Endoscopic step up: When and how.

The management of (peri)pancreatic collections has undergone a paradigm shift from open surgical drainage to minimally invasive endoscopic, percutaneous, or surgical interventions. Minimally invasive interventions are associated with less morbidity and mortality compared to open necrosectomy. The (peri)pancreatic collections are currently treated with a "step-up approach" of an initial drainage procedure followed, if necessary, by a more invasive debridement. The step-up approach for management of (peri)pancreatic collections is mainly of two types, namely, surgical and endoscopic. Surgical step up includes initial image-guided percutaneous catheter drainage followed, if necessary, by minimally invasive video-assisted retroperitoneal debridement. Endoscopic step-up approach includes endoscopic transluminal drainage followed, if necessary by direct endoscopic necrosectomy. The development of endoscopic ultrasound and lumen apposing metal stents (LAMSs) has revolutionized the endoscopic management of (peri)pancreatic collections. Compared to surgical step-up approach, endoscopic step-up treatment approach has been reported to be associated with less new-onset organ failure, pancreatic fistula, enterocutaneous fistula, or perforation of visceral organ and shorter hospital/intensive care unit stay. This review will mainly focus on indications, techniques, timing, and recent advances related to endoscopic step-up approach in management of symptomatic(peri)pancreatic collections.

Portal Cavernoma Cholangiopathy in Children: An Evaluation Using Magnetic Resonance Cholangiography and Endoscopic Ultrasound.

BACKGROUND: Portal cavernoma cholangiopathy (PCC) refers to abnormalities of the extra- and intrahepatic bile ducts in patients with portal cavernoma. The literature on PCC in children is very scarce. This study aimed at characterizing PCC in children with extrahepatic portal venous obstruction (EHPVO) using endoscopic ultrasound (EUS) and magnetic resonance cholangiography/portovenography (MRC/MRPV). METHODS: A total of 53 consecutive children diagnosed with EHPVO were prospectively evaluated for PCC using MRC/MRPV and EUS. Chandra classification was used for type of involvement and Llop classification for grading of severity. RESULTS: All 53 children (100%) had PCC changes on MRC/EUS, but none were symptomatic. Extrahepatic ducts (EHDs) and intrahepatic ducts were involved in majority (85%), and 58.5% had severe changes. Periductal thickening/irregularity (71%) was the commonest change in intrahepatic ducts, whereas irregular contour of the duct with scalloping (68%); common bile duct (CBD) angulation (62.3%) were the frequent changes in the EHDs. Increased CBD angulation predisposed to CBD strictures (P = 0.004). Both left and right branches of portal vein were replaced by collaterals in all children. Among the EUS biliary changes, para-pericholedochal, intrapancreatic, and intramural gall bladder collaterals had significant association with severity, with higher frequency of occurrence in children with the most severe Llop Grade. CONCLUSIONS: PCC develops early in the disease course of EHPVO, in children, but is asymptomatic despite severe changes. EUS biliary changes are more likely to be observed with increasing severity of PCC.

Contrast-enhanced endoscopic ultrasound features of tubercular lymphadenopathy.

There is lack of data on the contrast-enhanced endoscopic ultrasound features of tubercular lymph node; our retrospective analysis of 37 patients with enlarged mediastinal and abdominal lymph nodes showed heterogeneous enhancement in the great majority (70%).

Differentiating benign and malignant pancreatic masses: Ga-68 PSMA PET/CT as a new diagnostic avenue.

