Stability of infundibular dilatations: a single center follow-up study and systematic review of the literature.

PURPOSE: Although infundibular dilatations (IDs) have been thought to be benign anatomical variants, case reports suggest that they can grow and rupture. The aim of this study was to determine whether IDs have a tendency to grow or rupture. METHODS: The study population was collected from the Tampere University Hospital (TAUH) Aneurysm Database. The presence of IDs was screened from the medical records and imaging studies of 356 intracranial aneurysm patients left to follow-up from 2005 to 2020. The imaging studies were reviewed to confirm the IDs, and their clinical course. Finally, we performed a systematic review of published cases of ID leading to aneurysmatic rupture from PubMed. RESULTS: We found 97 typical IDs in 83 patients and 9 preaneurysmal lesions resembling ID in 9 patients. Out of the typical cone-shaped IDs, none grew or ruptured in a total follow-up of 409 patient-years. One preaneurysmal lesion ruptured during a follow-up: this lesion had components of both infundibular dilatation and aneurysm at the beginning of follow-up. In the systematic literature search, we found 20 cases of aneurysmatic SAHs originating from an ID. Of those, only 7 had imaging available prerupture. All 7 IDs were typically cone-shaped, but a branching vessel originating from the apex of ID was only seen in 4/7. CONCLUSION: Typical infundibular dilatations seem to be benign anatomical variants that are stable and, thus, do not need prophylactic treatment or imaging follow-up. Likely, the SAHs reported from IDs were actually caused by misdiagnosed preaneurysmal lesions.

Working towards risk stratification for ascending aortic dilatation in pediatric Turner syndrome patients: results of a longitudinal echocardiographical observation.

This study aimed to longitudinally evaluate aortic root dimensions and elasticity in pediatric Turner syndrome (TS) in relation to known cardiac implications such as coarctation of the aorta (CoA) and bicuspid aortic valves (BAV) in order to create an improved risk profile for the presumed underlying vessel pathology in childhood. We report on the longitudinal findings of our pediatric TS outpatient clinic over a period of up to 7.6 years. Forty-nine TS patients (median age at baseline 9.7 ± 5.9 years, range 0-19.8) were followed-up for on average 2.9 ± 1.1 examinations and a median time of 3.4 ± 1.6 years. Aortic root (AoR) diameters and corresponding Z-scores were determined echocardiographically, and elasticity parameters as well as annual progression rates were calculated. At baseline, 16.3% of patients showed Z-scores > 2 at one or more levels of the AoR (35.7% of patients with BAV, odds ratio of 4.2). There was net progression to be noted at all measuring levels, leading to 28.6% of patients (50% of patients with BAV) exhibiting aortic dilatation at the end of follow-up. Progression correlated with the presence of BAV, non-mosaic monosomy, and age. A levelling-off of progression was seen with the onset of adolescence. CONCLUSIONS: Marked progression of aortic diameters leading to the development of dilatation can be observed in TS patients during childhood and stresses the importance of close surveillance during childhood. Main risk factors are BAV and complete monosomy 45X0. A beneficial influence of estrogen substitution can be suspected but needs further investigation. WHAT IS KNOWN: • Patients with Turner syndrome are at an increased risk for aortic dilatation and dissection. • The presence of BAV and complete monosomy 45X are additional risk factors. WHAT IS NEW: • Aortic dilatation can be detected in pediatric patients with Turner syndrome. • Relevant progression in childhood is possible in at-risk individuals and warrants close surveillance.

A case of congenital biliary dilatation without pancreaticobiliary maljunction, so-called Type Ib according to Todani's classification.

Congenital biliary dilatation (CBD) is a congenital malformation of focal dilatation of the extrahepatic bile ducts, including the common bile duct, and is often associated with pancreaticobiliary maljunction (PBM). In this article, we report a CBD case that presented with focal dilation of the common bile duct without PBM (Todani's classification type Ib). The patient was a 32-year-old man who visited a doctor with a chief complaint of abdominal distension. Computed tomography revealed cystic dilatation of the common bile duct, and the patient was referred to our institution. Magnetic resonance cholangiopancreatography showed cystic dilatation of the common bile duct with a maximum diameter of 7 cm; however, evaluating the presence of PBM was challenging. Endoscopic ultrasonography showed small gallstones and debris in the dilated common bile duct and no thickening of the gallbladder wall. Endoscopic retrograde cholangiopancreatography revealed no PBM or markedly elevated bile amylase levels. Based on these findings, the patient was diagnosed with Todani Type Ib CBD. Since this patient did not have pancreatobiliary reflux, it was unclear whether the risk of developing biliary tract cancer was high, and since the treatment was highly invasive, the decision was to follow up without surgical treatment.

