Role of endoscopic ultrasound in the characterization of solid pseudopapillary neoplasm of the pancreas.

BACKGROUND: Solid pseudopapillary neoplasm (SPN) is an uncommon pathology of the pancreas with unpredictable malignant potential. Endoscopic ultrasound (EUS) assessment plays a vital role in lesion characterization and confirmation of the tissue diagnosis. However, there is a paucity of data regarding the imaging assessment of these lesions. AIM: To determine the characteristic EUS features of SPN and define its role in preoperative assessment. METHODS: This was an international, multicenter, retrospective, observational study of prospective cohorts from 7 large hepatopancreaticobiliary centers. All cases with postoperative histology of SPN were included in the study. Data collected included clinical, biochemical, histological and EUS characteristics. RESULTS: One hundred and six patients with the diagnosis of SPN were included. The mean age was 26 years (range 9 to 70 years), with female predominance (89.6%). The most frequent clinical presentation was abdominal pain (80/106; 75.5%). The mean diameter of the lesion was 53.7 mm (range 15 to 130 mm), with the slight predominant location in the head of the pancreas (44/106; 41.5%). The majority of lesions presented with solid imaging features (59/106; 55.7%) although 33.0% (35/106) had mixed solid/cystic characteristics and 11.3% (12/106) had cystic morphology. Calcification was observed in only 4 (3.8%) cases. Main pancreatic duct dilation was uncommon, evident in only 2 cases (1.9%), whilst common bile duct dilation was observed in 5 (11.3%) cases. One patient demonstrated a double duct sign at presentation. Elastography and Doppler evaluation demonstrated inconsistent appearances with no emergence of a predictable pattern. EUS guided biopsy was performed using three different types of needles: Fine needle aspiration (67/106; 63.2%), fine needle biopsy (37/106; 34.9%), and Sonar Trucut (2/106; 1.9%). The diagnosis was conclusive in 103 (97.2%) cases. Ninety-seven patients were treated surgically (91.5%) and the post-surgical SPN diagnosis was confirmed in all cases. During the 2-year follow-up period, no recurrence was observed. CONCLUSION: SPN presented primarily as a solid lesion on endosonographic assessment. The lesion tended to be located in the head or body of the pancreas. There was no consistent characteristic pattern apparent on either elastography or Doppler assessment. Similarly SPN did not frequently cause stricture of the pancreatic duct or common bile duct. Importantly, we confirmed that EUS-guided biopsy was an efficient and safe diagnostic tool. The needle type used does not appear to have a significant impact on the diagnostic yield. Overall SPN remains a challenging diagnosis based on EUS imaging with no pathognomonic features. EUS guided biopsy remains the gold standard in establishing the diagnosis.

Endoscopic ultrasound guided gastrojejunostomy in the treatment of gastric outlet obstruction: multi-centre experience from the United Kingdom.

BACKGROUND: Endoscopic ultrasound guided gastrojejunostomy (EUS-GJ) with lumen apposing metal stents has recently emerged as a viable option, as an alternative to surgical gastrojejunostomy and endoscopic enteral stenting, for managing gastric outlet obstruction (GOO). We aim to perform a retrospective analysis of the efficacy, safety and outcomes of EUS-GJ performed at three tertiary institutions in the United Kingdom. METHODS: Consecutive patients who underwent EUS-GJ between August 2018 and March 2021 were identified from a prospectively maintained database. Data were obtained from interrogation of electronic health records. RESULTS: Twenty five patients (15 males) with a median age of 63 years old (range 29-80) were included for analysis. 88% (22/25) of patients had GOO due to underlying malignant disease. All patients were deemed surgically inoperable or at high surgical risk. Both technical and clinical success were achieved in 92% (23/25) of patients. There was an improvement in the mean Gastric Outlet Obstruction Scoring System scores following a technically successful EUS-GJ (2.52 vs 0.68, p < 0.01). Adverse events occurred in 2/25 patients (8%), both due to stent maldeployment necessitating endoscopic closure of the gastric defect with clips. Long-term follow-up data were available for 21 of 23 patients and the re-intervention rate was 4.8% (1/21) over a median follow-up period of 162 (range 5-474) days. CONCLUSION: EUS-GJ in carefully selected patients is an effective and safe procedure when performed by experienced endoscopists.

