The Ultrasound Findings in a Rare Case of Nutcracker Syndrome, Wilkie's Syndrome, and Dunbar Syndrome Combination.

Vascular compression syndromes represent a group of rare and poorly understood diseases. Dunbar syndrome (DS) is caused by the median arcuate ligament of diaphragm originating lower than normal and causing compression of celiac artery. The Nutcracker is caused by the superior mesenteric artery (SMA) originating from aorta at an acute angle causing a restriction of aortomesenteric space that is traversed by the left renal vein and duodenum; if the compression involves only the left renal vein and becomes symptomatic it is called Nutcracker syndrome; if the symptomatic compression involves only the duodenum it is called Wilkie's syndrome or SMA syndrome. The knowledge of these rare pathologies is essential to reduce the false negatives which still remain very high; it is, therefore, necessary to promote greater knowledge as the lack of diagnosis can be very dangerous for the patient's health. We describe a rare case of a combination of DS, Nutcracker, and SMA or Wilkie's syndrome in a young patient.

Clinical and Radiological Features of Interstitial Lung Diseases Associated with Polymyositis and Dermatomyositis.

Polymyositis and dermatomyositis are autoimmune idiopathic systemic inflammatory diseases, characterized by various degrees of muscle inflammation and typical cutaneous lesions-the latter found in dermatomyositis. The underlying pathogenesis is characterized by a high level of uncertainty, and recent studies suggest diseases may have different immunopathological mechanisms. In polymyositis, components of the cellular immune system are involved, whereas in dermatomyositis, the pathogenesis is mainly mediated by the humoral immune response. The interstitial lung disease occurs in one-third of polymyositis and dermatomyositis patients associated with worse outcomes, showing an estimated excess mortality rate of around 40%. Lung involvement may also appear, such as a complication of muscle weakness, mainly represented by aspiration pneumonia or respiratory insufficiency. The clinical picture is characterized, in most cases, by progressive dyspnea and non-productive cough. In some cases, hemoptysis and chest pain are found. Onset can be acute, sub-acute, or chronic. Pulmonary involvement could be assessed by High Resolution Computed Tomography (HRCT), which may identify early manifestations of diseases. Moreover, Computed Tomography (CT) appearances can be highly variable depending on the positivity of myositis-specific autoantibodies. The most common pathological patterns include fibrotic and cellular nonspecific interstitial pneumonia or organizing pneumonia; major findings observed on HRCT images are represented by consolidations, ground-glass opacities, and reticulations. Other findings include honeycombing, subpleural bands, and traction bronchiectasis. In patients having Anti-ARS Abs, HRCT features may develop with consolidations, ground glass opacities (GGOs), and reticular opacities in the peripheral portions; nonspecific interstitial pneumonia or nonspecific interstitial pneumonia mixed with organizing pneumonia have been reported as the most frequently encountered patterns. In patients with anti-MDA5 Abs, mixed or unclassifiable patterns are frequently observed at imaging. HRCT is a sensitive method that allows one not only to identify disease, but also to monitor the effectiveness of treatment and detect disease progression and/or complications; however, radiological findings are not specific. Therefore, aim of this pictorial essay is to describe clinical and radiological features of interstitial lung diseases associated with polymyositis and dermatomyositis, emphasizing the concept that gold standard for diagnosis and classification-should be based on a multidisciplinary approach.

Corrigendum to "Rescue of renal function after endovascular revascularization of acute aorto-renal bypass occlusion in a patient with solitary kidney: A case report" [Radiology Case Reports 17 (2022) 2698-2701].

[This corrects the article DOI: 10.1016/j.radcr.2022.04.023.].

Vascular compression syndromes: a pictorial review.

Vascular compression syndromes include a group of rare vascular changes due to extrinsic compression of veins or arteries by surrounding structures. These pathologies are often underestimated due to their rarity, clinicians' poor level of knowledge, and the non-specificity of their symptoms. The best known are Eagle syndrome, thoracic outlet syndrome, nutcracker syndrome, May-Thurner syndrome, Dunbar syndrome, and popliteal entrapment syndrome. This work summarizes the main ultrasonographic characteristics, symptoms, and treatments of choice for these syndromes. Knowledge of these conditions' characteristic signs is essential for the differential diagnosis. Failure to diagnose these rare diseases can expose patients to serious complications and risks to their health.

Rescue of renal function after endovascular revascularization of acute aorto-renal bypass occlusion in a patient with solitary kidney: a case report.

Renal artery occlusion represents an early diagnostic urgency to prevent kidney injury or, even more grave, kidney failure. However, diagnosis is often a challenge due to nonspecific and sporadic symptoms and signs, resulting in misdiagnosis, missed, or delayed diagnosis. The patient ought to be evaluated by a multidisciplinary team to select the best treatment. We describe a 62-year-old man's case study. The patient had a left solitary kidney with an aortorenal artery bypass thrombosis. The renal function resumes 24 hours after the interventional radiology procedure. Based on our experience, revascularization of aortorenal artery bypass thrombosis may save renal function even after long ischemia times of over 24 hours.

