The European Rare Disease Network for HHT Frameworks for management of hereditary haemorrhagic telangiectasia in general and speciality care.

Hereditary haemorrhagic telangiectasia (HHT) is a complex, multisystemic vascular dysplasia affecting approximately 85,000 European Citizens. In 2016, eight founding centres operating within 6 countries, set up a working group dedicated to HHT within what became the European Reference Network on Rare Multisystemic Vascular Diseases. By launch, combined experience exceeded 10,000 HHT patients, and Chairs representing 7 separate specialties provided a median of 24 years' experience in HHT. Integrated were expert patients who focused discussions on the patient experience. Following a 2016-2017 survey to capture priorities, and underpinned by more than 40 monthly meetings, and new data acquisitions, VASCERN HHT generated position statements that distinguish expert HHT care from non-expert HHT practice. Leadership was by specialists in the relevant sub-discipline(s), and 100% consensus was required amongst all clinicians before statements were published or disseminated. One major set of outputs targeted all healthcare professionals and their HHT patients, and include the new Orphanet definition; Do's and Don'ts for common situations; Outcome Measures suitable for all consultations; COVID-19; and anticoagulation. The second output set span aspects of vascular pathophysiology where greater understanding will assist organ-specific specialist clinicians to provide more informed care to HHT patients. These cover cerebral vascular malformations and screening; mucocutaneous telangiectasia and differential diagnosis; anti-angiogenic therapies; circulatory interplays between anaemia and arteriovenous malformations; and microbiological strategies to counteract loss of normal pulmonary capillary function. Overall, the integrated outputs, and documented current practices, provide frameworks for approaches that augment the health and safety of HHT patients in diverse health-care settings.

Percutaneous biliary stone clearance: is there still a need? A 10-year single-centre experience.

AIM: To evaluate the safety and efficacy of percutaneous biliary stone clearance in a single hepatopancreaticobiliary (HPB) centre. MATERIALS AND METHODS: All patients who underwent percutaneous biliary stone clearance between 2010 and 2020 at a HPB centre were identified from the radiology information system. Their demographic data, presentation, previous surgery, number/size of biliary calculi, success and complications were collected from patient records. Unpaired student's t-test was used to compare numerical variables and the Chi-square test was used to compare categorical data. RESULTS: Sixty-eight patients aged between 58.5-91.1 years underwent the procedure, and 42.6% (29/68) had the procedure due to surgically altered anatomy precluding endoscopic retrograde cholangiopancreatography (ERCP). The most common presentation was cholangitis (62%). The success rate of percutaneous stone clearance was 92.7%. The average number of calculi was two (range 1-12). Of the patients included, 4.4% developed pancreatitis, 4.4% developed cholangitis, and 1.5% had hepatic artery branch pseudoaneurysm successfully treated with transarterial embolisation. There was no significant difference in success or complication rates between the different access sites (right lobe, left lobe, roux-loop, T-tube, p=0.7767). CONCLUSION: Percutaneous biliary stone clearance is safe and effective and will continue to play an important role where ERCP fails or is impossible due surgically altered anatomy.

Splenocystojejunostomy for the treatment of a symptomatic splenic pseudocyst.

Pancreatic pseudocyst is a widely recognised local complication following acute pancreatitis. Typically occurring more than four weeks after acute pancreatitis, a pseudocyst is a mature, encapsulated collection found within the peripancreatic tissues manifesting as abdominal pain, structural compression, gastroparesis, sepsis and organ dysfunction. Therapeutic interventions include endoscopic transpapillary or transmural drainage, percutaneous catheter drainage and open surgery. We present our management of idiopathic chronic pancreatitis complicated by a pancreatic pseudocyst extending to the splenic capsule in a 38-year-old man. A trial of conservative management was sought, but later escalated to percutaneous fluoroscopic drainage. Despite a period of volume reduction of the pseudocyst, reaccumulation occurred. We describe successful surgical treatment via means of a splenocystojejunostomy and subsequent pain reduction.

Transpleural systemic artery-to-pulmonary artery communications in the absence of chronic inflammatory lung disease. A case series and review of the literature.

