Autoimmune pancreatitis (AIP) can be chronic or recurrent, but frequently completely reversible after steroid treatment. A cystic lesion in AIP is a rare finding, and it can mimic a pancreatic cystic neoplasm. Difficulties in an exact diagnosis interfere with treatment, and surgery cannot be avoided in some cases. We report the history of a 63-year-old male presenting with jaundice and pruritus. AIP was confirmed by imaging and elevated IgG4 blood levels, and the patient completely recovered after corticosteroid therapy. One year later, he presented with a recurrent episode of AIP with elevated IgG4 levels, accompanied by the appearance of multiple intrapancreatic cystic lesions. All but 1 of these cysts disappeared after steroid treatment, but the remaining cyst in the pancreatic head was even somewhat larger 1 year later. Pancreatoduodenectomy was finally performed. Histology showed the wall of the cystic lesion to be fibrotic; the surrounding pancreatic tissue presented fibrosis, atrophy and lymphoplasmacytic infiltration by IgG4-positive cells, without malignant elements. Our case illustrates the rare possibility that cystic lesions can be part of AIP. These pseudocysts appear in the pancreatic segments involved in the autoimmune disease and can be a consequence of the local inflammation or related to ductal strictures. Steroid treatment should be initiated, after which these cysts can completely disappear with recovery from AIP. Surgical intervention may be necessary in some exceptional cases.
Autoimmune pancreatitis; Pseudocyst; Neoplastic cystic lesion; Intraductal papillary mucinous neoplasm; Pancreatic cancer
We report a rare case of spontaneous pneumoperitoneum. An 82-year-old Japanese male patient was referred to our hospital because of constipation and abdominal pain. Abdominal computed tomography revealed a large amount of feces in the colon and rectum, and free air in the abdomen. Based on these findings, the patient was diagnosed with gastrointestinal perforation. Emergency exploratory laparotomy was performed. Neither perforation nor ischemic changes were recognized in the digestive tract. The patient's defecation was managed postoperatively until discharge on the 13th postoperative day. The authors assumed that free air, which was released after a mucosal injury due to the internal pressure caused by the presence of a large amount of feces in the colon and rectum, had penetrated the bowel wall through the bowel mucosa. We herein report the present case while also reviewing the pertinent literature.
Spontaneous pneumoperitoneum; Constipation; Abdominal pain; Intestinal cystic pneumatosis; Differentiation
ALPPS (associating liver partition and portal vein ligation for staged hepatectomy) is a new surgical technique for patients in whom conventional treatment is not feasible due to insufficient future remnant liver (FRL). During the first stage of ALPPS, accelerated hypertrophy of the FRL is induced by ligation of the portal vein and in situ split of the liver. In the second stage, the deportalized liver is removed when the FRL volume has reached ≥25% of total liver volume. However, FRL volume does not necessarily reflect FRL function. 99mTc-mebrofenin hepatobiliary scintigraphy (HBS) with SPECT-CT is a quantitative test enabling regional assessment of parenchymal uptake function using a validated cut-off value for the prediction of postoperative liver failure (2.7%/min/m2). This paper describes the changes in FRL function and FRL volume in a 79-year-old patient diagnosed with metachronous colonic liver metastases who underwent ALPPS. We have observed a substantial difference between the increase in FRL volume and FRL function suggesting that HBS with SPECT-CT enables monitoring of the FRL function and could be a useful tool in the timing of resection in the second stage of the ALPPS procedure.