Initial United Kingdom experience of endoscopic ultrasound-directed transgastric endoscopic retrograde cholangiopancreatography.

Backgrounds/Aims: Gallstone disease is a recognized complication of bariatric surgery. Subsequent management of choledocholithiasis may be challenging due to altered anatomy which may include Roux-en-Y gastric bypass (RYGB). We conducted a retrospective service evaluation study to assess the safety and efficacy of endoscopic ultrasound-directed transgastric endoscopic retrograde cholangiopancreatography (EDGE) in patients with RYGB anatomy. Methods: All the patients who underwent EDGE for endoscopic retrograde cholangiopancreatography after RYGB at two tertiary care centers in the United Kingdom between January 2020 and October 2021 were included in the study. Clinical and demographic details were recorded for all patients. The primary outcome measures were technical and clinical success. Adverse events were recorded. Hot Axios lumen apposing metal stents measuring 20 mm in diameter and 10 mm in length were used in all the patients for creation of a gastro-gastric or gastro-jejunal fistula. Results: A total of 14 patients underwent EDGE during the study period. The majority of the patients were female (85.7%) and the mean age of patients was 65.8 ± 9.8 years. Technical success was achieved in all but one patient at the first attempt (92.8%) and clinical success was achieved in 100% of the patients. Complications arose in 3 patients with 1 patient experiencing persistent fistula and weight gain. Conclusions: In patients with RYGB anatomy, EDGE facilitated biliary access has a high rate of clinical success with an acceptable safety profile. Adverse events are uncommon and can be managed endoscopically.

Increased ERCP volume improves cholangiogram interpretation: a new performance measure for ERCP training?

BACKGROUND/AIMS: Cholangiogram interpretation is not used as a key performance indicator (KPI) of endoscopic retrograde cholangiopancreatography (ERCP) training, and national societies recommend different minimum numbers per annum to maintain competence. This study aimed to determine the relationship between correct ERCP cholangiogram interpretation and experience. METHODS: One hundred fifty ERCPists were surveyed to appropriately interpret ERCP cholangiographic findings. There were three groups of 50 participants each: "Trainees," "Consultants group 1" (performed >75 ERCPs per year), and "Consultants group 2" (performed >100 ERCPs per year). RESULTS: Trainees was inferior to Consultants groups 1 and 2 in identifying all findings except choledocholithiasis outside the intrahepatic duct on the initial or completion/occlusion cholangiogram. Consultants group 1 was inferior to Consultants group 2 in identifying Strasberg type A bile leaks (odds ratio [OR], 0.86; 95% confidence interval [CI], 0.77-0.96), Strasberg type B (OR, 0.84; 95% CI, 0.74-0.95), and Bismuth type 2 hilar strictures (OR, 0.81; 95% CI, 0.69-0.95). CONCLUSION: This investigation supports the notion that cholangiogram interpretation improves with increased annual ERCP case volumes. Thus, a higher annual volume of procedures performed may improve the ability to correctly interpret particularly difficult findings. Cholangiogram interpretation, in addition to bile duct cannulation, could be considered as another KPI of ERCP training.

10-year experience of Paediatric varicocele embolization in a tertiary centre with long-term follow-up.