OBJECTIVE: Differentiation of malignant and benign pancreatic lesions on anatomical imaging is difficult in some cases with overlapping features. Prostate-specific membrane antigen (PSMA) is overexpressed during angioneogenesis in many tumors. We aimed to evaluate the PSMA expression in pancreatic lesions to differentiate these lesions and explore the performance of Ga-68 PSMA-PET/CT vis-a-vis F-18 FDG-PET/CT. METHODS: Patients with pancreatic lesions on conventional imaging were prospectively recruited. All the patients underwent a whole-body F-18 FDG-PET/CT and a regional abdominal Ga-68 PSMA-PET/CT. Focal tracer uptake (FDG or PSMA) on PET images was considered positive. Histopathology and/or cytopathology were considered the reference standard. RESULTS: A total of forty patients (27 males, mean age 55.3 ± 9.8, range 37-71 years) were enrolled. Of these, 19 were diagnosed as malignant on histopathology/cytology. Patients with benign lesions showed no worsening of symptoms for at least 6 months on follow-up. FDG-PET/CT revealed 17 true-positive (TP), 9 false-positive (FP), 12 true-negative (TN), and 2 false-negative (FN) findings, whereas PSMA-PET/CT had 18 TP, 2 FP, 19 TN, and 1 FN finding. The sensitivity, specificity, PPV, NPV, and accuracy for FDG-PET/CT were 89.5%, 57.1%, 65.4%, 85.7%, and 72.5%, respectively, while for PSMA-PET/CT were 94.7%, 90.5%, 90%, 95%, and 92.5%, respectively. ROC curve analysis showed that the SUVmax value of 4.8 on PSMA-PET/CT could predict the malignant potential of a lesion with a specificity of 90.5% and a sensitivity of 84.2%. CONCLUSIONS: Ga-68 PSMA-PET/CT imaging helped in establishing a non-invasive pre-operative diagnosis of primary pancreatic malignancy with a higher degree of specificity and accuracy compared with FDG-PET/CT. KEY POINTS: • Conventional imaging such as CT and MRI are unable to reliably differentiate localized malignant pancreatic lesion from benign lesions mimicking malignancy such as mass-forming pancreatitis. • FDG PET/CT helps in detecting malignant foci in view of their increased glucose metabolism. However, it may be falsely positive in inflammatory lesions which may occasionally hinder its ability to differentiate between benign and malignant lesions. • Apart from prostatic malignancy, PSMA is overexpressed in neovasculature of many non-prostatic malignancies. The present study highlights that Ga68 PSMA PET/CT performed better in diagnosing malignancy non-invasively than FDG-PET/CT with a higher PPV (90.5% vs. 65.4%) and accuracy (92.5% vs. 72.5%).

Disconnected pancreatic duct syndrome: Updated review on clinical implications and management.

Disconnected Pancreatic Duct Syndrome (DPDS) is an important but often overlooked complication of acute necrotising pancreatitis (ANP) that occurs due to necrosis of the main pancreatic duct (PD). This segmental necrosis leads on to disconnection between the viable upstream pancreatic parenchyma and the duodenum. The disconnected and functional segment of pancreas continues to secrete pancreatic juice that is not drained into the gastrointestinal tract and lead on to recurrent pancreatic fluid collections (PFC), refractory external pancreatic flstulae and chronic abdominal pain/recurrent pancreatitis. Because of lack of awareness of this important complication of ANP, the diagnosis of DPDS is usually delayed. The delay in diagnosis increases the morbidity of the disease as well as increase the cost of treatment and duration of hospital stay. Surgery has remained the cornerstone for management of patients with DPDS. The conventional surgical approaches have been either resection or internal drainage procedures. Surgery for DPDS in the setting of ANP is often difficult due to presence of local inflammation and extensive venous collaterals in the operative field due to splenic vein thrombosis and therefore is associated with significant morbidity. Advancement in therapeutic endoscopy, especially advent of therapeutic endoscopic ultrasound has opened an exciting new field of minimally invasive therapeutic options for management of DPDS. The present review discusses the current understanding of the clinical manifestations, imaging features and management strategies in patients with DPDS.

Paediatric acute pancreatitis: Clinical profile and natural history of collections.

BACKGROUND & AIMS: Acute Pancreatitis (AP) tends to have a benign course in children. However there is a paucity of information with respect to severity of AP in children, the categorization of collections viz., walled off necrosis (WON)& pseudocyst and their natural history & outcomes. METHODS: A retrospective medical record review of 187 children with pancreatitis diagnosed and managed at our centre was performed. RESULTS: 101 children (59% boys, Median age 9yrs) had AP of which 37.6%, 60.4% and 2% had mild, moderately severe and severe AP. 61.4%(62) had PFC at diagnosis; 34%(21) acute pancreatic fluid collections (APFC) and 66%(41) acute necrotic collections (ANC). 52.3%(11of21) of APFC evolved into pseudocysts & 68.2%(28of41) of ANC into WON's. Drainage was required in 31%(12of39) of persisting collections, more frequently in children with traumatic AP. Percutaneous catheter drainage (PCD) was done in 6 children and endoscopic ultrasound (EUS) guided cystogastrostomy with placement of plastic or self expanding metal stents (SEMS) in 6 children. CONCLUSIONS: Moderately severe AP is common in hospitalized children with AP with PFC developing in 61.4%, majority being APFC. 48% of APFC and 32% of ANC will resolve and the rest evolve into pseudocyst or WON. Spontaneous resolution is more likely in children with non -traumatic AP having pseudocysts rather than WON's.

