{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,28]],"date-time":"2026-03-28T16:24:58Z","timestamp":1774715098726,"version":"3.50.1"},"reference-count":14,"publisher":"BMJ","issue":"4","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2019,3,3]]},"published-print":{"date-parts":[[2019,4]]},"abstract":"<jats:p>General lymphatic anomaly (GLA) is a very rare disorder, characterised by multifocal lymphatic malformations into various tissues that is due to congenital abnormalities of lymphatic development. No treatment has ever proved its efficiency.<\/jats:p>\n               <jats:p>We report a 22-year-old man with recurrent bronchial casts due to thoracic involvement of GLA. After a 6-month treatment with sildenafil (20\u2009mg three times a day), a phosphodiesterase 5 inhibitor, chest CT scan showed a complete regression of ground-glass opacities and lung function test results improved substantially and remained stable for 1\u2009year. The treatment was well tolerated.<\/jats:p>\n               <jats:p>This observation suggests that sildenafil may be a therapeutic approach to be tested in thoracic involvement of GLA.<\/jats:p>","DOI":"10.1136\/bcr-2018-228523","type":"journal-article","created":{"date-parts":[[2019,4,23]],"date-time":"2019-04-23T16:14:48Z","timestamp":1556036088000},"page":"e228523","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":8,"title":["Thoracic involvement of diffuse lymphangiomatosis successfully treated with sildenafil"],"prefix":"10.1136","volume":"12","author":[{"given":"Arnaud","family":"Maurac","sequence":"first","affiliation":[{"name":"Centre Hospitalier Universitaire de Nancy, D\u00e9partement de pneumologie, CHRU de Nancy, Vandoeuvre-l\u00e8s-Nancy, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marie-Pierre","family":"Debray","sequence":"additional","affiliation":[{"name":"Service de Radiologie, H\u00f4pital Bichat, Assistance Publique - H\u00f4pitaux de Paris, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bruno","family":"Crestani","sequence":"additional","affiliation":[{"name":"Service de Pneumologie et Centre de R\u00e9f\u00e9rence Constitutif des Maladies Pulmonaires Rares, H\u00f4pital Bichat, Assistance Publique - H\u00f4pitaux de Paris, Paris, France"},{"name":"D\u00e9partement Hospitalo-Universitaire FIRE; INSERM UMR 1152; LabEx Inflamex, Universit\u00e9 Paris Diderot, UFR de M\u00e9decine Site Xavier-Bichat, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Camille","family":"Taill\u00e9","sequence":"additional","affiliation":[{"name":"Service de Pneumologie et Centre de R\u00e9f\u00e9rence Constitutif des Maladies Pulmonaires Rares, H\u00f4pital Bichat, Assistance Publique - H\u00f4pitaux de Paris, Paris, France"},{"name":"D\u00e9partement Hospitalo-Universitaire FIRE; INSERM UMR 1152; LabEx Inflamex, Universit\u00e9 Paris Diderot, UFR de M\u00e9decine Site Xavier-Bichat, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2019,4,23]]},"reference":[{"key":"2025082701471010000_12.4.e228523.1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2014-3673"},{"key":"2025082701471010000_12.4.e228523.2","doi-asserted-by":"publisher","DOI":"10.1183\/16000617.0018-2016"},{"key":"2025082701471010000_12.4.e228523.3","doi-asserted-by":"crossref","first-page":"632","DOI":"10.1164\/rccm.201503-0631IM","article-title":"Plastic bronchitis related to idiopathic thoracic lymphangiectasia. noncontrast magnetic resonance lymphography","volume":"192","author":"El Mouhadi","year":"2015","journal-title":"Am J Respir Crit Care Med"},{"key":"2025082701471010000_12.4.e228523.4","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.22869"},{"key":"2025082701471010000_12.4.e228523.5","doi-asserted-by":"crossref","first-page":"839","DOI":"10.7326\/0003-4819-156-11-201206050-00016","article-title":"Successful treatment of diffuse pulmonary lymphangiomatosis with bevacizumab","volume":"156","author":"Aman","year":"2012","journal-title":"Ann Intern Med"},{"key":"2025082701471010000_12.4.e228523.6","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1159\/000440939","article-title":"Lymphatic malformation in newborns as the first sign of diffuse lymphangiomatosis: successful treatment with sirolimus","volume":"109","author":"Laforgia","year":"2016","journal-title":"Neonatology"},{"key":"2025082701471010000_12.4.e228523.7","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc1112482"},{"key":"2025082701471010000_12.4.e228523.8","first-page":"1689","article-title":"Diagnosis and treatment of lymphatic plastic bronchitis in adults using advanced lymphatic imaging and percutaneous embolization","volume":"13","author":"Itkin","year":"2016","journal-title":"Ann Am Thorac Soc"},{"key":"2025082701471010000_12.4.e228523.9","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdermsci.2014.01.001"},{"key":"2025082701471010000_12.4.e228523.10","doi-asserted-by":"publisher","DOI":"10.1093\/cvr\/cvv158"},{"key":"2025082701471010000_12.4.e228523.11","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1001\/jamadermatol.2013.7002","article-title":"Expression of phosphodiesterase-5 in lymphatic malformation tissue","volume":"150","author":"Green","year":"2014","journal-title":"JAMA Dermatol"},{"key":"2025082701471010000_12.4.e228523.12","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1038\/jp.2015.10","article-title":"Congenital chylothorax treated with oral sildenafil: a case report and review of the literature","volume":"35","author":"Malleske","year":"2015","journal-title":"J Perinatol"},{"key":"2025082701471010000_12.4.e228523.13","doi-asserted-by":"crossref","first-page":"980","DOI":"10.1016\/j.ijporl.2015.03.034","article-title":"Sildenafil for microcystic lymphatic malformations of the head and neck: A prospective study","volume":"79","author":"Koshy","year":"2015","journal-title":"Int J Pediatr Otorhinolaryngol"},{"key":"2025082701471010000_12.4.e228523.14","doi-asserted-by":"crossref","first-page":"2015","DOI":"10.1002\/pbc.25600","article-title":"Caution is recommended prior to sildenafil use in vascular anomalies","volume":"62","author":"Rankin","year":"2015","journal-title":"Pediatr Blood Cancer"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2018-228523","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T08:47:21Z","timestamp":1756284441000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2018-228523"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,4]]},"references-count":14,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2019,4,23]]},"published-print":{"date-parts":[[2019,4]]}},"alternative-id":["10.1136\/bcr-2018-228523"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2018-228523","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,4]]}}}