99mTc-mebrofenin hepatobiliary scintigraphy; Monitoring of liver function; Postoperative liver failure; ALPPS (associating liver partition and portal vein ligation for staged hepatectomy)
Capsule endoscope aspiration into the respiratory tract is a rare complication of capsule endoscopy. Despite the potential seriousness of this complication, no accepted methods exist to accurately predict and therefore prevent it. We describe the case of an 85-year-old male who presented for evaluation of iron deficiency anemia. He complained of dysphagia while ingesting a patency capsule, with several attempts over a period of 5 min before he was successful. Five days later, he underwent capsule endoscopy, where he experienced similar symptoms in swallowing the capsule. The rest of the examination proceeded uneventfully. On reviewing the captured images, the capsule endoscope was revealed to be aspirated, remaining in the respiratory tract for approximately 220 s before images of the esophagus and stomach appeared. To our knowledge, this is the first documented case of a patient who experienced capsule endoscope aspiration after ingestion of a patency capsule. This case suggests that repeated attempts required for ingesting the patency capsule can predict capsule endoscope aspiration. We presume that paying sufficient attention to the symptoms of a patient who ingests a patency capsule could help us prevent serious complications such as aspiration of the capsule endoscope. In addition, this experience implies the potential risk for ingesting the patency capsule. We must be aware that the patency capsule could also be aspirated and there may be more unrecognized aspiration cases.
Capsule endoscopy; Complication; Obscure gastrointestinal bleeding; Respiratory aspiration; Patency capsule
Myelofibrosis and gallbladder carcinoma are both very rare diseases. This case report describes a patient with a history of myelofibrosis and colorectal carcinoma who was diagnosed with colorectal liver metastases. Surgery was performed to remove the metastases, and on site, the gallbladder was removed because of involvement in one of the liver lesions. After pathological examination, a primary gallbladder carcinoma and myelofibrosis were found in addition to the liver metastases. The combination of diseases was not likely to be interconnected but rather an unlucky course of events for the patient.
Colorectal liver metastasis; Gallbladder carcinoma; Myelofibrosis
Neuroendocrine carcinomas of the esophagus are very rare, and the majority are high grade (poorly differentiated). They occur most frequently in males in their sixth and seventh decades of life. There have been no concrete data published on clinical features or on prognosis. We report a case of large-cell neuroendocrine carcinoma of the esophagus in a 66-year-old Saudi female with progressive dysphagia and weight loss. Upper endoscopy revealed an esophageal ulcerated mass.
Neuroendocrine carcinoma; Esophageal tumor; Large cell
Superior mesenteric artery (SMA) dissection without aortic dissection is a rare condition, and its diagnosis is considered to be difficult. Intestinal infarction is a severe complication of the disease, which may require resection of the intestine. We present a case of isolated SMA dissection. A 53-year-old man experienced sudden pain in the abdomen while playing Japanese pinball and was admitted to our hospital due to acute abdominal symptoms of uncertain cause. Enhanced CT revealed a defect of the root of the SMA, while angiography and intravascular ultrasound findings showed dissection of the SMA wall. Conservative treatment was chosen at the time, while a part of the small intestine was eventually resected because of progressive ischemia. Although SMA dissection is a rare occurrence in cases with acute abdominal symptoms, awareness of the condition is important for differential diagnosis.
Superior mesenteric artery dissection; Ischemia; Small intestine
Hepatitis E virus (HEV) infection is an emerging health concern in developing and developed countries, such as Japan. Five cases have recently been diagnosed as hepatitis E. Of interest, 3 of them had rheumatoid arthritis (RA), although a previous study demonstrated a lack of association between HEV and RA. One of the other patients developed autoimmune hepatitis and was successfully treated with corticosteroids approximately 150 days after the diagnosis of hepatitis E. In RA patients with liver dysfunction, the presence of HEV infection should be evaluated immediately because these patients are often relatively old. Further investigation of the association between HEV and autoimmune hepatitis is needed.
