Analysis of the diagnostic accuracy of Belin/Ambrósio Enhanced Ectasia and Corvis ST parameters for subclinical keratoconus.

OBJECTIVE: To investigate the diagnostic accuracy of the parameters in the Belin/Ambrósio Enhanced Ectasia Display built in Pentacam, which is designed for the screening of subclinical keratoconus (SKC) built in Pentacam, and the parameters in Corneal visualization Scheimpflug technology (Corvis ST). METHODS: A retrospective study: The fellow eyes of unilateral keratoconus cases were diagnosed with SKC. Patients presented to Shanxi Eye Hospital with SKC from October 2020 to November 2021 were included as the SKC group, and myopic patients undergoing corneal refractive surgery at the Refractive Surgery Department in our hospital within the same period were included as the control group. The Belin/Ambrósio and Corvis ST parameters were extracted from the system and analyzed using independent samples t test. Receiver operating curves (ROCs) were also created to test the diagnostic accuracy of each parameter. RESULTS: There were 70 patients (70 eyes) in the SKC group and 137 patients (137 eyes) in the control group. For Corvis ST parameters, Radius (P = 0.021), PachySlope (P = 0.040), SP-A1 (P = 0.002), A2 Deformation Amp. (P = 0.028), A2 Deflection Length (P < 0.001), Max ICR (P = 0.005), DA Ratio Max (1 mm) (P = 0.023), IR (P = 0.016), CBI (P = 0.003) and TBI (P < 0.001) were statistically different between the two groups. For Belin/Ambrósio parameters, PPI min. Axis, ART min, ART max, ART avg, Pachy min, Front K2, Astig, BAD-Df, BAD-Db, BAD-Dp, BAD-Dt, BAD-Da, BAD-D, PPI min, PPI max, PPI max. Axis, PPI avg and Dist.Apex-Thin.Loc. were significantly different between the two groups (all p < 0.001). TBI and BAD-D showed the best diagnostic accuracy, with AUCs of 0.944 and 0.965, respectively. CONCLUSIONS: Some Belin/Ambrósio and Corvis ST parameters differed between SKC eyes and eyes with normal cornea. TBI and BAD-D showed the ideal diagnostic performance for SKC. In clinical practice, conventional corneal topography could not be replaced by Corvis ST.

Case Report: Bilateral Corneal Ectasia Developed during Pregnancy after Small-incision Lenticule Extraction.

SIGNIFICANCE: This case highlights that hormonal changes during pregnancy could affect the biomechanical stability of the cornea and lead to corneal ectasia during pregnancy after corneal refractive surgery. PURPOSE: We report an unusual case of bilateral corneal ectasia after small-incision lenticule extraction that developed during pregnancy. CASE REPORT: A 27-year-old woman experienced post-small-incision lenticule extraction corneal ectasia. Her pre-operative corneal topography was normal, with a minimum central corneal thickness of 538 µm in the right eye and 530 µm in the left eye. The manifest refraction was -7.75 -0.25 × 180 and -7.50 -0.75 × 10, and the lenticule thickness was 140 and 139 µm in the right and left eyes, respectively. After 11 months, in her first trimester, the patient began to experience gradually deteriorating blurred vision. Two years post-operatively, corneal ectasia was diagnosed based on topographic data. The automatic optometer examination was -7.25 -2.50 × 42 in the right eye and -11.00 -5.00 × 140 in the left eye. Later, the patient underwent corneal collagen cross-linking to control further progression and was recommended to wear rigid gas-permeable contact lenses. CONCLUSIONS: Surgeons should be alert for cornea ectasia after refractive surgery in pregnant patients, as hormonal changes during pregnancy may affect corneal biomechanical stability.

Coronary Dilatation and Endothelial Inflammation in Neonates Born to Mothers with Preeclampsia.

