Pancreatoscopy of intraductal papillary mucinous neoplasm of the pancreas.

Video 1Pancreatoscopy of intraductal papillary neoplasm of the pancreas.

Safety and feasibility of same day discharge after per oral endoscopic pyloromyotomy in refractory gastroparesis: a pilot study.

BACKGROUND: Gastroparesis is a debilitating medical condition with limited treatment options. Gastric per-oral pyloromyotomy (G-POEM) has emerged as a promising treatment option with remarkable short-term clinical success shown in multiple studies. While the post-procedure protocol is not standardized across many centers, the majority of the centers observes these patients in the hospital after the procedure for monitoring. In this single-center prospective study, we evaluated the safety and feasibility of same day discharge after the G-POEM procedure. METHODS: All the patients with refractory gastroparesis undergoing G-POEM from October 2019 to March 2020 were enrolled. A total of 25 patients were enrolled in the procedure. Based on the pre-defined criteria, patients were either discharged on the same day after the procedure or admitted to the hospital for further observation. The patient and procedure-related data were extracted from the chart review. Univariate analysis was performed (chi-squared test) on categorical variables after organizing categorical variables as numeric counts or percentages. The student t test was performed on continuous variables after reporting as mean and standard deviation. For analysis with a smaller sample size, Fisher exact and Mann-Whitney tests were used. RESULTS: A total of 25 patients were enrolled. The technical success of G-POEM was 100% and clinical success was 80% (20/25) at 1-month follow-up. Of the 25 patients, 9 patients (36%) were discharged on the same day according to the procedure from the recovery unit. Of the remaining 16 patients who were admitted to the hospital post-procedure, 10 (40%) were admitted due to procedure-related causes while other admissions were either pre-planned or due to social reasons. The average Charlson comorbidity index was lower in the same day discharge group ( P   <  0.05). The number of patients requiring double myotomy was higher in the same day discharge group ( P  < 0.05). The overall complication rate of G-POEM in the study cohort was 12% (3/25) with all complications being mild without any severe adverse events. CONCLUSION: G-POEM is a safe and effective method of treatment for refractory GP with higher clinical success in short-term follow-up. The same day discharge after G-POEM is safe and feasible in >50% of patients with close periprocedural monitoring.

Quantification of Interstitial Cells of Cajal in the Gastric Muscle of Patients with Gastroparesis at Per-Oral Endoscopic Pyloromyotomy: A Novel Approach for Future Research in Pathogenesis of Gastroparesis.

BACKGROUND: The role of Interstitial Cells of Cajal (ICC) in the pathogenesis of gastroparesis has been suggested by previous studies due to their involvement in the transmission of neuronal signaling to the smooth muscles of the GI tract. However, studies have been limited by the inability to obtain a gastric muscle sample, since routine endoscopy can only biopsy the mucosa. We present a new technique of muscle biopsy during per-oral endoscopic pyloromyotomy (GPOEM), a novel endoscopic procedure for treatment of gastroparesis. PATIENTS AND METHODS: All enrolled patients had diagnosed gastroparesis and had biopsies of the muscular layer at the antrum/pylorus during POEM. All GPOEM procedures took place from August 2019 to December 2019. Various demographic, disease-related, and procedure-related data were collected from chart review. ICC in the biopsy specimen was examined and quantitated. RESULTS: Through this method, we readily expose the gastric muscle of 21 patients through dissection of a gastric submucosal tunnel during GPOEM and provide reliable muscle sample for ICC quantification. Average number of ICC were higher in clinical responders (88 ICC ± 63 vs. 39 ICC ± 24, p = 0.02), defined as those who experienced significant improvement in nausea and vomiting symptoms after GPOEM. CONCLUSIONS: This study provides a reliable novel biopsy method for safely biopsy gastric muscle for quantitating the number of gastric ICC in patients with gastroparesis. The number of ICC may be related to the outcome of GPOEM therapy. However, further studies with larger number of patients are needed to confirm the results.