Autoimmune hepatitis; Hepatitis E virus; Rheumatoid arthritis; IgA anti-hepatitis E virus antibody
Descending necrotizing mediastinitis (DNM) is a rare condition in which oropharyngeal infection spreads to the mediastinum via the cervical fascia. Delayed diagnosis and surgery result in a high mortality rate among patients with DNM. We present a case of DNM resulting from odontogenic infection treated successfully with tooth extraction following mediastinal and cervical drainage. A 43-year-old, previously healthy Japanese man was admitted to our hospital for treatment of acute mediastinitis. Computed tomography revealed gas collection around the mid-thoracic esophagus and bilateral pleural effusion. We performed mediastinal drainage via right thoracotomy. Cervicotomy was performed on postoperative day 14 to drain a residual cervical abscess. The patient required the extraction of ten teeth over three procedures to address primary odontogenic infection before his fever resolved on postoperative day 40. Prompt diagnosis, aggressive drainage and removal of the source of infection can improve survival among patients with this life-threatening disease.
Descending necrotizing mediastinitis; Drainage; Tooth extraction
Pseudomyxoma peritonei (PMP) is a rare diagnosis with an incidence of 1–2 per million. Most cases originate from an appendix which ruptures and releases mucin into the peritoneal cavity. The progression of the disease results in obstruction and cutaneous leak. Abdominal compartment syndrome is an uncommon complication of peritoneal pseudomyxoma. In the present article, we report the case of a patient with PMP and abdominal compartment syndrome. A laparotomy to decrease the abdominal pressure was performed. Three months later, a peritonectomy with hyperthermic intraperitoneal chemotherapy was performed. The patient was still alive 1 year after the procedure without any recurrence. In conclusion, acute abdominal pain and respiratory failure in patients with peritoneal PMP should lead to the measurement of the abdominal pressure but are not a contra indication for curative treatment of PMP.
Pseudomyxoma; Compartment syndrome; Emergency; Hyperthermic intraperitoneal chemotherapy; Surgery
Patients on peritoneal dialysis (PD) are at increased risk for peritonitis. We report a case of a patient with end-stage renal disease on continuous ambulatory PD (CAPD) who developed peritonitis within 24 h of upper endoscopy with biopsy and colonoscopy with polypectomy. He had a previous history of peritonitis unrelated to invasive procedures and eventually was transitioned to hemodialysis because of his recurrent peritonitis. The International Society for Peritoneal Dialysis (ISPD) and newly revised American Society for Gastrointestinal Endoscopy (ASGE) guidelines recommend prophylactic antibiotics for CAPD patients undergoing endoscopic procedures. Other guidelines do not address this issue, and there has been limited evidence to support recommendations.
Ascites; Endoscopy; Peritonitis; Prophylactic antibiotics
In patients with portal hypertension, bleeding from rectal varices is rare. However, it can be life-threatening. We report a case of massive bleeding from large rectal varices in a 59-year-old man with alcoholic cirrhosis. Emergent transjugular intrahepatic portosystemic shunt (TIPS) placement was performed following failed local endoscopic therapy. Despite normalization of the portosystemic pressure gradient, the patient had another episode of massive bleeding on the following day. Embolization of the rectal varices via TIPS successfully stopped the bleeding. After the procedure, rapid decompensation of the cirrhosis led to severe encephalopathy, and death was observed. Although TIPSs have been reported to be useful in controlling bleeding from rectal varices, our case illustrates the potential pitfalls in using this technique in the treatment of rectal variceal bleeding. TIPSs may not be always successful in controlling massive bleeding from large rectal varices, even after normalization of portal hypertension. TIPSs can also be associated with life-threatening complications that may lead to early mortality.
Cirrhosis; Portal hypertension; Anorectal varices; Transjugular intrahepatic portosystemic shunt; Embolization; Liver failure; Hepatic encephalopathy
A 73-year-old female of Asian origin was diagnosed with ulcerative colitis (UC) after initial gastrointestinal symptoms of abdominal pain and bloody diarrhea. She had a relatively benign course over the subsequent 12 years. In 2009, she had increased left-sided abdominal pain, bloody diarrhea and progressive weight loss, due to a severe exacerbation. In spite of a variety of standard treatments, her condition continued to decline with a significant impact on normal life and functioning. In December of 2010, repeat colonoscopy and microscopy confirmed pancolitis, without diverticulitis. The Specific Carbohydrate Diet (SCD) was initiated due to failure of conventional therapies. Following this highly restricted diet, within a period of 3–6 months, improvement was noted, and within a year, no abdominal pain or diarrhea were present, and she returned to her baseline functioning and career. Two years later, repeat colonoscopy showed resolution of the pancolitis, confirmed with microscopic evaluation. Successful use of the SCD in children with UC has been documented. We describe previously unreported, highly beneficial results with both symptomatic and clinical improvement and complete remission of UC in an adult female with the SCD.