OBJECTIVES: To investigate the cardiovascular features and endothelium in neonates born to mothers with preeclampsia. STUDY DESIGN: In this combined observational cohort and case-control study, neonates born to mothers with normotension and mothers with preeclampsia were recruited at a neonatal intensive care unit of a tertiary medical center. Cardiovascular measurements by echocardiography and the clinical measures upon admission were analyzed. Vascular cell adhesion molecule-1 expression in umbilical arteries and in in vitro endothelial cell stimulation with plasma were examined. Continuous data were compared using nonparametric analysis, and their relationships were analyzed using linear regression. Binary logistic regression was performed in the model of adjustment of birth body weight and for multivariate analysis. RESULTS: In the cohort, almost all cardiovascular segments positively correlated to birth weight. Notably, neonates (n = 65) of mothers with preeclampsia had significantly larger coronary arteries at birth than neonates of mothers with normotension (n = 404) (median size of left main coronary artery 1.36 mm versus 1.08 mm, p <0.001; median size of right coronary artery, RCA 1.25 mm versus 1.0 mm, p <0.001). The size of the right coronary artery positively correlated to the maternal antepartum diastolic blood pressure (r = 0.298, P = .018) and was associated with in-hospital death (P < .001). Meanwhile, endothelial vascular cell adhesion molecule-1 expression was significantly increased in the umbilical arteries of the preeclamptic group and following preeclamptic cord-plasma stimulation. The latter also correlated with their relative coronary sizes. CONCLUSIONS: Neonates of mothers with preeclampsia had distinctive coronary dilatation at birth. Coronary size might be useful as a severity index of neonatal endothelial inflammation as a result of maternal preeclampsia.

Prevalence of Thoracic Aortic Aneurysms in Patients with Degenerative Abdominal Aortic Aneurysms: Results from the Prospective ACTA Study.

OBJECTIVE: There are no recommendations for screening for thoracic aortic aneurysms (TAAs), even in patients with infrarenal abdominal aortic aneurysms (AAAs). The aims of this study were to determine the prevalence of TAAs in patients with AAAs and to analyse the risk factors for this association. METHODS: This was a multicentre prospective study. The Aortic Concomitant Thoracic and Abdominal Aneurysm (ACTA) study included 331 patients with infrarenal AAAs > 40 mm between September 2012 and May 2016. These patients were prospectively enrolled in three French academic hospitals. RESULTS: Patients were classified as having a normal, aneurysmal, or ectatic (non-normal, non-aneurysmal) thoracic aorta according to their maximum aortic diameter indexed by sex, age, and body surface area. Thoracic aortic ectasia (TAE) was defined as above or equal to the 90th percentile of normal aortic diameters according to gender and body surface area. Descending TAA was defined as ≥ 150% of the mean normal value, and ascending TAA as > 47 mm in men and 42 mm in women; 7.6% (n = 25) had either an ascending (seven cases; 2.2%) or descending aortic TAA (18 cases; 5.4%), and 54.6% (n = 181) had a TAE. Among the 25 patients with TAAs, five required surgery; two patients had TAAs related to penetrating aortic ulcers < 60 mm in diameter, and three had a TAA > 60 mm. In the multinomial regression analysis, atrial fibrillation (AF) (odds ratio [OR] 11.36, 95% confidence interval [CI] 2.18 - 59.13; p = .004) and mild aortic valvulopathy (OR 2.89, 1.04-8.05; p = .042) were independent factors associated with TAAs. Age (OR 1.06, CI 1.02 - 1.09; p = .003) and AF (OR 4.36, 1.21 - 15.61; p = .024) were independently associated with ectasia. CONCLUSION: This study confirmed that TAAs coexisting with AAAs are not rare, and one fifth of these TAAs are treated surgically. Systematic screening by imaging the whole aorta in patients with AAAs is clinically relevant and should lead to an effective prevention policy.

Risk Factors, Dynamics, and Clinical Consequences of Aortic Neck Dilatation after Standard Endovascular Aneurysm Repair.