Prevalence and Angiographic Characteristics of Coronary Artery Ectasia among Patients with Coronary Artery Disease: A Retrospective Analysis between 2014 and 2022.

Coronary artery ectasia (CAE) is defined as segmental dilatation with a diameter of 1.5-fold greater than that of an adjacent normal segment. Whether CAE is a unique clinical finding or results from other clinical entities remains to be determined. The purpose of the study was to investigate the prevalence, and clinical and angiographic characteristics of CAE in patients with coronary artery disease (CAD). Among the 8,845 coronary angiograms reviewed between the years 2014 and 2022, 142 patients had CAE yielding a detection rate of 4.9% among 2,870 CAD angiograms, and 28 patients had isolated CAE showing a detection rate of 0.32% (28/8,845) among total coronary angiography procedures. Overall, the incidence of CAE was 1.92% (170/8,845). The most commonly affected coronary artery by ectasia was the right coronary artery (RCA) (46.28%) among CAE coexisting with CAD cohort. The proportion of obesity, family history of CAD, and the proportion of hyperlipidemia in CAD patients who had ectasia were significantly higher than that in CAD patients who did not have ectasia (P < 0.05). In conclusion, CAE is an uncommon finding in coronary angiography, most commonly affecting the RCA. The obesity, family history of CAD, and the coexistence of hyperlipidemia were independent variables associated with CAE in CAD patients.

Bilateral internal jugular vein ectasia: A rare cause of neck swelling.

Internal jugular vein ectasia or phlebectasia is a condition in which there is an isolated fusiform dilatation of the internal jugular vein. The patient usually presents with swelling in the neck, which aggravates in size while coughing or straining. This is a rare condition and is often mis-diagnosed. It can be diagnosed by proper history, clinical examination and imaging. We report a 5-year-old boy who had bilateral internal jugular vein ectasia aggravating in size while straining and coughing. Ultrasonography and computed tomography scan showed dilatation of internal jugular veins on both sides. Since the patient was asymptomatic and had no complications, he was advised regular follow-up. This rare benign condition should be kept in mind as a differential diagnosis of an expansile neck mass.

Ascending thoracic aortic aneurysm elongation occurs in parallel with dilatation in a nonsurgical population.

OBJECTIVES: Rapid diameter growth is a criterion for ascending thoracic aortic aneurysm repair; however, there are sparse data on aneurysm elongation rate. The purpose of this study was to assess aortic elongation rates in nonsyndromic, nonsurgical aneurysms to understand length dynamics and correlate with aortic diameter over time. METHODS: Patients with <5.5-cm aneurysms and computed tomography angiography imaging at baseline and 3-5 years follow-up underwent patient-specific three-dimensional aneurysm reconstruction using MeVisLab. Aortic length was measured along the vessel centreline between the annulus and aortic arch. Maximum aneurysm diameter was determined from imaging in a plane normal to the vessel centreline. Average rates of aneurysm growth were evaluated using the longest available follow-up. RESULTS: Over the follow-up period, the mean aortic length for 67 identified patients increased from 118.2 (95% confidence interval: 115.4-121.1) mm to 120.2 (117.3-123.0) mm (P = 0.02) and 15 patients (22%) experienced a change in length of ≥5% from baseline. The mean annual growth rate for length [0.38 (95% confidence interval: 0.11-0.65) mm/year] was correlated with annual growth rate for diameter [0.1 (0.03-0.2) mm/year] (rho = 0.30, P = 0.01). Additionally, annual percentage change in length [0.3 (0.1-0.5)%/year] was similar to percentage change in diameter [0.2 (0.007-0.4)%/year, P = 0.95]. CONCLUSIONS: Aortic length increases in parallel with aortic diameter at a similar percentage rate. Further work is needed to identify whether elongation rate is associated with dissection risk. Such studies may provide insight into why patients with aortic diameters smaller than surgical guidelines continue to experience dissection events.

