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.

Thoracic outlet syndrome: a rare case with bilateral cervical ribs and bilateral anterior scalene hypertrophy.

Thoracic outlet syndrome (TOS) is a rare neurovascular disorder generally caused by the presence of a cervical rib or hypertrophic scalene anterior muscle that can compress the brachial plexus and/or subclavian vessels. In the vascular form, the symptoms are caused by the compression of the artery and/or the subclavian vein. In the first case, the compression is caused by the cervical rib and leads to hypo-perfusion with cooling and cyanosis of the upper limb, while in the second case, the compression is caused by the anterior scalene muscle and leads to congestion, cyanosis, swelling and pain in the higher limb. In this paper, we describe a case with the simultaneous presence of a bilateral cervical rib and bilateral hypertrophy of the anterior scalene muscle. TOS diagnosis is based on neurological, clinical and instrumental tests, such as chest radiography and color Doppler ultrasonography. The treatment of these patients can be surgical or conservative.

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.

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 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.

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.

A Case of Nutcracker Syndrome Combined with Wilkie Syndrome with Unusual Clinical Presentation.

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 aorto-mesenteric 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.