Focal Nodules Research Articles

Dynamic contrast-enhanced ultrasound findings of focal nodular hyperplasia-like nodules in cirrhosis: a description of two cases and literature analysis.

Dynamic contrast-enhanced ultrasound findings of focal nodular hyperplasia-like nodules in cirrhosis: a description of two cases and literature analysis.

Polypoidal Choroidal Vasculopathy: Evaluation Based on 3-Dimensional Reconstruction of OCT Angiography

Three-dimensional (3D) reconstruction using swept-source OCT angiography (SS-OCTA) can provide insights into the nature and structure of polypoidal choroidal vasculopathy (PCV) and its component parts, the polypoidal lesion (PL) and the branching neovascular network (BNN). This study aims to describe novel observations of PCV using 3D reconstruction of SS-OCTA, and to compare these observations with similar images of type I macular neovascularization (MNV) typical neovascular age-related macular degeneration (nAMD). Clinical case series. Patients with PCV in either eye from clinical studies conducted in a tertiary retina center. Images with prespecified SS-OCTA imaging protocol were obtained and reconstructed in 3D. Forty neovascularization lesions (30 PCV and 10 typical nAMD) based on SS-OCTA were analyzed. The following 3 specific features were evaluated: (1) the pattern of flow signal within the PLs as either homogenous or showing internal vascular architecture; (2) the configuration of the BNN as hypermature, mature, or immature; and (3) the spatial arrangement of the PLs in relation to the BNN. Comparisons were made between PCV and typical nAMD. All PLs exhibited internal vascular architecture in the form of coil-like loops and none exhibited homogenous flow. Small focal nodules were present within this internal vascular architecture in 70% of PLs. Branching neovascular networks exhibited a hypermature/mature configuration (100 vs. 50%, P < 0.01) and were associated with thicker choroid compared with typical nAMD type 1 MNV (238.7 ± 104.3 vs. 155.6 ± 49.2, P= 0.02). The BNN and PL were located at distinct anteroposterior planes in 81% of the eyes. We identified proliferating vasculature in both the PL and the BNN. Comparison of the configuration suggests that the BNN represents a more chronic and inactive lesion than the PL. Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

FRI253 Concurrent Adrenal Insufficiency And Hyperthyroidism In A Breast Cancer Patient Treated With Pembrolizumab Therapy

Abstract Disclosure: A.U. Kulkarni: None. D.T. Patel: None. E.B. Ruby: None. Anti-programmed cell death-1 (Anti-PD1) antibodies (e.g., Pembrolizumab) are immune checkpoint inhibitors that are widely in use for the treatment of many advanced malignancies. This has resulted in various side effects known as irAEs (immune-related adverse events). Endocrine irAEs include hypophysitis, adrenal insufficiency (AI), hypothyroidism, and hyperthyroidism. A 40-yo female with a history of recently diagnosed triple-negative breast cancer, status post chemotherapy, and immunotherapy with Pembrolizumab (last treatment a month prior to presentation) came in with fatigue, weight loss, and persistent vomiting for two weeks. Her initial BP was noted to be 80/50 mmHg, with HR 108bpm. She appeared weak and tired with no other abnormalities on physical exam. The thyroid was normal with no enlargement. Significant labs were creatinine 1.4mg/dL (0.4-1.3mg/dL), corrected calcium 12.8mg/dL, TSH of &amp;lt;0.01mUI/L and fT4 of 4.47ng/dL (0.61-1.12ng/dL) (thyroid tests were previously normal). The patient was admitted to the ICU for hypovolemic shock refractory to IV fluids and endocrinology was consulted for multiple hormonal abnormalities. AKI and hypercalcemia were likely from dehydration as they subsequently resolved with IV fluids. AM cortisol level was low at 0.7ug/dL(3-16ug/dL). The cosyntropin test was positive, confirming AI. ACTH level was &amp;lt;5pg/mL (normal&amp;lt;46pg/mL) indicating a diagnosis of secondary AI, likely from immunotherapy. She was initiated on IV hydrocortisone. MRI brain was obtained to evaluate for hypophysitis which was unremarkable. Further thyroid testing was pursued which showed negative Thyroid peroxidase and anti-thyroglobulin antibodies. Thyroid scan showed a focal hot nodule in the left lobe suspicious for an autonomous nodule. She was started on beta blockers and methimazole with the goal of optimizing her thyroid function prior to her elective mastectomy. Pembrolizumab was discontinued with the plan to re-challenge after surgery. BP stabilized and she was discharged home on hydrocortisone with close outpatient follow-up with Endocrinology and Oncology. The clinical manifestations of Anti-PD1-associated AI are very non-specific and difficult to diagnose as they can be confused with the cancer-related illness itself or chemotherapy. Multiple endocrine abnormalities on initial labs with recent Pembrolizumab therapy in our patient raised suspicion for irAEs. Even though the occurrence of AI from anti-PD1 drugs is extremely rare, being &amp;lt;1%, it can be life-threatening. Very few cases of Anti-PD1-induced thyroid dysfunction have been reported, however further testing in our patient confirmed hyperthyroidism from a toxic thyroid nodule. With the increasing popularity of these drugs, endocrinological complications are on the rise, and we as clinicians need to be vigilant and intervene at an early stage to prevent irreversible and fatal outcomes. Presentation: Friday, June 16, 2023

