Radio-pathologic correlation: no pial angioma-subarachnoid varicose network drainage pathway in Sturge-Weber syndrome.

PURPOSE: The traditional imaging findings reported in Sturge-Weber syndrome (SWS) include endpoints of cortical injury-cortical atrophy and cortical calcifications-but also what has been termed a "leptomeningeal angiomatosis," the latter recognized and reported as a leptomeningeal enhancement on magnetic resonance imaging (MRI). The objective of this study is to demonstrate through neuropathological correlation that the "leptomeningeal angiomatosis" in patients with Sturge-Weber syndrome (SWS), represents a re-opened primitive venous network in the subarachnoid space that likely acts as an alternative venous drainage pathway, seen separately to abnormal pial enhancement. MATERIALS AND METHODS: Retrospective review of MR imaging and surgical pathology of patients that underwent surgery for epilepsy at a tertiary, children's hospital. A pediatric radiologist with more than 20 years of experience reviewed the MR imaging. Surgically resected brain specimens that had been sectioned and fixed in 10% paraformaldehyde for histologic processing, following processing and paraffin embedding, were cut into 5-µm unstained slides which were subsequently stained with hematoxylin and eosin (H&E). Slides were re-examined by a board-certified pediatric neuropathologist, and histologic features specifically relating to cerebral surface and vascularity were documented for correlation with MR imaging of the resected region performed prior to resection. RESULTS: Five patients were reviewed (3 boys and 2 girls; the median age at the onset of seizures was 12 months (IQR, 7 to 45 months); the median age at surgery was 33 months (IQR, 23.5 to 56.5 months)). Surgical procedures included the following: 4, hemispherotomy (right: 2, left: 2) and 1, hemispherectomy (right). A subarachnoid space varicose network was present on both MRI and histology in 4 patients. Calcifications were seen on both MRI and histology in 3 patients. Abnormal leptomeningeal enhancement was present in 5 patients and seen separately from the subarachnoid vascular network in 4 patients. CONCLUSION: Histopathology confirmed the MRI findings of a subarachnoid space varicose network seen separately from leptomeningeal enhancement and presumed to represent an alternative venous drainage pathway to compensate for maldevelopment of cortical veins, the primary abnormality in SWS. No pial-based angioma was identified.

Impact of etiology on disease course in chronic pancreatitis.

BACKGROUND: Complications in chronic pancreatitis (CP) can be grouped in inflammatory (ICC) and fibrotic (FCC) clusters and pancreatic insufficiency cluster (PIC). However, the association between etiological risk factors and the development of complication clusters remains obscure. In this study, the impact of the etiology and disease duration on disease onset and development of complications was investigated. METHODS: This cross-sectional study recruited patients with CP from Mannheim/Germany (n = 870), Gießen/Germany (n = 100) und Donetsk/Ukraine (n = 104). Etiological risk factors, disease stage, age at disease onset, complications, need for hospitalization and surgery were noted. RESULTS: In 1074 patients diagnosed with CP, main risk factors were alcohol and nicotine abuse. An earlier onset of the disease was observed upon nicotine abuse (-4.0 years). Alcohol abuse was only associated with an earlier onset of the definite stage of CP. Alcohol abuse was the major risk factor for the development of ICC (p < 0.0001, multiple regression modeling). Abstinence of alcohol reduced ICC, whereas abstinence of nicotine showed no association. PIC correlated with efferent duct abnormalities and the disease duration. In contrast, FCC was mainly dependent on the disease duration (p < 0.0001; t-test). The presence of any complication cluster correlated with the need for surgery (p < 0.01; X2-test). However, only ICC correlated with a prolonged hospital stay (p < 0.05; t-test). CONCLUSIONS: ICC is mainly dependent on alcohol abuse. In contrast, FCC and PIC are mainly dependent on the disease duration. The etiology and disease duration can be used as predictors of the course of disease to provide individual treatment and surveillance strategies.

Imaging in a new pediatric brain tumor-a supratentorial neuroepithelial tumor with PLAGL1 fusion.

Molecular diagnostics have dramatically influenced the classification of tumor groups in the 2021 WHO CNS tumor classification. Studies focusing on molecular diagnostics continue to identify new tumors. Soon after the summary of the new classification was published, "Supratentorial Neuroepithelial Tumor with PLAGL1 Fusion" was described as a distinct entity. Although this new entity is defined pathologically, its imaging features are undefined. This case report discusses the imaging findings and possible differential diagnosis of the new tumor.

Acquired hump of the corpus callosum: a rare morphologic complication after CSF shunting.

