Endoscopic treatment of bile duct stones with benign choledochojejunal anastomotic stenosis.

[BACKGROUND AND AIMS]: Endoscopic interventions for bile duct stones (BDS) with benign choledochojejunal anastomotic stenosis (bCJS) are challenging. Therefore, we investigated endoscopic interventions for BDS with bCJS. [METHODS]: Seventeen patients with BDS with bCJS were retrospectively analyzed. Patient characteristics, technical success, adverse events (AEs), and recurrence were evaluated. [RESULTS]: In 17 patients, the median diameters of the bile duct and BDS were both 8 mm. The median number of BDS was 3. The technical success rate was 94% (16/17). Ten patients underwent balloon dilation at the choledochojejunal anastomotic site (CAS), the median diameter of balloon dilation was 10.5 mm, and waist disappearance was achieved in 2. Six patients had fully covered self-expandable metal stents (FCSEMS) with a diameter of 10 mm placed at the CAS. BDS were removed after balloon dilation or FCSEMS removal, and 6 out of 16 patients were treated with a combination of lithotripsy and 5 with peroral direct cholangioscopy (PDCS). Regarding AEs, perforation at the CAS by balloon dilation occurred in 1 patient. The median follow-up was 3701 days. Nine out of 16 patients (56%) had recurrence. The patients treated with combination of PDCS at BDS removal (p=0.022) and waist disappearance at the CAS by balloon dilation (p=0.035) had significantly fewer recurrences. [CONCLUSIONS]: Endoscopic interventions for BDS with bCJS are useful and relatively safe; however, long-term follow-ups showed frequent recurrences. Recurrence was common in patients not treated with the combination of PDCS at BDS removal and those without waist disappearance at the CAS by balloon dilation.

[A rare case of intraductal tumor of the pancreas in which an intraductal tubulopapillary neoplasm was mixed with a widely spreading gastric-type intraductal papillary-mucinous neoplasm].

A 70-year-old man receiving treatment for diabetes mellitus presented with a cystic mass in the border area of the pancreatic body and tail on plain computed tomography (CT) due to impaired glucose intolerance. Contrast-enhanced CT showed a faint hyperattenuated nodular mass extending from the dilated main pancreatic duct (MPD) to the branch duct. Endoscopic retrograde cholangiopancreatography revealed a mildly dilated orifice of the papilla of Vater and MPD stenosis with entire upstream and immediate downstream dilatations. The patient underwent distal pancreatectomy due to the suspicion of mixed-type intraductal papillary-mucinous carcinoma. A pathological examination showed an intraductal solid-nodular mass measuring 25mm in length, consisting of two types of neoplasms. One showed tubulopapillary growth with entirely high-grade (HG) atypical cuboidal epithelium, in which immunohistochemical examinations were positive for MUC6 but negative for human gastric mucin (HGM), MUC1, MUC2, and MUC5AC, fitting the concept of intraductal tubulopapillary neoplasm (ITPN). The other showed the same growth of low-grade (LG) atypical columnar cells positive for HGM and MUC5AC and negative for MUC1 and MUC2, which corresponded to gastric-type intraductal papillary-mucinous neoplasm (IPMN) -LG. The tumor had not invaded the duct walls, and no metastatic lymph nodes were observed. The ITPN was adjacent to the IPMN mainly composed of tubular glands mimicking pyloric glands with LG dysplasia that corresponded to the so-called IPMN-pyloric gland variant. Moreover, the proliferation of low-papillary gastric-type IPMN spread around the intraductal tumors. Consequently, the patient was diagnosed with an intraductal tubular neoplasm comprising a noninvasive ITPN and gastric-type IPMN-LG. ITPN is a recently identified intraductal neoplasm of the pancreas proposed by Yamaguchi et al. and is distinguished by intraductal tubulopapillary growth with HG cellular atypia without overt mucin production, in contrast to IPMN. To date, no cases of intraductal nodular tumors comprising ITPN and IPMN have been reported. We report this original case with imaging and pathological observations and discuss potential processes via which ITPN and IPMN may arise adjacent to each other in the same pancreatic duct.

Eosinophilic Granulomatosis with Polyangiitis Presenting as Steroid-Responsive Sclerosing Cholangitis and Cholecystitis: A Rare Case Report.