SAT483 Role of Cholestyramine in Refractory Amiodarone-induced Thyrotoxicosis

Abstract Disclosure: H. Zhao: None. J. Leung: None. Role of Cholestyramine in Refractory Amiodarone-Induced Thyrotoxicosis. Background: Amiodarone-induced thyrotoxicosis (AIT) is divided into type 1 (AIT1; treated with thioamides) and type 2 (AIT2; treated with glucocorticoids). Combined therapy is utilized in mixed or indefinite forms. When medical treatment is unsuccessful, radioiodine ablation or thyroidectomy are considered. Clinical Case: A 76-year-old gentleman, with a history of atrial fibrillation treated with amiodarone, presented with recurrent atrial fibrillation refractory to cardioversion. Investigations revealed TSH &amp;lt;0.01, FT4 58.1 (ref 10.0-20.0 pmol/L), FT3 16.0 (ref 3.5-6.5 pmol/L), TPO negative and TRAB negative. As thyroid ultrasound revealed absence of focal nodules or hypervascularity and technetium pertechnetate scan demonstrated no thyroid activity, AIT2 was suspected. Prednisone was initiated and amiodarone discontinued. Due to minimal response after a month, a mixed type was suggested and methimazole added. Despite intensification of the antithyroid regimen, FT4 increased to 59.5. Methimazole was discontinued for radioiodine ablation assessment. Given low thyroid uptake (&amp;lt;3%), ablation was deemed unlikely beneficial. After patient refused thyroidectomy, cholestyramine was initiated, resulting in significant improvement, with FT4 declining from 46.0 to 29.0 in 2 weeks. Euthyroidism was achieved after 2 months of cholestyramine treatment and medications were stopped. At 6 months post presentation, he developed amiodarone-related hypothyroidism and began levothyroxine. Conclusion: Previous reports propose that cholestyramine, an ion exchange resin, interrupts enterohepatic circulation of thyroid hormone and demonstrated the use of cholestyramine in refractory Grave’s hyperthyroidism. This is one of the first few reports demonstrating the possible role of cholestyramine in AIT refractory to first line medical therapy. Presentation Date: Saturday, June 17, 2023

Multimodal imaging and PD-OCT analysis of an isolated focal scleral nodule in a young female

Multimodal imaging and PD-OCT analysis of an isolated focal scleral nodule in a young female

Focal Liver Lesions in Budd-Chiari Syndrome: Spectrum of Imaging Findings.

Budd-Chiari syndrome (BCS) is a rare clinical entity characterized by hepatic venous outflow obstruction, resulting in liver congestion and subsequent chronic parenchymal damage. This condition often leads to the development of focal liver lesions, including benign focal nodular hyperplasia-like regenerative nodules, hepatocellular carcinoma, and perfusion-related pseudo-lesions. Computed tomography, ultrasound, and magnetic resonance are the commonly employed imaging modalities for the follow-up of BCS patients and for the detection and characterization of new-onset lesions. The accurate differentiation between benign and malignant nodules is crucial for optimal patient management and treatment planning. However, it can be challenging due to the variable and overlapping characteristics observed. This review aims to provide a comprehensive overview of the imaging features and differential diagnosis of focal liver lesions in BCS, emphasizing the key findings and discussing the challenges associated with their interpretation, with the purpose of facilitating the subsequent clinical decision-making.

