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Marfan syndrome
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===Surgery=== If the dilation of the aorta progresses to a significant-diameter [[aneurysm]], causes a dissection or a rupture, or leads to failure of the aortic or other valve, then surgery (possibly a [[Bentall procedure|composite aortic valve graft]] or [[valve-sparing aortic root replacement]]) becomes necessary. Although aortic graft surgery (or any vascular surgery) is a serious undertaking it is generally successful if undertaken on an elective basis.<ref>{{cite press release|url=http://www.docguide.com/news/content.nsf/news/852571020057CCF6852573E1005A0BDC|title=Elective Aortic Root Surgery in Marfan Syndrome Appears Safe and Durable: Presented at STS|date=January 31, 2008|publisher=Doctor's Guide|access-date=January 13, 2009|url-status=live|archive-url=https://web.archive.org/web/20081120151335/http://www.docguide.com/news/content.nsf/news/852571020057CCF6852573E1005A0BDC|archive-date=November 20, 2008}}<br />See also: * {{cite journal | vauthors = Cameron DE, Vricella LA | title = Valve-sparing aortic root replacement in Marfan syndrome | journal = Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual | volume = 8 | issue = 1 | pages = 103β111 | year = 2005 | pmid = 15818365 | doi = 10.1053/j.pcsu.2005.03.001 }} * {{cite journal | vauthors = Gott VL, Cameron DE, Alejo DE, Greene PS, Shake JG, Caparrelli DJ, Dietz HC | title = Aortic root replacement in 271 Marfan patients: a 24-year experience | journal = The Annals of Thoracic Surgery | volume = 73 | issue = 2 | pages = 438β443 | date = February 2002 | pmid = 11845856 | doi = 10.1016/S0003-4975(01)03336-7 }} * {{cite journal | vauthors = Bethea BT, Fitton TP, Alejo DE, Barreiro CJ, Cattaneo SM, Dietz HC, Spevak PJ, Lima JA, Gott VL, Cameron DE | display-authors = 6 | title = Results of aortic valve-sparing operations: experience with remodeling and reimplantation procedures in 65 patients | journal = The Annals of Thoracic Surgery | volume = 78 | issue = 3 | pages = 767β72; discussion 767β72 | date = September 2004 | pmid = 15336989 | doi = 10.1016/j.athoracsur.2004.03.040 | doi-access = free }}</ref> Surgery in the setting of acute aortic dissection or rupture is considerably more problematic. Elective aortic valve/graft surgery is usually considered when aortic root diameter reaches {{convert|50|mm|in|abbr=off|sp=us}}, but each case needs to be specifically evaluated by a qualified cardiologist. New valve-sparing surgical techniques are becoming more common.<ref name="mayo-heart">{{cite web|url=http://www.mayoclinic.org/marfan-syndrome/heartsurgery.html|title=Heart Surgery for Marfan Syndrome|access-date=January 12, 2007|publisher=Mayo Clinic|archive-url = https://web.archive.org/web/20061218031510/http://www.mayoclinic.org/marfan-syndrome/heartsurgery.html <!-- Bot retrieved archive -->|archive-date=December 18, 2006}}</ref> As people with Marfan syndrome live longer, other vascular repairs are becoming more common, e.g., repairs of descending thoracic aortic aneurysms and aneurysms of vessels other than the aorta.{{citation needed|date=June 2017}} The skeletal and ocular manifestations of Marfan syndrome can also be serious, although not life-threatening. These symptoms are usually treated in an appropriate manner for the condition, such as with pain medications or [[muscle relaxant]]s. Because Marfan syndrome may cause asymptomatic spinal abnormalities, any spinal surgery contemplated on a person with Marfan should only follow detailed imaging and careful surgical planning, regardless of the indication for surgery. The ocular complications of MFS can often be treated with surgery. [[Ectopia lentis]] can be treated, as artificial lenses can be surgically implanted. In addition, surgery can address [[glaucoma]] and [[cataract]]s.<ref name=":3" /> Treatment of a spontaneous pneumothorax is dependent on the volume of air in the pleural space and the natural progression of the individual's condition. A small pneumothorax might resolve without active treatment in one to two weeks. Recurrent pneumothoraces might require chest surgery. Moderately sized pneumothoraces might need [[Chest tube|chest drain]] management for several days in a hospital. Large pneumothoraces are likely to be medical emergencies requiring emergency decompression.<ref>{{Cite journal |last1=Zarogoulidis |first1=Paul |last2=Kioumis |first2=Ioannis |last3=Pitsiou |first3=Georgia |last4=Porpodis |first4=Konstantinos |last5=Lampaki |first5=Sofia |last6=Papaiwannou |first6=Antonis |last7=Katsikogiannis |first7=Nikolaos |last8=Zaric |first8=Bojan |last9=Branislav |first9=Perin |last10=Secen |first10=Nevena |last11=Dryllis |first11=Georgios |last12=Machairiotis |first12=Nikolaos |last13=Rapti |first13=Aggeliki |last14=Zarogoulidis |first14=Konstantinos |date=October 2014 |title=Pneumothorax: from definition to diagnosis and treatment |journal=[[Journal of Thoracic Disease]] |volume=6 |issue=Suppl 4 |pages=S372βS376 |doi=10.3978/j.issn.2072-1439.2014.09.24 |issn=2072-1439 |pmc=4203989 |pmid=25337391}}</ref> As an alternative approach, custom-built supports for the aortic root are also being used.<ref name="Tr2016">{{cite journal | vauthors = Treasure T, Petrou M, Rosendahl U, Austin C, Rega F, Pirk J, Pepper J | title = Personalized external aortic root support: a review of the current status | journal = European Journal of Cardio-Thoracic Surgery | volume = 50 | issue = 3 | pages = 400β404 | date = September 2016 | pmid = 27032474 | doi = 10.1093/ejcts/ezw078 | doi-access = free }}</ref> As of 2020 this procedure has been used in over 300 people with the first case occurring in 2004.<ref>{{cite journal | vauthors = Treasure T, Golesworthy T, Pepper J | title = Practical clinical applications of 3-D printing in cardiovascular surgery | journal = Journal of Thoracic Disease | volume = 9 | issue = 9 | pages = 2792β2797 | date = September 2017 | pmid = 29221242 | pmc = 5708385 | doi = 10.21037/jtd.2017.08.63 | doi-access = free }}</ref><ref>{{cite journal | vauthors = Nemec P, Pepper J, Fila P | title = Personalized external aortic root support | journal = Interactive Cardiovascular and Thoracic Surgery | volume = 31 | issue = 3 | pages = 342β345 | date = September 2020 | pmid = 32761056 | doi = 10.1093/icvts/ivaa111 | doi-access = free }}</ref>
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