Balloon-occluded retrograde transvenous obliteration for treatment of portal hypertensive related varices.

PURPOSE OF REVIEW: Balloon-occluded retrograde transvenous obliteration (BRTO) is becoming a more commonly used procedure to manage various complications of liver disease. It is important to understand the technique of the procedure, the indications for its use, and also potential associated complications. RECENT FINDINGS: BRTO is superior to endoscopic cyanoacrylate injection and transjugular intrahepatic portosystemic shunt for bleeding gastric varices associated with a portosystemic shunt and should be considered a first line therapy in these patients. In addition, it has been shown to be useful in controlling ectopic variceal bleeding, improving portosystemic encephalopathy, and also in modulating blood flow in the post liver transplant setting. Modified versions of BRTO, plug assisted retrograde transvenous obliteration and coil assisted retrograde transvenous obliteration, have been developed to reduce procedure time and improve complication rates. SUMMARY: As the use of BRTO expands in clinical practice it will be important for gastroenterologists and hepatologists to better understand the procedure. There are still many research questions left to answer regarding the use of BRTO in specific situations and for specific patient populations.

Female gender, dissatisfaction with weight, and number of IBD related surgeries as independent risk factors for eating disorders among patients with inflammatory bowel diseases.

BACKGROUND: The prevalence and risk factors of eating disorders among patients with IBD are poorly described in existing literature. Early recognition and intervention may influence clinical outcomes in both physical and mental health. The primary aims of this study were to describe the prevalence and identify risk factors for eating disorders among patients with IBD using a validated questionnaire, the EAT-26. METHODS: The EAT-26 was administered via email as an anonymous, unpaid, online survey to 1589 patients with an electronic medical record coded diagnosis of IBD (ulcerative colitis or Crohn's disease) who had visited our Digestive Health Center in the last 3 years. Demographics and IBD characteristics were also included in our survey. A score of 20 or higher on the EAT-26 portion of the survey was considered a positive screen for eating disorder risk. RESULTS: Fifteen (4.8%) survey participants screened positively for ED risk. These 15 participants who screened positively had statistically significant differences in self-identified gender (93% female, p = 0.031), happiness with current weight (80% dissatisfied with their current weight and trying to lose weight, p < 0.01), prior eating disorder diagnosis (20%, p < 0.01), and number of IBD related surgeries (27% having 3 or more, p = 0.013). CONCLUSIONS: This study identifies independent risk factors for eating disorder risk in patients with IBD including female gender, dissatisfaction with current weight, number of IBD related surgeries, and history of prior eating disorder diagnosis.

Nonalcoholic Fatty Liver Disease Is Common in IBD Patients However Progression to Hepatic Fibrosis by Noninvasive Markers Is Rare.

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is the most common liver disorder in western countries and an increasing cause of end-stage liver disease and hepatocellular carcinoma. NAFLD is known to coexist in patients with inflammatory bowel disease (IBD). This study aims to examine the prevalence of NAFLD, as well as trends in NAFLD-associated fibrosis, in a well-characterized IBD cohort utilizing a validated noninvasive test. METHODS: We conducted a single-center retrospective chart review of patients at a large academic IBD center between 2007 and 2017. Patients with IBD and concurrent hepatic steatosis were identified. Charts were reviewed for baseline characteristics and laboratory data in order to calculate and trend NAFLD progression over time by a noninvasive marker, the NAFLD fibrosis score (NFS). RESULTS: Of 207 patients with IBD and concurrent NAFLD, NFS was able to be calculated for 138 patients at index diagnosis. A subsequent NFS was able to be calculated at 5-year follow-up for 56 patients. Over 5 years, 9 patients (16%) had worsening in NFS category, 4 patients (7%) had improvement in NFS category, and the remaining 43 patients (77%) stayed within their index NFS category. CONCLUSIONS: IBD patients with NAFLD tend to have stable liver disease over 4-6 years, and the risk of liver disease progression is low. This is the first study to document the progression of NAFLD by noninvasive testing over time.