BACKGROUND Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitic condition characterized by bronchial asthma and eosinophilia. While biliary involvement is uncommon in EGPA, we present a unique case of EGPA presenting as steroid-responsive sclerosing cholangitis and cholecystitis. This case highlights the importance of considering EGPA in the differential diagnosis of biliary diseases, especially in patients with a history of bronchial asthma. CASE REPORT A 47-year-old man with a history of bronchial asthma presented with fatigue, weight loss, and epigastralgia. Blood tests revealed eosinophilia and elevated inflammatory markers, leading to the diagnosis of EGPA. Further imaging studies, including magnetic resonance cholangiopancreatography and contrast-enhanced computed tomography, confirmed the presence of sclerosing cholangitis and cholecystitis, a rare manifestation of EGPA. CONCLUSIONS Prompt treatment with prednisolone and azathioprine resulted in remission of symptoms and resolution of cholangitis and cholecystitis in this case. Our findings emphasize the importance of early recognition and appropriate management of EGPA-associated biliary involvement. Increased awareness of this rare manifestation may facilitate timely diagnosis and improve patient outcomes.

A Lethal Combination: Legionnaires' Disease Complicated by Rhabdomyolysis, Acute Kidney Injury, and Non-Occlusive Mesenteric Ischemia.

BACKGROUND Legionnaires' disease is one of the most common types of community-acquired pneumonia. It can cause acute kidney injury and also occasionally become severe enough to require continuous renal replacement therapy (CRRT). Non-occlusive mesenteric ischemia (NOMI) is a condition characterized by ischemia and necrosis of the intestinal tract without organic obstruction of the mesenteric vessels and is known to have a high mortality rate. CASE REPORT A 72-year-old man with fatigue and dyspnea was diagnosed with Legionnaires' disease after a positive result in the Legionella urinary antigen test pneumonia confirmed by chest radiography and computed tomography. He developed acute kidney injury, with anuria, rhabdomyolysis, septic shock, respiratory failure, and metabolic acidosis. We initiated treatment with antibiotics, catecholamines, mechanical ventilation, CRRT, steroid therapy, and endotoxin absorption therapy in the Intensive Care Unit. Despite ongoing CRRT, metabolic acidosis did not improve. The patient was unresponsive to treatment and died 5 days after admission. The autopsy revealed myoglobin nephropathy, multiple organ failure, and NOMI. CONCLUSIONS We report a fatal case of Legionnaires' disease complicated by rhabdomyolysis, acute kidney injury, myoglobin cast nephropathy, and NOMI. Legionella pneumonia complicated by acute kidney injury is associated with a high mortality rate. In the present case, this may have been further exacerbated by the complication of NOMI. In our clinical practice, CRRT is a treatment option for septic shock complicated by acute kidney injury. Thus, it is crucial to suspect the presence of NOMI when persistent metabolic acidosis is observed, despite continuous CRRT treatment.

[Carcinoma in situ arising in adenomyomatous hyperplasia of the ampulla of Vater: the first case report].

A man in his 70s was admitted to our hospital due to jaundice and upper abdominal pain. Laboratory findings indicated elevated serum hepatobiliary enzyme and amylase levels. Contrast-enhanced computed tomography revealed smooth wall thickening of the terminal bile duct (tBD) with a faintly enhanced inner line. ERCP revealed stenosis from the tBD to the ampulla of Vater (AV) with upstream dilatation. Intraductal ultrasound (IDUS) circumferentially revealed a thickened wall preserving a three-layered structure throughout the same region. Furthermore, a thick innermost hyperechoic layer was identified in the bile duct portion of the AV (Ab). Findings suggestive of adenocarcinoma were obtained from the tissue samples from the biliary stricture using biopsy forceps. Thus, pancreatoduodenectomy was performed. A pathological examination revealed a thickened AV wall spreading over the tBD with hyperplasia of the glands and smooth muscle fibers. In addition, low-grade biliary intraepithelial neoplasia (BilIN) was scattered throughout the lesion, and high-grade BilIN was partly observed in the peribiliary glands of the Ab. Based on these results, a diagnosis of carcinoma in situ arising in adenomyomatous hyperplasia (ADMH) of the AV was made. To date, there are no reports on ADMH-associated carcinoma of the BD or AV. We here report this original case with the IDUS findings, which are presumed to reflect the histologic features of ADMH showing ductal proliferation surrounded by smooth muscle fibers. Also, we discuss the process through which carcinoma arises from ADMH in AV.

Mucosal color changes on narrow-band imaging in esophageal eosinophilic infiltration.