AIUM Practice Parameter for the Performance and Interpretation of Diagnostic Ultrasound of the Thyroid and Extracranial Head and Neck.

AIUM Practice Parameter for the Performance and Interpretation of Diagnostic Ultrasound of the Thyroid and Extracranial Head and Neck.

LI-RADS version 2018 for hepatocellular carcinoma < 1.0cm on gadoxetate disodium-enhanced magnetic resonance imaging.

We aimed to develop and evaluate a modified Liver Imaging Reporting and Data System (LI-RADS) version 2018 using significant ancillary features for diagnosing hepatocellular carcinoma (HCC) < 1.0cm on gadoxetate disodium-enhanced magnetic resonance imaging (MRI). Patients who underwent preoperative gadoxetate disodium-enhanced MRI for focal solid nodules < 2.0cm within 1month of MRI between January 2016 and December 2020 were retrospectively analyzed. Major and ancillary features were compared between HCCs of < 1.0cm and 1.0-1.9cm using the chi-square test. Significant ancillary features associated with HCC < 1.0cm were determined by univariable and multivariable logistic regression analysis. The sensitivity and specificity of LR-5 were compared between LI-RADS v2018 and our modified LI-RADS (applying the significant ancillary feature) using generalized estimating equations. Of 796 included nodules, 248 were < 1.0cm and 548 were 1.0-1.9cm. HCC < 1.0cm less frequently showed an enhancing capsule (7.1% vs. 31.1%, p < .001) and threshold growth (0% vs. 8.3%, p = .007) than HCC of 1.0-1.9cm. Restricted diffusion was the only ancillary feature significant for diagnosing HCC < 1.0cm (adjusted odds ratio = 11.50, p < .001). In the diagnosis of HCC, our modified LI-RADS using restricted diffusion had significantly higher sensitivity than LI-RADS v2018 (61.8% vs. 53.5%, p < .001), with similar specificity (97.3% vs. 97.8%, p = .157). Restricted diffusion was the only significant independent ancillary feature for diagnosing HCC < 1.0cm. Our modified LI-RADS using restricted diffusion can improve the sensitivity for HCC < 1.0cm. • The imaging features of hepatocellular carcinoma (HCC) < 1.0cm differed from those of HCC of 1.0-1.9cm. • Restricted diffusion was the only significant independent ancillary feature for HCC < 1.0cm. • Modified Liver Imaging Reporting and Data System (LI-RADS) with the addition of restricted diffusion can improve the sensitivity for HCC < 1.0cm.

Bilobed Focal Scleral Nodule in a Young Girl

Bilobed Focal Scleral Nodule in a Young Girl

Pathological and immunohistochemical characterization of pancreatic carcinoma in cats

The aim of this study was to characterize the pathological and immunohistochemical aspects of pancreatic carcinoma in cats, through a retrospective study. From January 2010 to December 2021, 1,908 cat necropsies were performed, in which 20 cases of exocrine pancreatic neoplasia were diagnosed (1.04%). Affected cats were mature adults and seniors, except for one 1-year-old cat. In 11 cases the neoplasm was a soft, focal nodule on the left (8/11) or right (3/11) lobe. In nine cases there were multifocal nodules throughout the pancreatic parenchyma. The size of the single masses ranged from 2 to 12 cm, and the multifocal masses from 0.5 to 2 cm. The most frequent tumour type was acinar carcinoma (11/20), followed by ductal carcinoma (8/20), undifferentiated carcinoma (1/20) and carcinosarcoma (1/20). On immunohistochemical evaluation, all the neoplasms were remarkably reactive to pancytokeratin antibody. The ductal carcinomas were strongly reactive for cytokeratins 7 and 20, which proved to be a good marker for pancreatic ductal carcinoma in cats. The main form of metastasis was abdominal carcinomatosis, with a marked invasion of blood and lymphatic vessels by neoplastic cells. Our findings reinforce the fact that pancreatic carcinoma should be rated highly in the differential diagnosis in mature adult and senior cats with abdominal masses, ascites and/or jaundice.

Radiologic Subtypes and Treatment Outcome of Unclassifiable Type Mycobacterium avium Complex Pulmonary Disease.

