Endoscopic ultrasound-guided radiofrequency ablation of pancreatic insulinoma: a state of the art review.

INTRODUCTION: Insulinomas are the most common functional pancreatic neuroendocrine tumors (PNETs) that lead to incapacitating hypoglycemia. Guidelines recommend surgical resection as the mainstay of management. However, surgery is fraught with complications, causing significant peri/post-operative morbidity. Since insulinomas are usually benign, solitary, small (<2 cm), and do not need lymphadenectomy, hence, in this regard, endoscopic ultrasound-guided radiofrequency ablation (EUS-RFA) is now being increasingly performed, to circumvent these adverse events and impairment of pancreatic function. AREAS COVERED: A comprehensive literature search was undertaken across various databases (PubMed/MEDLINE, Embase, Scopus), with no language restriction, for relevant articles (case series, reviews, case reports) pertaining to EUS-RFA for insulinoma and PNETs, till October 2023. In this review, we have explicated the role of EUS-RFA for insulinoma management, detailing thoroughly its mechanism of action, EUS-RFA devices with data on its safety and efficacy, and an algorithmic approach for its management. EXPERT OPINION: EUS-RFA is being advocated as a 'mini-invasive' option with the potential to replace surgery as a first-line approach for benign, sporadic, solitary, and small (<2 cm) insulinomas. Under real-time guidance, EUS-RFA has immense precision, is safe, predictable, with acceptable safety profile. Presently, it is being frequently performed for high-risk or inoperable candidates. Current need-of-the-hour is a randomized controlled trial to substantiate its role in the therapeutic algorithm for insulinoma management.

Lumen-apposing metal stents for the treatment of pancreatic and peripancreatic fluid collections and bleeding risk: a propensity matched study.

INTRODUCTION: Endoscopic ultrasound (EUS)-guided drainage of symptomatic pancreatic fluid collections (PFCs) using the Hot-Axios device has recently been associated with a significant risk of bleeding. This adverse event (AE) seems to occur less frequently with the use of a different device, the Spaxus stent. The aim of the current study was to compare the rates of bleeding between the two stents. METHODS: Patients admitted for treatment of PFCs by EUS plus lumen-apposing metal stent in 18 endoscopy referral centers between 10 July 2019 and 28 February 2022 were identified and their outcomes compared using a propensity-matching analysis. RESULTS: 363 patients were evaluated. After a 1-to-1 propensity score match, 264 patients were selected (132 per group). The technical and clinical success rates were comparable between the two groups. Significantly more bleeding requiring transfusion and/or intervention occurred in the Hot-Axios group than in the Spaxus group (6.8% vs. 1.5%; P = 0.03); stent type was a significant predictor of bleeding in both univariate and multivariate regression analyses (P = 0.03 and 0.04, respectively). Bleeding necessitating arterial embolization did not however differ significantly between the two groups (3.0% vs. 0%; P = 0.12). In addition, the Hot-Axios was associated with a significantly higher rate of overall AEs compared with the Spaxus stent (9.8% vs. 3.0%; P = 0.04). CONCLUSION: Our study showed that, in patients with PFCs, bleeding requiring transfusion and/or intervention occurred significantly more frequently with use of the Hot-Axios stent than with the Spaxus stent, although this was not the case for bleeding requiring embolization.

Role of Novel Motorized Enteroscopy in the Evaluation of Small Bowel Diseases: A Systematic Review and Meta-analysis.

BACKGROUND AND AIM: Motorized spiral enteroscopy (MSE) has recently been introduced for small bowel evaluation. In this systematic review and meta-analysis, we aim to evaluate the safety and efficacy of MSE for evaluation of small bowel diseases. METHODS: A literature search was performed in Embase, PubMed, Medline databases for studies evaluating MSE between January -2010 and October-2022. The primary outcome of the study was diagnostic yield with MSE. Secondary outcomes included technical success, procedure duration, depth of maximum insertion (DMI), rate of pan-enteroscopy and adverse events. RESULTS: 10 studies with 961 patients [581 (60.5%) males] were included in the analysis. 1068 MSE procedures were performed by antegrade route in 698, retrograde route in 215 and bidirectional in 155 patients. Technical success was achieved in 94.9% (95% CI 92.9% to 96.4%) procedures. The pooled diagnostic yield of MSE was 73.7% (95% CI 70.7% to 76.4%). Pooled rate of pan-enteroscopy by antegrade route was 21.9% (95% CI 18.1% to 26.1%), retrograde route was 6.9% (95% CI 2.4% to 18.3%) and combined route was 61.2% (95% CI 52.4% to 69.3%). Pooled rate of major adverse events was 1.9% (95% CI 1.2% to 3.2%). CONCLUSIONS: MSE is a safe and effective tool for evaluating small bowel disorders. High diagnostic yield and low rate of adverse events make it a potential alternative to balloon enteroscopy. However, comparative trials are required in the future.

