphangiomaIntestinal lymphangiomatosis |
| spellingShingle | Marc Assaad Roshan Acharya Wasif Shamsi Elspeth Springsted Mahtab Foroozesh Maria del Mar Cirino-Marcano Mediastinal lymphangioma and intestinal lymphangiomatosis presenting with chylothorax: a systematic review of therapeutic modalities BMC Pulmonary Medicine Lymphangioma Chylothorax Chylous effusion Cystic hygroma Cavernous lymphangioma Intestinal lymphangiomatosis |
| title | Mediastinal lymphangioma and intestinal lymphangiomatosis presenting with chylothorax: a systematic review of therapeutic modalities |
| title_full | Mediastinal lymphangioma and intestinal lymphangiomatosis presenting with chylothorax: a systematic review of therapeutic modalities |
| title_fullStr | Mediastinal lymphangioma and intestinal lymphangiomatosis presenting with chylothorax: a systematic review of therapeutic modalities |
| title_full_unstemmed | Mediastinal lymphangioma and intestinal lymphangiomatosis presenting with chylothorax: a systematic review of therapeutic modalities |
| title_short | Mediastinal lymphangioma and intestinal lymphangiomatosis presenting with chylothorax: a systematic review of therapeutic modalities |
| title_sort | mediastinal lymphangioma and intestinal lymphangiomatosis presenting with chylothorax a systematic review of therapeutic modalities |
| topic | Lymphangioma Chylothorax Chylous effusion Cystic hygroma Cavernous lymphangioma Intestinal lymphangiomatosis |
| url | https://doi.org/10.1186/s12890-025-03664-3 |
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