Immunoglobulin G4-related disease mimicking gallbladder cancer with associated choledochal cyst: A case report of a malignant masquerade.

Immunoglobulin G4 (IgG4)-related disease is a recently described autoimmune disease that can involve diverse organ systems, causing pancreatitis, cholangitis, retroperitoneal fibrosis, and thyroiditis to name a few. Key histological features include storiform fibrosis, obliterative venulitis, and intense inflammatory infiltrate composed of lymphoplasmacytic cells. The disease has a tendency to present with mass-forming lesions, often difficult to differentiate from malignant processes. We report the case of a 48-year-old male from an area endemic for gallbladder cancer (north India). He presented with a short history of abdominal pain and was found to have asymmetric thickening of the gallbladder wall with a soft-tissue mass invading the adjacent liver. In addition, the bile duct was dilated throughout its extent. A clinical and radiological diagnosis of gallbladder cancer with choledochal cyst was made, and the patient underwent radical cholecystectomy with bile duct excision. Histopathology surprisingly demonstrated IgG4-related disease with no evidence of malignancy. Notably, serum levels of immunoglobulins were found to be normal. Preoperative diagnosis was challenging due to the absence of other manifestations. IgG4-related disease is a possible diagnostic pitfall and should be included as a possible differential diagnosis for gallbladder masses.

Pleural metastasis detected by transesophageal endoscopic ultrasonography.

Patients with pleural effusion and inconclusive repeated pleural fluid biochemical and cytological analysis are a diagnostic dilemma that is usually solved by invasive procedures such as diagnostic thoracoscopy. We report a 62-year-old male with malignant pleural effusion as well as ascites who was diagnosed using endoscopic ultrasound-guided fine-needle aspiration of pleural deposit. We also report contrast enhanced endoscopic ultrasound findings of pleural deposits.

An international, multi-institution survey of the use of EUS in the diagnosis of pancreatic cystic lesions.

BACKGROUND AND OBJECTIVES: Currently, pancreatic cystic lesions (PCLs) are recognized with increasing frequency and have become a more common finding in clinical practice. EUS is challenging in the diagnosis of PCLs and evidence-based decisions are lacking in its application. This study aimed to develop strong recommendations for the use of EUS in the diagnosis of PCLs, based on the experience of experts in the field. METHODS: A survey regarding the practice of EUS in the evaluation of PCLs was drafted by the committee member of the International Society of EUS Task Force (ISEUS-TF). It was disseminated to experts of EUS who were also members of the ISEUS-TF. In some cases, percentage agreement with some statements was calculated; in others, the options with the greatest numbers of responses were summarized. RESULTS: Fifteen questions were extracted and disseminated among 60 experts for the survey. Fifty-three experts completed the survey within the specified time frame. The average volume of EUS cases at the experts' institutions is 988.5 cases per year. CONCLUSION: Despite the limitations of EUS alone in the morphologic diagnosis of PCLs, the results of the survey indicate that EUS-guided fine-needle aspiration is widely expected to become a more valuable method.

Unusual tubular cystic lesion compressing inferior vena cava.

Fluid collections are important local complications of acute pancreatitis (AP). They are usually located in peri-pancreatic region but can also be found in various atypical locations like mediastinum, spleen, liver, pelvis or neck. Such pancreatic fluid collections in atypical locations can cause diagnostic dilemma and may cause delay in management. We report an interesting case of AP who presented to us with a walled-off necrosis mimicking a long cystic mass lesion surrounding the IVC.

Significance of ileal and/or cecal wall thickening on abdominal computed tomography in a tropical country.