Device-assisted endoscopic full-thickness resection in colorectum: Systematic review and meta-analysis.

OBJECTIVES: Endoscopic full-thickness resection (EFTR) is emerging as an effective modality for mucosal and submucosal lesions in the colorectum. In this systematic review and meta-analysis, we aimed to analyze the success and safety of device-assisted EFTR in the colon and rectum. METHODS: A literature search was performed in the Embase, PubMed, and Medline databases for studies evaluating device-assisted EFTR between inception to October 2022. The primary outcome of the study was clinical success (R0 resection) with EFTR. Secondary outcomes included technical success, procedure duration, and adverse events. RESULTS: In all, 29 studies with 3467 patients (59% male patients, 3492 lesions) were included in the analysis. The lesions were located in right colon (47.5%), left colon (28.6%), and rectum (24.3%). EFTR was performed for subepithelial lesions in 7.2% patients. The pooled mean size of the lesions was 16.6 mm (95% confidence interval [CI] 14.9-18.2, I2 98%). Technical success was achieved in 87.1% (95% CI 85.1-88.9%, I2 39%) procedures. The pooled rate of en bloc resection was 88.1% (95% CI 86-90%, I2 47%) and R0 resection was 81.8% (95% CI 79-84.3%, I2 56%). In subepithelial lesions, the pooled rate of R0 resection was 94.3% (95% CI 89.7-96.9%, I2 0%). The pooled rate of adverse events was 11.9% (95% CI 10.2-13.9%, I2 43%) and major adverse events requiring surgery was 2.5% (95% CI 2.0-3.1%, I2 0%). CONCLUSION: Device-assisted EFTR is a safe and effective treatment modality in cases with adenomatous and subepithelial colorectal lesions. Comparative studies are required with conventional resection techniques, including endoscopic mucosal resection and submucosal dissection.

The role of endoscopy in malignant hilar obstruction.

Malignant hilar biliary obstruction (MHO) is a medical challenge as regards both forming a correct diagnosis and its adequate management, in terms of treatment alternatives and palliative options. Surgical resection is the only curative treatment for the underlying disease, but the majority of patients are not suitable candidates because of an unresectable tumor or poor performance status. Biliary drainage (BD) can be achieved through the percutaneous transhepatic route or endoscopically, and the choice depends on a host of factors, including biliary anatomy and comorbidity of the patient. Though there is no consensus, the endoscopic approach is usually preferred over the former. Endoscopy can aid in both diagnosis (collection of histological as well as cytological samples, direct visualization of suspected malignant pathology, or use of endoscopic ultrasound [EUS] for evaluation and locoregional staging), and in achieving internal BD. Advances in the development of various stents, accessories and, more recently, the use of EUS have in fact further expanded its application in MHO management. The choice of stents to be used (type, make, and number), palliation methods, deployment techniques and the use of local ablative strategy are still evolving and require more data. The complexity of management of MHO mandates that each patient should receive a "personalized approach", all the way from establishing a diagnosis until final treatment, with the help of a multidisciplinary team effort. Herein, we provide a comprehensive literature review of the current role of endoscopy for MHO, according to its applications in various clinical settings.

Cholangiocarcinoma in the Era of Immunotherapy.

Cholangiocarcinoma (CCA) is a rare malignancy of the gastrointestinal tract, with aggressive behavior, and portends a poor prognosis. Traditionally, it is classified according to its site of involvement as intrahepatic, perihilar, and distal cholangiocarcinoma. A host of genetic and epigenetic factors have been involved in its pathogenesis. Chemotherapy has remained the standard first-line treatment over the last decade, with a disappointing median overall survival of 11 months for locally advanced and metastatic CCA. The advent of immunotherapy has revolutionized the treatment of many pancreaticobiliary malignancies, offering durable responses with a safe therapeutic profile. To date, there have been no significant advances in the management of CCA. Novel immunotherapeutic methods, such as cancer vaccines, adoptive cell therapy, and combinations of immune checkpoint inhibitors with other agents, are currently under investigation and may improve prognosis with overall survival. Efforts to find robust biomarkers for response to treatment along with multiple clinical trials are also ongoing in this regard. In this review, we present an overview of the current advances and the future perspectives of immunotherapy in the management of CCA.

