Interventional Management of a Rare Combination of Nutcracker and Wilkie Syndromes

Abstract

Nutcracker and Wilkie syndromes are rare mesoaortic compression entities, and their association is even less common. Data on interventional treatment of these pathologies are still scarce, but results from limited case series are encouraging. We report the case of a previously healthy 45-year-old woman diagnosed with nutcracker and Wilkie syndromes who presented with macroscopic hematuria, intermittent pain in the left flank and hypogastric region, postprandial nausea, and unexplained significant weight loss. A successful endovascular approach with stent implantation in the left renal vein was performed, but the stent migrated toward the left kidney, and this acute complication was managed through an interventional strategy as well. At the three-month follow-up, the patient described a marked improvement in all symptoms, except for the macroscopic hematuria. As it was our strong belief that the approach was efficient, we further investigated the "hematuria", which eventually led to the diagnosis of endometrial carcinoma. A hysterectomy and bilateral adnexectomy were planned, and chemoradiotherapy was initiated with the goal of preoperative tumor reduction. To our knowledge, this is the first reported case in which both Wilkie and nutcracker syndromes were effectively treated by stent implantation in the left renal vein, complicated with very early stent migration due to inadequate apposition to the less compliant venous lumen. The treatment of the duodenal compression was indirectly included in the stenting of the left renal vein, as reclaiming the venous lumen widened the aortomesenteric angle. The aim of this review is to discuss our center's transcatheter experience with these rare disorders and explore the literature in order to establish the benefits and limitations of such an approach.

Keywords: Wilkie; duodenal compression; endovascular treatment; hematuria; left renal vein compression; mesoaortic angle; nutcracker.

Figure 1

Illustration of the aortomesenteric angle in relation to the left renal vein and the terminal duodenum, tangling the two simulates the shape of a nutcracker (overlaid).

Figure 2

Computed tomography (CT) angiography findings. (A) Sagittal view revealing an aortomesenteric angle of 16 degrees; the blue circle represents the left renal vein and the yellow circle represents the location of the terminal duodenum. (B) Sagittal view revealing an aortomesenteric distance of 4.4 mm at the renal vein level and 7.1 mm at the duodenum level. (C) Coronal view showing a dilated left renal vein (arrow). (D) Coronal view showing an important distention of the stomach, with gastric stasis and gas distension of intestines.

Figure 3

Left renal vein angioplasty-initial procedure. (A) Stent placement at the ostium of the LRV. (B) Stent inflation. (C) Stent partially migrated toward left kidney. (D) Stent repositioning using a partially inflated balloon. (E) Stent repositioned at the ostium of the vessel and balloon inflation (arrow). (F) Final result, with the stent partially migrated again towards LRV (arrow).

Figure 4

Left renal vein angioplasty optimization of the initial angioplasty. Note that the images are mirrored because the patient was positioned upside down on the operating table for jugular access. (A) Initial stent repositioning and post-dilation. (B) Second stent positioning and (C) stent deployment. (D) Second stent post dilation. (E) Final result.

Figure 5

Abdominal ultrasound findings at the three-month follow-up. (A) LRV short axis showing well-expanded and well-apposed stent (green arrow), with enlarged aortomesenteric space. (B) Doppler ultrasound showing regular aortic (green arrow) and mesenteric (red arrow) flow, with enlarged aortomesenteric space. (C) LRV long axis confirming good expansion and apposition of the vein stent (red arrows). (D) Doppler ultrasound confirming regular flow in the LRV (green arrow), inferior vena cava (red arrow), and aortic artery (blue arrow).