AIM: To describe the causes and computed tomography (CT) and angiographic appearances of transpleural systemic artery-to-pulmonary artery shunts in patients without chronic inflammatory lung disease and determine their best management. MATERIALS AND METHODS: All patients referred to a tertiary referral unit between January 2009 and January 2020 in whom a diagnosis of a systemic-to-pulmonary artery communication without underlying chronic inflammatory lung disease was subsequently made have been included in this report. Medical records and imaging findings were reviewed retrospectively. RESULTS: Ten patients (male: female ratio = 7:3; median age 42 years [range 22-70 years]) with systemic artery-to-pulmonary artery shunts without chronic inflammatory lung disease were identified. Five were misdiagnosed as having a pulmonary arteriovenous malformation and had been referred for embolisation. In six patients, there was either a history of accidental or iatrogenic thoracic trauma or of inflammatory disease involving the pleura, and in two patients, in whom a previous medical history could not be obtained, there were CT features suggesting previous pleural inflammatory disease. Two shunts were thought to be congenital. All individuals were asymptomatic other than one with localised thoracic discomfort that dated from the time of surgery. All patients were managed conservatively and have remained well with a median follow-up of 4.5 years (range 1-11.3 years). CONCLUSIONS: Localised transpleural systemic artery-to-pulmonary artery shunts in the absence of chronic inflammatory lung disease are usually related to previous thoracic trauma/intervention or abdominal or pulmonary sepsis involving a pleural or diaphragmatic surface. Congenital shunts are rare. The present study and much of the literature supports conservative management.

The changing face of reoperative parathyroidectomy: a single-centre comparison of 147 parathyroid reoperations.

INTRODUCTION: Reoperative parathyroidectomy for persistent and recurrent primary hyperparathyroidism is dependent on radiology. This study aimed to compare outcomes in reoperative parathyroidectomy at a single centre using a combination of traditional and newer imaging studies. MATERIALS AND METHODS: Retrospective case note review of all reoperative parathyroidectomies for persistent and recurrent primary hyperparathyroidism over five years (June 2014 to June 2019; group A). Imaging modalities used and their positive predictive value, complications and cure rates were compared with a published dataset spanning the preceding nine years (group B). RESULTS: From over 2000 parathyroidectomies, 147 were reoperations (101 in group A and 46 in group B). Age and sex ratios were similar (56 vs 62 years; 77% vs 72% female). Ultrasound use remains high and shows better positive predictive value (76% vs 57 %). 99mTc-sestamibi use has declined (79% vs 91%) but the positive predictive value has improved (74% vs 53%). 4DCT use has almost doubled (61% vs 37%) with better positive predictive value (88% vs 75%). 18F-fluorocholine positron emission tomography-computed tomography and ultrasound-guided fine-needle aspiration for parathyroid hormone are novel modalities only available for group A. Both carried a positive predictive value of 100%. Venous sampling with or without angiography use has decreased (35% vs 39%) but maintains a high positive predictive value (86% vs 91%). Cure rates were similar (96% vs 100%). Group A had 5% permanent hypoparathyroidism, 1% permanent vocal cord palsy and 1% haematoma requiring reoperation. No complications for group B. CONCLUSION: Optimal imaging is key to good cure rates in reoperative parathyroidectomy. High-quality, non-interventional imaging techniques have produced a shift in the preoperative algorithm without compromising outcomes.

Assessing the effects of manual dexterity and playing computer games on catheter-wire manipulation for inexperienced operators.

AIM: To investigate the effect of playing computer games and manual dexterity on catheter-wire manipulation in a mechanical aortic model. MATERIAL AND METHODS: Medical student volunteers filled in a preprocedure questionnaire assessing their exposure to computer games. Their manual dexterity was measured using a smartphone game. They were then shown a video clip demonstrating renal artery cannulation and were asked to reproduce this. All attempts were timed. Two-tailed Student's t-test was used to compare continuous data, while Fisher's exact test was used for categorical data. RESULTS: Fifty students aged 18-22 years took part in the study. Forty-six completed the task at an average of 168 seconds (range 103-301 seconds). There was no significant difference in the dexterity score or time to cannulate the renal artery between male and female students. Students who played computer games for >10 hours per week had better dexterity scores than those who did not play computer games: 9.1 versus 10.2 seconds (p=0.0237). Four of 19 students who did not play computer games failed to complete the task, while all of those who played computer games regularly completed the task (p=0.0168). CONCLUSION: Playing computer games is associated with better manual dexterity and ability to complete a basic interventional radiology task for novices.