Endoscopic ultrasound-guided vascular interventions: An expanding paradigm.

Endoscopic ultrasound (EUS) has expanded its arena from a mere diagnostic modality to an essential therapeutic tool in managing gastrointestinal (GI) diseases. The proximity of the GI tract to the vascular structures in the mediastinum and the abdomen has facilitated the growth of EUS in the field of vascular interventions. EUS provides important clinical and anatomical information related to the vessels' size, appearance and location. Its excellent spatial resolution, use of colour doppler with or without contrast enhancement and ability to provide images "real-time" helps in precision while intervening vascular structures. Additionally, structures such as venous collaterals or varices can be dealt with optimally using EUS. EUS-guided vascular therapy with coil and glue combination has revolutionized the management of portal hypertension. It also helps to avoid radiation exposure in addition to being minimally invasive. These advantages have led EUS to become an upcoming modality to complement traditional interventional radiology in the field of vascular interventions. EUS-guided portal vein (PV) access and therapy is a new kid on the block. EUS-guided portal pressure gradient measurement, injecting chemotherapy in PV and intrahepatic portosystemic shunt has expanded the horizons of endo-hepatology. Lastly, EUS has also forayed into cardiac interventions allowing pericardial fluid aspiration and tumour biopsy with experimental data on access to valvular apparatus. Herein, we provide a comprehensive review of the expanding paradigm of EUS-guided vascular interventions in GI bleeding, portal vein access and its related therapeutic interventions, cardiac access, and therapy. A synopsis of all the technical details involving each procedure and the available data has been tabulated, and the future trends in this area have been highlighted.

Same-session double EUS-guided bypass versus surgical gastroenterostomy and hepaticojejunostomy: an international multicenter comparison.

BACKGROUND AND AIMS: Gastric outlet and biliary obstruction are common manifestations of GI malignancies and some benign diseases for which standard treatment would be surgical gastroenterostomy and hepaticojejunostomy (ie, "double bypass"). Therapeutic EUS has allowed for the creation of an EUS-guided double bypass. However, same-session double EUS-guided bypass has only been described in small proof-of-concept series and lacks a comparison with surgical double bypass. METHODS: A retrospective multicenter analysis was performed of all consecutive same-session double EUS-guided bypass procedures performed in 5 academic centers. Surgical comparators were extracted from these centers' databases from the same time interval. Efficacy, safety, hospital stay, nutrition and chemotherapy resumption, long-term patency, and survival were compared. RESULTS: Of 154 identified patients, 53 (34.4%) received treatment with EUS and 101 (65.6%) with surgery. At baseline, patients undergoing EUS exhibited higher American Society of Anesthesiologists scores and a higher median Charlson Comorbidity Index (9.0 [interquartile range {IQR}, 7.0-10.0] vs 7.0 [IQR, 5.0-9.0], P < .001). Technical success (96.2% vs 100%, P = .117) and clinical success rates (90.6% vs 82.2%, P = .234) were similar when comparing EUS and surgery. Overall (11.3% vs 34.7%, P = .002) and severe adverse events (3.8% vs 19.8%, P = .007) occurred more frequently in the surgical group. In the EUS group, median time to oral intake (0 days [IQR, 0-1] vs 6 days [IQR, 3-7], P < .001) and hospital stay (4.0 days [IQR, 3-9] vs 13 days [IQR, 9-22], P < .001) were significantly shorter. CONCLUSIONS: Despite being used in a patient population with more comorbidities, same-session double EUS-guided bypass achieved similar technical and clinical success and was associated with fewer overall and severe adverse events when compared with surgical gastroenterostomy and hepaticojejunostomy.

Dynamics of Serum Procalcitonin Can Predict Outcome in Patients of Infected Pancreatic Necrosis: A Prospective Analysis.