Simultaneous Examination of Eosinophil Infiltration in Esophageal Mucosa and Muscle in Patients with Achalasia: Direct Biopsy of the Esophageal Muscle at Per-oral Endoscopic Myotomy.

BACKGROUND: The relationship between eosinophilic esophagitis (EoE) and achalasia is not completely understood. There have been reports of eosinophilic infiltration of all esophageal layers in patients with achalasia. However, a routine endoscopic biopsy of the muscular layer is usually not feasible. We evaluate the safety and efficacy of muscle layer biopsy during per-oral endoscopic myotomy (POEM) as well as the prevalence of eosinophilic infiltration of the esophageal mucosa and muscular layer in patients with achalasia. PATIENTS AND METHODS: All enrolled patients had diagnosed achalasia and had simultaneous biopsies of the muscular layer at the middle esophagus and distal esophageal sphincter as well as the mucosal layer of the proximal and distal esophagus during POEM. All POEM procedures took place from August 2018 to December 2018 or September 2019 to November 2019. Various demographic, disease-related, and procedure-related data were collected from chart review. Eosinophilic infiltration in the biopsy specimen was examined. KEY RESULTS: Twenty consecutive patients (65% female, age range: 21-84) with a pre-procedure Eckardt score of >6 were enrolled during the study period, with the duration of their achalasia ranging from 1 to 32 years. Eighteen patients had clinical symptomatic improvement after POEM, as defined by an Eckardt score <3. Endoscopic examination did not reveal any signs of eosinophilic esophagitis. Pathologic examination of biopsies revealed eosinophilic infiltration in three of 20 patients (15%) in the distal esophageal mucosa (all <15 eosinophils/HPF) and none in the proximal esophageal mucosa. There was no eosinophilic infiltration in the distal esophageal sphincter and the middle esophageal muscle. No complication was noted due to muscle biopsy. CONCLUSIONS AND INFERENCES: Submucosal tunneling during POEM provides a safe access for direct esophageal muscle biopsy. This is the first report of the simultaneous biopsy of the esophageal mucosa and muscle in patients with achalasia. Contrary to all previously published studies, the association of esophageal eosinophilic infiltration and achalasia was not observed in this small sample study. Based on our findings, immune or autoimmune reaction rather than direct eosinophilic infiltration in the muscle is more likely the cause of achalasia.

Gas Leak and Mucosal Injury During Endoscopic Esophageal Myotomy After Previous Myotomy: A Single-Center Experience.

PURPOSE: Peroral endoscopic myotomy (POEM) after prior myotomy (PM-POEM) can be technically challenging with possible increased adverse events. We aimed to assess gas leak and mucosal injury incidence during PM-POEM, compared to an index POEM (iPOEM), and post-procedure extubation time. PATIENTS AND METHODS: A retrospective study comparing PM-POEM to iPOEM from March 2016 to August 2018. RESULTS: There were 21 subjects in the PM-POEM and 56 subjects in the iPOEM. The PM-POEM group was younger (average age 44.33 vs 57.57 years, p=0.0082). Gas leak incidence did not differ between groups (28.6% in PM-POEM vs 14.3% in iPOEM, p=0.148). For cases with imaging available postoperatively, there was a trend towards higher incidence of gas leak in the PM-POEM, but it was not statistically significant (60% vs 42.1%, p=0.359). The post-procedure extubation time was not different between PM-POEM and iPOEM (11.38 vs 9.46 minutes, p=0.93), but it was longer when gas leak occurred (15.92 vs 8.67 minutes, p=0.027). The odds of mucosal injury were four-fold higher (OR, 4.31; 95% CI, 1.32-14.08), and more clips were used to close mucosal injuries (0.62 vs 0.14 clips, p=0.0053) in the PM-POEM group. More procedures were deemed difficult or challenging in the PM-POEM (33.3% vs 7.1%, p=0.007). The number of clips used to close the mucosotomy was not different between groups (4.05 vs 3.84 clips, p=0.498). Although the myotomy was shorter in PM-POEM, it was not statistically significant (6.38 vs 7.14 cm, p=0.074). However, the procedure was longer in PM-POEM (61.28 vs 45.39 minutes, p=0.0017). There was no intervention or ICU admission required pertinent to the procedure. CONCLUSION: Performing PM-POEM can be more difficult with more mucosal injuries. Gas leak was associated with a slightly longer post-procedure extubation time, but clinical relevance is unclear given incidence of gas leak was unknown at time of extubation.