Although the "unclassifiable type" is categorized as one of the radiologic classifications in Mycobacterium avium complex (MAC) pulmonary disease (PD), there have been few studies of this type thus far. We aimed to investigate the radiologic subtypes and treatment outcome of unclassifiable type MAC-PD. Ninety-six patients with unclassifiable type MAC-PD who initiated a macrolide-containing regimen from 2001 to 2020 were identified at a tertiary referral center in South Korea. Among these 96 patients, 1-year culture conversion rate was analyzed for 48 patients who received standard treatment (three-drug oral-antibiotic combination with or without an injectable agent) for ≥ 1 year. The mean age of the 96 patients was 65.4 ± 10.8 years, and 72.9% of them were male. These patients were classified into four major radiologic subtypes; the most common subtype was the focal cavity subtype (n = 31, 32.3%), followed by the focal mass or nodule (n = 23, 24.0%), consolidation upon emphysema (n = 21, 21.9%), and bronchiolitis (n = 21, 21.9%) subtypes. For the 48 patients who received standard treatment for ≥ 1 year, the overall rate of culture conversion at 1-year was 93.8%. All patients in the focal cavity subtype and focal mass or nodule subtype categories achieved 1-year culture conversion. Additionally, 1-year culture conversion rate was 92.9% in consolidation upon emphysema subtype and 75.0% in bronchiolitis subtype. Unclassifiable type MAC-PD can be radiologically further categorized into four major radiologic subtypes. The treatment outcome of all of these subtypes seems to be favorable.

Recurrent primary cutaneous anaplastic large cell lymphoma in young female patient from Ethiopia: A Case Report

Primary Cutaneous Anaplastic Large Cell Lymphoma (PC-ALCL) is a CD30+ lymphoproliferative disease of the skin characterized by single or focal nodules or plaques that ulcerate over time. Diagnosis of PC-ALCL relies heavily on clinicopathological correlations because of the potential morphological, clinical, and molecular overlap with other cutaneous CD30+ LPDs. Histopathologic features include diffuse nonepidermotropic infiltrates with an adherent layer of large undifferentiated CD30+ tumor cells. The Exact incidence of PC-ALCL is not known partially because of the difficulty differentiating from the variety of CD30+ Lymphoproliferative disorders. Only a few cases are reported even from developed countries &amp; reports from developing countries are lacking. We present a possible case of primary cutaneous Anaplastic Large cell Lymphoma in a 20 years old Female patient who presented with a 2 months history of lateral neck swelling.

An attentive and adaptive 3D CNN for automatic pulmonary nodule detection in CT image

Automatic pulmonary nodule detection is the most crucial technology in early diagnosis of lung cancer and early treatment. However, the various nodule type, shape and size, especially the diameter of lung nodules (ranging from 3 mm to 30 mm), make the lung nodule detection results with high false positives. It significantly affects the detection performance of lung nodules. In this paper, an adaptive and attentive 3D Convolutional Neural Network (CNN) is proposed for automatic pulmonary nodule detection, which contains two parts: the candidate nodule detection and false positive reduction. In the first stage, the globule, spital and fine-grained information of focal nodules are scratched by the high-resolution fused attention module in the proposed method. In the second stage, an adaptive 3D CNN structure is designed to further reduce the false positives, which extracts the multilevel contextual information via an adaptive 3D convolution kernel. Extensive experiments are conducted on publicly available LUNA16. The results demonstrate that the proposed method can increase the sensitivity and decrease the false positives rate for automated pulmonary nodule detection effectively.

Simplified LI-RADS for Hepatocellular Carcinoma Diagnosis at Gadoxetic Acid-enhanced MRI.

