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Cornea Oct 2023Customized photorefractive keratectomy with minimal tissue ablation combined with corneal cross-linking seems to be a long-term safe and effective strategy for...
Customized photorefractive keratectomy with minimal tissue ablation combined with corneal cross-linking seems to be a long-term safe and effective strategy for anatomical and visual management of keratoconus, postsurgical ectasia, and other ectasia management. Multiple published studies, many with long-term follow-up, have supported the Athens Protocol and its various forms as a means to manage corneal ectatic disorders, which not only stabilize corneal shapes but also improve functional vision.
Topics: Humans; Keratoconus; Photorefractive Keratectomy; Corneal Cross-Linking; Dilatation, Pathologic
PubMed: 37669421
DOI: 10.1097/ICO.0000000000003320 -
Circulation Jun 2024Ascending aorta dilation and aortic valve degeneration are common complications in patients with bicuspid aortic valve. Several retrospective studies have suggested the... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Ascending aorta dilation and aortic valve degeneration are common complications in patients with bicuspid aortic valve. Several retrospective studies have suggested the benefit of statins in reducing these complications. This study aimed to determine whether atorvastatin treatment is effective in reducing the growth of aortic diameters in bicuspid aortic valve and if it slows the progression of valve calcification.
METHODS
In a randomized clinical trial, 220 patients with bicuspid aortic valve (43 women; 46±13 years of age) were included and treated with either 20 mg of atorvastatin per day or placebo for 3 years. Inclusion criteria were ≥18 years of age, nonsevere valvular dysfunction, nonsevere valve calcification, and ascending aorta diameter ≤50 mm. Computed tomography and echocardiography studies were performed at baseline and after 3 years of treatment.
RESULTS
During follow-up, 28 patients (12.7%) discontinued medical treatment (15 on atorvastatin and 13 taking placebo). Thus, 192 patients completed the 36 months of treatment. Low-density lipoprotein cholesterol levels decreased significantly in the atorvastatin group (median [interquartile range], -30 mg/dL [-51.65 to -1.75 mg/dL] versus 6 mg/dL [-4, 22.5 mg/dL]; <0.001). The maximum ascending aorta diameter increased with no differences between groups: 0.65 mm (95% CI, 0.45-0.85) in the atorvastatin group and 0.74 mm (95% CI, 0.45-1.04) in the placebo group (=0.613). Similarly, no significant differences were found for the progression of the aortic valve calcium score (=0.167) or valvular dysfunction.
CONCLUSIONS
Among patients with bicuspid aortic valve without severe valvular dysfunction, atorvastatin treatment was not effective in reducing the progression of ascending aorta dilation and aortic valve calcification during 3 years of treatment despite a significant reduction in low-density lipoprotein cholesterol levels.
REGISTRATION
URL: https://www.clinicaltrialsregister.eu; Unique identifier: 2015-001808-57. URL: https://www.clinicaltrials.gov; Unique identifier: NCT02679261.
Topics: Humans; Atorvastatin; Female; Male; Middle Aged; Aortic Valve; Disease Progression; Calcinosis; Bicuspid Aortic Valve Disease; Heart Valve Diseases; Adult; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Dilatation, Pathologic; Follow-Up Studies; Double-Blind Method; Treatment Outcome; Aorta; Aortic Valve Disease; Aortic Valve Stenosis
PubMed: 38804148
DOI: 10.1161/CIRCULATIONAHA.123.067537 -
Pediatric Surgery International Mar 2024Congenital esophageal stenosis (CES) associated with esophageal atresia (EA) is rare, and no standard treatment has been established. We reviewed cases of EA-associated... (Review)
Review
PURPOSE
Congenital esophageal stenosis (CES) associated with esophageal atresia (EA) is rare, and no standard treatment has been established. We reviewed cases of EA-associated CES to assess the clinical characteristics and treatment outcomes, especially the feasibility of endoscopic dilatation.
METHODS
We retrospectively examined patients with EA-associated CES. We also compared treatment outcomes of EA-associated CES with those of EA patients without CES who developed postoperative anastomotic stricture.
