Spigelian-cryptorchidism syndrome: Lesson based on a case report.

The Spigelian hernia is a abdominal wall hernia that originates from a discontinuity of the Spigelian fascia located lateral to the rectus abdominis muscle. It can be acquired in adults or congenital in newborns. In very rare cases in male it can be associated with cryptorchidism, in which case it is known as "Spigellian-Cryptorchidism Syndrome". It can be clinically highlighted with abdominal swelling wall along the semilunar line and intestinal obstruction. The diagnosis, as in all pediatric emergencies, must be timely and the method of choice is ultrasound which allows a rapid localization of the hernia breach and herniated structures. The treatment of choice is surgical with herniopexy and repositioning of the testicle into the scrotal sac, or orchipessy in cases of testicular necrosis. We describe ultrasound characteristics of Spigellian-cryptorchidism syndrome presenting with acute intestinal obstruction in a newborn.

Suppurative thyroiditis, a sign of branchiogenic fistula? Lesson based on a case report.

Branchiogenic fistulas are congenital alterations that affect the cervical compartments. Those of the fourth branchial cleft are rarest and can begin late with very serious complications. The suppurative thyroiditis can be a complication of these alterations. We describe a case of 3-year-old girl with high fever, left cervical swelling and increased inflammation indices. The neck ultrasound showed an abscess of the left thyroid lobe and a fluid mass with aerial content in laterocervical region. On MRI, the fluid mass extended from the left piriform sinus to the mediastinum. Fluoroscopy also highlighted a fistolose trait that extended from the left side wall of the esophagus, anteriorly towards the trachea. Treatment of these pathologies must be early and a late diagnosis can put patients' lives at risk.

Renal extramedullary hematopoiesis as an epiphenomenon of bone marrow dysfunction in a patient with primary myelofibrosis: A rare case report.

Extramedullary hematopoiesis represents a clinical compensatory condition characterized by the growth of hematopoietic tissue outside the bone marrow. It can mainly occur in patient with myeloproliferative disorders where alteration or neoplastic invasion of the bone marrow causes ineffective production of blood cells with the recruitment of progenitrix blood cells in non-hematopoietic organs, including kidneys. Renal extramedullary hematopoiesis is a rare condition manifesting as parenchymal or perirenal soft tissue masses with different patterns mimicking neoplasms, infectious or vascular diseases. We describe a unique case of a patient affected by primary myelofibrosis underwent ultrasound and magnetic resonance examinations showing bilateral perirenal alterations to be related to hemopoietic tissue. We also focused on the pathophysiology of this condition with imaging correlation. The case we present emphasises the importance of recognising the main radiological features of renal extramedullary hematopoiesis. MR examination should become part of the diagnostic pathway of the patient with primary myelofibrosis.

Lemmel's Syndrome: Lesson Based on a Case Report.

Lemmel's syndrome is a bile duct disease caused by periampullary duodenal diverticula that develop within 2-3 cm of the Vater papilla. This disease manifests itself as nonobstructive jaundice. In most cases, duodenal diverticula do not cause disease, and only in a small percentage of patients, diverticula cause biliary tract obstruction by extrinsic compression. If the compression is severe, in the long term, it can become complicated with lithiasis and cholangitis. Diagnosis is very difficult, and recurrent biliary symptoms must be directly related to the compression of the duodenal diverticula. Imaging is essential for differential diagnosis and includes conventional contrast radiographs, endoscopic retrograde cholangiopancreatography, computed tomography, and magnetic resonance imaging. The investigations show the dilation of the intra- and extra-hepatic bile ducts in the absence of lithiasis or main pancreatic duct dilatation, compressed by the diverticula, which most frequently originate from the medial wall of the second duodenal tract. The treatment of choice is surgical with removal of the diverticula. Failure to diagnose can cause serious health complications for the patient.

Quantitative Diffusion-Weighted MR Imaging: Is There a Prognostic Role in Noninvasively Predicting the Histopathologic Type of Uveal Melanomas?