Background Although various modifications to the Liver Imaging Reporting and Data System (LI-RADS) at gadoxetic acid-enhanced MRI have been suggested, LI-RADS shows suboptimal sensitivity for hepatocellular carcinoma (HCC) and is perceived to be too complex. Purpose To evaluate clinical usefulness of a simplified LI-RADS for diagnosing HCCs of 30 mm or smaller at gadoxetic acid-enhanced MRI. Materials and Methods Patients who underwent gadoxetic acid-enhanced MRI examination and subsequent resection, transplantation, or biopsy for focal solid nodules of 30 mm or smaller between January 2019 and December 2020 at a single tertiary referral institution were retrospectively analyzed. Two strategies for simplified LI-RADS using one size criterion (≥10 mm) were evaluated (strategy A, using classifications for nodules of 10-19 mm for nodules both 10-19 mm and ≥20 mm; strategy B, using classifications for nodules ≥20 mm for nodules both 10-19 mm and ≥20 mm). Multivariable analysis was performed to determine significant ancillary features for HCC. Generalized estimating equations were used to compare diagnostic performance for LR-5 (definite HCC) between LI-RADS version 2018 and simplified LI-RADS. The time required for LI-RADS category assignment was compared between the two systems with use of a paired t test. Results A total of 645 nodules from 510 patients (mean age ± SD, 60 years ± 10; 393 men) were evaluated. Compared with strategy A, strategy B had a higher sensitivity of 74% (347 of 470 nodules [95% CI: 70, 78]) vs 73% (342 of 470 nodules [95% CI: 69, 77]) (P = .02) with the same specificity of 96% (168 of 175 nodules [95% CI: 92, 98]) vs 96% (168 of 175 nodules [95% CI: 92, 98]) (P > .99). In strategy B, transitional phase hypointensity was an independent ancillary feature for HCC (P = .04) in LR-4 of at least 10 mm with arterial phase hyperenhancement and no other major features. In all 645 nodules, simplified LI-RADS with use of both strategy B and transitional phase hypointensity had a higher sensitivity of 82% (387 of 470 nodules [95% CI: 79, 86]) vs 73% (343 of 470 nodules [95% CI: 69, 77]) (P < .001) than LI-RADS version 2018, without lower specificity (94%, 165 of 175 nodules [95% CI: 90, 97] vs 96%, 168 of 175 nodules [95% CI: 92, 98], P = .08). Compared with LI-RADS version 2018, simplified LI-RADS reduced the time for LI-RADS category assignment (44 seconds ± 23 vs 74 seconds ± 22, P < .001). Conclusion A simplified Liver Imaging Reporting and Data System was found to be clinically useful for diagnosing hepatocellular carcinomas of 30 mm or smaller at gadoxetic acid-enhanced MRI. © RSNA, 2022 Online supplemental material is available for this article.

Clinical utility of histogram analysis of grayscale sonograms in assessing sonographic suspicious malignant thyroid nodules.

BackgroundWhen sonographic suspicious malignant thyroid nodules initially proven to be inconsistent by aspiration, repeat fine-needle aspiration cytology (FNAC) is commonly recommended. Although FNAC is simple and reliable, it is also an invasive operation with inevitable false negative results. To determine which sonographic features and histogram parameters based on gray-scale ultrasonic images need repeat aspiration.MethodsAll of the nodules were suspicious for malignancy by preoperative ultrasound (US), and their initial FNAC findings indicated Bethesda category II, or III. Histogram analyses were performed for gray-scale ultrasonic images. Mean, variance, skewness, kurtosis, percentiles (1st, 10th, 50th, 90th, 99th), and sonographic features were compared between pathological benign nodules and malignant ones. Thereafter, receiver operator characteristic (ROC) curves were analyzed for the valuable indicators.ResultsOne hundred and nineteen consecutive patients with 123 histopathologically diagnosed focal thyroid nodules were included in the cohort. The factors associated with malignancy were taller-than-wide shape [odds ratio (OR) =15.165; 95% confidence interval (CI): 3.157–72.854], irregular margins (OR =11.492; 95% CI: 1.747–75.573), microcalcifications (OR =5.107; 95% CI: 1.455–17.927) and skewness (OR =25.800; 95% CI: 1.034–76.422). The skewness of malignant thyroid nodules is higher than that of benign thyroid nodules and has an area under the curve (AUC) of 0.776 in the diagnosis.ConclusionsFor thyroid nodules suspicious on US but not on cytology, skewness, together with taller-than-wide shape, irregular margins and microcalcifications might be a promising marker for clinicians to perform repetitive FNACs.