RESULTS
Among 44 patients with EA, ten had CES (23%). Postoperative complications were not significantly different between EA patients with CES and those without CES but with anastomotic stricture. All CES patients underwent balloon dilatation as initial treatment. Eight of nine patients (89%) were successfully treated by dilatation only, and one patient underwent surgical resection. The median number of balloon dilatations for CES was five (2-17), which was higher than that for anastomotic stricture in patients without CES (p = 0.012). Esophageal perforation occurred in five patients with CES (5/9, 56%) after dilatation, but all perforations were successfully managed conservatively with an uneventful post-dilatation course.
CONCLUSIONS
Twenty-three percent of patients with EA had CES. Although balloon dilatation for EA-associated CES required multiple treatments and carried a risk of perforation, balloon dilatation showed an 89% success rate and all perforations could be managed conservatively.
Topics: Humans; Esophageal Atresia; Esophageal Stenosis; Dilatation; Retrospective Studies; Constriction, Pathologic; Treatment Outcome; Postoperative Complications; Anastomosis, Surgical
PubMed: 38517524
DOI: 10.1007/s00383-024-05652-w -
Indian Journal of Gastroenterology :... Feb 2024Up to 50% of patients with Crohn's disease develop a stricture within 10 years of diagnosis. Crohn's strictures can compose of inflammation, fibrosis or smooth muscle... (Review)
Review
Up to 50% of patients with Crohn's disease develop a stricture within 10 years of diagnosis. Crohn's strictures can compose of inflammation, fibrosis or smooth muscle expansion and usually a combination of these. There have been numerous new developments in imaging modalities in determining the composition of Crohn's strictures. Magnetic resonance imaging remains the best upfront imaging modality to characterize Crohn's strictures. Gastrointestinal ultrasound (GIUS) has an increasing role in clinical practice, particularly for monitoring stricture response as a treat-to-target tool. Novel imaging techniques to differentiate between fibrosis and inflammatory strictures have been developed including contrast-enhanced GIUS, strain or shear wave elastography with GIUS and multiple new magnetic resonance imaging (MRI) protocols, including diffusion weighted, delayed contrast enhancement and magnetization transfer MR protocols. However, these techniques require further validation and standardization. Regarding therapeutics, anti-tumor necrosis agents with a treat-to-target strategy have the highest quality evidence in treating strictures and can lead to stricture regression in some cases. Endoscopic balloon dilatation remains a mainstay in the treatment algorithm of treating predominantly fibrostenotic Crohn's strictures, particularly those which are symptomatic, < 5 cm in length and not causing prestenotic dilatation. Endoscopic balloon dilatation has greater effectiveness in anastomotic strictures. Surgery remains an important treatment option in Crohn's strictures, with segmental resection and stricturoplasty having their own advantages and disadvantages. Kono-S anastomosis may be superior to conventional anastomosis for endoscopic recurrence; however, further high-quality studies are required to confirm this. Using risk stratification models such as the BACARDI risk model is important to guide management decisions between a medical and surgical approach. Early post-operative medical prophylaxis with an advanced therapy is an important consideration to prevent disease recurrence. This review expands on the above topics, highlights research gaps and provides a suggested investigation and management pathway in stricturing Crohn's disease.
Topics: Humans; Crohn Disease; Constriction, Pathologic; Endoscopy; Fibrosis; Magnetic Resonance Imaging; Treatment Outcome; Dilatation
PubMed: 38277070
DOI: 10.1007/s12664-023-01508-8 -
Prenatal Diagnosis Feb 2024Urinary tract dilation (UTD), which refers to the abnormal dilation of the urinary collection system, is the most common finding on prenatal ultrasound and presents with... (Review)
Review
Urinary tract dilation (UTD), which refers to the abnormal dilation of the urinary collection system, is the most common finding on prenatal ultrasound and presents with varying severity, presentation, etiology, and prognosis. Prenatal classification and risk stratification aim to prevent postnatal complications, such as urinary tract infections and further kidney dysfunction. Parents expecting a child with UTD should be counseled by a multidisciplinary team consisting of maternal-fetal medicine specialists, and pediatric urology and nephrology providers. This review summarizes the key points in the diagnostic evaluation and management during the prenatal and initial postnatal period, focusing on the information that should be provided to future parents. We address frequently asked parental questions and concerns that our multidisciplinary clinical practice faces.