Anterior segment ultrasound biomicroscopy image analysis using ImageJ software: Intra-observer repeatability and inter-observer agreement.

PURPOSE: In this novel study, we demonstrate a standardized imaging and measurement protocol of anterior segment (AS) structures with reliability analysis using ultrasound biomicroscopy (UBM) and ImageJ software. METHODS: Ten pediatric and young adult patients undergoing examination under anesthesia for AS pathology were imaged using UBM. Four trained observers analyzed 20 images using ImageJ. Forty-five structural parameters were measured. Those that relied on the trabecular-iris angle (TIA) as a reference landmark were labeled TIA-dependent (TD) and all others were labeled non-TIA dependent (NTD). Intra-observer repeatability (IOR) and inter-observer agreement (IOA) of measurements were determined using coefficient of variation (CV) and intra-class correlation (ICC) followed by assessment of Bland-Altman plots (BAP) for each pair of observers, respectively. RESULTS: For NTD parameters, non-ciliary body (CB) related measurements showed CV range 0.60-16.22% and ICC range 0.84-0.89, whereas CB-related parameters showed CV range 2.86-23.40% and ICC range 0.29-0.92. For TD parameters, parameters < 2 degrees removed from reference showed CV range 0.02-5.40% and ICC range 0.89-1.00, whereas parameters > 1 degree removed showed CV range 0.63-27.44% and ICC range 0.22-1.00. No systematic proportional bias was detected by BAPs. CONCLUSIONS: Preplaced landmarks yielded good IOR and IOA in quantitative assessment of AS structures that were NTD and non-CB-related or less removed from the reference. CB-related NTD measurements varied greatly in IOR and IOA, indicating protocol modifications or CB qualitative assessments needed to improve accuracy. Variability in TD measurements increased the further removed from the reference, which supports implementation of a reliable reference landmark to minimize variation.

Repeatability and Reliability of Quantified Ultrasound Biomicroscopy Image Analysis of the Ciliary Body at the Pars Plicata.

Most of the ciliary body and ciliary processes of the eye cannot be directly visualized in vivo because of the posterior location of the pars plicata to the posterior chamber and iris. However, ciliary anatomy can be effectively imaged using ultrasound biomicroscopy (UBM) by placing the probe close to the limbus, perpendicular to this structure. Previous studies measuring ciliary body parameters in meridian UBM images found that these parameters were measured with poor reliability and repeatability. This study evaluates the intra-observer reliability and inter-observer agreement of a standardized protocol for measuring six ciliary parameters in transverse or quadrant UBM images that capture an entire row of ciliary processes. All six ciliary parameters have high intra-observer reliability, with ciliary body thickness, ciliary process length and ciliary process density measurements being the most consistent for each observer. The coefficient of variation for each observer ranged from 1.4%-15%. Inter-observer agreement was also high for all six parameters, with an intra-class correlation coefficient >0.8. Utilizing transverse UBM images of the pars plicata allows for consistent quantitative analysis in control subjects.

Rapid Resolution of Vulvar Crohn's Disease With Ustekinumab.

In the evaluation of patients with inflammatory bowel disease, assessment of extraintestinal manifestations of disease is integral to clinical management. The patient described in this case is a 24-year-old woman with a history of inflammatory colonic Crohn's disease (CD) who presented to the hospital with one week of vaginal pain and swelling. Initial assessment focused on infectious etiologies, though final diagnosis of vulvar CD was made on biopsy. Clinical course and treatment of vulvar CD are poorly defined in existing literature. This case highlights the multidisciplinary management of inflammatory bowel disease patients and successful treatment of a rare extraintestinal manifestation with anti-interleukin 12/23 therapy.

An Unusual Presentation of Baker's Cyst Rupture in an Inflammatory Bowel Disease Patient.

