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.

Role of Magnetic Resonance Imaging in Pelvic Organ Prolapse Evaluation.

Background and Objectives: The primary method for assessing pelvic floor defects is through physical examination. Magnetic resonance imaging (MRI) is a radiological technique that is useful for identifying the underlying defects of pelvic floor structures that require surgery. The primary aim of this study was to find correspondence between the clinical and radiological staging of pelvic organ prolapse (POP) before and after vaginal surgery. A secondary endpoint was to investigate, through clinical and MRI findings, whether surgery influences continence mechanisms. Finally, we reported changes in the quality of life of women who underwent surgery for prolapse. Materials and Methods: Twenty-five women with prolapse stage ≥ 2 POP-Q were recruited in this prospective study. They underwent preoperative clinical examination, MRI at rest and under the Valsalva maneuver, and quality of life questionnaires. Three months after vaginal surgery, they repeated clinical and radiological evaluation. Results: Twenty women completed the study. Both clinical and MRI evaluations showed an improvement in prolapse and symptoms after surgery. There were some discrepancies between clinical and radiological staging. MRI parameters did not show differences between pre- and postoperative values at rest; under the Valsalva maneuver, instead, the measurements changed after surgery. Continence was not worsened by the widening of the vesicourethral angle. Patients reported an improvement in quality of life. Conclusions: MRI is an accurate and objective method for defining the stage of prolapse, but clinical evaluation alone is sufficient for staging prolapse before surgery and evaluating the result at follow-up. It is an accurate method for visualizing some pelvic structures that can be compromised because of pelvic organ prolapse. MRI showed that vaginal surgery does not affect continence mechanisms.

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.

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.

A unique posterior nutcracker syndrome combined with Wilkie syndrome: A singular case.

Left renal vein variants are not commonly observed in the general population. Usually, the renal vein runs in front of the aorta before entering the inferior vena cava, while the most common variants include the presence of a circumaortic or retroaortic renal vein. However, when present, left venal rein variants are important to recognize due to their potential clinical and surgical relevance. In this regard, CE-CT is an instrument with high sensitivity and specificity in detecting vascular anomalies and can certainly help diagnose. In this article, we present a unique case of a left venal rein compressed between the left iliac artery and vertebral bodies associated with the presence of a superior mesenteric artery Syndrome, another rare entity that occurs when the duodenum is compressed between the aorta and the superior mesenteric artery.

Beyond the bowel: Wernicke's encephalopathy as a neurological complication of Crohn's disease: A case report.

Wernicke encephalopathy (WE) is a rare but severe neurological syndrome characterized, in its classic form, by the acute onset of ocular disturbances, ataxia, and cognitive impairment. It is caused by a deficiency of thiamine (vitamin B1) and mainly affects chronic alcoholics, although it can also affect patients with pathologies that lead to malnutrition. We present a case of a 58-year-old woman, who presented with significant weight loss over the past 6 months and who came to the emergency department for episodes of repetitive vomiting and a sleepy state. The patient underwent blood chemistry tests and a brain CT scan, which revealed symmetrical and bilateral hypodensity of the medial portion of the thalamus, tectal plate, and periaqueductal gray matte, suggestive of WE. She was subsequently referred to the Department of Neurology and underwent a brain MRI, which confirmed the clinical suspicion. She also had an abdominal CT scan and ileo-colonoscopy and was diagnosed with Crohn's disease. Immediately after the clinical diagnosis of WE, a replacement therapy based on intravenous thiamine at high doses was promptly set up, and the patient improved from a clinical point of view. Wernicke encephalopathy can be difficult to diagnose when it occurs in non-alcoholic patients; WE associated with IBD is a rare condition, and it can present with atypical and more subtle symptoms. Radiologists and physicians must be aware of this condition and imaging findings for rapid diagnosis and treatment.

Corrigendum: Neuroimaging in PRUNE1 syndrome: a mini-review of the literature.

[This corrects the article DOI: 10.3389/fneur.2023.1301147.].