Topics: Pregnancy; Female; Child; Humans; Dilatation; Urinary Tract; Dilatation, Pathologic; Urinary Tract Infections; Parents; Hydronephrosis; Ultrasonography, Prenatal
PubMed: 38117007
DOI: 10.1002/pd.6497 -
Graefe's Archive For Clinical and... Sep 2023Osteogenesis imperfecta (OI) is a rare inherited disease affecting collagen-rich tissues. Ocular complications have been reported such as thin corneas, low ocular...
PURPOSE
Osteogenesis imperfecta (OI) is a rare inherited disease affecting collagen-rich tissues. Ocular complications have been reported such as thin corneas, low ocular rigidity, keratoconus, among others. The purpose of this study is to characterize corneal tomographic features in OI patients compared to unaffected patients, with particular focus on commonly studied keratoconus indices.
METHODS
Cross-sectional case-control study including 37 OI patients and 37 age-matched controls. Patients and controls underwent comprehensive ophthalmological examination including corneal Scheimpflug tomography with a Pentacam HR device (Oculus Optikgeräte GmbH, Wetzlar, Germany) to analyse and compare topometric, tomographic, pachymetric and Belin-Ambrósio Enhanced Ectasia Display III (BAD-D) data of both eyes of each patient.
RESULTS
Most OI patients had type I disease (n = 24; 65%) but type III-VII patients were also included. Two patients had clinically overt bilateral keratoconus. OI patients had significantly higher maximum keratometry (45.2 ± 2.1 vs. 43.7 ± 1.2; p = 0.0416), front and back elevation (3.0 ± 3.3 vs. 2.1 ± 1.3, p = 0.0201; 11.1 ± 8.2 vs. 5.0 ± 3.7, p < 0.0001), index of surface variance (25.5 ± 13 vs. 17.4 ± 8.3; p = 0.0016), index of vertical asymmetry (0.21 ± 0.14 vs. 0.15 ± 0.06; p = 0.0215), index of height asymmetry (9.2 ± 14 vs. 6.0 ± 4.5; p = 0.0421), index of height decentration (0.02 ± 0.01 vs. 0.01 ± 0.01; p < 0.0001) and average pachymetric progression (1.01 ± 0.19 vs. 0.88 ± 0.14; p < 0.0001) readings. Thinnest corneal thickness and maximum Ambrósio relational thickness were significantly lower (477 ± 52 vs. 543 ± 26; 387 ± 95 vs. 509 ± 49; p < 0.0001). Two-thirds of OI patients had corneas with a minimum thickness < 500 µm. BAD-D value was significantly higher in OI patients (2.1 ± 1.4 vs. 0.9 ± 0.2; p < 0.0001).
CONCLUSION
OI patients showed significant changes in corneal profiles compared with healthy subjects. A high proportion of patients had tomographically suspect corneas when using keratoconus diagnostic indices. Further studies are warranted to assess the true risk of corneal ectasia in OI patients.
Topics: Humans; Keratoconus; Corneal Topography; Case-Control Studies; Corneal Pachymetry; Osteogenesis Imperfecta; Dilatation, Pathologic; Cross-Sectional Studies; ROC Curve; Cornea; Tomography; Retrospective Studies
PubMed: 37074408
DOI: 10.1007/s00417-023-06059-4 -
European Archives of... Nov 2023This study aimed to assess the changes in spirometry parameters or indices after relieving laryngotracheal stenosis (LTS) in adult patients. (Meta-Analysis)
Meta-Analysis Review
PURPOSE
This study aimed to assess the changes in spirometry parameters or indices after relieving laryngotracheal stenosis (LTS) in adult patients.
METHODS
A systematic review and meta-analysis of studies from PubMed, Scopus, Web of Science, Cochrane Library, and EBSCO databases was conducted for assessing changes in spirometry values after endoscopic balloon dilatation of LTS in adults. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed. Relevant data, such as changes in mean spirometry values between preoperative and postoperative interventions, and findings of receiver operating characteristic curve analyses for predicting the need for surgical intervention, were extracted.