Prevalence and Risk Factors for Intraductal Papillary Mucinous Neoplasm in Patients With Chronic Kidney Disease: A Single-Center Case-Control Study.

OBJECTIVES: The exact prevalence for intraductal papillary mucinous neoplasm (IPMN) in patients with chronic kidney disease (CKD) remains unknown. In this single-center case-control study, we aimed to study the prevalence and risk factors for IPMN in patients with CKD. METHODS: We performed a retrospective case-control study comparing patients with and without CKD who had magnetic resonance imaging of the abdomen performed between January 2018 and December 2018. Patient demographic, clinical, and imaging metrics were extracted from chart review. The prevalence of IPMN was compared between the 2 groups. RESULTS: A total of 800 patient charts were reviewed. There were 400 patients with CKD compared with an age-matched control group of 400 patients without CKD. The total prevalence of IPMN in patients with CKD was 13.7% (55/400) compared with 7.8% (29/400; P = 0.002) in non-CKD patients. The prevalence of diabetes mellitus was significantly higher in the CKD group (41% vs 14%, P = 0.0001). The percentage of patients consuming alcohol was significantly higher in the non-CKD group (23% vs 35%, P = 0.002). CONCLUSIONS: Patients with CKD have a significantly higher prevalence of IPMN compared with non-CKD patients. Larger population-based studies are needed to confirm these findings.

Long-term Outcome of Gastric Per-Oral Endoscopic Pyloromyotomy in Treatment of Gastroparesis.

BACKGROUND & AIMS: Gastric per oral endoscopic pyloromyotomy (GPOEM) is a promising treatment for gastroparesis. There are few data on the long-term outcomes of this procedure. We investigated long-term outcomes of GPOEM treatment of patients with refractory gastroparesis. METHODS: We conducted a retrospective case-series study of all patients who underwent GPOEM for refractory gastroparesis at a single center (n = 97), from June 2015 through March 2019; 90 patients had more than 3 months follow-up data and were included in our final analysis. We collected data on gastroparesis cardinal symptom index (GCSI) scores (measurements of postprandial fullness or early satiety, nausea and vomiting, and bloating) and SF-36 questionnaire scores (measures quality of life). The primary outcome was clinical response to GPOEM, defined as a decrease of at least 1 point in the average total GCSI score with more than a 25% decrease in at least 2 subscales of cardinal symptoms. Recurrence was defined as a return to baseline GCSI or GCSI scores of 3 or more for at least 2 months after an initial complete response. The secondary outcome was the factors that predict GPOEM failure (no response or gastroparesis recurrence within 6 months). RESULTS: At initial follow-up (3 to 6 months after GPOEM), 73 patients (81.1%) had a clinical response and significant increases in SF-36 questionnaire scores (indicating increased quality of life) whereas 17 patients (18.9%) had no response. Six months after GPOEM, 7.1% had recurrence. At 12 months, 8.3% of patients remaining in the study had recurrence. At 24 months, 4.8% of patients remaining in the study had a recurrence. At 36 months, 14.3% of patients remaining in the study had recurrence. For patients who experienced an initial clinical response, the rate of loss of that response per year was 12.9%. In the univariate and multivariate regression analysis, a longer duration of gastroparesis reduced the odds of response to GPOEM (odds ratio [OR], 0.092; 95% CI, 1.04-1.3; P = .001). On multivariate logistic regression, patients with high BMIs had increased odds of GPOEM failure (OR, 1.097; 95% CI, 1.022-1.176; P = .010) and patients receiving psychiatric medications had a higher risk of GPOEM failure (OR, 1.33; 95% CI, 0.110-1.008; P = .052). CONCLUSIONS: In retrospective analysis of 90 patients who underwent GPOEM for refractory gastroparesis, 81.1% had a clinical response at initial follow-up of their procedure. 1 year after GPOEM, 69.1% of all patients had a clinical response and 85.2% of initial responders maintained a clinical response. Patients maintained a clinical response and improved quality of life for as long as 3 years after the procedure. High BMI and long duration gastroparesis were associated with failure of GPOEM.