OBJECTIVE: Aortic neck dilatation (AND) occurs after endovascular aneurysm repair (EVAR) with self expanding stent grafts (SESs). Whether it continues, ultimately exceeding the endograft diameter leading to abdominal aortic aneurysm (AAA) rupture, remains uncertain. Dynamics, risk factors, and clinical relevance of AND were investigated after EVAR with standard SESs. METHODS: All intact EVAR patients treated from 2000 to 2015 at a tertiary institution were included. Demographic, anatomical, and device related characteristics were investigated as risk factors for AND. Outer to outer diameters were measured at a single standardised aortic level on reconstructed computed tomography (CT) images. RESULTS: A total of 460 patients were included (median follow up 5.2 years, interquartile range [IQR] 3.0, 7.7 years; CT imaging follow up 3.3 years, IQR 1.3, 5.4). Baseline neck diameter was 24 mm (IQR 22, 26) and increased 11.1% (IQR 1.5%, 21.9%) at last CT imaging. Endograft oversizing was 20.0% (IQR 13.6, 28.0). AND was greater during the first year (5.2% [IQR 0, 11.7]) decreasing subsequently (two to four years to 1.4%/year [IQR 0.0, 4.5%], p ≤ .001) and was associated with suprarenal fixation endografts (t value = 7.9, p < .001) and oversizing (t value = 4.4, p < .001). AND exceeding the endograft was 3.5% (95% CI 2.2% - 4.8%) and 14.4% (95% CI 11.0% - 17.8%) at five and eight years, respectively. Excessive AND was associated with baseline neck diameter (OR 1.2/mm, 95% CI 1.05 - 1.41) while the Excluder endograft had a protective effect (OR 0.15, 95% CI 0.04 - 0.58). Excessive AND was associated with type 1A endoleak (HR 3.3, 95% CI 1.1 - 9.7) and endograft migration > 5 mm (HR 3.1, 95% CI 1.4 - 6.9). CONCLUSION: AND after EVAR with SES is associated with endograft oversizing and radial force but decelerates after the first post-operative year. Baseline aortic neck diameter and suprarenal stent bearing endografts were associated with an increased risk of AND beyond nominal stent graft diameter. However, it remains unclear whether patient selection, differences in endograft radial force or the suprarenal stent are accountable for this difference.

ROLE OF DILATED SUBFOVEAL CHOROIDAL VEINS IN EYES WITH MYOPIC MACULAR NEOVASCULARIZATION.

PURPOSE: To investigate the dilated choroidal veins (DCVs) at or around myopic macular neovascularizations (MNVs) and to determine whether there is a hemodynamic relationship between them. METHODS: Fifty-eight eyes of 57 patients with myopic MNVs were examined. Dilated choroidal veins were defined as choroidal veins whose diameter was 2X larger than adjacent veins. Indocyanine green angiography and swept-source optical coherence tomography images were reviewed to detect DCVs that crossed the subfoveal area. The filling sequence of the DCVs and MNVs was determined. RESULTS: Patients' mean age was 71.4 ± 10.6 years. The mean axial length was 29.3 ± 1.8 mm. Dilated choroidal veins below or around the MNV were found in 17 eyes (29.3%). Emissaries of the short posterior ciliary arteries were seen at or around MNVs in 8 of the 17 eyes. In these eyes, the short posterior ciliary artery was filled first or almost simultaneously with the filling of the MNV, followed by a laminar filling of the DCVs. In one eye, afferent arterioles from the short posterior ciliary arteries and efferent venules connected to DCVs were seen. CONCLUSION: Dilated choroidal veins are present below or around MNVs in about 30% of eyes with myopic MNVs. Our findings suggest that an MNV might be a vascular unit consisting of short posterior ciliary arteries, afferent arterioles, efferent venules, and DCVs.

Epidemiology and Risk Factors for Giant Coronary Artery Aneurysms Identified After Acute Kawasaki Disease.

A giant coronary artery (CA) aneurysm is a potentially fatal cardiac complication resulting from Kawasaki disease (KD). We aimed to identify epidemiologic characteristics and potential risk factors associated with giant CA aneurysms identified after acute KD. We analyzed 90,252 patients diagnosed with KD from 2011 to 2018, using data obtained in nationwide KD surveys conducted in Japan. Multivariable logistic regression analyses were performed to evaluate potential risk factors associated with subsequent giant CA aneurysm complications (defined as lumen size ≥ 8 mm), adjusting for all potential factors. Giant CA aneurysms were identified in 144 patients (0.16%) after acute KD. The annual prevalence ranged from 0.07 to 0.20% during the study period. In the multivariate analyses, male sex (adjusted odds ratio 2.09 [95% confidence interval 1.41-3.11], recurrent KD (1.90 [1.09-3.33]), IVIG administration at 1-4 days of illness (1.49 [1.04-2.15]) and ≥ 8 days after KD onset (2.52 [1.38-4.60]; reference, 5-7 days), detection of CA dilatations and aneurysms at initial echocardiography (4.17 [1.85-5.41] and 46.5 [28.8-74.8], respectively), and resistance to IVIG treatment (6.09 [4.23-8.75]) were significantly associated with giant CA aneurysm complications identified after acute KD. The annual prevalence of giant CA aneurysms identified after acute KD did not increase during the study period. Patients with larger CA abnormalities detected at initial echocardiography were independently associated with progression to giant CA aneurysm complications after acute KD regardless of the number of days from onset at treatment initiation.