RESULTS
Ten studies including 330 patients overall met the inclusion criteria. Significant improvements were observed from preoperative to postoperative mean values of different spirometry parameters and indices. The overall mean differences in peak expiratory flow (ΔPEF), expiratory disproportion index (ΔEDI), and peak inspiratory flow (ΔPIF) were 2.26 L/s (95% CI 2.14-2.38), 27.94 s (95% CI 26.36-29.52), and 1.21 L/s (95% CI 0.95-1.47), respectively. ΔPEF and ΔPIF values increased, while ΔEDI decreased. In predicting the need for surgical intervention, EDI had the highest sensitivity (88%), and forced expiratory volume per second/forced vital capacity had the highest specificity (85%).
CONCLUSION
Spirometry is a valuable tool for assessing patients with LTS. PEF, EDI, and PIF were the most commonly reported spirometry parameters that significantly improved after airway stenosis was relieved.
Topics: Adult; Humans; Constriction, Pathologic; Spirometry; Tracheal Stenosis; Respiratory Function Tests; ROC Curve; Laryngostenosis
PubMed: 37522909
DOI: 10.1007/s00405-023-08159-7 -
The British Journal of Surgery Sep 2023Active surveillance is being investigated as an alternative to standard surgery after neoadjuvant chemoradiotherapy for oesophageal cancer. It is unknown whether...
BACKGROUND
Active surveillance is being investigated as an alternative to standard surgery after neoadjuvant chemoradiotherapy for oesophageal cancer. It is unknown whether dysphagia persists or develops when the oesophagus is preserved after neoadjuvant chemoradiotherapy. The aim of this study was to assess the prevalence and severity of dysphagia during active surveillance in patients with an ongoing response.
METHODS
Patients who underwent active surveillance were identified from the Surgery As Needed for Oesophageal cancer ('SANO') trial. Patients without evidence of residual oesophageal cancer until at least 6 months after neoadjuvant chemoradiotherapy were included. Study endpoints were assessed at time points that patients were cancer-free and remained cancer-free for the next 4 months. Dysphagia scores were evaluated at 6, 9, 12, and 16 months after neoadjuvant chemoradiotherapy. Scores were based on the European Organisation for Research and Treatment of Cancer oesophago-gastric quality-of-life questionnaire 25 (EORTC QLQ-OG25) (range 0-100; no to severe dysphagia). The rate of patients with a (non-)traversable stenosis was determined based on all available endoscopy reports.
RESULTS
In total, 131 patients were included, of whom 93 (71.0 per cent) had adenocarcinoma, 93 (71.0 per cent) had a cT3-4a tumour, and 33 (25.2 per cent) had a tumour circumference of greater than 75 per cent at endoscopy; 60.8 to 71.0 per cent of patients completed questionnaires per time point after neoadjuvant chemoradiotherapy. At all time points after neoadjuvant chemoradiotherapy, median dysphagia scores were 0 (interquartile range 0-0). Two patients (1.5 per cent) underwent an intervention for a stenosis: one underwent successful endoscopic dilatation; and the other patient required temporary tube feeding. Notably, these patients did not participate in questionnaires.
CONCLUSION
Dysphagia and clinically relevant stenosis are uncommon during active surveillance.
Topics: Humans; Deglutition Disorders; Neoadjuvant Therapy; Watchful Waiting; Constriction, Pathologic; Esophageal Neoplasms; Chemoradiotherapy
PubMed: 37418342
DOI: 10.1093/bjs/znad211 -
European Heart Journal Sep 2023International guidelines recommend screening of first-degree relatives (FDR) of people with bicuspid aortic valves (BAVs). However, the prevalence of BAV and of aortic... (Meta-Analysis)
Meta-Analysis
AIMS
International guidelines recommend screening of first-degree relatives (FDR) of people with bicuspid aortic valves (BAVs). However, the prevalence of BAV and of aortic dilatation amongst family members is uncertain.
