{"title":"再做VATS治疗birt - hogg - dub<s:1>综合征复发性气胸1例。","authors":"Mohan Pulle, Harsh Puri, Belal Asaf, Sukhram Bishnoi, Deepika Bhardwaj, Arvind Kumar","doi":"10.1510/mmcts.2025.062","DOIUrl":null,"url":null,"abstract":"<p><p>Birt-Hogg-Dubé syndrome (BHDS) is an autosomal dominant inherited disorder caused by mutation in a tumour suppressor gene, FLCN, leading to skin tumours (fibrofolliculomas), renal tumours and pulmonary cysts. Lung involvement is predominantly observed in 70% of the cases of BHDS, manifesting in the form of recurrent primary spontaneous pneumothorax. This video tutorial showcases the surgical management of recurrent right primary spontaneous pneumothorax in a young adult with a history of multiple episodes of bilateral pneumothorax managed by surgical intervention previously. The study underscores the efficacy of video-assisted thoracic surgery surgical management coupled with talc pleurodesis in restoring incomplete pleural symphysis in BHDS patients, as evident from successful surgery and uneventful recovery of the patient.</p>","PeriodicalId":53474,"journal":{"name":"Multimedia manual of cardiothoracic surgery : MMCTS / European Association for Cardio-Thoracic Surgery","volume":"2025 ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2025-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Re-do VATS for recurrent pneumothorax in a Birt-Hogg-Dubé syndrome patient.\",\"authors\":\"Mohan Pulle, Harsh Puri, Belal Asaf, Sukhram Bishnoi, Deepika Bhardwaj, Arvind Kumar\",\"doi\":\"10.1510/mmcts.2025.062\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Birt-Hogg-Dubé syndrome (BHDS) is an autosomal dominant inherited disorder caused by mutation in a tumour suppressor gene, FLCN, leading to skin tumours (fibrofolliculomas), renal tumours and pulmonary cysts. Lung involvement is predominantly observed in 70% of the cases of BHDS, manifesting in the form of recurrent primary spontaneous pneumothorax. This video tutorial showcases the surgical management of recurrent right primary spontaneous pneumothorax in a young adult with a history of multiple episodes of bilateral pneumothorax managed by surgical intervention previously. The study underscores the efficacy of video-assisted thoracic surgery surgical management coupled with talc pleurodesis in restoring incomplete pleural symphysis in BHDS patients, as evident from successful surgery and uneventful recovery of the patient.</p>\",\"PeriodicalId\":53474,\"journal\":{\"name\":\"Multimedia manual of cardiothoracic surgery : MMCTS / European Association for Cardio-Thoracic Surgery\",\"volume\":\"2025 \",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-09-08\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Multimedia manual of cardiothoracic surgery : MMCTS / European Association for Cardio-Thoracic Surgery\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1510/mmcts.2025.062\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Multimedia manual of cardiothoracic surgery : MMCTS / European Association for Cardio-Thoracic Surgery","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1510/mmcts.2025.062","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
Re-do VATS for recurrent pneumothorax in a Birt-Hogg-Dubé syndrome patient.
Birt-Hogg-Dubé syndrome (BHDS) is an autosomal dominant inherited disorder caused by mutation in a tumour suppressor gene, FLCN, leading to skin tumours (fibrofolliculomas), renal tumours and pulmonary cysts. Lung involvement is predominantly observed in 70% of the cases of BHDS, manifesting in the form of recurrent primary spontaneous pneumothorax. This video tutorial showcases the surgical management of recurrent right primary spontaneous pneumothorax in a young adult with a history of multiple episodes of bilateral pneumothorax managed by surgical intervention previously. The study underscores the efficacy of video-assisted thoracic surgery surgical management coupled with talc pleurodesis in restoring incomplete pleural symphysis in BHDS patients, as evident from successful surgery and uneventful recovery of the patient.
期刊介绍：
The Multimedia Manual of Cardio-Thoracic Surgery (MMCTS) is produced by The European Association for Cardio-Thoracic Surgery (EACTS). MMCTS is the world’s premier video-based educational resource for cardiovascular and thoracic surgeons; freely accessible - and essential - for all. MMCTS was launched more than ten years ago under the leadership of founding editor Professor Marko Turina. It was Professor Turina’s vision that the European Association for Cardio-Thoracic Surgery (EACTS), already the world-leader in CT surgery education, should take advantage of the Internet’s rapidly improving video publication capabilities and create a new step-by-step manual of surgical procedures. Professor Turina and EACTS agreed that the manual, MMCTS, should be freely accessible to all users, regardless of association membership status, nationality, or affiliation. MMCTS was self-published by EACTS for some years before being transferred to Oxford University Press, which hosted it until the end of 2016. In November 2016, the Manual returned home to EACTS and it has now relaunched in a completely new format. Since its birth in 2005, MMCTS has published some 400 detailed, video-based demonstrations of cardio-thoracic surgical procedures. Tutorials published prior to 2012 have been archived and we are working with the authors of these tutorials to update their work pending republication on the new site. Our mission is to make MMCTS the best online reference for cardio-thoracic surgeons – residents and experienced surgeons alike. Our aim is to include tutorials presenting procedures at both a fundamental and an advanced level. Truly innovative procedures are also included and are identified as such.
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