Transposition der A. mesenterica superior an der infrarenalen Aorta beim „Nussknacker“-Syndrom

Zusammenfassung

Hintergrund

Das „Nussknacker“-Syndrom ist ein sicher unterdiagnostiziertes Krankheitsbild, mit dem die Patienten häufig einen längeren Leidensweg zurücklegen, ehe es adäquat diagnostiziert und behandelt wird. Hinsichtlich der Behandlung existiert eine Fülle unterschiedlicher therapeutischer Maßnahmen, wobei die am ehesten kausal anmutende Maßnahme, die Transposition der A. mesenterica superior, im Verhältnis wenig eingesetzt wird.

Ziel

Basierend auf selektiven Referenzen der einschlägigen medizinisch-wissenschaftlichen Literatur und eigenen klinisch-gefäßmedizinischen Erfahrungen sollen die wesentlichen Charakteristika des „Nussknacker“-Syndroms dargestellt werden.

Methode

Es wurde ein narrativer Review unter Literatursuche in PubMed unter Nutzung der Stichworte „Nussknacker-Syndrom“, „May-Turner-Syndrom“, „Wilkie-Syndrom“, „aortomesenterialer Winkel“, „pelvic congestion syndrome“ zum Thema verfasst.

Ergebnisse

Die Beschwerden des Nussknacker-Syndroms sind unspezifisch und mannigfaltig und sollten gerade in ihrer Kombination an ein Nussknacker-Syndrom denken lassen. Duplexsonografie und CT sind Methoden hoher diagnostischer Genauigkeit und können zudem relativ häufig simultan auftretende weitere Gefäßanomalien nachweisen. In Zusammenschau der Klinik und Paraklinik kann patientenspezifisch ein Therapieplan – a. e. im Rahmen eines Stufenkonzeptes – festgelegt werden, in dem auch die Transposition der A. mesenterica superior ein kausales, effektives und sicheres Verfahren gerade bei kombinierten Gefäßanomalien wie May-Turner- oder Wilkie-Syndrom darstellt.

Schlussfolgerung

Das Nussknacker-Syndrom als venöse Abflussbehinderung der Niere stellte, medizinisch gesehen, bislang selbst eine recht „harte Nuss“ dar, wofür die moderne Medizin durch Evidenzfindung nicht zuletzt durch Fallberichte und retrospektive Kohortenanalysen mittlerweile einen verifizierten diagnostischen und therapeutischen Algorithmus bereithält.

Abstract

Background

The nut cracker syndrome (NCS) can be considered as an under-diagnosed disease, which the patients have usually suffered from for a prolonged time prior to correct diagnosis and subsequent initiation of adequate therapy. There are several possible therapies, the most promising – transposition of the superior mesenteric artery (SMA) – from the perspective of its aetiopathogenesis – has rarely been used.

Aim

Based on selective references from the scientific medical literature and our own clinical experiences in vascular medicine, the important characteristics of the “nut cracker” phenomenon and syndrome are described.

Method

A topic-related narrative review has been based on a literature search in PubMed using the key words “Nut cracker syndrome”, “May-Turner syndrome“, “Wilkie syndrome”, “aorto-mesenteric angle“, “pelvic congestion syndrome”.

Results

There are diverse symptoms in NCS, which is unspecific; however, in particular, the combination of signs and symptoms associated with a suspicious pathological urine finding may indicate NCS. Duplex ultrasonography and CT-scan are very accurate diagnostic procedures and include an adequate search for simultaneously occurring vascular anomalies. Taken together, clinical findings and laboratory parameters may lead to a patient-specific and graduated therapeutic plan, in which transposition of SMA is a causal, effective and safe procedure, particularly when there are combined vascular anomalies, e.g., the combination of NCS with May-Turner syndrome in young patients.

Conclusion

The nutcracker syndrome as venous congestion of the kidney may be a “hard nut”. Modern medicine aims at achieving evidence for the best approach by case reports and retrospective cohort studies and can – in the meanwhile – provide a verified diagnostic work-up. In addition, is a therapeutic algorithm comprising a spectrum of highly effective measures, particularly for young patients, who frequently exhibit additional vascular anomalies.

Schlüsselwörter

Nussknacker-Syndrom - May-Turner-Syndrom - Wilkie-Syndrom - aorto-mesenterialer Winkel - Schnabelzeichen - „pelvic congestion syndrome“

Keywords

nut cracker-syndrome - May-Turner syndrome - Wilkie syndrome - aorto-mesenteric angle - pelvic congestion syndrome

Publikationsverlauf

Eingereicht: 27. März 2024

Angenommen nach Revision: 15. November 2024

Artikel online veröffentlicht:
17. Juli 2025

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