Differences in post-operative complications after reconstruction for congenital biliary dilatation in a single institution-Roux-en-Y hepaticojejunostomy versus hepaticoduodenostomy.

BACKGROUND: The standard surgical procedure for congenital biliary dilatation (CBD) consists of excision of extrahepatic bile duct and Roux-en-Y hepaticojejunostomy (HJ). However, alternative reconstructive operations for CBD includes hepaticoduodenostomy (HD). We compared postoperative complications and therapeutic outcomes of these two operations at our institution. METHODS: From 1981 to 2009, there were 23 traceable patients who underwent operation for CBD, They were divided into an HJ Group (n = 15) and an HD Group (n = 8). Demographic and outcome data were compared. RESULTS: There were no significant differences in postoperative complications (cholangitis, pancreatitis, and anastomotic stenosis) and current blood test data (AST, ALT, Total Bilirubin, Direct Bilirubin, Amylase) between the two groups. Current abdominal pain and carcinogenesis were not observed in either group. Intrahepatic stones occurred in one patient in each group, both > 25 years post-operation. CONCLUSION: HD is considered to be an acceptable reconstruction method compared to HJ, based on our results. There has been a suggestion that inadequate diversion in HD might increase the risk of cholangiocarcinoma. To date, in this series, that has not happened.

Marfan syndrome: Evolving organ manifestations-A 10-year follow-up study.

The age-dependent penetrance of organ manifestations in Marfan syndrome (MFS) is not known. The aims of this follow-up study were to explore how clinical features change over a 10-year period in the same Norwegian MFS cohort. In 2003-2004, we investigated 105 adults for all manifestations in the 1996 Ghent nosology. Ten years later, we performed follow-up investigations of the survivors (n = 48) who consented. Forty-six fulfilled the revised Ghent criteria. Median age: females 51 years, range 32-80 years; males 45 years, range 30-67 years. New aortic root dilatation was detected in patients up to 70 years. Ascending aortic pathology was diagnosed in 93 versus 72% at baseline. Sixty-five percent had undergone aortic surgery compared to 39% at baseline. Pulmonary trunk mean diameter had increased significantly compared to baseline. From inclusion to follow-up, two patients (three eyes) developed ectopia lentis, four developed dural ectasia, four developed scoliosis, three developed incisional or recurrent herniae, and 14 developed hindfoot deformity. No changes were found regarding protrusio acetabuli, spontaneous pneumothorax, or striae atrophicae. The study confirms that knowledge of incidence and progression of organ manifestations throughout life is important for diagnosis, treatment, and follow-up of patients with verified or suspected MFS.

Risk factors for postlaser refractive surgery corneal ectasia.

PURPOSE OF REVIEW: To summarize newly reported and update previously known risk factors for post laser vision correction (LVC) corneal ectasia. RECENT FINDINGS: Advances in corneal topography and tomography have improved detection of preclinical ectasia-related changes. This has allowed for enhanced screening of surgical candidates. There are also known genetic predispositions for corneal ectasia, and recent genetic analyses have revealed various genes and inflammatory biomolecular markers related to corneal biomechanical integrity. Other models of risk assessment such as finite element biomechanical modeling and artificial intelligence tools have shown promise in detecting more subtle risk factors, such as those related to mechanical strain. SUMMARY: Risk factors for post-LVC corneal ectasia involve both surgery and patient-specific characteristics, such as topographic and tomographic abnormalities, younger age, thinner preoperative corneas, and residual stromal beds, high myopia, and higher percentage tissue altered. Preoperative risk stratification has helped to decrease the incidence of postoperative ectasia, a trend which will likely continue with advancements in analytic tools and better understanding of underlying genetic predispositions.

Diagnosis and surgical treatment of pathologic nipple discharge using ultrasound-guided wire localization of focal ductal dilatation.