Ulcerative colitis; Inflammatory bowel disease; Dietary therapy
Sorafenib is an effective treatment for unresectable hepatocellular carcinoma (HCC) characterized by disease stabilization. However, the response rates are very low (<9%percnt;), and a complete response is rarely achieved. We report an extremely rare case of a HCC patient with multiple lung metastases treated with sorafenib who achieved a complete response for a long period. A 77-year-old woman was diagnosed with chronic hepatitis C in 1990. In 2007, a HCC detected in the liver was treated with percutaneous ethanol injection therapy. Subsequently, recurrence of HCC in the liver was treated with microwave coagulonecrotic therapy in 2010. In April 2011, a computed tomography (CT) scan revealed innumerable multiple metastases spread diffusely in both lungs. Tumor marker levels were extremely high [α-fetoprotein (AFP) 76,170 ng/ml, lens culinaris agglutinin-reactive fraction of AFP 7.5%percnt;, des-γ-carboxyprothrombin (DCP) 63,400 mAU/ml]. Sorafenib was administered at a reduced dose of 400 mg/day because of old age. Four months after sorafenib treatment, AFP and DCP had decreased to within normal levels, and the multiple lung metastases had disappeared. Currently, sorafenib is administered at a reduced dose of 400 mg/day, and the complete response has been maintained for 48 months.
Hepatocellular carcinoma; Sorafenib; Complete response; Lung metastases; Liver; Chemotherapy
We report 2 cases of ulcerative colitis (UC) with intestinal tract dilatation treated with tacrolimus. They were 53- and 64-year-old males, who had been admitted to local hospitals for increasing severity of their UC symptoms. Treatment for severe UC was immediately started, but both cases were refractory to corticosteroid therapy; they were then transferred to our hospital. When they were referred to our hospital, they had frequent bloody diarrhea, fever, severe abdominal pain, and even dilatation of the transverse colon on abdominal X-ray test. They were treated with oral tacrolimus medication, and their symptoms improved immediately. Dilatation of the transverse colon was improved on plain X-ray at 2 weeks after starting therapy, and emergency colectomy could be avoided. These 2 cases may suggest that tacrolimus is effective for UC with colonic dilatation as a rescue therapy.
Ulcerative colitis; Tacrolimus; Toxic megacolon; Intestinal tract dilatation
Metastatic breast cancer is typically identified in the bones, lymph nodes, lungs and liver. Rarely does metastatic breast cancer involve the common bile duct (CBD) without direct extension from liver metastasis into the CBD. We present a woman diagnosed with metastatic breast cancer in the CBD after presenting with obstructive jaundice. Patients with a history of primary breast cancer who present with obstructive jaundice secondary to CBD mass need identification of the mass in order to provide appropriate treatment.
Metastatic breast cancer; Common bile duct; Obstructive jaundice
Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare variant of hepatocellular carcinoma, usually presenting in the younger population (<40 years) without underlying liver disease. Although it has a better prognosis than hepatocellular carcinoma, it has a high rate of recurrence months to years after primary resection. While sites of recurrence usually involve the liver, regional lymph nodes, peritoneum, and lung, metastasis to the pancreas is extremely rare, with only 2 other cases reported in the literature. We present the case of a 46-year-old patient with metastatic FL-HCC to the pancreas 30 years after diagnosis and 26 years since his last resected liver recurrence.