Histopathologically, uveal melanomas (UMs) can be classified as spindle cell, mixed cell and epithelioid cell type, with the latter having a more severe prognosis. The aim of our study was to assess the correlation between the apparent diffusion coefficient (ADC) and the histologic type of UMs in order to verify the role of diffusion-weighted magnetic resonance imaging (DWI) as a noninvasive prognostic marker. A total of 26 patients with UMs who had undergone MRI and subsequent primary enucleation were retrospectively selected. The ADC of the tumor was compared with the histologic type. The data were compared using both one-way analysis of variance (ANOVA) (assessing the three histologic types separately) and the independent t-test (dichotomizing histologic subtypes as epithelioid versus non-epithelioid). Histologic type was present as follows: the epithelioid cell was n = 4, and the spindle cell was n = 11, the mixed cell type was n = 11. The mean ADC was 1.06 ± 0.24 × 10-3 mm2/s in the epithelioid cells, 0.98 ± 0.19 × 10-3 mm2/s in the spindle cells and 0.96 ± 0.26 × 10-3 mm2/s in the mixed cell type. No significant difference in the mean ADC value of the histopathologic subtypes was found, either when assessing the three histologic types separately (p = 0.76) or after dichotomizing the histologic subtypes as epithelioid and non-epithelioid (p = 0.82). DWI-ADC is not accurate enough to distinguish histologic types of UMs.

Combination between Dunbar Syndrome and May-Thurner Syndrome: A Rare Case Report.

Dunbar syndrome (DS) and May-Thurner syndrome (MTS) are part of a group of rare vascular disorders known as "vascular compression syndromes." Dunbar's syndrome is caused by the median arcuate ligament of diaphragm, which, due to an abnormal course, causes celiac artery compression. MTS is caused by the left common iliac vein compression pushed against the spine by the right common iliac artery causing progressive flow congestion and leading to thrombosis. Ultrasound is the first-level examination for the diagnosis of these rare pathologies and allows to recognize vascular compressions and to obtain an estimate of stenosis degree. We describe a very rare case of DS and MTS combination in a young man with postprandial pain and left lower limb thrombosis.

The Role of Ultrasound in Venous Thoracic Outlet Syndrome: Lesson Based on a Case Report.

Thoracic outlet syndrome is a rare neurovascular disease, usually caused by compression of subclavian vessels and/or brachial plexus by a cervical rib or hypertrophic scalene muscles. When the subclavian artery is compressed, it can cause perfusion deficiency with cyanosis and greater fatigue of the upper limb; if the subclavian vein is compressed, it can cause venous congestion with swelling and pain in the upper limb. When compression affects the brachial plexus, it can cause tingling or paresthesia of the upper limb. It can be mono or bilateral, congenital, or acquired. The diagnosis of vascular alterations is difficult due to the rarity, poor knowledge, and nonspecificity of symptoms. Ultrasound allows to localize and measure the vascular stenosis degree. Failure to diagnose can expose patients to serious health risks. We describe a rare case of venous compression caused by anterior scalene muscle hypertrophy.

Double nutcracker syndrome in a patient with circumaortic venous ring: a rare case report.

Aim of the study: In this article, we describe a rare case of the nutcracker syndrome caused by combined compression of the left anteroaortic and retroaortic renal veins. Case description: A 42-year-old woman presented with microhematuria and left flank pain. The patient underwent computed tomography and Doppler ultrasound which showed the left renal veins with anteroaortic and retroaortic courses, with signs of compression. Compression of the anteroaortic renal vein was caused by a narrowing of the aortomesenteric space, whereas compression of the retroaortic renal vein was caused by a narrowing of the aortovertebral space. Conclusions: NCS is a rare disease, poorly understood and difficult to diagnose mainly due to the non-specificity of symptoms. Imaging is essential for diagnosis, and the combination of ultrasound and computed tomography allows for better classification of the disease. Increased disclosure of these cases can significantly contribute to a reduction of false negatives.

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.

Hepatic Sarcoidosis: Lesson Based on a Case Report.