Nipple discharge is the third breast complaint after pain and lumps. The modern high-resolution ultrasound techniques are becoming more sensitive for the visualization of intraductal changes especially focal ductal dilatation (FDD), hypothesized as a radiographic manifestation of the lesion itself and that ultrasound-guided wire localization of this finding would enable identification and excision of the causative lesion. The aim of this study was to evaluate the safety, feasibility, efficiency and outcome of ultrasound-guided wire localization of FDD as possible cause of pathological nipple discharge (PND). The present study was conducted on 56 patients with PND presented to Surgical Oncology Unit at General Surgery Department, Tanta University Hospital from January 2018 to January 2019. The patients subjected to ultrasound-guided wire localization of FDD on the day of surgery, the involved duct was cannulated with a lacrimal duct probe, the targeted tissue was excised, and the specimen was sent for histopathological examination. The patients' age ranged between 26 and 71 years with a mean age of 48 years. The bloody nipple discharge was the commonest presenting symptom in 44 out of 56 patients (78.5%). The duct dilatation on study ultrasound ranged from 2.1 to 3.7 mm with a mean of 2.6 mm. Preoperative ultrasound-guided wire localization of the site of FDD was successfully performed in all cases. Papilloma alone founded in 40 out of 56 patients (71.4%), papilloma + ductal carcinoma in situ (DCIS) in six patients (10.7%), papilloma + invasive ductal carcinoma in six patients (10.7%), DCIS in two patients (3.6%) and duct ectasia in two patients (3.6%). Ultrasound-guided wire localization of FDD is an easy and safe technique for evaluation, precise localization, and targeted excision of the underlying lesions of PND.

Expert consensus and evidence-based recommendations for the assessment of flow-mediated dilation in humans.

Endothelial dysfunction is involved in the development of atherosclerosis, which precedes asymptomatic structural vascular alterations as well as clinical manifestations of cardiovascular disease (CVD). Endothelial function can be assessed non-invasively using the flow-mediated dilation (FMD) technique. Flow-mediated dilation represents an endothelium-dependent, largely nitric oxide (NO)-mediated dilatation of conduit arteries in response to an imposed increase in blood flow and shear stress. Flow-mediated dilation is affected by cardiovascular (CV) risk factors, relates to coronary artery endothelial function, and independently predicts CVD outcome. Accordingly, FMD is a tool for examining the pathophysiology of CVD and possibly identifying subjects at increased risk for future CV events. Moreover, it has merit in examining the acute and long-term impact of physiological and pharmacological interventions in humans. Despite concerns about its reproducibility, the available evidence shows that highly reliable FMD measurements can be achieved when specialized laboratories follow standardized protocols. For this purpose, updated expert consensus guidelines for the performance of FMD are presented, which are based on critical appraisal of novel technical approaches, development of analysis software, and studies exploring the physiological principles underlying the technique. Uniformity in FMD performance will (i) improve comparability between studies, (ii) contribute to construction of reference values, and (iii) offer an easy accessible and early marker of atherosclerosis that could complement clinical symptoms of structural arterial disease and facilitate early diagnosis and prediction of CVD outcomes.

Proximal aorta longitudinal strain predicts aortic root dilation rate and aortic events in Marfan syndrome.

AIMS: Life expectancy in Marfan syndrome patients has improved thanks to the early detection of aortic dilation and prophylactic aortic root surgery. Current international clinical guidelines support the use of aortic root diameter as a predictor of complications. However, other imaging markers are needed to improve risk stratification. This study aim to ascertain whether proximal aorta longitudinal and circumferential strain and distensibility assessed by cardiac magnetic resonance (CMR) predict the aortic root dilation rate and aortic events in Marfan syndrome. METHODS AND RESULTS: One hundred and seventeen Marfan patients with no previous aortic dissection, cardiac/aortic surgery, or moderate/severe aortic regurgitation were prospectively included in a multicentre protocol of clinical and imaging follow-up. At baseline, CMR was performed and proximal aorta longitudinal strain and ascending aorta circumferential strain and distensibility were obtained. During follow-up (85.7 [75.0-93.2] months), the annual growth rate of aortic root diameter was 0.62 ± 0.65 mm/year. Fifteen patients underwent elective surgical aortic root replacement and four presented aortic dissection. Once corrected for baseline clinical and demographic characteristics and aortic root diameter, proximal aorta longitudinal strain, but not circumferential strain and distensibility, was an independent predictor of the aortic root diameter growth rate (P = 0.001, P = 0.823, and P = 0.997, respectively), z-score growth rate (P = 0.013, P = 0.672, and P = 0.680, respectively), and aortic events (P = 0.023, P = 0.096, and P = 0.237, respectively). CONCLUSION: Proximal aorta longitudinal strain is independently related to the aortic root dilation rate and aortic events in addition to aortic root diameter, clinical risk factors, and demographic characteristics in Marfan syndrome patients.