This study aimed to examine the range of beige colored mucosa (BCM) in patients with esophageal eosinophilic infiltration (EEI) using narrow-band imaging (NBI). In this retrospective study, EEI was confirmed histologically in 12 consecutive patients from January 2014 to December 2017. The BCM tone on NBI without magnifying endoscopy was evaluated, and red, green, and blue (RGB) values of BCM and normal mucosa were measured. BCM was macroscopically classified into 2 groups (bright and dark) using cluster analysis. Histopathological analysis was performed in 1 patient who underwent biopsy for both normal mucosa and BCM. All 12 patients presented with BCM. Endoscopy revealed fixed rings, longitudinal furrows, mucosal edema, and exudate in 3, 12, 10, and 8 patients, respectively. Strictures were absent. Five patients had findings suggestive of gastroesophageal reflux disease. In the cluster analysis, 5 and 7 patients had bright and dark BCM, respectively. Consistent results were noted when we categorized patients according to their macroscopic characteristics. RGB values of the BCM and normal mucosa were measured-normal mucosa: R: 99.8 ± 16.5, G: 121.7 ± 23.1, and B: 93.4 ± 19.2; BCM: R: 152.0 ± 31.3, G: 123.9 ± 35.0, and B: 97.5 ± 29.5. BCM had significantly higher R values than normal mucosa (P = .0001). All parameters were significantly lower in the dark BCM group than in the bright BCM group (P < .001). Histopathological analysis revealed expansion of the epithelial intercellular space, eosinophilic infiltration, and basal cell hyperplasia at the BCM sites. BCM was observed in all cases of EEI. RGB values differed between bright and dark BCM. Assessing BCM tone using NBI is a potentially novel diagnostic method for EEI.

Solitary extrahepatic hepatocellular carcinoma in vertebrae without a primary lesion in the liver might originate from bone marrow: a case report and new hypothesis based on a review of the literature and the latest findings.

Solitary extrahepatic hepatocellular carcinoma without a primary lesion in the liver is rare and unique. In such patients, in addition to hepatocellular carcinoma, hepatoid adenocarcinoma, hepatoid teratoma, and hepatoid yolk sac tumor must be considered as differential diagnoses, and patients must be investigated in detail by histopathological studies with immunohistochemistry, especially using epithelial markers for which tumor cells are generally negative in hepatocellular carcinoma. A case with a solitary neoplasm of the vertebrae, which was diagnosed histopathologically as hepatocellular carcinoma, without a primary lesion is presented. The primary lesion was not identified even on autopsy, and the liver was pathologically almost normal. Given the review of the literature and circumstantial evidence, we would like to propose a bold new hypothesis that hepatocellular carcinoma might primarily originate from bone marrow.

Image quality of abdominal ultrasonography after esophagogastroduodenoscopy is preserved by using carbon dioxide insufflation: A non-inferiority test in the same subject.

Because bowel gas deteriorates the image quality of abdominal ultrasonography (AUS), it is common to perform AUS prior to esophagogastroduodenoscopy (EGD). This one-way order limits the availability of examination appointments. To evaluate whether EGD using insufflation of carbon dioxide (CO2), which is rapidly absorbed by the gastrointestinal mucosa, preserves the image quality of AUS performed subsequently, we designed a non-inferiority test in which each subject underwent AUS, EGD with CO2 insufflation, and a second AUS, in that order. All saved AUS moving images were randomized and imaging quality was evaluated at 16 organs using a four-point Likert-like scale that divides the depiction rate by 25%. Sample size was calculated to be 26 using the following: non-inferiority margin of -0.40 corresponding to depiction rate of -10%, difference of means of 0.40, common standard deviation of 1.25, power of 90%, and 1-sided α-level of 0.025. We enrolled 30 subjects. The mean and 95% confidence interval (CI) of the image quality score of all 16 organs at pre- and post-EGD AUS in the 30 subjects were 3.54 [3.48-3.60] and 3.46 [3.39-3.52], respectively. The difference in the means was 0.08 of the scores, corresponding to a 2% depiction rate. The effect size was 0.172. The image quality of post-EGD AUS was not inferior, as demonstrated by the 97.5% CI of the difference, which did not cross the non-inferiority margin of -0.40. In conclusion, the use of CO2 for insufflation in EGD does not cause much deterioration in the image quality of AUS performed subsequently. Therefore, it is permissible to perform EGD prior to AUS, which is expected to improve the efficiency of examination setup.

Biliary stones or ulcers at the choledochojejunal anastomotic site involving the jejunal mucosa at stent removal may be recurrent factors in patients with benign choledochojejunal anastomotic stenosis undergoing endoscopic biliary stenting using fully covered self-expandable metal stents.