Endoscopic Ultrasound-Guided Management of Chronic Pancreatitis.

PURPOSE OF REVIEW: The purpose of this review is to discuss the role of endoscopic ultrasound (EUS) in the diagnosis and treatment of chronic pancreatitis (CP). RECENT FINDINGS: EUS has evolved and become invaluable in diagnosing early CP with the use of elastography and contrast enhancement. Lumen-apposing metal stents have allowed for easier transmural drainage and necrosectomy for pancreatic pseudocyst and walled of necrosis. EUS-guided pancreatic duct drainage is being utilized for pancreatic duct complications including stenosis, stones, and duct disruptions that are not amendable to endoscopic retrograde cholangiopancreatography. EUS is an effective tool that assists with the diagnosis and treatment of CP. The technology continues to evolve allowing for diagnosis of CP in earlier stages, which enables more effective therapy. The development of new EUS-guided tools and techniques has improved the treatment of complications from CP.

Digestive system involvement of novel coronavirus infection: Prevention and control infection from a gastroenterology perspective.

An epidemic of an acute respiratory syndrome caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in Wuhan, China, now known as coronavirus disease 2019 (COVID-19), beginning in December 2019, has attracted an intense amount of attention worldwide. As the natural history and variety of clinical presentations of this disease unfolds, extrapulmonary symptoms of COVID-19 have emerged, especially in the digestive system. While the respiratory mode of transmission is well known and is probably the principal mode of transmission of this disease, a possibility of the fecal-oral route of transmission has also emerged in various case series and clinical scenarios. In this review article, we summarize four different aspects in published studies to date: (a) gastrointestinal manifestations of COVID-19; (b) microbiological and virological investigations; (c) the role of fecal-oral transmission; and (d) prevention and control of SARS-CoV-2 infection in the digestive endoscopy room. A timely understanding of the relationship between the disease and the digestive system and implementing effective preventive measures are of great importance for a favorable outcome of the disease and can help climnicians to mitigate further transmission by taking appropriate measures.

Retrospective Multicenter Matched Controlled Comparison of Endoscopic Retrograde Cholangiopancreatography in Pediatric Patients: A 10-year Experience.

INTRODUCTION: This study was designed to evaluate outcomes in pediatric patients undergoing endoscopic retrograde cholangiopancreatography (ERCP) as compared with an American Society of Gastrointestinal Endoscopy (ASGE) complexity grade-matched adult cohort. METHOD: In this retrospective case-control study, ERCPs performed in pediatric patients from January 2008 to December 2018 in 2 tertiary referral hospitals were compared with a complexity-matched adult control group with similar procedural indications. Primary outcomes included the clinical success rate, technical success rate, and complication rate. Secondary outcomes included postprocedural admission rates, mode of sedation, procedure time, fluoroscopy time, hospitalization length, and the number of repeat procedures. RESULTS: Two hundred thirty-two ERCPs performed in 110 pediatric patients (average age 13.3) and 318 ERCPs performed in 160 ASGE grade-matched adult controls (average age 47.2 years) were analyzed. All procedures were therapeutic. There was no difference in the technical success rate (P = 0.2), clinical success rate (P = 0.5), complication rates (P = 0.1), and fluoroscopy time (P = 0.4), between the pediatric and adult cohorts. General anesthesia use and length of stay were significantly higher in the pediatric group (P = 0.0001). In subgroup analysis, technical (P = 0.2) and clinical success (P = 0.2) as well as complication rates (P = 0.6) were comparable between patients 10 years or less and patients 11 to 18 years within pediatric cohort. CONCLUSIONS: ERCP in pediatric cohorts appears to be safe and effective with equivalent outcomes relative to an ASGE complexity-matched adult cohort. Pediatric patients are more likely to require general anesthesia and have a longer average length of stay relative to adult controls.