Value of aortic volumes assessed by automated segmentation of 3D MRI data in patients with thoracic aortic dilatation: A case-control study.

PURPOSE: The purpose of this study was to investigate the benefit of aortic volumes compared to diameters or cross-sectional areas on three-dimensional (3D) magnetic resonance imaging (MRI) in discriminating between patients with dilated aorta and matched controls. MATERIALS AND METHODS: Sixty-two patients (47 men and 15 women; median age, 66 years; age range: 33-86 years) with tricuspid aortic valve and ascending thoracic aorta aneurysm (TAV-ATAA) and 43 patients (35 men and 8 women; median age, 51 years; age range: 17-76 years) with bicuspid aortic valve and dilated ascending aorta (BAV) were studied. One group of 54 controls matched for age and sex to patients with TAV-ATAA (39 men and 15 women; median age, 68 years; age range: 33-81 years) and one group of 42 controls matched for age and sex to patients with BAV (34 men and 8 women; median age, 50 years; age range: 17-77 years) were identified. All participants underwent 3D MRI, used for 3D-segmentation for measuring aortic length, maximal diameter, maximal cross-sectional area (CSA) and volume for the ascending aorta. RESULTS: An increase in ascending aorta volume (TAV-ATAA: +107%; BAV: +171% vs. controls; P < 0.001) was found, which was three times greater than the increase in diameter (TAV-ATAA: +29%; BAV: +40% vs. controls; P < 0.001). In differentiating patients with TAV-ATAA from their controls, the indexed ascending aorta volume showed better performances (AUC, 0.935 [95% confidence interval (CI): 0.882-0.989]; accuracy, 88.7% [95% CI: 82.9-94.5]) than indexed ascending aorta length (P < 0.001), indexed ascending aorta maximal diameter (P = 0.003) and indexed ascending aorta maximal CSA (P = 0.03). In differentiating patients with BAV from matched controls, indexed ascending aorta volume showed significantly better performances performance (AUC, 0.908 [95% CI: 0.829-0.987]; accuracy, 88.0% [95% CI: 80.9-95.0]) than indexed ascending aorta length (P = 0.02) and not different from indexed ascending aorta maximal diameter (P = 0.07) or from indexed ascending aorta maximal CSA (P = 0.27) CONCLUSION: Aortic volume measured by 3D-MRI integrates both elongation and luminal dilatation, resulting in greater classification performance than maximal diameter and length in differentiating patients with dilated ascending aorta or aneurysm from controls.

Congenital segmental dilatation of the colon in a newborn: a radiologic perspective of an unusual entity.

A newborn with congenital segmental dilatation of the intestine affecting the colon is presented. This rare condition, unrelated to Hirschsprung's disease, may affect any portion of the bowel and is characterized by focal dilatation of a segment of bowel flanked by normal proximal and distal bowel. While reported in the surgical literature, congenital segmental dilatation of the intestine has not been reported in the pediatric radiology literature even though pediatric radiologists may be the first to encounter imaging suggesting the diagnosis. We therefore present the characteristic imaging findings, including abdominal radiographs and images from a contrast enema, and discuss the clinical presentation, pathology findings, associations, treatment, and prognosis of congenital segmental dilatation of the intestine to increase awareness of this unusual diagnosis.

Custom anterior segment optical coherence tomography indices for detection of corneal ectasia.