Fibrolamellar hepatocellular carcinoma; Metastasis; Liver transplantation
Refeeding syndrome is defined as electrolyte and fluid abnormalities that occur in significantly malnourished patients when they are refed orally, enterally, or parenterally. The principal manifestations include hypophosphatemia, hypokalemia, vitamin deficiencies, volume overload and edema. This can affect multiple organ systems, such as the cardiovascular, pulmonary, or neurological systems, secondary to the above-mentioned abnormalities. Rarely, patients may develop gastrointestinal symptoms and show abnormal liver function test results. We report the case of a 52-year-old woman with anorexia nervosa who developed refeeding syndrome and simultaneous elevations of liver function test results, which normalized upon the resolution of the refeeding syndrome.
Refeeding syndrome; Anorexia nervosa; Abnormal liver function tests
Lichen planus (LP) is an idiopathic disorder that presents with cutaneous and genital manifestations. Esophageal LP (ELP) was first described by Al-Shihabi and Jackson [J Laryngol Otol 1982;96:567–571] in 1982. Only approximately 80 cases have been documented in the literature since. It is a rare and underrecognized disorder, leading to a delay in diagnosis and a lack of standardized management. We describe the presentation, diagnosis, and management of 6 cases of ELP, at a tertiary institution, because we believe that an increasing awareness of this condition can help identify more cases and increase our understanding of this interesting condition.
Esophageal lichen planus; Idiopathic disorder; Squamous cell carcinoma
Primary gastrointestinal T-cell lymphoma is an uncommon entity and primary colon T-cell lymphoma is even rarer. The majority of enteropathy-associated T-cell lymphomas present predominantly as ulcers or strictures in the endoscopic examinations, while primary B-cell lymphomas commonly present as exophytic lesions. Ulcerative colon T-cell lymphoma may mimic Crohn's disease (CD), which is a chronic inflammatory disease of the intestines with ulcer and fistula formations difficult for clinicians to diagnose based on endoscopic observations alone. Like CD, T-cell lymphoma may be characterized by the presence of multiple skipped ulcers distributed from the terminal ileum to the descending colon. Furthermore, it is difficult to diagnose this unusual lymphoma by a single endoscopic biopsy. Typically, the histological composition of T-cell lymphoma is made of medium to large atypical cells located in the base of the ulcer with extension to the muscle layer and the adjacent mucosa. However, it is common that biopsy specimens show only mixed inflammatory changes where the lymphoma cells are hard to be identified. The differential diagnosis of malignant lymphoma must be considered when clinically diagnosed CD is refractory to the medical treatment or when its clinical behavior becomes aggressive. The current study presents a rare case of primary colon T-cell lymphoma in a 56-year-old male with marked recent weight loss, watery diarrhea and bilateral neck lymphadenopathy, who received a laboratory checkup and endoscopic workup for colon biopsy. The initial pathological report was consistent with mucosal inflammation and benign colon ulcers. Interestingly, the blood test showed a prominent eosinophilia. A biopsy of the enlarged neck lymph nodes done approximately 1 month after the colon biopsy unexpectedly showed T-cell lymphoma, which led to a review of the initial colonic biopsy specimens. Additional immunohistochemical stains were used accordingly, which showed positive results for CD3, CD45RO and LCA antibodies confirming the diagnosis of lymphoma. The endoscopic diagnosis of ulcerative colon T-cell lymphoma is frequently confused with inflammatory conditions of the large bowel such as CD, and tuberculosis colitis. Our study aims to emphasize the difficulty in differentiating this ulcerative form of colon T-cell lymphoma from the inflammatory bowel diseases and the importance of its differential diagnosis due to the much more aggressive clinical behavior of the T-cell lymphoma.
T-cell lymphoma; Colitis; Eosinophilia; Crohn's disease
Mixed adenoneuroendocrine carcinoma is a rare condition comprising at least 30%percnt; of each component of exocrine and endocrine tumors. The denominations were defined in the 2000 WHO classification of endocrine tumors. We report an 83-year-old male with a polypoid gastric tumor in the gastric high body who received total gastrectomy and died 8 months after the diagnosis from local recurrence and distal metastases. A review of the literature for this infrequent condition is presented.