Performance Characteristics of Magnetic Resonance Imaging in Patients With Pancreas Divisum.

OBJECTIVE: This study was designed to assess the diagnostic accuracy of standard nonsecretin-enhanced preprocedural magnetic resonance imaging/cholangiopancreatography (MRI/MRCP) in patients with and without pancreas divisum. METHODS: Patients undergoing MRI/MRCP followed by endoscopic retrograde cholangiopancreatography with between 2009 and 2016 were reviewed. The diagnostic accuracy of the MRI/MRCP was evaluated against the pancreatography. A subsequent independent blinded re-review performed by an expert abdominal radiologist was also evaluated. Multivariate binary logistic regression was performed to assess the impact of clinicopathologic factors on the diagnostic accuracy. RESULTS: A total of 189 patients were included in analysis. The sensitivity, specificity, positive predictive value, and negative predictive value of MRI/MRCP for pancreas divisum were 63%, 97%, 94%, and 82% initially and 81%, 91%, 91%, and 82% on the expert review. Motion artifact, the presence of pancreatic tumor, and pancreatic necrosis were not found to significantly impact the accuracy. A normal diameter pancreatic duct (P = 0.04) and complete divisum anatomy were correlated with improved accuracy (P = 0.001). CONCLUSIONS: Although expert review, normal duct diameter, and complete divisum are associated with increased sensitivity, pancreas divisum may be uncharacterized by preprocedural MRI in 19% to 37% of patients before the index endoscopic retrograde cholangiopancreatography.

Functional lumen imaging probe in gastrointestinal motility diseases.

Dysfunction of gastrointestinal (GI) sphincters, including the lower esophageal sphincter (LES) at the esophagogastric junction (EGJ) and the pyloric sphincter, plays a vital role in GI motility disorders, such as achalasia, gastroesophageal reflux disease (GERD), gastroparesis, and fecal incontinence. Using multi-detector high-resolution impedance planimetry, the functional luminal imaging probe (FLIP) system measures simultaneous data on tissue distensibility and luminal geometry changes in the sphincter in a real-time manner. In this review we focus on the emerging data on FLIP, which can be used as an innovative diagnostic method during endoscopic or surgical procedures in GI motility disorders. Subsequent large, prospective, standardizing studies are needed to validate these findings before it can be put to routine clinical use.

Extreme hyperbilirubinemia: An indicator of morbidity and mortality in sickle cell disease.

