Gallstone Enteropathy: An Unusual Cause of Bowel Obstruction.

Gallstones causing bowel obstruction, known as gallstone ileus, are rare and account for less than 0.5% of small bowel obstruction cases. Additionally, it is a rare complication affecting only 0.3% of patients who have gallstones. Fistula formation between the biliary system, most commonly between the gallbladder and duodenum because of their proximity, facilitates the migration of gallstones into the enteric system with subsequent impaction in the small intestine, usually in the distal ileum close to the ileocecal valve, promoting the development of mechanical small bowel obstruction. Computerized tomography of the abdomen and pelvis is a confirmatory and widely used imaging study when there are two signs of Rigler's triad, which includes pneumobilia, evidence of small bowel obstruction and the presence of radiopaque stones. We report a case of a 75-year-old Caucasian man who presented with abdominal distention with signs of severe dehydration secondary to intractable nausea and vomiting complicated with severe acute kidney injury and was found to have a 4.7-centimeter gallstone-induced small intestinal obstruction.

Distinction between Mitochondrial Antibody-Positive and -Negative Primary Biliary Cholangitis.

Antimitochondrial antibody-positive primary biliary cholangitis (AMA-pos PBC) is an autoimmune disorder in which monoclonal antibodies are produced against epitopes in the mitochondrial membranes of biliary epithelial cells, resulting in progressive nonsuppurative biliary cholangitis. Up to 5% of patients lack these autoantibodies, termed antimitochondrial antibody-negative (AMA-neg) PBC. Although a somewhat new variant of AMA-pos PBC, it is not an overlapping syndrome. Few studies to date have described this phenomenon. An 87-year-old woman was referred to our clinic with elevated serum alkaline phosphatase (714 U/L). She reported fatigue but no other symptoms. A physical examination revealed a benign lesion and bilateral lower extremity swelling secondary to lymphedema. The serological profile was significant for a high antinuclear antibody titer (>1:2,560) with a centromere pattern and negative for antimitochondrial antibody (AMA). The hepatitis panel was negative for viruses A, B, and C. Her serum immunoglobulin G level was 871 mg/dL (normal, <1,600 mg/dL). The rest of the serological tests, including anti-smooth muscle antibodies (ASMA) and anti-liver/kidney microsomal antibodies, were negative. Computed tomography of the abdomen and pelvis without contrast showed normal liver parenchyma and no acute intra-abdominal pathology. Histopathology indicated florid duct lesions. The background parenchyma showed no significant steatosis, and inflammatory changes were limited to the portal areas. Periodic acid-Schiff staining revealed intact hepatic parenchyma and architecture. The patient was diagnosed with AMA-neg PBC and responded well to ursodeoxycholic acid therapy. This case highlights the importance of recognizing AMA-neg PBC as a variant of AMA-pos PBC and differentiating between them. Autoimmune cholangitis is a vague and imprecise condition. All patients with AMA-negative PBC should be tested for other PBC-specific autoantibodies. Although the prognosis and bile duct damage and loss are worse in AMA-neg PBC for unknown reasons, treatment remains the same for both.

A rare synchrony of adenocarcinoma of the ampulla with an ileal gastrointestinal stromal tumor: A case report.