Possible new endoscopic finding in patients with achalasia: "Gingko leaf sign".

BACKGROUND: The diagnosis of achalasia can occasionally be difficult because of the low prevalence of apparent endoscopic abnormal findings, such as dilation and food residue, and lack of "esophageal rosette" in some patients. We have found a new endoscopic finding "Gingko leaf sign", which consists of not being able to see the full extent of the esophageal palisade vessels and a Gingko leaf-shaped morphology of a longitudinal section of the esophagogastric junction at the end of a deep inspiration, in some achalasia patients without "esophageal rosette". The aim of the study was to investigate the prevalence of "Gingko leaf sign" in these patients. METHODS: We retrospectively compared the prevalence of "Gingko leaf sign" between 11 achalasia patients without "esophageal rosette" and 22 age-/gender-matched healthy subjects. The diagnoses of achalasia were based on the results of high-resolution manometry. We also investigated the characteristics of the patients with "Gingko leaf sign". RESULTS: All the patients had "Gingko leaf sign", in contrast to none of the healthy subjects (p < 0.001). Four of 11 patients did not require any therapy. Six of seven patients did not relapse after balloon dilatation, but one patient required per-oral endoscopic myotomy 8 months after balloon dilatation. CONCLUSION: All our achalasia patients without "esophageal rosette" had "Gingko leaf sign". It is possibly a useful endoscopic finding in achalasia patients without "esophageal rosette".

Intrahepatic cystic biliary dilatation constitutes a significant prognostic factor in patients with primary sclerosing cholangitis.

AIMS: To evaluate the prognostic value of cystic dilatation (CD) of the intrahepatic biliary ducts in patients with primary sclerosing cholangitis (PSC). METHODS: A single-center cohort of 205 patients with PSC from 2003 to 2016 was analysed. CD was defined by quantitative and qualitative criteria. Radiological and clinical courses were assessed. A Kaplan-Meier analysis was used to estimate cumulative survival without liver transplantation (LT) from the date of PSC diagnosis. A log-rank test was performed to compare survival time of PSC patients with and without CD. RESULTS: A total of 15 (7.3%) PSC patients (12 males) with a median age of 23 years at diagnosis had CD. Five patients had one CD; seven patients had two or three CDs; and three patients had diffuse CD. CDs ranged in small diameter size from 12 to 32 mm. Radiological evolution of CD was markedly variable. However, a radiological worsening of PSC over time was observed in all patients. The clinical course was characterized by the occurrence of complications in most patients. Half of the patients with CD underwent LT at a median time of 40 months from diagnosis of CD and the median survival time from PSC diagnosis was significantly lower than in PSC without CD (10.7 vs. 23.4 years; HR 3.8, 95% confidence interval: 1.7-8.3, p = 0.001). CONCLUSIONS: CD in PSC is an unusual condition that mostly affects young patients. It is characterized by a rapid, unfavorable course and constitutes a significant prognostic factor. KEY POINTS: • Cystic dilatation of the intrahepatic biliary ducts affects young patients with primary sclerosing cholangitis and is characterized by a markedly variable radiological evolution. • Biliary wall inflammation, found in explanted livers, could be a key feature in the pathogenesis of cystic dilatation. • Cystic dilatation of the intrahepatic biliary ducts is characterized by an unfavorable course and constitutes a significant prognostic factor of primary sclerosing cholangitis.

Coexistence of cervical vertebral scalloping, pedicle deficiencies and dural ectasia in type I neurofibromatosis.

