Objective: Rare disease Background: Nutcracker syndrome and Wilkie's syndrome are rare vascular diseases due to the abnormal course of the superior mesenteric artery originating from the abdominal aorta with reduced angle (<22°) and consequent compression of the left renal vein (nutcracker) and duodenum (Wilkie). Here, we report the case of a patient with a rare combination of these 2 syndromes and with unusual clinical manifestation of post-prandial pain. Case Report: We describe the case of a young male patient with rapid weight loss, coupled with post-prandial abdominal pain, with sub-acute onset, not associated with other symptoms. The ultrasound examination found an aortomesenteric angle of 18° and compression of the left renal vein and left varicocele. A CT study was performed to exclude oncological diseases and/or other pathologies responsible for the pain and weight loss, which confirmed the ultrasound findings and showed compression of the third part of the duodenum. The patient underwent endovascular treatment, with stent placement in the left renal vein, which resolved the vascular compression and of the duodenum, with regression of symptoms. Conclusions: The ultrasound scan promptly highlighted the reduction of the aorto-mesenteric angle and the signs of venous congestion of the left renal vein. Based on this experience, in patients with weight loss and post-prandial pain, in our opinion, diagnostic investigations should also be extended to the study of the aorto-mesenteric angle to confirm or exclude any vascular and/or duodenal compression.
Vascular compression syndromes are rare alterations that have in common the compression of an arterial and/or venous vessel by contiguous structures and can be congenital or acquired. The best known are the Thoracic Outlet Syndrome, Nutcracker Syndrome, May–Thurner Syndrome, and Dunbar Syndrome. The incidence of these pathologies is certainly underestimated due to the non-specific clinical signs and their frequent asymptomaticity. Being a first-level method, Ultrasound plays a very important role in identifying these alterations, almost always allowing a complete diagnostic classification. If in expert hands, this method can significantly contribute to the reduction of false negatives, especially in the asymptomatic population, where the finding of the aforementioned pathologies often happens randomly following routine checks. In this review, we briefly discuss the best known vascular changes, the corresponding ultrasound anatomy, and typical ultrasound patterns.
Background Posterior nutcracker syndrome is defined by the compression of the left renal vein between the abdominal aorta and a lumbar vertebral body. It can be clinically manifest with intermittent hematuria, gonadal or spermatic reflux resulting in varicocele. Ultrasound is the first-line imaging which require more accurate study with contrast-enhanced computed tomography. Management can be conservative in younger patients with mild hematuria due to the high spontaneous remission rate and invasive with open surgical and endovascular interventions. We describe a very rare case with compression of the left renal vein due to an osteophyte of the spine. Case presentation A 62-year-old Caucasic male came to our radiology department for chronic hepatitis B virus (HBV)-related liver disease follow-up and mild scrotal pain. The ultrasound examination revealed a compression of the left retro-aortic renal vein in the aorto-vertebral space caused by an osteophyte. Duplex Doppler ultrasound revealed flow congestion in the left renal vein and renal failure; power Doppler ultrasound showed left varicocele. Conclusions Doppler ultrasound is the first-line imaging and allows the detection of all the typical signs of posterior nutcracker: left renal vein stenosis, flow congestion and renal failure. Nutcracker syndrome should be suspected in older patients with left varicocele associated with hematuria. Failure to diagnose and treat these patients could have serious consequences for their health.
Patient: Male, 55-year-old Final Diagnosis: Dunbar syndrome Symptoms: Epigastric pain • weight loss Medication: — Clinical Procedure: None Specialty: Gastroenterology and Hepatology • Radiology Objective: Rare disease Background: Dunbar syndrome is a rare vascular alteration caused by the abnormal course of the median arcuate ligament of the diaphragm, which in some patients causes chronic compression of the celiac artery and can cause non-specific symptoms such as diarrhea, retro-sternal pain, vomiting, swelling, and nausea, or a typical symptomatic triad with weight loss, post-prandial abdominal pain, and epigastric murmur. Color Doppler ultrasound and duplex Doppler ultrasound provide a complete diagnostic framework of this disease. Case Report: We describe a case of 55-year-old man with post-prandial epigastric pain, significant weight loss, and several episodes of retro-sternal pain. He underwent multidetector computed tomography of the abdomen and color duplex Doppler ultrasound examination of the celiac artery that highlighted stenosis of the celiac artery, more severe in expiratory apnea. The computed tomography showed the typical aspect of the celiac artery, with the “hook sign”. A duplex Doppler ultrasound examination showed a significant increase in peak speed (226 cm/s) due to severe stenosis of the celiac artery by the median arched ligament of the diaphragm. Conclusions: This case is unique due to the severity of the celiac artery stenosis and the unusual clinical presentation of the patient who had frequent episodes of retro-sternal pain. The significant increase in peak velocity in the celiac artery in expiratory apnea, if associated with the typical symptomatology in the absence of other alterations of the splanchnic vessels, can be considered, in our opinion, sufficient for the diagnosis of Dunbar syndrome.
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