BACKGROUND: Sickle cell disease (SCD) is a disorder that results in increased hospitalizations and higher mortality. Advances in management have resulted in increases in life expectancy and led to increasing awareness of sickle cell hepatopathy (SCH). However, its impact in patients on the natural history and outcomes of SCD is not known. Our study aims to describe the prevalence of extreme hyperbilirubinemia (EH), one form of SCH, its effect on morbidity and mortality, and correlations between sickle cell genotype and SCH type. We hypothesize that EH is associated with higher morbidity and mortality. AIM: To investigate the effects of EH on morbidity and mortality among patients with SCD. METHODS: This retrospective cohort study was performed using a database of patients with SCD treated at Grady Memorial Hospital between May 2004 and January 2017. Patients with EH (defined as total bilirubin above 13.0 mg/dL) were identified. A control group was identified from the same database with patients with total serum bilirubin ≤ 5.0 mg/dL. Electronic medical records were used to extract demographic information, laboratory values, radiology results, current medications, need for transfusions and mortality data. Two samples T-test, chi-squared test and Fisher's exact test were then used to compare the parameters between the two groups. RESULTS: Out of the database, fifty-seven charts were found of patients with bilirubin > 13 mg/dL. Prevalence of severe SCH as defined by EH was 4.8% (57/1172). There were no demographic differences between patients with and without EH. Significant genotypic differences existed between the two groups, with hemoglobin SS SCD being much higher in the EH group (P < 0.001). Patients with severe EH had a significant elevations in alanine aminotransferase (157.0 ± 266.2 IU/L vs 19.8 ± 21.3 IU/L, P < 0.001), aspartate aminotransferase (256.5 ± 485.9 U/L vs 28.2 ± 14.7 U/L, P < 0.001) and alkaline phosphatase (218.0 ± 176.2 IU/L vs 85.9 ± 68.4 IU/L, P < 0.001). Patients with EH had significantly higher degree of end organ failure measured with quick Sequential Organ Failure Assessment scores (0.42 ± 0.68 vs 0.01 ± 0.12, P < 0.001), increased need for blood products (63% vs 5%, P < 0.001), and exchange transfusions (10.5% vs 1.3%, P = 0.022). CONCLUSION: Among patients with SCD, elevated levels of total bilirubin are rare, but indicative of elevated morbidity, mortality, and need for blood transfusions. Large differences in sickle cell genotype also exist, but the significance of this is unknown.

Outcomes of per oral endoscopic pyloromyotomy in gastroparesis worldwide.

Per oral endoscopic pyloromyotomy (POP), also known as gastric per-oral endoscopic myotomy (GPOEM), is a novel procedure with promising potential for the treatment of gastroparesis. As more data emerge and the procedure is becoming more recognized in clinical practice, its safety and efficacy need to be carefully evaluated. Appropriate patient selection for favorable clinical success prediction after GPOEM also needs additional research. This review aims to systemically summarize the existing data on clinical outcomes of POP. Symptomatologic responses to the procedure, its adverse effects, procedural techniques, and predictive factors of clinical success are also discussed.

Early vs late endoscopic retrograde cholangiopancreatography in patients with acute cholangitis: A nationwide analysis.

AIM: To assess the effect of early vs late endoscopic retrograde cholangiopancreatography (ERCP) on mortality and readmissions in acute cholangitis, using a nationally representative sample. METHODS: We used the 2014 National Readmissions Database to identify adult patients hospitalized with acute cholangitis who underwent therapeutic ERCP within one week of admission. Early ERCP was defined as ERCP performed on the same day of admission or the next day (days 0 or 1, < 48 h), and late ERCP was performed on days 2 to 7 of admission. Patients with severe cholangitis had any of the following additional diagnoses: Severe sepsis, septic shock, acute renal failure, acute respiratory failure, or thrombocytopenia. Multivariate logistic regression was used to calculate the adjusted odds of association of ERCP timing with in-hospital mortality, 30-d mortality, and 30-d readmissions, controlling for age, sex, severe disease and comorbidities. RESULTS: Four thousand five hundred and seventy patients satisfied the inclusion criteria; with a mean age of 64.1 years. Of these, 66.6% had early ERCP, while 33.4% had late ERCP. Early ERCP was associated with lower in-hospital mortality [1.2% vs 2.4%, adjusted odds ratio (aOR) = 0.50, 95%CI: 0.76-0.83, P = 0.001] and lower 30-d mortality (1.5% vs 3.3%, aOR = 0.48, 95%CI: 0.33-0.69, P < 0.0001) compared to the late ERCP group. Similarly, early ERCP was associated with lower 30-d readmissions (9.7% vs 15.1%, aOR = 0.58, 95%CI: 0.49-0.7, P < 0.0001). When stratified by severity of cholangitis, there was a similar benefit of early ERCP on all outcomes in those with and without severe cholangitis. The mean length of stay was higher in the late ERCP group compared to the early ERCP group (6.9 d vs 4.5 d, P < 0.0001). The mean hospitalization cost was higher in the late ERCP group ($21459 vs $16939, P < 0.0001). CONCLUSION: Early ERCP is associated with lower in-hospital and 30-d mortality in those with or without severe cholangitis. Regardless of severity, we suggest performing early ERCP.