Neurofibromatosis type 1 (NF-1; also known as Von Recklinghausen's disease) is a common autosomal dominant disease that occurs in the general population at the rate of 1 in 3000. Many NF-1 patients present with spinal malformations. A 54-year-old female patient was admitted to the Outpatient Clinic of Dermatology with gradually increasing swelling and spots on the body that had been present for a long period of time. Cervical vertebral scalloping, pedicle deficiencies and dural ectasia (DE) were also detected. She was diagnosed with NF-1. NF-1 is routinely seen in dermatology practice. Coexistence of NF-1 with vertebral scalloping, pedicle deficiencies and DE rarely occurs. Our case is the second reported instance in the literature of NF-1 with a spinal anomaly in the cervical region, and the first reported instance of the coexistence of NF-1 with cervical vertebral scalloping, pedicle deficiencies and DE.

Magnetic resonance cholangiogram patterns and clinical profiles of ketamine-related cholangiopathy in drug users.

BACKGROUND & AIMS: Recreational ketamine use has emerged as an important health and social issue worldwide. Although ketamine is associated with biliary tract damage, the clinical and radiological profiles of ketamine-related cholangiopathy have not been well described. METHODS: Chinese individuals who had used ketamine recreationally at least twice per month for six months in the previous two years via a territory-wide community network of charitable organizations tackling substance abuse were recruited. Magnetic resonance cholangiography (MRC) was performed, and the findings were interpreted independently by two radiologists, with the findings analysed in association with clinical characteristics. RESULTS: Among the 343 ketamine users referred, 257 (74.9%) were recruited. The mean age and ketamine exposure duration were 28.7 (±5.8) and 10.5 (±3.7) years, respectively. A total of 159 (61.9%) had biliary tract anomalies on MRC, categorized as diffuse extrahepatic dilatation (n = 73), fusiform extrahepatic dilatation (n = 64), and intrahepatic ductal changes (n = 22) with no extrahepatic involvement. Serum alkaline phosphatase (ALP) level (odds ratio [OR] 1.007; 95% CI 1.002-1.102), lack of concomitant recreational drug use (OR 1.99; 95% CI 1.11-3.58), and prior emergency attendance for urinary symptoms (OR 1.95; 95% CI 1.03-3.70) had high predictive values for biliary anomalies on MRC. Among sole ketamine users, ALP level had an AUC of 0.800 in predicting biliary anomalies, with an optimal level of ≥113 U/L having a positive predictive value of 85.4%. Cholangiographic anomalies were reversible after ketamine abstinence, whereas decompensated cirrhosis and death were possible after prolonged exposure. CONCLUSIONS: We have identified distinctive MRC patterns in a large cohort of ketamine users. ALP level and lack of concomitant drug use predicted biliary anomalies, which were reversible after abstinence. The study findings may aid public health efforts in combating the growing epidemic of ketamine abuse. LAY SUMMARY: Recreational inhalation of ketamine is currently an important substance abuse issue worldwide, and can result in anomalies of the biliary system as demonstrated by magnetic resonance imaging. Although prolonged exposure may lead to further clinical deterioration, such biliary system anomalies might be reversible after ketamine abstinence. Clinical trial number: NCT02165488.

Multiple sites of vascular dilation or aneurysmal disease and matrix metalloproteinase genetic variants in patients with abdominal aortic aneurysm.

OBJECTIVE: The objective of this study was to assess whether functional genetic polymorphisms of matrix metalloproteinases (MMPs) 1, 3, 9, and 12 are associated with arterial enlargements or aneurysms of the thoracic aorta or popliteal arteries in patients with abdominal aortic aneurysm (AAA). METHODS: The associations between MMP1 (-1607 G in/del, rs1799750), MMP3 (-1171 A in/del rs35068180), MMP9 (13-26 CA repeats around -90, rs2234681, rs917576, rs917577), and MMP12 (G/T missense variation, rs652438) polymorphisms and enlargements or aneurysms of the thoracic aorta and popliteal arteries were tested in 169 consecutive AAA patients. RESULTS: Thoracic aorta enlargement or aneurysm (TE/A; maximum diameter, >35 mm) was detected in 34 patients (20.1% prevalence). MMP9 rs2234681 microsatellite was the only genetic determinant of TE/A in AAA patients (P = .003), followed by hypercholesterolemia and antiplatelet use. Carriers of both alleles with ≥22 CA repeats had a 5.9 (95% confidence interval, 1.9-18.6; P < .0001) increased odds of TE/A, and a score considering all three variables showed 98% negative predictive value and 30% positive predictive value for thoracic aortic aneurysm detection. Eighty-two popliteal artery enlargements or aneurysms (diameter >10 mm) occurred in 55 patients (33.1% prevalence). Carriers of MMP12 rs652438 C allele showed an 18% (P = .006) increased diameter in popliteal arteries and a 2.8 (95% confidence interval, 1.3-6; P = .008) increased odds of popliteal artery enlargement or aneurysm compared with TT genotype. CONCLUSIONS: Among patients with AAA, carriers of homozygous ≥22 CA repeats in MMP9 rs12234681 and of C allele in MMP12 rs652438 have a substantial risk of carrying thoracic and popliteal enlargements, respectively.