INTRODUCTION: Paediatric varicocele embolization has many benefits over surgical ligation, but lacks published long-term data. We investigated technical and clinical outcomes in this under reported patient group. OBJECTIVE: To evaluate technical success, complications and recurrence rates following varicocele embolization in paediatric patients. MATERIALS AND METHODS: A single-centre retrospective review of procedural data and electronic notes of consecutive patients referred for varicocele embolization over a 10-year period was performed (February 2010-March 2020). The primary outcomes were technical success and clinical efficacy (lack of symptom recurrence). Secondary outcomes included complications, testicular vein size reduction and procedural parameters including radiation exposure. Chi-square analysis was used to identify predictors of clinical success. Follow-up involved outpatient clinical assessment and telephone interview. RESULTS: 40 patients (median age 15) were referred for left-sided symptomatic varicocele. Technical embolization success was achieved in 36/40 patients (90%), with 4 procedures abandoned (inaccessible vein). Embolization technique was platinum-based coils ± sclerosant. There were no immediate or long-term procedural complications. 32/36 patients completed short term follow-up at a median interval of 2.8 months. 30/32 (93.78%) experienced early clinical success. We found a significant reduction in peritesticular vein size following embolization (pre-3.70 vs post-2.56 mm, p = 0.00017) and a significant relationship between varicocele grade and early clinical success (χ2 = 4.2, p = 0.04), but not pre-treatment peritesticular vein size (χ2 = 0.02, p = 0.88). 33/36 patients completed long-term follow-up (median 4.2 years, range 0.36-9.9 years) producing a late clinical success rate of 93.9% (31/33). No post procedural complications including hydroceles were identified. DISCUSSION: This study demonstrates technical success, matching rates described in adult patients which is reassuring and in support of embolization in the younger patient cohort. More importantly, the overall clinical success rate is comparable with previous embolization studies. Reassuringly, all symptom recurrences occurred early in follow-up, and there is a cogent argument for a single follow-up appointment at this juncture. Our long-term average follow-up duration, primarily gained via telephone interview, exceeds other studies. Although our study has the longest follow-up for varicocele embolization in children, it is limited by a few patients being lost to early and long-term follow-up. This is a recognised issue faced by studies attempting to follow-up benign conditions with a high clinical success rate. CONCLUSION: Paediatric varicocele embolization is a successful alternative to surgical ligation, with no complications and good clinical outcomes over a long-term follow-up.

Attitudes to radiation safety and cholangiogram interpretation in endoscopic retrograde cholangiopancreatography (ERCP): a UK survey.

BACKGROUND: Fluoroscopy during endoscopic retrograde cholangiopancreatography (ERCP) exposes staff and patients to potentially harmful ionizing radiation. We performed a UK survey to explore trainee and trainer attitudes to radiation protection and cholangiogram interpretation in ERCP. METHODS: An electronic 10-point survey was prospectively distributed to endoscopy unit leads, training programme directors between October and November 2019. Only UK-based ERCP trainees and trainers with hands-on procedural exposure were eligible for the survey. RESULTS: The survey was completed by 107 respondents (58 trainees and 49 trainers), with an estimated overall response rate of 46%. Overall, 49% of respondents were up to date with their radiation protection course, 38% were aware of European Basic safety standards directive (BSSD), 38% wore radiation protection goggles, and 40% were aware of the average radiation screening dose per ERCP procedure. Compared with trainers, trainees were less likely to routinely wear thyroid protection shields (76% vs 92%; p=0.028), have awareness of the BSSD (20% vs 49%; p=0.037) or know their average procedural radiation dosages (21% vs 63%; p<0.001). With regard to cholangiogram interpretation, only 26% had received formal training, with 97% of trainees expressing a desire for further training. CONCLUSION: This survey highlights a relative complacency in safety attitudes to radiation protection during ERCP. These data provide impetus to improve training and quality assurance in radiation protection, which should be regarded as a mandatory safety aspect prior to commencing hands-on ERCP training.

Management of infected post-pancreatic resection fluid collections under endoscopic ultrasound guidance using lumen apposing metal stent: A case series and review of the literature.