BACKGROUND: This is a unique case of a patient who was found to have two extremely rare primary malignancies synchronously, i.e., an ampullary adenocarcinoma arising from a high-grade dysplastic tubulovillous adenoma of the ampulla of Vater (TVAoA) with a high-grade ileal gastrointestinal stromal tumor (GIST). Based on a literature review and to the best of our knowledge, this is the first report of this synchronicity. Primary ampullary tumors are extremely rare, with an incidence of four cases per million population, which is approximately 0.0004%. Distal duodenal polyps are uncommon and have a preponderance of occurring around the ampulla of Vater. An adenoma of the ampulla ( AoA) may occur sporadically or with a familial inheritance pattern, as in hereditary genetic polyposis syndrome such as familial adenomatous polyposis syndrome (FAPS). We report a case of a 77-year-old male who was admitted for painless obstructive jaundice with a 40-pound weight loss over a two-month period and who was subsequently diagnosed with two extremely rare primary malignancies, i.e., an adenocarcinoma of the ampulla arising from a high-grade TVAoA and a high-grade ileal GIST found synchronously. CASE SUMMARY: A 77-year-old male was admitted for generalized weakness with an associated weight loss of 40 pounds in the previous two months and was noted to have painless obstructive jaundice. The physical examination was benign except for bilateral scleral and palmar icterus. Lab results were significant for an obstructive pattern on liver enzymes. Serum lipase and carbohydrate antigen-19-9 levels were elevated. Computed tomography (CT) of the abdomen and pelvis and magnetic resonance cholangiopancreatography were consistent with a polypoid mass at the level of the common bile duct (CBD) and the ampulla of Vater with CBD dilatation. The same lesions were visualized with endoscopic retrograde cholangiopancreatography. Histopathology of endoscopic forceps biopsy showed TVAoA. Histopathology of the surgical specimen of the resected ampulla showed an adenocarcinoma arising from the TVAoA. Abdominal and pelvic CT also showed a coexisting heterogeneously enhancing, lobulated mass in the posterior pelvis originating from the ileum. The patient underwent ampullectomy and resection of the mass and ileo-ileal side-to-side anastomosis followed by chemoradiation. Histopathology of the resected mass confirmed it as a high-grade, spindle cell GIST. The patient is currently on imatinib, and a recent follow-up positron emission tomography (PET) scan showed a complete metabolic response. CONCLUSION: This case is distinctive because the patient was diagnosed with two synchronous and extremely rare high-grade primary malignancies, i.e., an ampullary adenocarcinoma arising from a high-grade dysplastic TVAoA with a high-grade ileal GIST. An AoA can occur sporadically and in a familial inheritance pattern in the setting of FAPS. We emphasize screening and surveillance colonoscopy when one encounters an AoA in upper endoscopy to check for FAPS. An AoA is a premalignant lesion, particularly in the setting of FAPS that carries a high risk of metamorphism to an ampullary adenocarcinoma. Final diagnosis should be based on a histopathologic study of the surgically resected ampullary specimen and not on endoscopic forceps biopsy. The diagnosis of AoA is usually incidental on upper endoscopy. However, patients can present with constitutional symptoms such as significant weight loss and obstructive symptoms such as painless jaundice, both of which occurred in our patient. Patient underwent ampullectomy with clear margins and ileal GIST resection. Patient is currently on imatinib adjuvant therapy and showed complete metabolic response on follow up PET scan.

Ampullary and Pancreatic Neuroendocrine Tumors: A Series of Cases and Review of the Literature.

The ampulla of Vater is a unique, highly vascularized pouch. Its anatomic transition is halfway along the second part of the duodenum from the foregut to the midgut. According to the World Health Organization's (WHO) latest nomenclature, carcinoid tumors are now called neuroendocrine tumors (NETs). Knowledge of NETs is important because of their rarity, reclassification, prognosis, and management. NETs involving the ampulla of Vater are extremely rare, constituting <0.05% of gastrointestinal NETs, and involving the pancreas are rare to our knowledge. There are only a few reports of ampullary NETs. We report two rare NET cases involving the ampulla and pancreas and review the relevant literature. A 71-year-old patient with neurofibromatosis and multiple comorbidities presented with chronic intermittent abdominal pain. Abdominal imaging studies showed a suspicious mass at the level of the ampulla. Endoscopic retrograde cholangiopancreatography and endoscopic ultrasound revealed bulging papillae. Histopathology of the biopsied mass revealed a low-grade NET. Given his multiple comorbidities, the patient was scheduled for endoscopic resection. Our second patient was an 83-year-old lady presenting with nausea and vomiting. Abdominal imaging studies revealed a lobular mass over the body of the pancreas. Histopathological examination of fine-needle aspiration of the mass confirmed a well-differentiated low-grade NET. Octreoscan and dotatate scans showed pancreatic, multiple hepatic and metastatic lesions in the left lung and left shoulder. The patient is currently stable after completing peptide receptor radioligand therapy at a tertiary oncology center. Because of the patients' comorbidities and staging, their management has taken different approaches. More data and more research are needed for accurate assessment of prognosis; however, a review of the latest literature recommends Whipple resection with lymphadenectomy for all ampullary NETs provided patients can tolerate the procedure. Endoscopic resection or surgical ampullary resection should be performed on contraindicated patients. The majority of ampullary and pancreatic NETs would have metastasized by the time patients sought treatment. Because of their rarity and ill-defined and highly variable presentation, NET diagnosis is always delayed and sometimes incidental; therefore, we emphasize the importance of early diagnosis and management to reduce mortality and morbidity.

Jejunal Diverticulitis: A Rare Diverticular Disease of the Bowel.