PURPOSE: To present the longitudinal MR imaging of 4 children with an acquired corpus callosum hump, in order to demonstrate graphically that this represents a dysmorphology caused through a constellation of pre-existing pathology, timing, and complications of treatment. MATERIALS AND METHODS: Four cases with a corpus callosum hump were evaluated for common findings in the clinical history and on MRI scans. Those patients with available follow-up imaging were specifically evaluated for the presence of the hump on initial neonatal imaging and for evidence of development and progression of the deformity over time. Corpus callosum length was measured and compared against normal standards. RESULTS AND CONCLUSION: Congenital hydrocephalus, chronic ventricular over-shunting, white matter volume loss, and lateral ventricle communication were common to all cases. Corpus callosum length was above normal values. The corpus callosum hump term was previously described as dysplasia but was not present on initial scans in our cases. We conclude that the corpus callosum hump can be acquired as a complication of over-shunting in children with congenital hydrocephalus. Thus, we present our examples as "acquired hump of the corpus callosum," which differs from the prior example. We postulate that the lengthening of the stretched corpus callosum due to chronic hydrocephalus in the pre-myelinated state renders it unable to return to its normal shape when the ventricles are drained. Over-shunting of both lateral ventricles simultaneously in the absence of a septum pellucidum results in collapse and folding in of the corpus callosum on itself, resulting in the hump.

A Comparative Review of Imaging Journal Policies for Use of AI in Manuscript Generation.

RATIONALE AND OBJECTIVES: Artificial intelligence (AI) technologies are rapidly evolving and offering new advances almost on a day-by-day basis, including various tools for manuscript generation and modification. On the other hand, these potentially time- and effort-saving solutions come with potential bias, factual error, and plagiarism risks. Some journals have started to update their author guidelines in reference to AI-generated or AI-assisted manuscripts. The purpose of this paper is to evaluate author guidelines for including AI use policies in radiology journals and compare scientometric data between journals with and without explicit AI use policies. MATERIALS AND METHODS: This cross-sectional study included 112 MEDLINE-indexed imaging journals and evaluated their author guidelines between 13 October 2023 and 16 October 2023. Journals were identified based on subject matter and association with a radiological society. The authors' guidelines and editorial policies were evaluated for the use of AI in manuscript preparation and specific AI-generated image policies. We assessed the existence of an AI usage policy among subspecialty imaging journals. The scientometric scores of journals with and without AI use policies were compared using the Wilcoxon signed-rank test. RESULTS: Among 112 MEDLINE-indexed radiology journals, 80 journals were affiliated with an imaging society, and 32 were not. 69 (61.6%) of 112 imaging journals had an AI usage policy, and 40 (57.9%) of 69 mentioned a specific policy about AI-generated figures. CiteScore (4.9 vs 4, p = 0.023), Source Normalized Impact per Paper (1.12 vs 0.83, p = 0.06), Scientific Journal Ranking (0.75 vs 0.54, p = 0.010) and Journal Citation Indicator (0.77 vs 0.62, p = 0.038) were significantly higher in journals with an AI policy. CONCLUSION: The majority of imaging journals provide guidelines for AI-generated content, but still, a substantial number of journals do not have AI usage policies or do not require disclosure for non-human-created manuscripts. Journals with an established AI policy had higher citation and impact scores.

Third Ventricular Diameter is Inversely Related to Thalamic Massa Intermedia Thickness in Hydrocephalus Caused by Congenital Aqueductal Stenosis.

BACKGROUND AND PURPOSE: In fetuses with lateral ventriculomegaly and normal posterior fossa cerebrospinal spaces, third ventricular distention is a compelling clue that supports a diagnosis of aqueductal stenosis. However, this association assumes normal ventricular anatomy. Structural constraints can impair pressure-induced compliance. We aimed to determine how thalamic massa intermedia (TMI) size alterations may impact the size of the 3rd ventricle in the setting of congenital aqueductal stenosis (CAS). MATERIALS AND METHODS: This retrospective study was performed at a single academic pediatric hospital after IRB approval. We searched our brain MRI reports for all exams describing "aqueductal stenosis" and included all the patients who had both fetal and postnatal exams. Patients with interhypothalamic adhesions and hydrocephalus unrelated to CAS were excluded from this study. We evaluated all the MRIs for the presence of TMI and documented third ventricle diameters (supraoptic recess, central, and suprapineal recess) and the TMI circumference. Spearman correlation was used to identify the potential relationship between the TMI circumference and 3rd ventricle size in fetal and postnatal MRIs. Patients were also stratified into two groups based on the presence or absence of TMI. Mann-Whitney U tests were used to compare third ventricle diameters between these groups. RESULTS: The study included both fetal and postnatal studies from 59 patients. The overall third ventricular diameter was inversely proportional to the circumference of the TMI in both groups (fetal: p=0.001, rho=-0.422, CI=[-0.628-0.181]; postnatal: p<0.001, rho=-0.653, CI= [-0.782-0.479]). Nonetheless, dilation of anterior and posterior recesses still occurred when the mid third ventricle was non-dilated or less severely dilated in patients with an enlarged TMI. Third ventricular dilation was most severe in patients lacking a TMI compared to patients with a TMI (p<0.001). CONCLUSIONS: In patients with suspected congenital aqueductal stenosis, lack of significant third ventriculomegaly as conventionally measured can sometimes be explained by thickening of the TMI. In this circumstance, it is important to evaluate the extreme recesses of the 3rd ventricle for evidence of dilatation on fetal MRI.ABBREVIATIONS: TMI = Thalamic massa intermedia; CAS = Congenital aqueductal stenosis.