Nodular Liver in Portosinusoidal Vascular Disease: A Case of Mistaken Identity

Background Portosinusoidal vascular disease (PSVD) has been proposed as a unifying term for vascular abnormalities of the small portal veins and sinusoids that occur in the noncirrhotic liver. This term supersedes other designations including incomplete septal cirrhosis, nodular regenerative hyperplasia, hepatoportal sclerosis, and obliterative portal venopathy. Most patients have noncirrhotic portal hypertension, but the latter is not required to establish a diagnosis of PSVD. Diagnosis requires a liver biopsy of adequate size (≥20 mm long) in the presence of accompanying clinical signs of noncirrhotic portal hypertension and/or histological changes of PSVD. We report a case of severe PSVD that caused severe liver atrophy and required transplantation for intractable hepatic encephalopathy. The explanted liver showed numerous focal nodular hyperplasia-like nodules. Case Report A 57-year-old man with neurofibromatosis type 2 and diabetes mellitus type 2 controlled with insulin developed ascites and signs of portal hypertension. He was presumed to have cirrhosis due to nonalcoholic fatty liver disease and underwent liver transplantation for intractable portal hypertension and severe hepatic encephalopathy. The explanted liver had a nodular appearance; however, histological sections showed no bridging fibrosis. Thin, incomplete curvilinear fibrous septa were seen emanating from portal tracts in a patchy distribution. Our case illustrates that PSVD may cause severe hepatic atrophy and lead to architectural abnormalities that may radiologically mimic the parenchymal nodularity of cirrhosis.

Septicaemic Listeriosis in a White-Faced Saki (Pithecia pithecia)

A 27-year-old female white-faced saki (Pithecia pithecia) died following an onset of vomiting and ptyalism. Necropsy revealed lesions of suppurative ventriculitis, choroid plexitis, periventricular encephalitis and meningitis with intralesional gram-positive coccobacilli and paired rods. The saki also had suppurative to mononuclear hepatitis, mild intestinal crypt necrosis, proliferative glomerulonephritis, aortic arteriosclerosis, pulmonary interstitial fibrosis, chronic mild epicarditis, ovarian medullary arteriopathy and a focal superficial cerebral fibrotic nodule with surrounding chronic mixed cell inflammation. Listeria monocytogenes was cultured from liver and spinal cord. Intralesional Listeria bacteria were immunolabelled in brain sections and real-time polymerase chain reaction of brain tissue detected L. monocytogenes. Whole genome multilocus sequence typing characterized the cultured bacterial isolates as sequence type 6 and clonal complex 6. A database search for related clinical and food listerial outbreaks identified genetically related isolates but, because these isolates were more than 20 alleles distant from the saki isolates, they were not a related cluster. Reports of listeriosis in non-human primates are infrequent, and when infections do occur, they tend to be haematogenous with the propensity to cause meningoencephalitis. This saki likely ingested environmental L. monocytogenes, which resulted in disease that may have been facilitated by pre-existing co-morbidities and age.

Clinical Features and Multimodal Imaging Findings of Focal Scleral Nodule: Potential Association between Lesion Characteristics and Age

To describe the clinical course and anatomic features of focal scleral nodules (FSNs) based on their clinical characteristics and multimodal imaging findings and analyze their potential associations with the patient's age. Single-center, retrospective, observational case series. Twenty-three lesions of 23 patients with FSN. Clinical characteristics were analyzed, and multimodal imaging was performed, including color fundus photography, OCT, fundus autofluorescence imaging, near-infrared imaging, B-scan ultrasonography, fluorescein and indocyanine green angiography, and OCT angiography of FSNs. Qualitative features, including the slope of anterior curvature (curvature of the dome shape is less than nodular curvature, which is less than volcanic curvature; i.e., volcanic curvature has the steepest slope), retinal pigment epithelium (RPE) status, and fluid compartments, were analyzed. Quantitative measurements, including maximum linear basal diameter, maximum horizontal length at the choroidoscleral border, maximum vertical length (Vmax) from the choroidoscleral border, and choroidal thickness, were analyzed. The mean age at presentation was 44 ± 17 years (range, 4-68 years), and the mean follow-up duration was 32.2 ± 29.5 months. On OCT, all lesions were confined to the sclera. Patients with volcanic or nodular anterior curvature were younger than those with the dome-shaped phenotype (35 ± 19 years vs. 52 ± 9 years, respectively; Mann-Whitney U test; P= 0.015). The Spearman rank correlation analysis displayed a significant correlation between age at diagnosis and Vmax (r=-0.466; P= 0.025). The Wilcoxon matched paired t test for the patients demonstrated a significant decrease in Vmax at the final follow-up visit (initial, 555 ± 228 μm vs. final, 517 ± 202 μm, respectively; P= 0.028). We observed subretinal fluid, intraretinal fluid, pigment epithelial detachment, and severe outer retinal atrophy in a relatively older population with sustained nodular or volcanic anterior curvature (age, 52 ± 7 years; range, 43-60 years). One lesion diagnosed in a patient aged 4 years demonstrated spontaneous regression. Young patients tended to have protruded lesions (high Vmax) with nodular or volcanic anterior curvature. Older patients manifested the dome-shaped phenotype, and those with sustained nodular or volcanic curvature tended to develop fluid compartments or atrophic changes of the overlying RPE and retina because of possible chronic mechanical compression. Thus, there could be a significant association between FSN phenotype and age.