BACKGROUNDS/AIMS: Post-operative pancreatic fistulas (POPF) and fluid collections (POPFC) remain significant sources of morbidity and mortality after pancreatic resections. There remains a paucity of literature describing endoscopic ultrasound (EUS) guided drainage of POPFC using a Hot AXIOS™ lumen apposing metal stent (LAMS). METHODS: We conducted a retrospective study, encompassing all consecutive patients with POPFC managed using Hot AXIOS™ LAMS at our institution between January 2017 and December 2019. Primary outcome measures were technical and clinical success. Secondary outcome measures were adverse events and recurrence rates. RESULTS: Five patients underwent EUS guided drainage using Hot AXIOS™ LAMS during the study period. Mean age of patients was 67.8 ± 2.16 years. The majority (60.0%) of patients were males. Median duration of symptom onset after surgery was 9 days. All patients presented with abdominal pain. Median size of the collection measured on computed tomography was 91 mm. Median interval time between symptom onset and EUS drainage was 30 days. Two patients required percutaneous drainage prior to EUS guided drainage. Technical and clinical success were achieved for all patients. No adverse events were observed. Median duration of follow-up was 90 days. No recurrence of collection occurred during the follow-up period. CONCLUSIONS: EUS guided drainage of POPFC using Hot AXIOS™ LAMS is a safe and effective treatment modality with technical and clinical success rates of 100% in our experience.

Experimental and numerical investigation of pulsed flows in asevere aortic stenosed model.

Steady and pulsatile aortic stenotic flows through stenosis tubes were experimentally and numerically investigated. The objective was the understanding of the fluid dynamics in arterial geometries most relevant in the context of atherosclerosis. Axisymmetric phantoms corresponding to significant artery stenosis of 50% in diameter and severe aortic stenosis of 75% were respectively machined from silicon. A water flow circuit was established, a steady flow was provided by gravity and a pulsed flow by a pulsatile pump. At inlet Reynolds numbers in the range of 85 to 1125, flows at the stenosis region were investigated using two-component Particle Image Velocimetry (PIV). For the unsteady flow, three different heartbeats (60, 69 and 90 beats per minute) were considered. The k-ω shear-stress-transport first-order turbulence model in Computational Fluid Dynamics (CFD) commercial software was adopted for simulations. Experimental measurements of the velocity fields show good agreements with CFD for both steady and pulsed flows. Recirculation regions were found near the stenosis in both cases. Reverse flow through the stenosis was also observed in pulsatile flow during the end diastolic phase of the cycle. CFD simulations allowed us to accurately assess wall shear stress in the stenotic region where the optical measurements are very noisy. High values of wall shear stress (with high variations both in space and time), are observed, which are indicators of possible future aortic wall damage.

Current assessment and management of endoleaks after advanced EVAR: new devices, new endoleaks?

INTRODUCTION: In recent years there has been an increasing application of advanced EVAR techniques to tackle complex clinical and anatomical scenarios. In a bid to overcome the limitations of the traditional stent-grafts, newer EVAR endografts and techniques have been developed and introduced into clinical practice, permitting endovascular management of difficult infrarenal, juxta-renal and thoracoabdominal aneurysms for which previously there was no endovascular solution. As a consequence, we are now confronted with unique patterns of endoleak requiring customized clinical-radiological assessment and treatment. Despite the increasing body of evidence regarding new EVAR techniques and related endoleaks, current guidelines do not specifically address these issues. OBJECTIVES: Our review aims to assess risk factors, development, and management strategies of these endoleaks, in the most recent infrarenal EVAR devices and in more complex fenestrated EVAR (FEVAR) and Chimney EVAR (Ch-EVAR). EXPERT OPINION: Most new devices have demonstrated types of endoleaks that need specific imaging and treatment, as in EVAS, FEVAR, and ChEVAR. Knowledge of specific stent-graft characteristics and the nature of endoleaks associated with the various procedures facilitates the application of relevant useful imaging. In addition, it should aid development of a customized and practically relevant approach to patient management during intervention and follow-up.

A Genetic-Based Extreme Gradient Boosting Model for Detecting Intrusions in Wireless Sensor Networks.