BACKGROUND AND AIM: Clinical significance of ileocecal thickening on computed tomography (CT) is uncertain. We conducted this prospective study to determine the clinical relevance of ileal and/or cecal thickening on CT. METHODS: All patients with ileocecal thickening on CT were prospectively evaluated with ileocolonoscopy, biopsy, and other relevant investigations. RESULTS: Fifty patients (29 males, mean age 36.8 ± 13.21 years) were studied. Thirty nine (78%) patients presented with abdominal pain. On CT, 46 (92%) had a thickened wall of terminal ileum, 25 (50%) cecum, and 21 (42%) of both cecum and ileum. The mean wall thickness of ileum and cecum on CT was 7.23 + 3.2 mm and 5.5 + 3.1 mm, respectively. Final diagnosis was tuberculosis in 24 (48%) patients, Crohn's disease (CD) in 10 (20%), and adenocarcinoma in 1 patient. Colonoscopy demonstrated abnormal findings in 41 patients (82% patients with mucosal ulcerations being most common (n = 20 (40%). Of 15 (30%) patients with ileocecal bowel wall thickening, 4 (8%) patients had normal colonoscopy and histopathology (incidental ileocecal wall thickening), and in the remaining 11 patients, histopathology showed non-specific findings and these patients were asymptomatic without any specific treatment on last follow up ranging from 3 to 24 months. Involvement of cecum with ileocecal junction, ascending colon involvement, peri-ileocecal stranding, and long-segment stricture was significantly more common in patients with underlying disease as compared to nondiseased patients (P < 0.05). CONCLUSION: A majority of patients with ileocecal wall thickening on CT have an underlying disease and should be further investigated by ileocolonoscopy and biopsy.

Endoscopic Ultrasound-Guided Tissue Acquisition: Techniques and Challenges.

Endoscopic ultrasound-guided fine needle aspiration (EUS FNA) has made pathological diagnosis of pancreatic neoplasms, diseases involving lymph nodes at various mediastinal and abdominal sites, gastrointestinal submucosal lesions, perirectal lesions, adrenal lesions, and mediastinal masses easy. EUS-guided FNA is a multistep procedure that involves assessment of proper clinical indication, correct selection of FNA needles, and adoption of evidence-based techniques for tissue sampling. EUS FNA is done by needles that are available in different sizes, mainly 25, 22, and 19-gauge needle. The need of onsite cytopathologist, dependence on histology/core biopsy occasionally to get a diagnosis, and inability to reliably assess for molecular markers are important limitations of EUS FNA. EUS-guided fine needle biopsy (FNB) that samples the core of tissue is an exciting new development in the field of diagnostic EUS. FNB needles are expensive than FNA needles, and although the initial results are encouraging, more studies with robust evidence proving their superiority beyond any doubt are needed before they can be widely used.

A multi-institution consensus on how to perform EUS-guided biliary drainage for malignant biliary obstruction.

BACKGROUND AND OBJECTIVES: EUS-guided biliary drainage (EUS-BD) was shown to be useful for malignant biliary obstruction (MBO). However, there is lack of consensus on how EUS-BD should be performed. METHODS: This was a worldwide multi-institutional survey among members of the International Society of EUS conducted in February 2018. The survey consisted of 10 questions related to the practice of EUS-BD. RESULTS: Forty-six endoscopists of them completed the survey. The majority of endoscopists felt that EUS-BD could replace percutaneous transhepatic biliary drainage after failure of ERCP. Among all EUS-BD methods, the rendezvous stenting technique should be the first choice. Self-expandable metal stents (SEMSs) were recommended by most endoscopists. For EUS-guided hepaticogastrostomy (HGS), superiority of partially-covered SEMS over fully-covered SEMS was not in agreement. 6-Fr cystotomes were recommended for fistula creation. During the HGS approach, longer SEMS (8 or 10 cm) was recommended. During the choledochoduodenostomy approach, 6-cm SEMS was recommended. During the intrahepatic (IH) approach, the IH segment 3 was recommended. CONCLUSION: This is the first worldwide survey on the practice of EUS-BD for MBO. There were wide variations in practice, and randomized studies are urgently needed to establish the best approach for the management of this condition.