Congenital Lung Malformations: A Pictorial Review of Imaging Findings and a Practical Guide for Diagnosis.

The term congenital lung malformation (CLM) is used to describe a wide range of pathological conditions with different imaging and clinical manifestations. These anomalies stem from abnormal embryological lung development, potentially occurring across various stages of prenatal life. Their natural history can be variable, presenting in a wide range of severity levels and encompassing asymptomatic individuals who remain so until adulthood, as well as those who experience respiratory distress in the neonatal period. Through the PubMed database, we performed an extensive review of the literature in the fields of congenital lung abnormalities, including their diagnostic approach and findings. From our RIS-PACS database, we have selected cases with a final diagnosis of congenital lung malformation. Different diagnostic approaches have been selected, including clinical cases studied using plain radiograph, CT scan, prenatal ultrasound, and MR images. The most encountered anomalies can be classified into three categories: bronchopulmonary anomalies (congenital pulmonary airway malformations (CPAMs), congenital lobar hyperinflation, bronchial atresia, and bronchogenic cysts), vascular anomalies (arteriovenous malformation), and combined lung and vascular anomalies (scimitar syndrome and bronchopulmonary sequestration). CLM causes significant morbidity and mortality; therefore, the recognition of these abnormalities is necessary for optimal prenatal counseling and early peri- and postnatal management. This pictorial review aims to report relevant imaging findings in order to offer some clues for differential diagnosis both for radiologists and pediatric consultants.

The Immunomodulatory Potential of Concurrent High-Dose Radiotherapy and Immune Checkpoint Inhibitor Cemiplimab in Advanced Cutaneous Squamous Cell Carcinoma: Initial Results.

In this retrospective case series, we investigate the synergistic effect and the immunomodulatory potential of combination radiotherapy and immunotherapy on 11 patients affected by locally advanced or metastatic cutaneous squamous cell carcinoma (CSCC), treated at our institution between 2020 and 2023. The primary endpoints of this study are objective tumor response, assessed by Immunotherapy Response Evaluation Criteria in Solid Tumors (iRECIST), and time to treatment failure (disease progression). In all patients, surgery was deemed not amenable, due to its potential functional and aesthetic impact. Therefore, upon multidisciplinary agreement, radiotherapy and immunotherapy with cemiplimab were alternatively administered. After 6 months, an early objective tumor response was observed in 9/11 patients, with 17/20 cutaneous lesions (85%) presenting either a complete or partial response. Only 2/11 patients, with a total of 3/20 cutaneous lesions (15%), had stable disease. These benefits persisted at a longer follow-up (21.4 ± 9.7 months), with no patients presenting disease progression. Despite the retrospective nature of this study and small sample size, our experience highlights the ability of concomitant radiotherapy and cemiplimab to promote an early objective response in patients with advanced CSCC. Moreover, in our population, the clinical benefits were also related to a longer progression-free survival, without any safety alert reported.

Prostate Artery Embolization as Minimally Invasive Treatment for Benign Prostatic Hyperplasia: An Updated Systematic Review.

Background/Objectives: Benign prostatic hyperplasia (BPH) has a significant impact on the quality of life of symptomatic patients. In patients manifesting lower urinary tract symptoms (LUTS), prostatic arterial embolization (PAE) has become a topic of interest in recent years. The purpose of this systematic review is to analyze and review techniques and clinical outcomes of patients who underwent endovascular treatment of BPH, with a special focus on the comparison of surgical and endovascular procedures. Methods: Through the major scientific databases, 1225 articles were selected from the initial research utilizing specific keywords and medical subject headings. Based on the inclusion and exclusion criteria established for selecting relevant studies for our purposes, the systematic analysis of the literature was conducted on a total of seven articles. Results: We collected data on 718 male patients (408 patients underwent PAE and 310 received TURP). The technical success rate varied from 86% to 100% for TAE and was 100% for TURP. During a 12-month follow-up period, both PAE and TURP were comparable on the reduction in IPSS and QoL questionnaire, while TURP showed significant improvements in Qmax and in the reduction in prostate volume. Length and cost of hospitalization were lower for PAE. Complication and adverse events rates were higher in the TURP group rather than in the PAE group (60.6% vs. 35.5%). Conclusions: Prostatic artery embolization represents an emerging minimally invasive procedure for BPH. According to previously released clinical studies, quality-of-life and urological symptom scores of the PAE group were comparable to those of the TURP group. Our research strengthens the evidence supporting the effectiveness and safety of PAE as a therapy for LUTS related to BPH.