INTRODUCTION: Corneal thinning and changes in the corneal thickness profile are major symptoms of corneal ectasia. The anterior seg-ment optical coherence tomography is currently widely used, and the development of additional indices may lead to improvements in the diagnostics of keratoconus. AIM: To determine the diagnostic value of newly developed custom anterior segment OCT indices in diagnosing corneal ectasia. PATIENTS AND METHODS: Two sets of patients were included in the current study - healthy controls in the first and patients with corneal ectasia in the second, 80 eyes per group of 43 patients each. The groups were age- and sex-matched. Each patient underwent a standard ophthalmological examination (visual acuity, tonometry, slit lamp examination, fundus biomicroscopy), a corneal topography with OCULUS Keratograph 5M, and an anterior segment optical coherence tomography with RTVue-100. Besides the indices automatically generated by the software of the device, we measured the following custom parameters: partial corneal area (PCA), partial chamber area (PCA), and an index that reflects the relation between the two (CpC). All measurements were performed in two axial pachymetric scans, one vertical and one horizontal using the built-in software. RESULTS: A statistically significant difference was found between the two groups (p<0.001, confidence Interval 95%) for all the proposed indices in both the vertical and the horizontal scans. The ROC analysis showed promising results for differentiation between the groups with the area under the curve (AUC) in the range from 0.892 for the vertical partial anterior chamber area to 0.984 for the vertical CpC index. CONCLUSIONS: The proposed indices can be used to differentiate between normal and ectatic corneas.

Fetal intestinal loop dilatation: Follow-up and outcome of a series of 133 consecutive cases (the DILDIG study).

OBJECTIVES: To define the prognostic markers of fetal dilated bowel loops. METHODS: National non-interventional study of 133 consecutive prenatal observations of dilated loops including ultrasound examinations, complementary laboratory tests, magnetic resonance imaging (MRI), outcomes, and postnatal diagnosis. RESULTS: One hundred twenty seven cases were classified according to outcome: Group 1, very severe (n = 43), Group 2, children needing specific care (n = 39), and Group 3, healthy children (n = 45). Prenatal ultrasound scan suggested duodenal obstruction in 30 cases, small bowel obstruction in 81, colonic obstruction in 11, and diffuse dilatation in 5. Diameter of dilated loops did not significantly differ between the groups. A poor prognosis was significantly associated with duodenal obstruction, genetic anomalies (53% vs. 21.8%), including aneuploidies or CFTR gene mutations and abnormal amniotic fluid biochemistry (86.4% vs. 38.7%). A good prognosis was associated with regression of dilatation and normal MRI. CONCLUSION: In this study, postnatal outcomes for fetuses with intestinal dilatation were best predicted by assessing the level of obstruction with prenatal ultrasound and MRI, determining the presence of associated malformations, amniotic fluid biochemical and genetic testing, and monitoring for regression of bowel dilatation. These results should help inform future guidelines on the prenatal and neonatal management of congenital intestinal obstruction.

Main pancreatic duct dilatation and pancreatic cysts in relatives and spouses of patients with pancreatic cancer.

Although main pancreatic duct dilatation and pancreatic cysts are risk factors for developing pancreatic cancer, limited data exist regarding these findings in relatives and spouses of pancreatic cancer patients. The frequency of these findings was examined using long-term follow-up data and transabdominal ultrasonography focusing on the pancreas. We prospectively enrolled 184 relatives and spouses of pancreatic cancer patients and performed special pancreatic ultrasonography to detect main pancreatic duct dilatation and pancreatic cysts. First-degree relatives (148 participants) of patients with pancreatic cancer were significantly younger than the spouses (36 participants; 41 vs. 65 years old). The frequency of ultrasonographic findings was significantly different between the relative (8.8%) and spouse (33.3%) groups. Main pancreatic duct dilatation and pancreatic cysts were observed in seven (4.7%) and seven (4.7%) participants in the relative group, and in nine (25.0%) and five (13.9%) participants in the spouse group, respectively. On multivariate analysis, age was an independent risk factor for the ultrasonographic findings. The frequency of ultrasonographic findings was significantly higher in spouses than in first-degree relatives of patients with pancreatic cancer and was strongly influenced by the age gap between the groups. Main pancreatic duct dilatation was frequently observed, especially in the spouse group.

Systemic Immune Inflammation Index is a Novel Marker in Predicting the Presence and Severity of Isolated Coronary Artery Ectasia. / O Índice de Inflamação Imune Sistêmica é um Novo Marcador na Previsão da Presença e Gravidade aa Ectasia Coronariana Isolada.