Ureteral metastasis from gastric cancer after two years of subtotal gastrectomy: a case report.

True ureteral metastases from gastric cancer are extremely rare. Only a few cases of this condition have been reported. CT is the first-line imaging technique and may aid the diagnosis, even if the definitive diagnosis is histologic. We report a case of a 45-year-old female with a history of gastric cancer who underwent subtotal gastrectomy and presented 2 years later with ureteral metastasis and subsequently renal pelvis metastasis in absence of peritoneal involvement. A biopsy was required to rule out primary urothelial carcinoma and make a well-timed and proper diagnosis. We describe the pathologic and radiological features of this case, followed by a brief review of the literature included in the discussion.

True congenital multicystic disease of the pancreas in the infant: a very rare case.

The most frequent pancreatic cysts in patients of developmental age are pseudocysts, neoplastic cysts, posttraumatic cysts, and parasitic cysts. Retention cysts, duplication cysts, and congenital cysts are called true cysts. Among the true cysts, congenital cysts are very rare and include 1% of all pancreatic cysts. The finding of true congenital pancreatic cysts in children under 3 years represents a very rare event; less than 30 have been described in the literature. We describe the case of a 3-year-old male who came to our observation for abdominal pain and underwent an abdomen ultrasound examination. The ultrasound examination showed unilocular sub-centimeter cystic lesions, distributed throughout the pancreatic parenchyma. The patient was, therefore, not subjected to any treatment, and after a few days, the symptoms regressed spontaneously. This description probably represents the first case in the literature of "congenital multicystic disease" of the pancreas.

May Thurner syndrome: description of a case with unusual clinical onset.

May-Thurner syndrome (MTS) is a congenital vascular alteration that is part of a restricted category of very rare vascular syndromes that have in common the compression of an arterial or venous vessel. MTS, first described in 1957, is due to compression of the left common iliac vein against the lumbar spine by the adjacent common iliac artery. It can cause chronic thrombosis of the left lower limb and can give edema, pain, claudication, thrombophlebitis, and, in severe cases, pulmonary embolism. Color Doppler and duplex Doppler ultrasound allow us to easily locate the deep vein thrombosis, to measure its extension, and to highlight the vascular changes typical of MTS: compression and consequent hypertension of the left common iliac vein. The therapy depends on the degree of venous stasis and on the presence of venous thrombosis; generally, it consists of the administration of short- or long-term anticoagulant and thrombolytic drugs. In cases of severe stenosis of the left common iliac vein, the first-choice treatment consists of positioning a vascular stent, which resolves compression and significantly reduces chronic thrombotic episodes. We describe a case of MTS with an unusual clinical onset with pulmonary embolism.

Wilkie Syndrome and Pseudo-Nutcracker Syndrome a Rare Combination: Description of a Case.

Wilkie's syndrome is a very rare vascular alteration caused by congenital or acquired reduction of space between the superior mesenteric artery (SMA) and aorta. In acquired form, it is caused by perivascular adipose tissue reduction due to rapid weight loss and, if symptomatic, causes postprandial vomiting and weight loss. The left renal vein (LRV) when it has a retro-aortic course can be compressed in aorto-vertebral space (AVS); if the stenosis is severe it can cause venous congestion symptoms with left flank pain, microhematuria, and thrombosis, this vascular alteration is known as a pseudo-nutcracker syndrome. The combination of Wilkie's and pseudo-nutcracker's syndrome (PNCS) is very rare and has not yet been described in the literature. We describe a case of a 62-year-old woman who presented symptoms and alterations typical of two syndromes.

Non traumatic intrathoracic liver herniation mimicking a pulmonary metastasis in patient with breast cancer: A case report.

Non-traumatic hepatic hernia is defined as hepatic protrusion through acquired or congenital defects on diaphragm without prior trauma. This event is rare among adults and infrequently reported in literature. 52-year-old Caucasian woman with surgically treated breast cancer with suspected lung metastasis detected during a routine Multidetector Computed Tomography lung exam. Ultrasound and subsequently Magnetic Resonance Imaging (MRI) was performed which revealed an overdiaphragmatic mass in contiguity with liver parenchyma compatible with overdiaphragmatic hepatic hernia. Differential diagnosis should be made with diaphragmatic or pulmonary nodule. Correct diagnosis can avoid further diagnostic investigations or invasive procedures such as biopsy. Magnetic Resonance Imaging is a non-risky method and can clarify interpretative doubts. Currently there are still controversies about traumatic or idiopathic nature of this hernia.

Superior mesenteric artery syndrome (Wilkie Syndrome) with unusual clinical onset: Description of a rare case.