METHODS AND RESULTS
A systematic review and meta-analysis of original reports of screening for BAV. Databases including MEDLINE, Embase, and Cochrane CENTRAL were searched from inception to December 2021 using relevant search terms. Data were sought on the screened prevalence of BAV and aortic dilatation. The protocol was specified prior to the searches being performed, and standard meta-analytic techniques were used. Twenty-three observational studies met inclusion criteria (n = 2297 index cases; n = 6054 screened relatives). The prevalence of BAV amongst relatives was 7.3% [95% confidence interval (CI) 6.1%-8.6%] overall and per family was 23.6% (95% CI 18.1%-29.5%). The prevalence of aortic dilatation amongst relatives was 9.4% (95% CI 5.7%-13.9%). Whilst the prevalence of aortic dilatation was particularly high in relatives with BAV (29.2%; 95% CI 15.3%-45.1%), aortic dilatation alongside tricuspid aortic valves was a more frequent finding, as there were many more family members with tricuspid valves than BAV. The prevalence estimate amongst relatives with tricuspid valves (7.0%; 95% CI 3.2%-12.0%) was higher than reported in the general population.
CONCLUSION
Screening family members of people with BAV can identify a cohort substantially enriched for the presence of bicuspid valve, aortic enlargement, or both. The implications for screening programmes are discussed, including in particular the substantial current uncertainties regarding the clinical implications of aortic findings.
Topics: Humans; Bicuspid Aortic Valve Disease; Heart Valve Diseases; Dilatation; Aortic Valve; Aortic Diseases; Dilatation, Pathologic; Retrospective Studies
PubMed: 37288540
DOI: 10.1093/eurheartj/ehad320 -
Journal of the American College of... Jan 2024Neo-aortic root dilatation can lead to significant late morbidity after the arterial switch operation (ASO) for dextro-transposition of the great arteries (d-TGA).
BACKGROUND
Neo-aortic root dilatation can lead to significant late morbidity after the arterial switch operation (ASO) for dextro-transposition of the great arteries (d-TGA).
OBJECTIVES
We sought to examine the growth of the neo-aortic root in d-TGA.
METHODS
A single-center, retrospective cohort study of patients who underwent the ASO between July 1, 1981 and September 30, 2022 was performed. Morphology was categorized as dextro-transposition of the great arteries with intact ventricular septum (d-TGA-IVS), dextro-transposition of the great arteries with ventricular septal defect (d-TGA-VSD), and double-outlet right ventricle-transposition of the great arteries type (DORV-TGA). Echocardiographically determined diameters and derived z scores were measured at the annulus, sinus of Valsalva, and sinotubular junction immediately before the ASO and throughout follow-up. Trends in root dimensions over time were assessed using linear mixed-effects models. The association between intrinsic morphology and the composite of moderate-severe aortic regurgitation (AR) and neo-aortic valve or root intervention was evaluated with univariable and multivariable Cox proportional hazards models.
RESULTS
Of 1,359 patients who underwent the ASO, 593 (44%), 666 (49%), and 100 (7%) patients had d-TGA-IVS, d-TGA-VSD, and DORV-TGA, respectively. Each patient underwent a median of 5 echocardiograms (Q1-Q3: 3-10 echocardiograms) over a median follow-up of 8.6 years (range: 0.1-39.3 years). At 30 years, patients with DORV-TGA demonstrated greater annular (P < 0.001), sinus of Valsalva (P = 0.039), and sinotubular junction (P = 0.041) dilatation relative to patients with d-TGA-IVS. On multivariable analysis, intrinsic anatomy, older age at ASO, at least mild AR at baseline, and high-risk root dilatation were associated with moderate-severe AR and neo-aortic valve or root intervention at late follow-up (all P < 0.05).
CONCLUSIONS
Longitudinal surveillance of the neo-aortic root is warranted long after the ASO.
Topics: Humans; Infant; Transposition of Great Vessels; Retrospective Studies; Aorta, Thoracic; Follow-Up Studies; Prognosis; Heart Septal Defects, Ventricular; Aortic Valve Insufficiency; Dilatation, Pathologic; Treatment Outcome
PubMed: 37939977
DOI: 10.1016/j.jacc.2023.10.023