Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2017 Aug 3;20(4):339-342.
doi: 10.1007/s40477-017-0257-2. eCollection 2017 Dec.

Wilkie's syndrome

Renato Farina  1 , Pietro Valerio Foti  1 , Giuseppe Cocuzza  1 , Valeria Costanzo  1 , Giuliana Costanzo  1 , Alessandro Conti  1 , Alfredo Torcitto  1 , Monica Pennisi  1
Affiliations
Case Reports

Wilkie's syndrome

Renato Farina et al. J Ultrasound. .

Abstract
in English, Italian

Superior mesenteric artery syndrome, also known as Wilkie's syndrome, is a rare vascular disease caused by the anomalous course of the superior mesenteric artery arising from the abdominal aorta with a smaller angle than the norm (<22°). The reduced angle compresses the structures situated between the aorta and the superior mesenteric artery, such as the duodenum and left renal vein; this can determine painful crises, intestinal subocclusions, and left varicocele. This syndrome can be congenital or acquired. The acquired type is more common and is generally caused by reduced perivascular fat surrounding the abdominal aorta and the superior mesenteric artery; this form is common among anorexic patients that have had a rapid weight loss. We present the case of a female patient who suffered from repeated postprandial vomiting and who lost 12 kg in 4 months. B-mode ultrasound imaging revealed evidence of a reduced angle between the aorta and the superior mesenteric artery, as found in Wilkie's syndrome. After diagnosis, the patient followed a high-calorie diet, and 2 months later an ultrasound scan proved the restoration of the aorto-mesenteric angle as a consequence of increased perivascular fat with regression of symptoms.

La sindrome dell’arteria mesenterica superiore o sindrome di Wilkie è una patologia vascolare rara dovuta all’anomalo decorso dell’arteria mesenterica superiore che nasce dall’aorta addominale con un angolo ridotto rispetto alla norma (inferiore a 22°). L’angolazione ridotta provoca la compressione delle strutture che passano tra l’aorta e l’arteria mesenterica superiore, il duodeno e la vena renale sinistra; questo può determinare crisi dolorose, sub-occlusioni intestinali e varicocele sinistro. Questa sindrome può essere congenita o acquisita. La forma acquisita, più frequente, è dovuta in genere alla riduzione del pannicolo adiposo peri-vascolare che circonda l’aorta addominale e l’arteria mesenterica superiore; questa forma è comune soprattutto nei pazienti anoressici che hanno subito una rapida perdita di peso. Presentiamo un caso di una paziente affetta da crisi ripetute di vomito post-prandiale che ha subito una perdita di 12 kg in 4 mesi. Durante l’esame ecografico B-Mode si evidenziava una riduzione dell’angolo tra aorta ed arteria mesenterica superiore, tipico della sindrome di Wilkie. Successivamente la paziente ha eseguito una dieta ipercalorica e dopo due mesi l’ecografia dimostrava il ripristino dell’angolo aorto-mesenterico come conseguenza dell’aumento del grasso peri-vascolare con regressione dei sintomi.

Keywords: Superior mesenteric artery; Ultrasound; Wilkie’s syndrome.

PubMed Disclaimer

Conflict of interest statement

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individuals participating in the study.

Figures

Fig. 1
Fig. 1
Ultrasound examination before treatment: longitudinal subxiphoidal scan of the abdominal aorta. a The aorto–mesenteric angle is 15°. b The thickness of the perivascular fat (arrow) appears reduced. AO abdominal aorta, L liver, SMA superior mesenteric artery
Fig. 2
Fig. 2
CT: the axial scanning proves the constriction of the aorto–mesenteric angle (long arrow) and the compression of the third portion of the duodenum (short arrow)
Fig. 3
Fig. 3
CT: the sagittal reconstruction shows the reduction of the aorto–mesenteric angle with “beak sign” (arrow)
Fig. 4
Fig. 4
Reconstruction CT coronal that shows left pelvic varicocele (arrows)
Fig. 5
Fig. 5
Ultrasound examination after the treatment: longitudinal subxiphoidal scan of the abdominal aorta. The aorto–mesenteric angle (A) is 32°. The ultrasound scan shows increased thickness of the perivascular fat (arrow). AO abdominal aorta, L liver, SMA superior mesenteric artery
See this image and copyright information in PMC

References

    1. Wilkie DPD. Chronic duodenal ileus. Am J Med Sci. 1927;173:643. doi: 10.1097/00000441-192705000-00006. - DOI
    1. Kwan E. Wilkie’s syndrome. Surgery. 2004;135(2):225–227. doi: 10.1016/S0039-6060(03)00367-2. - DOI - PubMed
    1. Gthrie RH., Jr Wilkie’s syndrome. Ann Surg. 1971;173(2):290–293. doi: 10.1097/00000658-197102000-00017. - DOI - PMC - PubMed
    1. Fong JK, Poh AC, Tan AG, et al. Imaging findings and clinical features of abdominal vascular compression syndromes. Am J Roentgenol. 2014;203:29–36. doi: 10.2214/AJR.13.11598. - DOI - PubMed
    1. Gebhart T. Superior mesenteric artery syndrome. Gastroenterol Nurs. 2015;38:189–193. doi: 10.1097/SGA.0000000000000107. - DOI - PubMed

Publication types

MeSH terms

LinkOut - more resources