BACKGROUND: The underlying pathology of isolated coronary artery ectasia (CE) has not been fully elucidated. OBJECTIVE: We aimed to examine the relationship between the systemic immune inflammation index (Sii), which corresponds to the multiplying of the neutrophil-to-lymphocyte ratio (NLR) and the platelet counts, and isolated CE. METHOD: The retrospective study population included 200 patients with isolated CE, 200 consecutive with obstructive coronary artery disease, and 200 consecutive with a normal coronary artery angiogram. A 2-sided p-value of <0.05 was considered significant. RESULTS: Sii, NLR, platelet-to-lymphocyte ratio (PLR), and monocyte-to-high density lipoprotein cholesterol ratio (MHR) were significantly higher in the CE group compared with the other groups (all p<0.001). In multivariate analysis, Sii (p<0.001, OR = 1.005, 95% CI =1.004-1.005) was found to be an independent predictor of isolated CE. In Receiver Operating Characteristic curve analysis, Sii had a higher Area Under the Curve than NLR, PLR, and MHR. Sii value of >517.35 has 79% sensitivity, 76% specificity for the prediction of the CE [AUC: 0.832, (p<0.001)]. Sii had a significant correlation with the number of ectatic coronary arteries and Markis classification (r:0.214 p=0.002; r:-0.195, p=0.006, respectively). CONCLUSION: To the best of our knowledge, this is the first study that Sii was significantly associated with isolated CE presence and anatomical severity.

FUNDAMENTO: A patologia subjacente da ectasia da artéria coronária (EC) isolada não foi totalmente elucidada. OBJETIVO: Nosso objetivo foi examinar a relação entre o índice de inflamação imune sistêmica (Sii), que corresponde à multiplicação da razão neutrófilos-linfócitos (RNL) e as contagens de plaquetas, e EC isolada. MÉTODO: A população do estudo retrospectivo incluiu 200 pacientes com EC isolada, 200 consecutivos com doença arterial coronariana obstrutiva e 200 consecutivos com angiografia coronária normal. Um valor de p bicaudal <0,05 foi considerado significativo. RESULTADOS: Sii, RNL, razão plaqueta-linfócito (RPL) e razão monócito-colesterol de lipoproteína de alta densidade (MHR) foram significativamente maiores no grupo EC em comparação com os outros grupos (todos p<0,001). Na análise multivariada, Sii (p<0,001, OR = 1,005, IC 95% =1,004-1,005) foi considerado um preditor independente de EC isolada. Na análise da curva Receiver Operating Characteristic (ROC), Sii teve uma área sob a curva maior em comparação com RNL, RPL e MHR. O valor de Sii >517,35 tem 79% de sensibilidade, 76% de especificidade para a predição do EC [AUC: 0,832, (p<0,001)]. Sii teve correlação significativa com o número de artérias coronárias ectásicas e classificação de Markis (r: 0,214 p=0,002; r:-0,195, p=0,006, respectivamente). CONCLUSÃO: Até onde sabemos, este é o primeiro estudo em que Sii foi significativamente associado à presença isolada de EC e gravidade anatômica.

Clinical Predictors of Coronary Artery Ectasia.

Coronary artery ectasia (CAE) is defined as abnormal dilation of a coronary artery ≥1.5 times the normal segment. We aimed to determine the prevalence and clinical predictors of CAE. This was a prospective analysis performed on 6465 patients undergoing coronary angiography. Patients were divided based on the presence or absence of CAE and compared for angiographic characteristics and clinical risk factors. The prevalence of CAE was 7%, CAE associated with coronary artery stenosis was 5.4%, and isolated CAE was 1.6%. The mean age of presentation in CAE patients was 60 years, with male predominance (83.8%) and stable angina was the most common presentation. The left anterior descending artery (LAD) (51.7%) was the most commonly involved vessel, with diffuse ectasia more commonly seen in right coronary artery and discrete ectasia in LAD. Type 4 CAE was the most common type (92.4%). Hypertension, diabetes, smoking, dyslipidemia, and obesity were found in 62.4%, 35.3%, 45.3%, 54.9%, and 23.3%, respectively in CAE patients, with significant association with smoking (Odds Ratio = 3.06). The prevalence of CAE was 7% and was frequently associated with atherosclerotic coronary disease. Smoking was a significant predisposing factor for CAE.