An Intrusion detection system is an essential security tool for protecting services and infrastructures of wireless sensor networks from unseen and unpredictable attacks. Few works of machine learning have been proposed for intrusion detection in wireless sensor networks and that have achieved reasonable results. However, these works still need to be more accurate and efficient against imbalanced data problems in network traffic. In this paper, we proposed a new model to detect intrusion attacks based on a genetic algorithm and an extreme gradient boosting (XGBoot) classifier, called GXGBoost model. The latter is a gradient boosting model designed for improving the performance of traditional models to detect minority classes of attacks in the highly imbalanced data traffic of wireless sensor networks. A set of experiments were conducted on wireless sensor network-detection system (WSN-DS) dataset using holdout and 10 fold cross validation techniques. The results of 10 fold cross validation tests revealed that the proposed approach outperformed the state-of-the-art approaches and other ensemble learning classifiers with high detection rates of 98.2%, 92.9%, 98.9%, and 99.5% for flooding, scheduling, grayhole, and blackhole attacks, respectively, in addition to 99.9% for normal traffic.

A constellation of orthopaedic deformities in connection with cartilage oligomeric matrix protein mutation.

BACKGROUND: Trendelenburg's gait can be observed in Legg-Calvé-Perthes disease, antalgic gait observed in osteoarthropathy and waddling gait is usually seen in genu varum and circumduction gait in patients with genu valgum. Disabling pain was a prime manifestation in slipped capital femoral epiphysis (SCFE). Limited joint range of motion with an inability to bear full weight on an affected extremity with swaying and wide-based gait is seen in patients with malalignment of the lower limbs. All the above-mentioned deformities have been labelled as idiopathic. The main objective of this article is to approach to the aetiology understanding. PATIENTS AND METHODS: Ten children (3 girls and 7 boys with age average of 9 years) presented with variable deformities; Perthes-like deformity, genu varum/valgum and osteoarthropathy and one patient with SCFE. Clinical and radiological phenotypes were the baseline tool of diagnosis. Genotypic characterisations were performed. RESULTS: Diverse clinical presentations of Perthes-like disease, osteoarthropathy, genu varum/valgum and SCFE were the most prominent skeletal abnormalities in patients manifested cartilage oligomeric matrix protein (COMP) gene mutation. CONCLUSION: : The value of presenting this article is fourfold; first to signify that mutation study was essential for the increment of knowledge related to the genotype-phenotype relationships. Second, to indicate that professional awareness is needed to differentiate between the hidden pathologies in patients with Perthes-like deformity, genu varum, genu valgum and early osteoarthritis in correlation with COMP gene mutation. Third, it is mandatory to question the validity of the term idiopathic. Fourth, this article is an attempt to sensitise orthopaedic physicians and surgeons that deformities might be stemmed from diverse forms of intrinsic bone disorders.

Endoscopic ultrasound-guided pancreatic fluid collections' transmural drainage outcomes in 100 consecutive cases of pseudocysts and walled off necrosis: a single-centre experience from the United Kingdom.

BACKGROUND AND AIM: Endoscopic ultrasound-guided drainage is a minimally invasive first-line modality for the drainage of pancreatic fluid collection (PFC) resulting in a shorter hospital stay and less morbidity compared with surgical cystogastrostomy. Our aim is to evaluate potential differences in the outcomes of endoscopic ultrasound (EUS) guided transmural drainage (EUS-TD) drainage of pancreatic pseudocyst (PP) and walled-off necrosis (WON). METHOD: We retrospectively reviewed 100 consecutive EUS-guided drainages of PFC utilising EUS reports; clinical notes and imaging with follow-up (FU) to 12 months. All procedures were undertaken under conscious sedation with EUS guidance alone (without fluoroscopy) and placement of plastic double pigtail stents. RESULTS: In these 100 sequential cases, there were 78 cases of PP and 22 cases of WON. All 22/22(100%) cases of WON had successful EUS-guided stent placement. In 2/22(9%), there was little or no clinical improvement. These two patients required further computed tomography (CT)-guided drainage and one of these patients (1/22) (4.5%) developed recurrence within 12 months FU after removal of stents. In case of PP, overall stent placement was successful in 76/78 (97%) patients, but 6/78(8%) required 2nd EUS procedure after failure to show clinical improvement; 3/78(2.5%) required further CT-guided drainage. The overall complication rate was 9%(9/100) with 4%(4/100) requiring endoscopic or CT-guided intervention with no overall 30-day mortality. CONCLUSION: This is the largest series from a single UK centre demonstrating that EUS-guided cystogastrostomy of PFC drainage using plastic double pigtail stents is sufficient in majority of cases with PFC including that of WON, with or without infection.