Focal solitary necrotic nodules in fatty liver: characteristics on conventional and contrast-enhanced ultrasonography

PurposeFocal lesions in fatty liver are difficult to diagnose using conventional ultrasonography (CVUS). The aim of this study was to investigate the characteristics of solitary necrotic nodules (SNNs) in fatty liver using CVUS and contrast-enhanced ultrasonography (CEUS) and to evaluate the diagnostic value of CEUS for SNNs in fatty liver.MethodsFifteen SNNs in the fatty liver of fifteen patients were examined by both CVUS and CEUS. The contrast agent SonoVue was used for CEUS. The characterization and shape of these SNNs in the fatty liver were analyzed using CEUS.ResultsCVUS revealed eight oval-shaped, six irregularly shaped, and one wedge-shaped SNN in the fatty liver. The six irregularly shaped lesions on CVUS were revealed to comprise four gourd-shaped, one serpiginous, and one 3-pin socket-shaped nodule on CEUS. One of these SNNs showed a subcapsular wedge shape, with peripheral and distinct internal septal hyperenhancement in the arterial phases that washed out in the portal phase; moreover, most areas of th lesion showed no internal enhancement in any of the three phases. Fourteen of the lesions were characterized as lacking internal enhancement, and 12 of them had mild–moderate peripheral thin enhancement in the arterial phases. Twelve of the 15 nodules could be considered for diagnosis as SNNs by CEUS, which was further proved by US-guided biopsy and histopathology. However none of them could be considered for diagnosis as SNNs by CVUS.ConclusionsCEUS is a valuable tool for visualizing the characteristics of SNNs in fatty liver to improve the differential diagnosis.

Porto-Sinusoidal Vascular Disease and Focal Nodular Hyperplasia-like Nodules in a Patient with Neurofibromatosis and Non-Cirrhotic Portal Hypertension

Abstract Introduction/Objective Non-cirrhotic portal hypertension (NCPH) is uncommon. The underlying pathophysiology appears to lie at the level of intrahepatic portal veins and sinusoids, hence the term “porto-sinusoidal vascular disease” (PSVD). We report a rare case of PSVD with focal nodular hyperplasia (FNH)-like nodules in a patient with neurofibromatosis type 2 (NF2). Methods/Case Report A 57-year-old male with NF2 and type 2 diabetes, presented with a large variceal bleed requiring blood transfusion and subsequent transjugular intrahepatic portosystemic shunt (TIPS). Imaging showed a nodular liver, presumed to be cirrhosis due to non-alcoholic liver disease. Liver biopsy was not done. Thereafter, he had several episodes of hepatic encephalopathy and TIPS was downsized to prevent recurrences. The patient required liver transplantation for intractable portal hypertension and severe hepatic encephalopathy; his liver synthetic function was near normal and MELD was 11. Portal vein was patent. The explanted liver was micronodular, soft and weighed 946 grams. Unencapsulated nodules, a few mm to 1 cm in size, were present. Microscopically, there was diffuse nodularity in the absence of bridging fibrosis. Thin, incomplete curvilinear fibrous septa were present. There were aberrant veins, hypervascular portal tracts, herniated portal veins and rare occluded portal veins. Trichrome and reticulin stains confirmed architectural abnormalities including nodularity, lack of bridging fibrosis and approximation of portal tracts. Immunohistochemistry for glutamine synthetase accentuated architectural distortion and revealed nodules with FNH-like geographic areas of staining. Results (if a Case Study enter NA) NA Conclusion This is a rare case of NCPH due to PSVD in a patient with NF2. Microscopy suggested incomplete septal cirrhosis (ISC), a pattern associated with both PSVD and regression of fibrosis in a cirrhotic liver. Isolated portal hypertension without loss of synthetic function favors primary PSVD over regression of fibrosis. FNH-like nodules are consistent with regenerative changes caused by localized abnormalities of blood flow.