When evaluating a patient with acute onset unilateral leg pain and concurrent inflammatory bowel disease (IBD), keeping a broad differential diagnosis will allow for prompt diagnosis and management. The patient described in this case report is a 32-year-old male with inflammatory ileocolonic Crohn's disease (CD) status after ileocecectomy with perianal involvement and known Type 1 arthropathy. He presented with a three-day history of unilateral leg swelling and tenderness. Initial evaluation focused on possible thrombosis given the development of erythema and systemic symptoms. Final diagnosis was ruptured Baker's (popliteal) cyst. This pathology is not well described in existing literature, but should be considered in IBD patients given their chronic inflammatory state and common associated intra-articular pathology.

Pediatric Corneal Structural Development During Childhood Characterized by Ultrasound Biomicroscopy.

PURPOSE: To quantitatively describe the structural corneal changes from infancy to early adulthood using ultrasound biomicroscopy. METHODS: In this prospective study, 168 ultrasound biomicroscopy images were obtained from 24 healthy eyes of 24 patients who consented and enrolled in the Pediatric Anterior Segment Imaging Innovation Study. Their ages ranged from birth to 26 years. An established ultrasound biomicroscopy imaging protocol including seven views of one eye per patient were obtained and measured using ImageJ software (National Institutes of Health). Twelve corneal structural parameters were measured. Means were compared between younger and older groups. RESULTS: Among the 12 measured structures, 5 demonstrated statistically significant differences (P < .05) between patients younger than 1 year and patients older than 1 year. The mean values for corneal cross-sectional width and length, central corneal thickness, and radii of curvature (anterior and posterior) were significantly different in patients younger than 1 year. Curvature and limbus-to-limbus dimensions changed more dramatically than thickness and tissue density. When comparing the youngest to oldest subgroups, anterior curvature flattened (6.14 to 7.55 radius), posterior curvature flattened (5.53 to 6.72 radius), angle-to-angle distance increased (8.93 to 11.40 mm), and endothelial cross-sectional distance increased (10.63 to 13.61 mm). CONCLUSIONS: Pediatric corneal structures change with age. The most significant changes occur in the first months of life, with additional changes later in childhood. This study further demonstrates the importance of age in pediatric corneal imaging analysis. [J Pediatr Ophthalmol Strabismus. 2020;57(4):238-245.].

Characterization of Mycophenolate Mofetil Gastrointestinal Toxicity and Risk Factors for Severe Disease and Poor Prognosis.

Authors performed a chart review to identify cases of mycophenolate mofetil gastrointestinal toxicity at our institution. In this cohort, friability was associated with severe disease; and nausea and erythema were associated with poor prognosis.

Dysfunctional autophagy in RPE, a contributing factor in age-related macular degeneration.

Age-related macular degeneration (AMD) is a devastating neurodegenerative disease and a major cause of blindness in the developed world. Owing to its complexity and the lack of an adequate human model that recapitulates key aspects of the disease, the molecular mechanisms of AMD pathogenesis remain poorly understood. Here we show that cultured human retinal pigment epithelium (RPE) from AMD donors (AMD RPE) are functionally impaired and exhibit distinct phenotypes compared with RPE cultured from normal donors (normal RPE). Accumulation of lipid droplets and glycogen granules, disintegration of mitochondria, and an increase in autophagosomes were observed in AMD RPE cultures. Compared with normal RPE, AMD RPE exhibit increased susceptibility to oxidative stress, produce higher levels of reactive oxygen species (ROS) under stress conditions, and showed reduced mitochondrial activity. Measurement of the ratio of LC3-II/ LC3-I, revealed impaired autophagy in AMD RPE as compared with normal RPE. Autophagic flux was also reduced in AMD RPE as compared with normal RPE, as shown by inability of AMD RPE to downregulate p62 levels during starvation. Impaired autophagic pathways were further shown by analyzing late autophagic vesicles; immunostaining with lysosome-associated membrane protein 1 (LAMP-1) antibody revealed enlarged and annular LAMP-1-positive organelles in AMD RPE as opposed to smaller discrete puncta observed in normal RPE. Our study provides insights into AMD cellular and molecular mechanisms, proposes dysfunctional autophagy as an underlying mechanism contributing to the pathophysiology of the disease, and opens up new avenues for development of novel treatment strategies.