ASL Perfusion Lightbulb Sign: An Imaging Biomarker of Pediatric Posterior Fossa Hemangioblastoma.

BACKGROUND AND PURPOSE: Hemangioblastoma is a rare vascular tumor that occurs within the central nervous system in children. Differentiating hemangioblastoma from other posterior fossa tumors can be challenging on imaging, and preoperative diagnosis can change the neurosurgical approach. We hypothesize that a 'lightbulb sign' on the ASL sequence (diffuse homogenous intense hyperperfusion within the solid component of the tumor) will provide additional imaging finding to differentiate hemangioblastoma from other posterior fossa tumors. MATERIALS AND METHODS: In this retrospective comparative observational study, we only included pathology-proven cases of hemangioblastoma, while the control group consisted of other randomly selected pathology-proven posterior fossa tumors from January 2022 to January 2024. Two blinded neuroradiologists analyzed all applicable MRI sequences, including ASL sequence if available. ASL was analyzed for the 'lightbulb sign'. Disagreements between the radiologists were resolved by a third pediatric neuroradiologist. Chi-square and Fisher's exact test were used to analyze the data. RESULTS: 95 patients were enrolled in the study; 57 (60%) were male. The median age at diagnosis was 8 years old (IQR: 3-14). Out of the enrolled patients, 8 had hemangioblastoma, and 87 had other posterior fossa tumors, including medulloblastoma (n=31), pilocytic astrocytoma (n=23), posterior fossa ependymoma type A (n=16), and other tumors (n=17). The comparison of hemangioblastoma vs non-hemangioblastoma showed that peripheral edema (p=0.02) and T2 flow void (p=0.02) favors hemangioblastoma, whereas reduced diffusion (low ADC) (p=0.002) and ventricular system extension (p=0.001) favors nonhemangioblastoma tumors.Forty-two cases also had ASL perfusion sequences. While high perfusion favors hemangioblastoma (p=0.03), the lightbulb sign shows a complete distinction since all the ASL series of hemangioblastoma cases (n=4) showed the lightbulb sign, whereas none of the nonhemangioblastoma cases (n=38) showed the sign (p<0.001). CONCLUSIONS: Lightbulb-like intense and homogenous hyperperfusion patterns on ASL are helpful in diagnosing posterior fossa hemangioblastoma in children.ABBREVIATIONS ASL = Arterial spin labelling; pASL = Pulsed arterial spin labelling; pCASL = Pseudocontinuous arterial spin labelling; DCE = Dynamic contrast-enhanced; DSC = Dynamic susceptibility contrast; VHL = Von Hippel Lindau.

Impact of alcohol and smoking cessation on the course of chronic pancreatitis.

OBJECTIVES: Alcohol and nicotine are the two most important risk factors of chronic pancreatitis and they often occur together. It is still unclear how much they influence the severity of the disease and which of the two addictions should be treated with priority. METHODS: We performed a single-center, retrospective, cross-sectional study in a mixed medicosurgical cohort of 870 patients diagnosed with chronic pancreatitis (CP). We analyzed the impact of the drinking pattern and abstinence for alcohol and nicotine on the course of the disease. Patients with alcoholic CP were subdivided in I) patients with "life-time drinking history" (LTDH), II) "current drinkers" with current alcohol abuse without signs of LTDH, and III) "former drinkers" who stopped or reduced alcohol intake dramatically. RESULTS: Compared to patients with LTDH, "former drinkers" had a lower rate of exocrine insufficiency (29% vs. 59%) and pseudocysts (33% vs. 49%), were more often relapse-free (37% vs. 5%) and had less abdominal pain. There was no correlation detected between the quantity of alcohol consumption and the severity or progression of the disease. Regarding nicotine, 29 pack years are the threshold for developing early stage of CP. Under nicotine abstinence, only slightly more patients were relapse-free (37% vs. 22%). In contrast, the cumulative amount of nicotine consumed correlated with overall disease severity and the development of pseudocysts. The need for surgery was increased with odds ratios of 1.8 for both, alcohol and nicotine abuse. CONCLUSIONS: Alcohol cessation in chronic pancreatitis reduces exocrine insufficiency, abdominal pain and local complications. The effect of nicotine cessation is less pronounced in our cohort. However, nicotine abuse represents an important factor for the development of the disease.

Hamlet and radiology.

After I started my radiology training, searching for art in radiology became a passion for me. One of the most concrete examples of my search was a case of CT-guided biopsy we encountered recently. In a patient with metastatic cancer, we searched for the primary lesion. PET/CT showed a focus in the upper lobe of the right lung. During the CT-guided biopsy, this lesion was like a smiling face in shape. The fact that this cute-looking mass was metastatic cancer reminded me of a character from Hamlet. In William Shakespeare's famous work, Prince Hamlet refers to Claudius as a 'smiling villain' and draws attention to the evil behind his smile. In this article, we discuss the similarity of our daily practice with Hamlet through a case.