Clinical profile and treatment outcomes in autoimmune pancreatitis: a report from North India.

BACKGROUND: Autoimmune pancreatitis (AIP) is a rare disease, and data from countries like India concerning its clinical presentation and long-term outcomes are scarce. We retrospectively evaluated the clinical presentation, imaging features and treatment outcomes of patients with AIP. METHODS: We carried out a retrospective analysis of our database to identify patients diagnosed with and treated for AIP at our unit in a tertiary care hospital in North India. RESULTS: Eighteen patients with AIP (mean age: 54.9±11.1 years; 13 male) were evaluated. Of these, 9 (50%) patients had probable type 1 AIP, 2 (11%) patients probable type 2 AIP, and 4 (22%) definite type 1 AIP. Patients with type 2 AIP were significantly younger than patients with type 1 (40.0±2.8 vs. 58.4±9.6 years). In type 1 AIP, other organ involvement was observed in 3/18 (17%) patients, whereas both patients with type 2 AIP had coexisting ulcerative colitis. The diagnosis of AIP was made after resective surgery in 6/18 (33.0%) patients. An accurate diagnosis of AIP could be made in all patients who underwent resection or core biopsy, but cytological examination after endoscopic ultrasound-guided fine-needle aspiration could not provide a definitive diagnosis in any patient. Initial treatment with steroids was given to 12 (67%) patients, with a 100% response, but the disease relapsed in 5/13 (38%) patients over a mean follow-up period of 34.2±21.6 weeks. CONCLUSION: AIP is not rare in India and the majority of clinical manifestations, imaging features, treatment response and long-term outcomes are similar to those reported in the literature.

Chylous ascites due to idiopathic chronic pancreatitis managed with endoscopic stenting.

Chylous ascites is an uncommon clinical entity, which usually results from congenital or acquired abnormality in proper lymphatic flow. While the common causes are related to trauma, malignancy or tuberculosis, pancreatic disorders can occasionally result in chylous ascites. Sporadic cases of chylous ascites due to acute or chronic pancreatitis or surgery for pancreatitis have been reported. The usual management is with medium chain triglycerides (MCT), total parenteral nutrition and octreotide. We report a case of a young male with idiopathic chronic pancreatitis who presented with chylous ascites which did not respond to MCT based diet and octreotide. The patient improved with endoscopic pancreatic duct stenting.

Frequency & predictors of pancreatitis in symptomatic primary hyperparathyroidism.

BACKGROUND & OBJECTIVES: The frequency and predictors of pancreatitis in primary hyperparathyroidism (PHPT) are not well understood. The objective of the present study was to evaluate the frequency of pancreatitis in patients with PHPT and its association with clinical and biochemical parameters of the disease. METHODS: In this retrospective study all consecutive patients with PHPT registered in the PHPT registry (www.indianphptregistry.com) from the year 2004 to 2013 were included. The clinical, biochemical and radiological parameters related to pancreatitis were evaluated in histologically proven PHPT patients. RESULTS: A total of 218 patients (63 men; mean age: 40.6±14.4 yr) underwent surgery for PHPT during the study. Pancreatitis occurred in 35 [16%, 18 acute and 17 chronic pancreatitis (CP)] patients and male:female ratio was 1:0.94. Skeletal manifestations were seen less frequently in PHPT with pancreatitis as compared to that of PHPT without pancreatitis. PHPT with pancreatitis had significantly higher serum calcium (12.4±2.0 vs. 11.7±1.5 mg/dl, P <0.05) in comparison to PHPT without pancreatitis. PHPT with acute pancreatitis (AP) had higher serum calcium (P <0.05) and parathyroid hormone (PTH) (P <0.05) levels than PHPT with CP. Curative parathyroidectomy improved the symptoms associated with pancreatitis as there was no recurrence in AP group, whereas recurrence was observed only in about 10 per cent patients of the CP group. INTERPRETATION & CONCLUSIONS: Pancreatitis was observed in 16 per cent of PHPT patients with male predominance in the study population. No recurrence of AP was observed after curative surgery. It may be proposed that serum amylase with calcium and PTH should be measured in all patients of PHPT with pain abdomen to rule out pancreatitis.