Diverticulosis is an out-pocketing of the bowel wall that can affect the small bowel through the large bowel. Small bowel diverticulosis is rare and not as common as colonic diverticulosis, which is an important diagnosis for hospitalizations. Moreover, jejunal diverticulosis is rare among cases of small bowel diverticulosis. Jejunal diverticulitis is one of the complications of jejunal diverticulosis that can be conservatively managed with antibiotics instead of surgery. We report a case of a 41-year-old African American man who presented with vague epigastric pain and was diagnosed with adhesive jejunal diverticulitis upon contrast-enhanced computed tomography of the abdomen. The patient did not develop any life-threatening complications such as perforation or peritonitis, and recovered after conservative management with antibiotics. Adhesive jejunal diverticulitis with fat stranding was the distinctive finding in our patient, as he might have had multiple asymptomatic episodes. Initial diagnostic modalities include radiography and contrast-enhanced computed tomography. Enteroclysis is the most reliable and accurate diagnostic modality, but is not available in all urgent settings. Recently, endoscopy has replaced radiological studies. Conservative management is adequate for uncomplicated cases of jejunal diverticulitis. However, surgical intervention is required in most cases of complicated jejunal diverticulosis, or mortality rates will be high.

Chemoprophylaxis to Prevent Deep Venous Thrombosis in Patients Hospitalized for Pancreatitis: Beneficial or Harmful?

INTRODUCTION:  Vascular complications in pancreatitis generally occur in the form of hemorrhage or thrombosis. Pancreatitis resulting in splanchnic thrombosis has been well studied, but the cause of this correlation has not been studied in the current era of increasing anticoagulant use for deep venous thrombosis (DVT) prophylaxis. Hemorrhagic pancreatitis and peri-pancreatic bleeding are also known phenomena encountered in relation to pancreatitis, but these risks are not well established in the setting of chemical prophylaxis for DVT. OBJECTIVES:  Our objective was to identify whether chemical DVT prophylaxis in pancreatitis harms the patient by increasing the risk of hemorrhagic conversion of pancreatitis or peri-pancreatic hemorrhage or if it is beneficial by preventing splanchnic venous thrombosis in the abdominal vasculature that surrounds the pancreas. METHODS:  We undertook a retrospective chart review with approval from the Institutional Review Board on patients who were hospitalized for or developed pancreatitis during their hospital stay from April 2014 to July 2015. We reviewed the charts for imaging suggestive of venous thrombosis or the development of intra-abdominal hemorrhage at admission during hospitalization and within 30 days after hospitalization. We also reviewed the methods of DVT prophylaxis to identify any correlation with the risk of hemorrhage or thrombosis. A bedside index of severity in acute pancreatitis score was used within 24 hours of admission to calculate the severity of the patients' pancreatitis. The data collected were analyzed for descriptive statistics, correlation using Pearson's coefficient, and multivariate regression analysis using Microsoft Excel and SPSS Inc. Released 2009. PASW Statistics for Windows, Version 18.0. Chicago: SPSS Inc. RESULTS:  This study included 389 patients who met the inclusion criteria. Of these, 74.6% of patients received chemical prophylaxis, mostly low molecular weight heparin, and 18.5% of patients were not on chemical or mechanical means of DVT prophylaxis. Only 12 patients (3%) had complications related to thrombosis and hemorrhage. Seven patients had splanchnic venous thrombosis, one had a hemorrhagic conversion of pancreatitis, three had a peri-pancreatic hemorrhage, and one had both the hemorrhagic conversion of pancreatitis and peri-pancreatic hemorrhage. Ten patients out of 12 patients had complications before admission, and nine of the 12 patients were on chemical prophylaxis. Pearson's coefficient showed no statistically significant correlation between the incidence of complications and the use of chemical DVT prophylaxis. Multivariate analysis showed no specific variable that increased the risk of complications. CONCLUSIONS:  Our study showed that chemoprophylaxis for DVT in patients hospitalized for acute pancreatitis is neither harmful by causing hemorrhagic conversion of pancreatitis, peri-pancreatic hemorrhage nor beneficial by preventing splanchnic venous thrombosis.

Normoglycemic diabetic ketoacidosis in a type 2 diabetes patient on dapagliflozin: A case report.

A 48-year-old male patient with Type 2 diabetes mellitus(T2D), on insulin replacement therapy, glipizide, and dapagliflozin presented with generalized weakness with weight loss of 40 pounds in 6 months ever since he was started on dapagliflozin. He was hemodynamically stable on arrival with a finger stick glucose of 121 gm%. Physical examination was unremarkable except for dry mucus membranes. His laboratory results on arrival are shown in Table 1. His serum osmolar gap was within the normal range. He was treated insulin drip per DKA protocol and gap was closed, the patient was clinically and biochemically back to baseline, and he was discharged home. Delayed diagnosis of normoglycemic diabetic ketoacidosis (DKA) in adults with diabetes treated with multiple antidiabetic drugs (eg, sodium-glucose cotransporter-2 [SGLT-2] inhibitors) can potentially increase morbidity and mortality. Patient education in terms of symptoms and signs, physician awareness of early recognition of ketoacidosis in the setting of paradoxically normal or near-normal blood glucose levels in these patients is the primary focus of this case study. This is paradoxical DKA because theoretically patient is not meeting one of the criteria for DKA which include triad of hyperglycemia, Ketoacidosis with widened anion gap, Ketonemia. This is a short case report of presumed SGLT-2 inhibitor euglycemic diabetic ketoacidosis. The main teaching point is recognition and early diagnosis of this issue when multiple diabetic medications are present with the absence of hyperglycemia. This is, by current definition, not DKA because theoretically, the patient does not meet one of the criteria for DKA as the patient was apparently not hyperglycemic, albeit with, ketoacidosis and widened anion gap. (ketonemia).