Sarcoidosis is a multisystemic disease of unknown etiology that can involve lungs, abdominal organs, and lymph nodes. The incidence of sarcoidosis is highest between the ages of 20 and 40 years, and it affects both sexes equally. The most frequent localization is in the lungs, and about half of the affected patients are generally asymptomatic but can involve in small percentages of various other parts such as the biliary tract, pancreas, stomach, and urinary tract. Hepatic and splenic localization is infrequent, and lesions are often mistaken for metastases due to their morphologic similarity. The histological lesion of sarcoidosis is a noncaseous granuloma always associated with high levels of angiotensin-converting enzyme, hypercalcemia, hypercalciuria, and, in a significant percentage of cases (35%-40%), liver enzyme alteration. The pathological evolution of granulomas is fibrosis, and the most severe cases of hepatic sarcoidosis can develop into cirrhosis or portal hypertension. Imaging is essential for lesion localization and is represented by ultrasound, computed tomography (CT), magnetic resonance imaging, and positron emission tomography/CT. The differential diagnosis is very difficult and is almost always histological. We describe a case of hepatic sarcoidosis in an asymptomatic patient with elevated liver enzymes.

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.

Histopathologic and MR Imaging Appearance of Spontaneous and Radiation-Induced Necrosis in Uveal Melanomas: Initial Results.

Necrosis in uveal melanomas can be spontaneous or induced by radiotherapy. The purpose of our study was to compare the histopathologic and MRI findings of radiation-induced necrosis of a group of proton beam-irradiated uveal melanomas with those of spontaneous necrosis of a control group of patients undergoing primary enucleation. 11 uveal melanomas who had undergone proton beam radiotherapy, MRI and secondary enucleation, and a control group of 15 untreated uveal melanomas who had undergone MRI and primary enucleation were retrospectively identified. Within the irradiated and nonirradiated group, 7 and 6 eyes with histological evidence of necrosis respectively, were furtherly selected for the final analysis; the appearance of necrosis was assessed at histopathologic examination and MRI. Irradiated melanomas showed a higher degree of necrosis as compared with nonirradiated tumors. Irradiated and nonirradiated lesions differed based on the appearance and distribution of necrosis. Irradiated tumors showed large necrotic foci, sharply demarcated from the viable neoplastic tissue; nonirradiated tumors demonstrated small, distinct foci of necrosis. Radiation-induced necrosis, more pigmented than surrounding viable tumor, displayed high signal intensity on T1-weighted and low signal intensity on T2-weighted images. The hemorrhagic/coagulative necrosis, more prevalent in nonirradiated tumors (4 out of 6 vs. 1 out of 7 cases), appeared hyperintense on T2-weighted and hypointense on T1-weighted images. Our study boosts the capability to recognize radiation-induced alterations in uveal melanomas at MRI and may improve the accuracy of radiologists in the evaluation of follow-up MR examination after radiotherapy.

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.

Diagnostic methods and therapeutic options of uveal melanoma with emphasis on MR imaging-Part I: MR imaging with pathologic correlation and technical considerations.

Uveal melanoma is a malignant neoplasm that derives from pigmented melanocytes of the uvea and involves, in order of decreasing prevalence, the choroid, ciliary body and iris. Its prognosis is related to histopathologic and genetic features, tumor size and location, extraocular extension. The diagnosis is fundamentally based on clinical evaluation (ophthalmoscopy, biomicroscopy) and ultrasonography. MRI is useful in case of untransparent lens or subretinal effusion. Moreover, MRI has a significant role to confirm the diagnosis, in the evaluation of the local extent of the disease with implications for treatment planning, and in the follow-up after radiotherapy treatment. Uveal melanoma can show different morphologic features (lentiform, dome or mushroom shape) and often determines retinal detachment. MR appearance of uveal melanoma mainly depends on the melanin content. Uveal melanoma typically displays high signal intensity on T1-weighted images and low signal intensity on T2-weighted images. Nevertheless, imaging appearance may be variable based on the degree of pigmentation and the presence of areas of necrosis or cavitation. Differential diagnosis includes other uveal lesions. The radiologists and in particular MRI play a significant role in the clinical management of uveal melanoma. The purpose of this pictorial review is to provide the radiologists with awareness about diagnostic methods and therapeutic options of uveal melanoma. In the present first section we summarize the MR anatomy of the eye and describe ophthalmological and radiological imaging techniques to diagnose uveal melanomas, with emphasis on the role of MR imaging. Additionally, we review MR imaging appearance of uveal melanomas.