Association between duration or etiology of gastroparesis and clinical response after gastric per-oral endoscopic pyloromyotomy.

BACKGROUND AND AIMS: Gastric per-oral endoscopic pyloromyotomy (GPOEM) is a novel procedure with promising potential for the treatment of gastroparesis but with limited data regarding predictors of clinical response. This study aims to evaluate the safety and efficacy of the procedure and explore the impact of duration and etiology (diabetic vs nondiabetic) of gastroparesis on clinical outcome as measured by the Gastroparesis Cardinal Symptom Index (GCSI). METHODS: A single-center retrospective longitudinal study at a tertiary care hospital was performed over an 18-month period. Forty patients with refractory gastroparesis (25 nondiabetic and 15 diabetic patients) were included. RESULTS: GCSI significantly improved throughout the study period (F[2.176, 17.405] = 10.152, P = .001). The nausea/vomiting subscale showed sustained improvement through 18 months (F[2.213, 17.704] = 15.863, P < .00001). There was no significant improvement in bloating (F[2.099, 16.791] = 1.576, P = .236). Gastric scintigraphy retention was significantly reduced by 41.7% (t = -7.90; P < .00001). Multivariate linear regression modeling revealed a significant correlation between the duration of disease and a GCSI improvement at 12 months (P = .02), with a longer duration of disease associated with a poorer long-term response. The etiology of gastroparesis was not associated with clinical improvement (P = .16). Adverse events (7.5%) included 1 capnoperitoneum, 1 periprocedure chronic obstructive pulmonary disease exacerbation, and 1 mucosotomy closure site disruption. CONCLUSIONS: GPOEM appears to be a safe and effective minimally invasive therapy for refractory gastroparesis, especially for patients with predominant nausea/vomiting and shorter duration of disease, regardless of the etiology. We propose the clinical criteria for undergoing GPOEM should be a GCSI of at least 2.0 and a gastric retention of greater than 20%.

Orthotropic Liver Transplantation for Acute Intrahepatic Cholestasis in Sickle Cell Disease: Clinical and Histopathologic Features of a Rare Case.

Sickle cell disease has a wide range of hepatic manifestations, with acute intrahepatic cholestasis being one of the rarest and most fatal, often resulting in acute fulminant hepatic failure. Liver transplantation is an emerging but rarely utilized treatment for hepatic failure in the setting of sickle cell disease. Few such cases have been reported in the literature, with little emphasis on histopathologic correlation. We report a case of acute intrahepatic cholestasis in a patient with sickle cell disease who underwent orthotropic liver transplantation and describe novel correlating histopathologic features. The patient is a 29-year-old man who presented with hyperbilirubinemia, acute kidney injury, and coagulopathy. He was diagnosed clinically with acute intrahepatic cholestasis and received an orthotropic liver transplant. The explanted liver demonstrated marked sinusoidal expansion by sickled erythrocytes, hyperplastic Kupffer cells, and extramedullary hematopoiesis. There was extensive sinusoidal and centrizonal fibrosis with occlusion of central veins reminiscent of chronic sinusoidal obstructive syndrome, a previously undescribed pattern of injury. This case represents one of the few reported cases of sickle cell intrahepatic cholestasis treated by transplantation and demonstrates the rarely reported histopathologic features and gives insight to a potentially new mechanism of injury in these patients. Familiarity with the morphologic features of sickle cell hepatopathy and its clinical manifestations is important as transplantation in sickle cell-related liver injury increases in frequency.