BACKGROUND: Temporary fully covered self-expandable metal stent (FCSEMS) placement is performed for benign choledochojejunal anastomotic stenosis (bCJS). However, recurrence may develop after stricture resolution. We investigated endoscopic biliary stenting using FCSEMS for bCJS. METHODS: Sixteen bCJS patients with FCSEMS placement were retrospectively analyzed. FCSEMS was removed endoscopically after 2 months. Technical success, stricture resolution, recurrence, and adverse events were evaluated. RESULTS: The technical success rate for FCSEMS placement was 94% (15/16). Biliary stones were detected and extracted in four patients. FCSEMS removal was successfully performed on 14 patients, excluding one with stent migration. At FCSEMS removal, stricture resolution was noted in 14 patients; however, four had anastomotic ulcers. The median follow-up was 319 days. Three patients with a history of repeated plastic stent placement had no recurrence. Four out of 15 patients (27%) had recurrence, and three had no recurrence after additional interventions. Biliary stones before first FCSEMS placement (P = .003) or anastomotic ulcers at FCSEMS removal (P = .018) were associated with recurrence. CONCLUSIONS: Although FCSEMS placement was useful for stricture resolution, recurrence was detected in patients with biliary stones before first FCSEMS placement or anastomotic ulcers at FCSEMS removal. Anastomotic ulcers are a risk factor for recurrence and only detected by endoscopy.

Long-term outcomes after endoscopic retrograde pancreatic drainage for symptomatic pancreaticojejunal anastomotic stenosis.

There is limited evidence supporting the usefulness of endoscopic retrograde pancreatic drainage (ERPD) for symptomatic pancreaticojejunal anastomotic stenosis (sPJS). We examined the usefulness of ERPD for sPJS. We conducted a retrospective analysis of 10 benign sPJS patients. A forward-viewing endoscope was used in all sessions. Following items were evaluated: technical success, adverse events, and clinical outcome of ERPD. The technical success rate was 100% (10/10) in initial ERPD; 9 patients had a pancreatic stent (no-internal-flap: n = 4, internal-flap: n = 5). The median follow-up was 920 days. Four patients developed recurrence. Among them, 3 had a stent with no-internal-flap in initial ERPD, the stent migrated in 3 at recurrence, and a stent was not placed in 1 patient in initial ERPD. Four follow-up interventions were performed. No recurrence was observed in 6 patients. None of the stents migrated (no-internal-flap: n = 1, internal-flap: n = 5) and no stents were replaced due to stent failure. Stenting with no-internal-flap was associated with recurrence (p = 0.042). Mild adverse events developed in 14.3% (2/14). In conclusions, ERPD was performed safely with high technical success. Recurrence was common after stenting with no-internal-flap. Long-term stenting did not result in stent failure.Clinical trial register and their clinical registration number: Nos. 58-115 and R2-9.

Tumor lysis syndrome in a patient with metastatic melanoma treated with nivolumab.

A 79-year-old man with metastatic melanoma of the right maxillary sinus and multiple liver metastases received a single dose of nivolumab. Eight days later, he experienced impaired consciousness, accompanied by abnormal laboratory and electrocardiographic findings. He was therefore diagnosed with tumor lysis syndrome (TLS). Laboratory and electrocardiographic findings improved immediately after continuous hemodiafiltration; however, he died 22 days after receiving nivolumab. Autopsy revealed massive tumor necrosis in the liver. There are few case reports of TLS associated with immune checkpoint inhibitors, indicating that we should be prepared to manage especially in a patient with liver involvement of high tumor burden.

More than half of hypoxemia cases occurred during the recovery period after completion of esophagogastroduodenoscopy with planned moderate sedation.

Guidelines advise precautionary measures for possible adverse events that may occur due to sedation during endoscopic procedures. To avoid complications, intraprocedural and postprocedural monitoring during recovery is considered important. However, since not many studies have reported on hypoxemia during the recovery period, findings for specific monitoring methods are insufficient. The aim of this retrospective study was to determine the incidence of hypoxemia during the recovery period using continuous central-monitoring by pulse oximetry and to characterize the hypoxemia cases. Among the 4065 consecutive esophagogastroduodenoscopy (EGD) procedures under planned moderate sedation, 84 (2.1%) procedures developed unexpected hypoxemia (SpO2 ≤ 90%). Hypoxemia was observed during the procedure, at the end of the procedure, and during the recovery period in 21, 17, and 46 (1.1%) procedures, respectively. More than half of the hypoxemia cases occurred during the recovery period. Many hypoxemia cases were characterized by neither serious co-morbid illness nor low body mass index which have been reported as risk factors of hypoxemia. The lack of risk factors is no guarantee that hypoxemia will not occur. Therefore, continuous monitoring by pulse oximetry is more important during the recovery period and is recommended in all EGD procedures under planned moderate sedation.