Adenoneuroendocrine carcinoma; MANECs; Gastric cancer
Pancreatic and gastric heterotopias are rare clinical entities which have been identified throughout the entire length of the gastrointestinal tract. Combined gastric and pancreatic heterotopias, although unusual, have been described in the duodenum and jejunum, and in other structures, including Meckel's diverticulum and the ampulla of Vater. We report a novel case of pancreatic and gastric heterotopia with an associated submucosal lipoma in a 38-year-old female with a recent history of rectal cancer and chronic crampy abdominal pain. On computed tomography, a 7-cm luminal polypoid mass extending into the distal ileum was discovered. The mass was successfully resected using retrograde double balloon enteroscopy. We believe this is the first report of all three histological entities co-existing in an obstructive ileal lesion in an adult. It highlights endoscopic resection trough double enteroscopy as a safe alternative to more invasive surgical approaches for this type of lesion.
Gastric heterotopia; Pancreatic heterotopia; Double balloon enteroscopy; Intermittent bowel obstruction
A bezoar is a mass of indigestible material. Bezoars can present with a gradual onset of non-specific gastrointestinal symptoms including abdominal pain, nausea and vomiting. However, bezoars can result in more serious conditions such as intestinal bleeding or obstruction. Without quick recognition, particularly in susceptible individuals, the diagnosis and treatment can be delayed. Currently resolution is achieved with enzymatic dissolution, endoscopic fragmentation or surgery. We describe, to our knowledge, the first pediatric patient with lymphoma to have had a bezoar treated with Coca-Cola.
Bezoar; Coca-Cola; Oncology; Intestinal dysmotility
Glycogenic hepatopathy (GH) remains underrecognized in adults as most clinicians mistake it for the more common hepatic abnormality associated with uncontrolled diabetes mellitus in this age group, non-alcoholic fatty liver disease. This is also complicated by the fact that both entities are indistinguishable on liver ultrasound. We herein describe a similar predicament in which a young adult female presented with bilateral upper quadrant abdominal pain, tender hepatomegaly, lactic acidosis and a >10-fold increase in liver enzymes, which worsened after the administration of high-dose steroids. Despite intravenous normal saline resuscitation, serum transaminitis persisted in a fluctuating manner. Ultimately, a liver biopsy confirmed GH. Biochemically, GH is driven by high amounts of both circulating glucose and insulin or by the administration of high-dose steroids. Improving glycemic control is the mainstay of treatment for GH. However, in our case, improvement in glycated hemoglobin of just 0.6% was enough to achieve symptomatic relief, supporting recent claims of the involvement of other identified factors in disease development.
Glycogenic hepatopathy; Hepatomegaly; Elevated liver enzymes; Non-alcoholic fatty liver disease; Celiac disease; Dual-echo magnetic resonance imaging
Direct-acting antivirals with or without peginterferon α (PEG-IFN α) plus ribavirin are now available for the treatment of hepatitis C virus (HCV) infection. Direct-acting antivirals are potent inhibitors of HCV replication, but some of them occasionally possess serious adverse events. We experienced a 64-year-old female with chronic HCV genotype 1b infection who showed elevated alanine aminotransferase of 528 IU/l at week 9 after the commencement of treatment of simeprevir with PEG-IFN α-2a plus ribavirin. However, she achieved sustained virological response at week 24 after the end of treatment. In Japan, we also have to treat elderly patients infected with HCV and/or advanced hepatic fibrosis. Until an effective interferon-free regimen is established, direct-acting antivirals with PEG-IFN plus ribavirin may still play a role in the treatment for certain patients. To avoid serious results from adverse events, careful attention and follow-up will be needed in the treatment course of simeprevir with PEG-IFN plus ribavirin for chronic HCV infection.
Hepatitis C virus; Simeprevir; Peginterferon; Ribavirin; Sustained virological response