{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T11:14:57Z","timestamp":1792062897979,"version":"4.4.0"},"reference-count":11,"publisher":"Georg Thieme Verlag KG","issue":"05","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Zentralbl Chir"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title>\n                  <jats:p>Das Midaortic-Syndrom (MAS) ist eine seltene vaskul\u00e4re Erkrankung, die durch segmentale Hypoplasien oder Stenosen der thorakoabdominellen Aorta und m\u00f6glicher Mitbeteiligung renoviszeraler \u00c4ste gekennzeichnet ist.<\/jats:p>\n                  <jats:p>In der hier pr\u00e4sentierten Kasuistik wurde bei einer 19-j\u00e4hrigen Patientin mit thorakoabdomineller Aortenhypoplasie in Kombination mit multiplen aortalen und viszeralen Aneurysmata bei genetisch nachgewiesener COL3A1-Variante (vaskul\u00e4res Ehlers-Danlos-Syndrom) ein offen chirurgischer thorakoabdomineller Typ-II-Aortenersatz durchgef\u00fchrt. Unter Anwendung eines multimodalen neuro- und organprotektiven Ansatzes einschl. perioperativer Liquordrainage, MEP-Ableitungen, maschineller selektiver und distaler Extremit\u00e4ten- und Organperfusion und gezieltem Blutprodukt- und Volumenmanagement erfolgte eine komplikationsfreie thorakoabdominelle Rekonstruktion.<\/jats:p>\n                  <jats:p>Der postoperative Verlauf war regelhaft, erforderte jedoch eine sp\u00e4tere interventionelle endovaskul\u00e4re Therapie einer Anastomosenstenose der rechten Nierenarterie.<\/jats:p>\n                  <jats:p>Der Fall unterstreicht die Notwendigkeit eines ma\u00dfgeschneiderten Therapieansatzes bei komplexer, genetischer Aortenpathologie. Er zeigt auf, dass nicht alle aortalen Erkrankungen einer endovaskul\u00e4ren Therapie zug\u00e4nglich sind und dass die offen chirurgische Therapie insbesondere bei j\u00fcngeren Patienten mit heredit\u00e4ren Aortopathien nach wie vor einen hohen Stellenwert einnimmt.<\/jats:p>","DOI":"10.1055\/a-2773-1827","type":"journal-article","created":{"date-parts":[[2026,1,23]],"date-time":"2026-01-23T00:11:36Z","timestamp":1769127096000},"page":"434-438","source":"Crossref","is-referenced-by-count":1,"title":["Offener thorakoabdomineller Aortenersatz bei Midaortic-Syndrom und COL3A1-Variante"],"prefix":"10.1055","volume":"151","author":[{"given":"Christos","family":"Tanasidis","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsklinikum Carl Gustav Carus, Deutschland","place":["Dresden"],"department":["Klinik und Poliklinik f\u00fcr Viszeral-, Thorax- und 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