Gut-derived Enterococcus faecium from ulcerative colitis patients promotes colitis in a genetically susceptible mouse host.

BACKGROUND: Recent metagenomic analyses have revealed dysbiosis of the gut microbiota of ulcerative colitis (UC) patients. However, the impacts of this dysbiosis are not fully understood, particularly at the strain level. RESULTS: We perform whole-genome shotgun sequencing of fecal DNA extracts from 13 healthy donors and 16 UC and 8 Crohn's disease (CD) patients. The microbiota of UC and CD patients is taxonomically and functionally divergent from that of healthy donors, with E. faecium being the most differentially abundant species between the two microbial communities. Transplantation of feces from UC or CD patients into Il10-/- mice promotes pathological inflammation and cytokine expression in the mouse colon, although distinct cytokine expression profiles are observed between UC and CD. Unlike isolates derived from healthy donors, E. faecium isolates from the feces of UC patients, along with E. faecium strain ATCC 19434, promotes colitis and colonic cytokine expression. Inflammatory E. faecium strains, including ATCC 19434 and a UC-derived strain, cluster separately from commercially available probiotic strains based on whole-genome shotgun sequencing analysis. The presence of E. faecium in fecal samples is associated with large disease extent and the need for multiple medications in UC patients. CONCLUSIONS: E. faecium strains derived from UC patients display an inflammatory genotype that causes colitis.

Acute liver failure due to severe Herpes simplex viral hepatitis in an elderly woman: A case of initial infection.

The high mortality rate due to severe Herpes simplex viral (HSV) hepatitis is associated with the difficulty of its diagnosis. We describe the extremely rapid disease course of a patient who died of severe HSV hepatitis. A 73-year-old woman was admitted for a bronchial asthma attack. Her symptoms improved with steroid treatment, but she developed a sore throat and painful swallowing. On day 12 after admission, she suddenly went into shock. Blood test results showed a significant increase in the liver enzyme levels, with remarkable disseminated intravascular coagulation. She died the same day. The autopsy revealed extensive coagulative necrosis of the liver. Viral inclusion of type A Cowdry bodies was found in the residual hepatocytes in the hepatic lobule. Immunostaining revealed HSV type 1 positivity. We diagnosed the cause of death as severe HSV hepatitis. On examination of a stored serum sample, the patient tested positive for the HSV immunoglobulin (Ig)-M antibody, and the HSV RNA level was very high (1 × 109 copies/mL). Remarkably, the HSV IgG test result was negative, and we diagnosed her as having had an initial HSV infection. Hepatitis due to HSV is very rare in healthy adults; however, there are many reports of immune-deficient cases. The presence of HSV IgG is decreasing in the elderly population because of the change in living environments/lifestyles. The increasing use of immunosuppressive drugs, such as steroids, for treating diseases in elderly patients could be linked to the increased prevalence of initial HSV infections, resulting in liver injury.

A case of macrocystic-type serous cystic neoplasm with repeated pancreatitis within a short period of time.

The patient was a 39-year-old woman in whom computed tomography (CT) in 201X had revealed a pancreatic cystic neoplasm (PCN) of 4.3 cm in diameter in the pancreatic body. In June 201X + 3, the patient consulted our hospital regarding severe acute pancreatitis. The condition improved through treatment with large-volume fluid replacement and continuous regional arterial infusion therapy. Thereafter, acute pancreatitis recurred twice, in November 201X + 3 and in January 201X + 4. During an 8-month period, acute pancreatitis occurred three times. The PCN was examined by endoscopic ultrasound, thin-slice contrast-enhanced CT, and T2-weighted magnetic resonance imaging, which led to the diagnosis of macrocystic-type serous cystic neoplasm (SCN). The SCN was found to be 5.8 cm in diameter with dilatation of the main pancreatic duct (MPD) caudal to the SCN for 3 years. We suspected that the repeated pancreatitis had been obstructive pancreatitis resulting from displacement of the MPD caused by the SCN, and therefore recommended that the patient undergo surgery for the SCN. In March 201X + 4, distal pancreatectomy was performed. In the resected specimen, a macrocystic-type SCN was diagnosed. No recurrence of acute pancreatitis has been observed postoperatively. A macrocystic-type SCN with repeated pancreatitis within a short period of time is rare.