Correlation of Smoking and Myocardial Infarction Among Sudanese Male Patients Above 40 Years of Age.

To find an association between smoking and the development of myocardial infarction in male patients above forty years of age presenting at the echocardiology department of Sudan heart center Khartoum. A prospective cohort study was carried out at the echocardiography department of Sudan Heart Center in Khartoum-Sudan between July 2012 and June 2014. The study population comprised a total of 168 adult male patients who underwent cardiac ultrasound scanning. Out of a total of 144 cases, 65% (94) of patients were smokers, 74% of the 94 cases smoked for more than 10 years, and 26% of the 94 cases smoked for less than 10 years. With this study it was concluded that smoking is a risk factor for the development of myocardial infarction. This study showed that patients with myocardial infarction are more likely to have a past history of smoking.

Study of the association between the incidences of congenital anomalies and hydrocephalus in Sudanese fetuses.

This study was designed with an aim to detect the congenital anomalies appear to be linked to and in conjunction with hydrocephalus fetuses in Sudan, when ultrasound is used to exam fetuses in the second and third trimesters of pregnancy. This prospective cohort study was performed from December 2011 to December 2013, in a group consists of 5000 single gestation pregnant Sudanese women. In all cases, maternal ages were 35 years up to 48 years; mean age of 42.5 years. Pelvic; obstetric ultrasound scanning protocol used should meet the standards established by the American Institute of Ultrasound in Medicine (AIUM) for scanning in the second and third trimesters of pregnancy. Statistical Package for the Social Sciences (SPSS) was used to analyze the results. Diagnosed hydrocephalus cases (0.4%) were found to be associated with other fetal anomalies as aqueduct stenosis (45%), spina bifida (30%), Arnold-Chiari malformation (20%) and Dandy-Walker malformation (5%). The incidence of congenital anomalies and hydrocephalus in Sudanese fetuses showed considerable variation among different regions of Sudan. Hydrocephalus is associated with certain congenital anomalies. In agreement with previous studies, hydrocephalus is predominantly in male rather than female fetuses. The prevalence of fetal anomalies and hydrocephalus are within previously reported ranges.

Massive localised lymphoedema: a rare vascular malformation.

Lymphatic malformations are a subset of congenital vascular malformations, and are caused by a defect in lymphatic development during embryogenesis. When lymphatic mesoderm development is prematurely arrested, it retains it proliferative potential. Stimulus in the future can cause the lesion to proliferate locally without coordination or regulation, resulting in the rare condition known as massive localised lymphoedema (MLL). We present a case report of MLL, a rare and ill-defined soft tissue mass reported in the morbidly obese, with reference to the existing literature.

A comparison of the quality of the information available on the internet on interventional radiology, vascular surgery, and cardiology.

CONTEXT AND AIMS: Internet use is rapidly expanding and increasingly plays a substantial role in patient education. We sought to evaluate and compare the quality of information available to patients online on three closely linked specialties: Interventional radiology (IR), cardiology, and vascular surgery. MATERIALS AND METHODS: We searched the leading three search engines for the terms: "Interventional Radiology", "cardiology," and "vascular surgery," collating the top 50 hits from each search. After excluding duplicates and irrelevant sites, 43, 25, and 36 sites remained, respectively. Sites were analyzed using the LIDA instrument (an online tool for assessing health-related websites) and Fleisch Reading Ease Scores (FRES) were compared across the different search terms and correlated with the country of origin and certification by the Health on the Net (HON) Foundation. RESULTS: There was no significant difference ( P>0.05) in the total LIDA, accessibility, usability or reliability scores between the three specialties. HONCode certification was associated with higher LIDA (83.1±1.6 vs. 71.53±0.8 ( P<0.0001)), reliability (75.7±3.6 vs. 49.0±1.6 ( P<0.0001)) and FRES (37.4±4.0 vs. 29.7±1.4 ( P=0.0441)). CONCLUSION: Websites are generally well designed and easy to use; the majority however, lacks currency and reliability. Despite similarity in quality of online information, there is a disparity in knowledge of IR; this may be due to low web-traffic figures of IR sites. Wikipedia's user-generated content, ranks highly in major search engines, as such; this could serve as means of disseminating reliable health information to patients.