Corneal crosslinking (CXL) with 18-mW/cm2 irradiance and 5.4-J/cm2 radiant exposure-early postoperative safety.

PURPOSE: To investigate safety of accelerated corneal crosslinking during the first postoperative month. METHODS: In this retrospective study, 76 eyes of 60 patients with verified progressive keratectasia were enrolled in this study and followed for 1 month after accelerated CXL (18 mW/cm2 for 5 min, radiant exposure 5.4 J/cm2) (A-CXL(5*18)). Preoperatively, objective refraction, slit lamp inspection, and corneal tomography were performed. Early postoperative slit lamp examinations were performed on days 1 and 4. At 1 month, objective refraction, slit lamp inspection, and corneal tomography were performed. RESULTS: Gender distribution was m:f = 55:21, OD:OS was 40:36, and the average age was 26.5 ± 8.6 years at surgery. Only 71 of the 76 eyes completed the 1-month follow-up, indicating a dropout rate of 6.6%. In 7.0% (n = 5), sterile infiltrates were observed; 5.6% of eyes (n = 4) showed delayed epithelial healing (> 4 days) in 2.8% (n = 2); an infection occurred and in 1 eye (1.4%), a stromal scar was detected; no other complications, neither a loss of two or more Snellen lines at 1 month postoperatively, were observed. As a risk factor for sterile infiltrates, thin preoperative pachymetry could be identified (p = 0.027). CONCLUSIONS: This study revealed no difference in early postoperative safety between CXL using 18 mW/cm2 and standard corneal CXL. Thinner preoperative pachymetry could be identified predicting a higher rate of postoperative sterile infiltrates.

Dilated Superior Ophthalmic Vein: Clinical and Radiographic Features of 113 Cases.

PURPOSE: Dilated superior ophthalmic vein (SOV) is an uncommon radiographic finding. The authors review the presentation, etiology, radiography, and visual implications of 113 patients with dilated SOV. METHODS: An observational case series and multicenter retrospective chart review were conducted. There were 113 patients with a dilated SOV. Outcome measures included patient demographics, clinical features, radiographic findings, diagnosis, and treatment, and treatment outcomes were assessed. RESULTS: Cases included 75 women (66%) and 38 men (34%) with a mean age of 49 ± 24 years (range, 0.4-90 years). Diagnoses fell under 6 categories: vascular malformation (n = 92, 81%), venous thrombosis (n = 11, 10%), inflammatory (n = 6, 5%), traumatic hemorrhage (n = 2, 2%), lymphoproliferative (n = 1, 1%), and infectious (n = 1, 1%). Imaging modalities utilized included MRI (n = 98, 87%), digital subtraction angiography (n = 77, 68%), CT (n = 29, 26%), and ultrasonography (n = 4, 4%). Disease status at last follow up included no evidence of disease (n = 57, 50%), alive with persistent disease (n = 53, 47%), and expired from disease (n = 3, 3%). Treatment and management was tailored to the underlying disease process with a mean follow up of 18 months (range, 1 day to 180 months). Visual impairment observed at presentation and last follow up across all cases was 26% and 22%, respectively. CONCLUSION: Dilated SOV is a rare radiographic finding resulting from a wide spectrum of etiologies with clinical implications ranging from benign to sight- and life-threatening. Dilated SOV is most often found with dural-cavernous fistula or carotid-cavernous fistula, orbital or facial arteriovenous malformation, and venous thrombosis. Recognition of this finding and management of the underlying condition is critical.