Planning Prostate Artery Embolisation: Is it Essential to Perform a Pre-procedural CTA?

PURPOSE: A highly variable prostatic artery origin coupled with frequent anastomoses to adjacent organs makes prostate artery embolisation a challenging procedure. Despite CT angiography facilitating procedural planning, it is not performed in all centres. Therefore, we explored the utility of prostatic CT angiography by assessing its capacity to identify the prostatic arteries and highlight troublesome anastomoses. MATERIALS AND METHODS: A retrospective review of patients undergoing prostate artery embolisation for symptoms of benign prostatic obstruction between June 2012 and October 2016 was conducted, with analysis of the pre-procedural CT angiography. CT findings were compared with subsequent intraprocedural angiography to assess the accuracy with which CT angiography predicts the origin of prostatic arteries and identifies anastomoses. RESULTS: In total, 110 patients underwent prostate artery embolisation, with pre-procedural CT angiography acquired in all patients, enabling assessment of 220 pelvic sides of hemiprostatic arterial supply. Mean dose length product was 808.4 mGycm. CT angiography successfully identified prostatic arterial supply in 214/220 pelvic sides, an accuracy of 97.3%. Anastomoses of prostatic vessels were suggested by CT angiography in 52 pelvic sides. These were confirmed by angiogram in 49/52 sides (94.2%). CT angiography demonstrated a sensitivity of 59.0% and specificity of 94.2% for anastomoses detection. CONCLUSION: CT angiography prior to embolisation reliably predicts the arterial anatomy and facilitates procedural planning. Therefore, it should be a considered as a pre-procedural investigation for patients undergoing prostate artery embolisation. Sensitivity is low for predicting anastomoses, so careful periprocedural evaluation of the target vessels is still required.

How frequent is osteogenesis imperfecta in patients with idiopathic osteoporosis?: Case reports.

RATIONALE: The term idiopathic osteoporosis itself is quite a non-specific disease label, which fails to address the etiological understanding. Bone mineral density alone is not a reliable parameter to detect patients at high risk of fracture. The diversity of the clinical phenotypes of discolored teeth, blueness of the sclera, back and joint pain, cardiovascular disease, Diabetes type II, hearing problems and a long list of orthopedic problems are have to be considered. PATIENTS CONCERNS: Our study has been designed in accordance with the clinical and radiological phenotype of eleven index cases with the provisional diagnosis of OI, which was followed by genotypic confirmation. This was followed by the invitation of siblings, parents, grandparents and other relatives to participate in the interviews, and to discuss the impact of the diagnosis. Proper collaboration with these families facilitated the process to identify other subjects with a history of fractures and other deformities/disabilities which were seemingly correlated to heritable connective tissue disorder. In total, 63 patients (27 children and 36 parents/grandparents and relatives) were enrolled in the study. Two groups of children were not included in our study. We excluded children with incomplete documentation and children who manifested de novo mutation. The term idiopathic osteoporosis (IOP) has been given to these families in other Institutes and was considered as a definite diagnosis. IOP was solely based on T scores, BMD and certain laboratory tests. Surprisingly, no single adult patient underwent clinical and or radiological phenotypic characterization. DIAGNOSES: A constellation of significant disease associations with osteoporotic fracture risk have been encountered. The index cases showed mutations in COL1A1 (17q21.31.q22) and COL1A2 (7q22.1), the genes encoding collagen type I. The phenotype/genotype confirmation in 11 children was the key factor to boost our research and to re-consult each family. Comprehensive clinical and radiological phenotypic documentation has been applied to most of other family subjects who principally received the diagnosis of IOP. INTERVENTIONS: All adult patients had normal serum calcium and only three patients showed an average of low serum phosphate of 0.7-0.61 mmol/l. Serumcrosslaps in six parents was in the average of (2.9-3.8 nM) and PTH levels were normal in all patients (the average showed 8.73 pg/ml). OUTCOMES: Our efforts to minimize and constrain the usage of the term idiopathic osteoporosis and to understand the sequence of pathological events that occurred in these families were emphasized. These efforts evolved into a remarkable and unique constellation of clinical findings. Strikingly, fracture represented a portion in a series of skeletal and extra-skeletal deformities and abnormalities which are all correlated to connective tissue disorder. This was achieved mainly through comprehensive phenotype/genotype confirmation, followed by scrutinizing the records of each family, clinical examination of the adults and revising the archives of our Hospitals and other Institutes. LESSONS: The sequence of diverse pathological events recorded within each family would be almost incomprehensible without a proper etiological understanding of the natural history of each child/family deformity that led to their occurrences. We wish to stress that, our current study is just an attempt to cover only a tiny fraction of the tip of the iceberg and to profoundly explore one of the most under-estimated causes of idiopathic osteoporosis.