Wilkie's Syndrome is a very rare disease caused by reduction of aorto-mesenteric space with consequent duodenum compression. It can combine with left renal vein stenosis which, when symptomatic, is known as "Nutcracker Syndrome". We describe a clinical onset case with epigastric pain without vomiting in a normal weight patient. 28-year-old woman who came to our observation for intense epigastric pain after a weight loss of 14 kg in 4 months. Multidetector Computed Tomography and Ultrasound revealed gastric and duodenal overdistension with hydro-air levels, severe duodenum stenosis, and left renal vein compression. Wilkie's Syndrome is common in anorexic individuals suffering from recurrent postprandial vomiting, onset with severe epigastric pain, without vomiting, is quite unusual. High-calorie diet must be first therapeutic approach, in case of failure treatment of first choice should be endovascular stenting and, only in selected cases, surgical treatment should be used because it is very invasive and burdened with numerous complications. Failure to diagnose this disease can expose patients to serious health risks.

Stylo-Jugular Venous Compression Syndrome: Lessons Based on a Case Report.

BACKGROUND Eagle syndrome is a vascular compression syndrome that is caused by a very elongated styloid process and/or calcification of the stylohyoid ligament compressing the vascular or nerve structures of the neck, resulting in vascular congestion, thrombosis, or neurological symptoms (eg, dysphagia, neck pain, ear pain). Stylo-jugular venous compression syndrome is a subtype of Eagle syndrome and is caused by compression of the internal jugular vein. Treatment varies according to the symptoms and the severity of the compression, and it can be pharmacological or surgical, with vascular stenting and/or removal of the styloid process. We describe a rare case of left cerebral venous sinus thrombosis and ipsilateral internal jugular vein stenosis sustained by excessive length of the left styloid process. CASE REPORT A 36-year-old woman presented with recurrent episodes of drug-resistant headache and recent memory disturbances. She underwent cerebral and neck multidetector computed tomography-angiography and Doppler ultrasound of the epiaortic vessels that respectively revealed thrombosis of the left cerebral venous sinus and left internal jugular vein stenosis due to a very long styloid process. The patient was treated with anticoagulant drugs and experienced a gradual remission of symptoms. CONCLUSIONS Compression of the jugular vein by the styloid process is a rare entity, and it often goes undiagnosed when it is asymptomatic. Doppler ultrasound is a sensitive method for identifying jugular vein stenosis and can provide an estimated degree of stenosis, which is useful for treatment planning. Doppler ultrasound should be combined with multidetector computed tomography-angiography to rule out compression of other vascular structures and other causes of compression. Failure to treat these patients could have serious health consequences for them.

A man with the rare simultaneous combination of three abdominal vascular compression syndromes: median arcuate ligament syndrome, superior mesenteric artery syndrome, and nutcracker syndrome.

Median arcuate ligament syndrome and superior mesenteric artery syndrome are well-known abdominal compression syndromes, the coexistence of which is rarely described in literature. In addition, due to the common pathogenesis, anterior nutcracker syndrome may occur simultaneously to superior mesenteric artery syndrome. To our knowledge, this is the first case reporting combination of these 3 syndromes detected with ultrasound, Computed Tomography and upper gastrointestinal fluoroscopic exam. A 69-year-old man came to our attention for rapid weight loss, postprandial epigastric pain and recurrent vomiting for at least 6 months. Doppler ultrasound showed both celiac artery and left renal vein stenosis with simultaneous left varicocele. Computed tomography showed a reduction of aortomesenteric space causing both left renal vein and duodenal stenosis, this latter confirmed by upper gastrointestinal fluoroscopic exam. The diagnosis of these three vascular compression syndromes (MALS, SMAS, and anterior NCS) has been formulated, based on clinical and imaging findings. We assumed that the postprandial crises caused by median arcuate ligament syndrome may induce a reduction of meals consumption and progressive weight loss which can be a cause of anterior nutcracker syndrome and superior mesenteric artery syndrome onset. Doppler ultrasound, in expert hands, allows to accurately diagnosing these syndromes which are often underestimated. Failure to recognize it and inadequate treatment could have serious consequences for patients' health.

May-Thurner Syndrome with Double Compression of the Iliac Vein: Lessons Based on a Case Report.

BACKGROUND May-Thurner syndrome, also known as Cockett's syndrome, is characterized by vascular alterations due to stenosis of the left iliac vein, usually caused by compression against the vertebral column by the right iliac artery. Doppler ultrasound represents the first level of examination for the study of this vascular pathology, and allows a very accurate study of the lower-limb vessels. We describe an unusual presentation with double stenosis of the left common iliac vein. CASE REPORT A 73-year-old woman came to the clinic for acute onset of worsening dyspnea, with lymphedema of the left lower limb, and was examined using ultrasound and multidetector computed tomography. The Doppler ultrasound exam showed 2 compressions of the common iliac vein by the right and left iliac artery due to a combination of osteophytosis of the vertebral column and reduced distance between the left iliac vein and the spine. CONCLUSIONS May-Thurner syndrome should be suspected in patients with symptoms of venous stasis of the left lower limb. Doppler ultrasound identified stenosis of the common iliac vein and the consequent flow changes. Failure to diagnose and treat May-Thurner syndrome could expose patients to very serious risks to their health.

The role of ultrasound imaging in vascular compression syndromes.

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.

Posterior nutcracker syndrome: a case report.

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.

The Role of Ultrasound in Dunbar Syndrome: Lessons Based on a Case Report.

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.