BACKGROUND: Timely intervention can alter outcome in patients of infected pancreatic necrosis (IPN) but lacks adequate biomarker. Role of serum procalcitonin (PCT) in the management of IPN is understudied, and hence, this study was planned. METHODOLOGY: All patients of acute pancreatitis with IPN without prior intervention were included. Baseline demographic, radiological and laboratory parameters were documented. PCT was measured at baseline, prior to intervention, and thereafter every 72 h. Patients were grouped into those having baseline PCT < 1.0 ng/mL and those with PCT ≥ 1.0 ng/mL and various outcome measures were compared. RESULTS: Of the 242 patients screened, 103 cases (66 males; 64.1%) with IPN were grouped into 2: PCT < 1.0 ng/mL (n = 29) and PCT ≥ 1.0 ng/mL (n = 74). Patients with baseline PCT ≥ 1.0 ng/mL had significantly more severe disease scores. 16 out of 19 patients with rise in PCT on day-7 post-intervention expired. PCT ≥ 1.0 ng/mL group had higher need for ICU (p = 0.001) and mortality (p = 0.044). PCT > 2.25 ng/mL (aOR 22.56; p = 0.013) at baseline and failure in reduction of PCT levels to < 60% of baseline at day-7 post-intervention (aOR 53.76; p = 0.001) were significant mortality predictors. CONCLUSION: Baseline PCT > 1.0 ng/mL is associated with poor outcome. PCT > 2.25 ng/mL and failure in reduction of PCT levels to < 60% of its baseline at day-7 post-intervention can identify high-mortality risk patients.

EUS-guided biliary drainage in patients with moderate-severe cholangitis is safe and effective: a multi-center experience.

BACKGROUND: Patients with moderate-severe cholangitis require urgent/early biliary drainage and failed endoscopic retrograde cholangiopancreatography (ERCP) warrants use of percutaneous drainage. While endoscopic ultrasound-guided biliary drainage (EUS-BD) has evolved as an effective salvage modality but its safety and efficacy data in moderate-severe cholangitis are limited. PATIENTS AND METHODS: All consecutive moderate-severe cholangitis patients, with failed/technically non-feasible ERCP requiring EUS-BD in two tertiary care centers were included. Baseline laboratory and demographic parameters were documented. Technical and clinical success were primary outcome measures. Additionally, effective biliary drainage, adverse events due to procedure, hospital stay, ICU stay, and mortality were noted. RESULTS: Of the 49 patients (23 male; 46.9%) presenting with moderate/severe cholangitis, 23 (46.9%) had severe cholangitis. The median Charleston comorbidity index was 7.0 (IQR 2.0). Majority had malignant disease (87.8%) and 25 (51.0%) had inaccessible papilla. Technical success was achieved in 48 cases (98.0%), while clinical success with improvement of cholangitis was noted in 44 of 48 cases (91.7%). Effective biliary drainage was noted in 85.4% (41/48) cases. Adverse events in the form of mostly bleeding and bile leak were noted in 5 cases (10.2%) but managed conservatively. Distal obstruction exhibited significantly better clinical success (100% vs. 78.9%; p = 0.02) than hilar obstruction. Severe cholangitis had significantly lower clinical success (81.8% vs. 100%; p = 0.04) than moderate cholangitis. CONCLUSION: EUS-BD can be a safe and effective alternative option for patients with moderate to severe cholangitis, even with significant pre-morbid conditions, with acceptable adverse events rate.

The expanding role of endoscopic ultrasound elastography.

Endoscopic ultrasound (EUS) is an invaluable tool for assessing various GI diseases. However, using just the conventional B-mode EUS imaging may not be sufficient to accurately delineate the lesion's character. Using the principle of stress-induced tissue strain, EUS elastography (EUS-E) can help in the real-time sonographic assessment of the level of tissue stiffness or hardness of any organ of interest during a routine EUS procedure. Thus, EUS-E can better characterize the lesion's nature and highlight the more suspicious areas within an individual lesion. The most commonly studied lesions with EUS-E are the pancreatic lesions, namely, chronic pancreatitis, pancreatic cancer, and lymph nodes. However, EUS-E is gradually expanding its use for lesion characterization of the liver, bile duct, adrenals, gastrointestinal tract, and even therapy response. Moreover, the use of EUS-E along with other image enhancement techniques such as harmonic EUS and contrast-enhanced EUS can improve the accuracy of the diagnosis. However, several technical aspects need to be standardized before EUS-E can be truly used as a tool for "virtual biopsy". This review focuses on the various technical aspects of the use of EUS-E, it is established and expanding indications and an extensive outline of the various studies on EUS-E. We also discuss the current pitfalls and future trends in EUS-E.