Prostate artery embolization: a new, minimally invasive treatment for lower urinary tract symptoms secondary to prostate enlargement.

Prostate artery embolization (PAE) is emerging as a safe and efficacious treatment which approaches benign prostatic obstruction (BPO) from a unique perspective. This brings with it distinct advantages and solutions, which we discuss along with cost, evidence, complications and disadvantages.

Corrections of diverse forms of lower limb deformities in patients with mucopolysaccharidosis type IVA (Morquio syndrome).

BACKGROUND: Thoracolumbar kyphosis has been considered as the first presenting deformity and is often a key diagnostic clue noted in children with mucopolysaccharidosis (MPS) type IV (Morquio's syndrome). However, we observed that the progressive irregularities of the epiphyses of the long bones were the most prominent skeletal pathology, causing effectively the development of diverse forms of lower limbs deformities with extreme variation in age of onset. MATERIALS AND METHODS: Ten patients (seven children and three adults) with an average age of 15 years have been enrolled in this study. Age of diagnosis of MPS IVA has a variable age of onset and a MISLEADING rate of severity. Hip dislocations, genu valgum, protrusio acetabuli and osteoarthritis were the most common lower limbs deformities in these patients. Clinical and radiographic phenotypes were the baseline tools of documentation. Urinary screening and genotypic characterizations have been applied accordingly. RESULTS: Combined pelvic and femoral procedures for hip dislocation, epiphysiodeses and supracondylar osteotomy for genu valgum and hip arthroplasty for protrusio acetabuli have been performed. All patients manifested insufficient activity of N-acetylgalactosamine-6-sulphate sulphatase, an enzyme that degrades keratin sulphate and chondroitin-6 sulphate. CONCLUSION: The extensive clinical heterogeneity contributed significantly in the delay in establishing the diagnosis particularly in adult patients with MPS IV. The epiphyseal irregularities of the long bones and the progressive flattening pathology of MPS IV A were the reason to falsely diagnose some patients as spondyloepiphyseal dysplasia congenital and/or tarda. Proximal femoral osteotomy, realignment osteotomy and total hip arthroplasty have been performed for coxa vara, genu valgum and protrusio acetabuli, respectively, in children and adult group of patients. The importance of early diagnosis on MPS IV A is to receive enzyme replacement therapy and plan for other therapeutic measures.

Updated Management of Malignant Biliary Tract Tumors: An Illustrative Review.

The management of malignant biliary tumors (MBTs) is complex and requires a multidisciplinary approach. Guidelines and methods of staging for biliary tumors have recently been released by main international societies, altering the clinical and radiologic approach to this pathologic condition. The aim of the present review is to detail the updated role of imaging in preoperative staging and follow-up and to illustrate clinical/therapeutic pathways. In addition, future perspectives on imaging and targeted/embolization therapies are outlined.