Magnetic Resonance Imaging of Central Nervous System Manifestations of Type 1 Neurofibromatosis: Pictorial Review and Retrospective Study of Their Frequency in a Cohort of Patients.

Background: type 1 neurofibromatosis (NF1) is the most common neurocutaneous disorder, and it is an inherited condition that causes a tumour predisposition. Central nervous system (CNS) manifestations are a significant cause of morbidity and mortality in NF1. We provide a pictorial review of neuroradiological features of NF1, with emphasis on magnetic resonance imaging (MRI), and we assess the frequency of those features on a cohort of NF1 patients. Methods: we retrospectively evaluated all patients with a diagnosis of NF1 who underwent MRI of the spine and brain in our centre over a period of almost 5 years. A total of 74 patients were enrolled, 28 males and 46 females, with a mean age of 21 ± 12.67 years. The frequency of CNS manifestations encountered in our cohort of NF1 patients was assessed and compared with the data found in other studies published in the literature. Results: many of our findings were in line with the literature, and possible interpretations for those that turned out to be different were suggested in the discussion. Conclusion: imaging plays a central role in the diagnosis and management of NF1, and the knowledge of CNS manifestations could be critical for its early detection and identification, such as for treatment planning and prognostic implications.

Spontaneous retroperitoneal hematoma treated with transarterial embolization: a systematic review and metanalysis.

PURPOSE: The purpose of this systematic review and meta-analysis was to evaluate the safety, technical, and clinical effectiveness of percutaneous Transarterial Embolization (TAE) in treating spontaneous retroperitoneal hematomas as well as assess treatment outcomes in patients who underwent target or empirical embolization. MATERIALS AND METHODS: Through the PubMed, Embase, and Google Scholar databases, an extensive search was performed in the fields of spontaneous retroperitoneal hematomas treated with transcatheter arterial embolization. We collected pooled data on 141 patients from 6 separate articles selected according to the inclusion and exclusion criteria. RESULTS: Technical success rate was 100% in all six studies, for both targeted and empirical embolization. The clinical success rate varied from 56.3 to 89.5%. The total number of complications related to the embolization procedure was 10 events out of 116 procedures analyzed. Empirical or empirical embolization was performed in three studies, where the source of active bleeding was not evident during DSA. A meta-analysis compared the rebleeding rates between targeted and empirical embolization groups. The odds ratio from pooled data from the three assessed studies (72 patients) showed no significant difference in rebleeding rates after empirical TAE compared with targeted TAE. CONCLUSIONS: TAE is a safe, effective, and potentially life-saving procedure for the treatment of life-threatening spontaneous retroperitoneal hematomas. Empirical and targeted TAE procedures demonstrate a relatively low risk of complications, compared to the high technical and relatively high clinical success rates.

Corrigendum to "Not always embolism: A case of pulmonary artery intimal sarcoma - The role of the radiologist in early diagnosis" [Respir. Med. Case Rep. 42 (2023) 101822].

[This corrects the article DOI: 10.1016/j.rmcr.2023.101822.].

Neuroimaging in PRUNE1 syndrome: a mini-review of the literature.

Prune exopolyphosphatase 1 (PRUNE1) is a short-chain phosphatase that is part of the aspartic acid-histidine-histidine (DHH) family of proteins. PRUNE1 is highly expressed in the central nervous system and is crucially involved in neurodevelopment, cytoskeletal rearrangement, cell migration, and proliferation. Recently, biallelic PRUNE1 variants have been identified in patients with neurodevelopmental disorders, hypotonia, microcephaly, variable cerebral anomalies, and other features. PRUNE1 hypomorphic mutations mainly affect the DHH1 domain, leading to an impactful decrease in enzymatic activity with a loss-of-function mechanism. In this review, we explored both the clinical and radiological spectrum related to PRUNE1 pathogenic variants described to date. Specifically, we focused on neuroradiological findings that, together with clinical phenotypes and genetic data, allow us to best characterize affected children with diagnostic and potential prognostic implications.