Comparison between endoscopic ultrasound-guided fine-needle biopsy and bite-on-bite jumbo biopsy for sampling of subepithelial lesions.

BACKGROUND AND AIMS: A direct comparison between endoscopic ultrasound (EUS) fine-needle biopsy (FNB) and current endoscopic biopsy techniques in patients with subepithelial lesions (SELs) is still lacking. Aim of this multicenter study was to compare the diagnostic performance and safety profile between EUS-FNB and bite-on-bite jumbo biopsy. METHODS: Out of 416 patients undergoing endoscopic sampling of SELs between 2017 and 2021, after propensity score matching two groups were compared: 120 undergoing EUS-FNB and 120 sampled with bite-on-bite jumbo biopsy. Primary outcome was sample adequacy. Secondary outcomes were diagnostic accuracy, sensitivity, specificity, and adverse events. RESULTS: Median age was 61 years and most patients were male in both groups. Final diagnosis was GIST in 65 patients (54.1%) in the EUS-FNB group and 62 patients in the bite-on-bite biopsy group (51.6%; p = 0.37). Sample adequacy was significantly higher in the EUS-FNB group as compared to the bite-on-bite biopsy group (94.1% versus 77.5%, p<0.001). EUS-FNB outperformed bite-on-bite biopsy also in terms of diagnostic accuracy (89.3% versus 67.1%, p<0.001) and sensitivity (89% vs 64.5%; p<0.001), whereas specificity was 100% in both groups (p = 0.89). These findings were confirmed in subgroup analysis according to SEL location, final diagnosis, and wall layers of the sampled SEL. Adverse event rate was 6.6% in the EUS-FNB group and 30% in the bite-on-bite biopsy group (p<0.001). CONCLUSION: EUS-FNB outperforms bite-on-bite biopsy both in terms of diagnostic yield and safety profile.

Endoscopic Ultrasound-guided Drainage of Patients With Infected Walled-off Necrosis: Which Stent to Choose?

BACKGROUND: Endoscopic ultrasound (EUS)-guided drainage is the preferred treatment of pancreatic fluid collections (PFC). However, the choice of the stent for EUS-guided drainage in critically ill PFC cases with infected walled-off necrosis (WON) and/or organ failure (OF) remains unknown. MATERIALS AND METHODS: Between January 2018 and December 2019, consecutive patients with symptomatic PFC subjected to EUS-guided drainage using biflanged metal stents (BFMS) or double-pigtail plastic stents (DPPS) were compared for technical success, clinical success, duration of the procedure, need for intensive care unit stay, duration of intensive care unit stay, ventilator need, resolution of OF, the duration for resolution of OF, complications, need for salvage percutaneous drainage or surgery and mortality. A subgroup of patients having infected WON with/without OF were analyzed separately. RESULTS: Among 120 patients (84.6% males) with PFC (108 WON, 22 pseudocyst) who underwent EUS-guided drainage, there was no difference in outcome parameters in BFMS and DPPS groups. Among patients with WON, clinical success was significantly higher (96.2% vs. 81.8%, P=0.04), with significantly shorter hospital stay (6 vs. 10 d) and procedure duration (17.18±4.6 vs. 43.6±9.7 min, P<0.0001) in the BFMS group. Among patients with infected WON with/without OF, the clinical success was significantly higher (100% vs. 73.9%, P=0.02), and the duration of the procedure was significantly lower (16.28±4.4 vs. 44.39±10.7, P<0.0001) in BFMS compared with DPPS group. CONCLUSION: EUS-guided drainage of WON using BFMS scores over DPPS. In patients having infected WON with/without OF, BFMS may be preferred over DPPS.

Role of therapeutic endoscopic ultrasound in gastrointestinal malignancy- current evidence and future directions.

