Management in der Therapie adulter Lungensequester – eine retrospektive multizentrische Analyse

Zusammenfassung

Einführung: Lungensequester sind seltene pulmonale Malformationen. Die komplexe Pathogenese ist nicht vollständig geklärt. Extralobäre Sequester sind immer kongenital und werden im Kindesalter diagnostiziert. Demgegenüber scheinen bei Erwachsenen ein Teil der intralobären Sequester erworben zu sein. Die unspezifische Klinik bedingt eine protrahierte Diagnose. In der Behandlung ist die Resektion weiterhin der Goldstandard. Trotz der geringen Inzidenz haben auch hier die technischen Entwicklungen der letzten Jahrzehnte, wie präoperative angiologische Interventionen und videoassistierte thorakoskopische Resektionen zu Veränderungen im Management der Erkrankung geführt. Methode: Die prospektiv erfassten Daten von erwachsenen Patienten, die aufgrund eines Lungensequesters im Zeitraum von 23 Jahren in 4 verschiedenen Kliniken operiert wurden, wurden retrospektiv analysiert. Ergebnisse: Insgesamt wurden 14 Fälle mit intralobären Sequestern (n = 11, 79 % linker Unterlappen) beobachtet. Das mittlere Alter betrug 50 Jahre (27–76 Jahre). Das männliche Geschlecht war häufiger betroffen (8/6). In der Symptomatik war die unspezifische pulmonale Infektsituation (n = 6, 42 %) führend. Bei 2 Patienten bestanden Hämoptysen. Drei Patienten waren symptomlos. Die Diagnostik (CT-Thorax, n = 14) wurde in 7 Fällen durch ein CT-Abdomen sowie bei 10 Patienten durch eine Angiografie ergänzt. Eine präoperative Embolisation wurde bei 5 dieser Patienten (2× Äste der abdominellen und 2× Äste der thorakalen Aorta und 1× Äste von beiden) durchgeführt. Die Resektion erfolgte zumeist offen-chirurgisch (n = 12, 86 %). Insgesamt überwogen die nicht anatomischen Resektionen (n = 8, 57 %). Die Mikrobiologie zeigte in 7 Fällen eine bakterielle Superinfektion, gehäuft mit Streptococcus pneumoniae. In einem Fall bestand der Nachweis einer Pilzinfektion mit Candida albicans. Schlussfolgerung: Bei unklaren pulmonalen Befunden, insbesondere im Bereich des Unterlappens, sollten Lungensequester auch im höheren Alter differenzialdiagnostisch berücksichtigt werden. Die operative Entfernung wird bei fehlenden Kontraindikationen grundsätzlich empfohlen und kann in ausgewählten Fällen minimalinvasiv erfolgen. Eine präoperative Abklärung der arteriellen Versorgung (Äste aus der Aorta thoracica, Aorta abdominalis oder aus beiden) kann die Planung der Resektion erleichtern. Bei Hämoptysen oder Versorgung aus der Aorta abdominalis ist die präoperative interventionell-radiologische Embolisation indiziert.

Abstract

Background: Pulmonary sequestration is a rare pulmonary malformation whose complex pathogenesis is not completely understood. Extrapulmonary sequestrations are always hereditary malformations and are usually diagnosed during childhood. Some intralobar sequestrations in adults, by contrast, seem to be acquired. The clinical presentation is non-specific and often misinterpreted, which results in delayed diagnosis. Surgical resection continues to be the gold standard. Despite the low incidence, new technologies developed in the past few decades, e.g. preoperative interventional angiology procedures and video-assisted lung resection, have changed the management of the disease. Methods: A prospective data collection was performed on adult patients who had undergone surgical resection of a pulmonary sequestration in four different centres during a period of 23 years. These data were retrospectively analysed. Results: A total of 14 patients with intralobar sequestrations (n = 11, 79 % left lower lobe) underwent surgical resection. The male/female ratio was 8/6 (median age 50 years). Non-specific pulmonary symptoms such as recurrent infections had a high prevalence (n = 6, 42 %). Two patients had haemoptysis. Three patients were asymptomatic. All patients had a chest CT, 7 patients had an additional abdominal CT, while 10 underwent angiography. Preoperative embolization was performed in 5 of these patients (branches of the thoracic aorta: in 2 cases, branches of abdominal aorta: in 2 cases, and branches of both: in 1 case). Resection was mostly performed with an open surgical approach (n = 12, 86 %). Most patients had a non-anatomic pulmonary resection (n = 8, 57 %). In 7 patients, a microbiologic examination revealed a superinfection with Streptococcus pneumoniae, while 1 patient had an invasive mycosis with Candida albicans. Conclusion: In patients with non-specific pulmonary symptoms and undetermined lesions, especially in the lower lobes, pulmonary sequestrations must be considered in the differential diagnostic evaluation even in adult and elderly patients. If there are no contraindications, surgical removal is basically recommended and may be minimally-invasive in selected cases. The planning of the resection can be facilitated by preoperative evaluation of the arterial supply (branches of the thoracic or abdominal aorta, or both). In cases with haemoptysis or blood supply over the abdominal aorta, preoperative embolization is indicated.

Schlüsselwörter

Thoraxchirurgie - Lungensequester - intralobäre Lungensequester im Erwachsenenalter - modernes Diagnostik- und Therapiemanagement - präoperative Embolisation - thorakoskopisch assistierte Resektion

Key words

intralobar lung sequestration in adulthood - modern diagnostic and therapeutic management - preoperative embolization - video-assisted thoracoscopical resection

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