Idiopathic Esophageal Lipomatosis: An Incidental Benign Discovery.

Gastrointestinal (GI) lipomatosis has been reported in the GI medicine literature, but esophageal lipomatosis has never been reported at all. We report the case of an 86-year-old man with multiple medical comorbidities who was admitted to our hospital for community-acquired pneumonia. Computed tomography angiography of his pulmonary arteries ruled out the possibility of pulmonary embolism but showed a 9-mm circumferential wall thickening in the proximal esophagus measuring -172 HU, which is similar in opacity to the adipose tissue. The patient was asymptomatic and without any current or prior symptoms of dysphagia or odynophagia. The barium esophagogram was unremarkable; there were no strictures, masses, or mucosal abnormalities. There was no evidence of esophageal dilatation on either imaging modality. Esophageal lipomatosis is only described in a few case reports in the radiological literature and, to our knowledge, has not been reported in the GI literature at all. It is important to highlight in the GI literature this as a benign entity that does not cause symptoms and typically does not warrant invasive diagnostic or therapeutic interventions.

Idiopathic Benign Impulsive Bilomas.

"Biloma" is a collection of bile outside of the biliary tree that could occur postoperatively in patients who undergo laparoscopic cholecystectomy or in patients with blunt trauma to the liver. Spontaneous or impulsive bilomas with no identifiable cause occur rarely. We report a case of a 60-year-old woman with no history of hepatobiliary surgery or trauma, who was admitted for right upper quadrant pain. An abdominal examination revealed tenderness in the right upper quadrant (RUQ). Her alkaline phosphatase level was 2,343 IU/L. Computed tomography of the abdomen and pelvis with contrast showed perihepatic, periduodenal, and right paracolic gutter ascites. The image-guided aspiration of the peritoneal cavity yielded greenish fluid that was determined to be bile. The cytological studies were negative for malignancy and microorganisms. The ultrasound images of the RUQ were negative for cholecystitis and gallstones, and the results of the hepatobiliary nuclear scan study (HIDA) were unremarkable. Magnetic resonance cholangiopancreatography (MRCP) revealed an intact intrahepatic and extrahepatic biliary tree and confirmed the presence of multiple lakes of bile ascites. During the entire hospital stay, the patient remained stable without any unifying diagnosis and she was discharged with a pigtail catheter. A follow-up abdominal CT scan revealed a complete resolution of the bilomas. We consider this as a spontaneous extra- and intrahepatic biloma of unknown etiology and should be in our differentials when a patient presents with right upper quadrant abdominal pain.

Chest Wall Swelling in a Baby Boomer: An Unusual Presentation of Primary Hepatocellular Carcinoma.

Primary hepatocellular carcinoma (HCC) is one of the most frequently diagnosed cancers in adult men and a leading cause of cancer-related deaths worldwide. It also has an association with patients with hepatitis C-related cirrhosis. HCC usually metastasizes within the liver as well as to the lungs, regional lymph nodes, and adrenal glands, whereas the involvement of the chest wall and thoracic musculoskeletal system are more unusual. Herein, we report the case of a 58-year-old man who presented with swelling of the right anterolateral lower chest wall. The final diagnosis was primary HCC with distant metastases involving the right anterolateral ribs and left scapula. Such a presentation of extrahepatic HCC of this size and at this site is unique and has never before been reported in the literature. It reinforces the urgency and importance of screening all adults (18 years and above), particularly baby boomers, because three out of 100 have been infected with hepatitis C, at least once in their lifetime. It is also a wake-up call, as the incidence of primary HCC secondary to hepatitis C-associated cirrhosis has doubled, with a resultant increase in mortality. This HCC-related death might have been prevented if the patient had been screened for hepatitis C virus in his lifetime, as recommended by the American Association for the Study of Liver Diseases. We also discuss the latest developments in the diagnosis and management of HCC.