Endoscopic ultrasound (EUS) has come a long way from a mere diagnostic tool to an advanced therapeutic modality. With the advent of better technologies and accessories, EUS has found ground in the management of gastrointestinal (GI) malignancies, not only for diagnosis but also for therapeutic purposes. EUS can tackle a host of conditions, including hepato-pancreatico-biliary malignancies. Advances and experience in various EUS-guided biliary drainage techniques have enabled the endosonologist to tackle biliary obstruction when conventional techniques of endoscopic retrograde cholangiopancreatography (ERCP) and/or percutaneous transhepatic biliary drainage (PTBD) fails. More and more emerging data not only establishes the safety of EUS-BD but also demonstrates superior efficacy over PTBD and sometimes even ERCP. Malignant gastric outlet obstruction can now be safely managed with EUS-guided gastroenterostomy. Starting from pain management in malignant tumors through celiac plexus neurolysis to various tumor ablative therapies, EUS has forged ahead over percutaneous treatment or surgical options in the management of GI malignancies. Additional data is now coming up on the prospects of EUS-guided immunotherapy and biological therapy for tumor management. The future of EUS therapeutics in the field of GI malignancies is bright. With increasing evidence, this modality becoming a key player in management of a host of complex clinical conditions arising out of GI malignancies is in the offing. This review focuses on elucidating the role of therapeutic EUS in the management of GI malignancies, a synopsis of various techniques, data on its safety and efficacy as well as future advancements in this domain.

Role of endoscopic ultrasound in the field of hepatology: Recent advances and future trends.

The role of endoscopic ultrasound (EUS) as a diagnostic and therapeutic modality for the management of various gastrointestinal diseases has been expanding. The imaging or intervention for various liver diseases has primarily been the domain of radiologists. With the advances in EUS, the domain of endosonologists is rapidly expanding in the field of hepatology. The ability to combine endoscopy and sonography in one hybrid device is a unique property of EUS, together with the ability to bring its probe/transducer near the liver, the area of interest. Its excellent spatial resolution and ability to provide real-time images coupled with several enhancement techniques, such as contrast-enhanced (CE) EUS, have facilitated the growth of EUS. The concept of "Endo-hepatology" encompasses the wide range of diagnostic and therapeutic procedures that are now gradually becoming feasible for managing various liver diseases. Diagnostic advancements can enable a wide array of techniques from elastography and liver biopsy for liver parenchymal diseases, to CE-EUS for focal liver lesions to portal pressure measurements for managing various liver conditions. Similarly, therapeutic advancements range from EUS-guided eradication of varices, drainage of bilomas and abscesses to various EUS-guided modalities of liver tumor management. We provide a comprehensive review of all the different diagnostic and therapeutic EUS modalities available for the management of various liver diseases. A synopsis of all the technical details involving each procedure and the available data has been tabulated, and the future trends in this area have been highlighted.

Corona Virus Disease-19 pandemic: The gastroenterologists' perspective.

The world is witnessing a serious public health threat in the wake of the third corona virus pandemic, a novel corona virus (severe acute respiratory syndrome coronavirus 2 [SARS-CoV-2]). The Corona Virus Disease-19 (COVID-19) is not limited to the respiratory system but has widespread involvement including the gastrointestinal (GI) tract and liver, with evidence of prolonged fecal shedding and feco-oral transmission. This finding has stirred up a hornet's nest of not only a newer modality of the spread of the virus but also a risk of the unpredictable duration of the infective potential of the shedders. We reviewed the literature on fecal shedding and possible implications on prevention and surveillance strategies. The pandemic is changing the management of underlying chronic diseases such as inflammatory bowel disease (IBD) and other diseases. Moreover, for the gastroenterologist, doing endoscopic procedures in this COVID-19 era poses a high risk of contamination, as it is an aerosol-generating procedure. There is a daily influx of data on this disease, and multiple societies are coming up with various recommendations. We provide a comprehensive review of all the reported GI manifestations of COVID-19 infection and the side effects of confounding drugs. We have summarized the management recommendations for diseases such as IBD with COVID-19 and nutritional recommendations and provided a concise review of the endoscopy guidelines by the various societies. This review provides a comprehensive account and a lucid guide covering various aspects of gastroenterology practice during this COVID-19 pandemic.

Priapism as a Debut Presentation of Chronic Myeloid Leukemia.

Chronic myeloid leukemia (CML) is a chronic myeloproliferative disorder that usually presents with high white blood cell counts and massive splenomegaly. Priapism is a rare manifestation of CML and is mostly due to hyperleukocytosis. Its debut appearance as a sign of hematological dyscrasia is a rare event. Priapism occurring in a setting of any leukemia is both a medical and a urological emergency that requires immediate local therapy, symptomatic treatment, cytoreductive therapy and early initiation of targeted therapy. This case report describes priapism as an unusual presentation of CML and its importance in the work-up and management of patients presenting with priapism.