Acute Colonic Diverticulitis: CT Findings, Classifications, and a Proposal of a Structured Reporting Template.

Acute colonic diverticulitis (ACD) is the most common complication of diverticular disease and represents an abdominal emergency. It includes a variety of conditions, extending from localized diverticular inflammation to fecal peritonitis, hence the importance of an accurate diagnosis. Contrast-enhanced computed tomography (CE-CT) plays a pivotal role in the diagnosis due to its high sensitivity, specificity, accuracy, and interobserver agreement. In fact, CE-CT allows alternative diagnoses to be excluded, the inflamed diverticulum to be localized, and complications to be identified. Imaging findings have been reviewed, dividing them into bowel and extra-intestinal wall findings. Moreover, CE-CT allows staging of the disease; the most used classifications of ACD severity are Hinchey's modified and WSES classifications. Differential diagnoses include colon carcinoma, epiploic appendagitis, ischemic colitis, appendicitis, infectious enterocolitis, and inflammatory bowel disease. We propose a structured reporting template to standardize the terminology and improve communication between specialists involved in patient care.

Neuroimaging features of WOREE syndrome: a mini-review of the literature.

The WWOX gene encodes a 414-amino-acid protein composed of two N-terminal WW domains and a C-terminal short-chain dehydrogenase/reductase (SDR) domain. WWOX protein is highly conserved among species and mainly expressed in the cerebellum, cerebral cortex, brain stem, thyroid, hypophysis, and reproductive organs. It plays a crucial role in the biology of the central nervous system, and it is involved in neuronal development, migration, and proliferation. Biallelic pathogenic variants in WWOX have been associated with an early infantile epileptic encephalopathy known as WOREE syndrome. Both missense and null variants have been described in affected patients, leading to a reduction in protein function and stability. The most severe WOREE phenotypes have been related to biallelic null/null variants, associated with the complete loss of function of the protein. All affected patients showed brain anomalies on magnetic resonance imaging (MRI), suggesting the pivotal role of WWOX protein in brain homeostasis and developmental processes. We provided a literature review, exploring both the clinical and radiological spectrum related to WWOX pathogenic variants, described to date. We focused on neuroradiological findings to better delineate the WOREE phenotype with diagnostic and prognostic implications.

Radiotherapy of Orbital and Ocular Adnexa Lymphoma: Literature Review and University of Catania Experience.

Orbital and ocular adnexa lymphomas are rare neoplasms confined to the orbital region. The prognosis is generally favorable, with a high proportion of localized disease, indolent clinical course, prolonged disease-free intervals, and low lymphoma-related mortality rate. We report our experience on eleven patients with confirmed histological diagnosis of lymphoma stage IE-IIE, treated between 2010 and 2021 with radiotherapy alone or in association with chemotherapy or immunotherapy. Eight patients were treated with primary radiotherapy only, while three received previous systemic treatments. Six patients were treated with Proton beam therapy (PBT), and five with external beam radiotherapy (EBRT). The five-year local control rate was 98%; only one patient developed an out-of-field recurrence. We also conducted a comprehensive literature review using electronic databases (PubMed, EMBASE, and Cochrane Library). Articles were selected based on their pertinence to treatment of the ocular and adnexal lymphoma focusing on radiotherapy techniques (electron beam radiotherapy, photon beam radiotherapy, or proton beam radiotherapy), treatment total dose, fractionation schedule, early and late radio-induced toxicities, and patient's clinical outcome. Radiotherapy is an effective treatment option for orbital lymphoma, especially as standard treatment in the early stage of orbital lymphoma, with excellent local control rate and low rates of toxicity.