Hepatocellular carcinoma risk in HBeAg-negative chronic hepatitis B patients with or without cirrhosis treated with entecavir: HepNet.Greece cohort.

Hepatocellular carcinoma (HCC) may still develop in chronic hepatitis B (CHB) patients treated with lamivudine. Whether HCC rates are comparable in patients treated with the current first-line antivirals remains uncertain. We estimated the incidence and evaluated predictors of HCC in a large nationwide prospective cohort (HepNet.Greece) of HBeAg-negative CHB patients treated with entecavir. HBeAg-negative CHB patients from the same cohort who were initially treated with lamivudine were used as controls. We included 321 patients treated with entecavir for a median of 40 months and 818 patients treated initially with lamivudine for a median of 60 months. In the entecavir group, HCC developed in 4 of 321 (1.2%) patients at a median of 1.5 (range: 1.0-4.5) years, while the cumulative HCC incidence was significantly higher in cirrhotics than noncirrhotics (1, 3, 5 years: 0%, 3%, 9% vs 1%, 1%, 1%; P = 0.024) and in older patients (P = 0.026). Entecavir compared with lamivudine group patients had lower HCC incidence (1, 3, 5 years: 0.3%, 1.2%, 2.8% vs 0.7%, 3.8%, 5.6%; P = 0.024). However, in multivariable Cox regression analysis, the HCC risk was independently associated with older age (P < 0.001), male gender (P = 0.011) and cirrhosis (P = 0.025), but not with the initial agent. In conclusion, our large nationwide study indicates that the HCC risk remains increased in entecavir-treated HBeAg-negative CHB patients with cirrhosis, particularly of older age, at least for the first 5 years. The HCC risk does not seem to be significantly reduced with entecavir compared with antiviral therapy starting with lamivudine.

Can capsule endoscopy be used as a diagnostic tool in the evaluation of nonbleeding indications in daily clinical practice? A prospective study.

OBJECTIVE: To evaluate the diagnostic yield of capsule endoscopy (CE) and its impact on treatment and outcome in patients without bleeding indications. SUBJECTS AND METHODS: One hundred and sixty-five nonbleeding patients were enrolled in the study. The most common indications for CE were chronic abdominal pain alone (33 patients) or combined with chronic diarrhea (31 patients) and chronic diarrhea alone (30 patients). Among the 165 patients, 129 underwent CE for evaluation of gastrointestinal symptoms and 36 for surveillance or disease staging. RESULTS: CE findings were positive, suspicious and negative in 73 (44.2%), 13 (7.9%) and 79 (47.9%) of cases, respectively. The diagnostic yield was highest in patients with refractory celiac disease (10/10, 100%) and suspected Crohn's disease (5/6, 83.3%), followed by patients with chronic abdominal pain and chronic diarrhea (13/31, 41.9%), established Crohn's disease (2/6, 33.3%), chronic diarrhea alone (8/30, 26.7%), chronic abdominal pain alone (8/33, 24.2%) and other indications (3/13, 23.1%) (p < 0.005). The CE findings led to a change of medication in 74 (47.7%) patients, surgery in 15 (9.7%), administration of a strict gluten-free or other special diet in 13 (8.4%) and had other consequences in 11 (6.7%). Management was not modified in 42 (27.1%) patients. Among symptomatic patients (n = 129), 29 (22.5%) were lost to follow-up. The remaining 100 patients were followed up for 8.7 ± 4.0 months (range 2-19). Among the latter, resolution or improvement of symptoms was observed in 86 (86%) patients, no change in 11 (11%) and 3 (3%) died. All 86 patients who experienced resolution or improvement of their symptoms had a modification of their management after CE; only 7/11 patients whose symptoms did not change (63.6%) and 2/3 patients who died (66.7%) had a modification of management (p < 0.001). CONCLUSIONS: CE appears to be a useful tool in the evaluation of patients with nonbleeding indications. The outcome of most patients with negative findings was excellent.

Real-time PCR detection of Helicobacter pylori clarithromycin resistance in Thrace, Greece.

BACKGROUND: Growing resistance to clarithromycin is a major concern regarding treating Helicobacter pylori (H. pylori) infection. Resistance rates have a great variation even in different geographic areas within the same country and are associated with point mutations of the microbial 23S rRNA (A2142C, A2142G, and A2143G). Given the absence of available data in Thrace, the objective of this study was to estimate the resistance of H. pylori to clarithromycin and identify specific mutations that contribute to clarithromycin resistance. METHODS: In this prospective study, we enrolled consecutive patients referred for dyspeptic complaints who underwent upper gastrointestinal endoscopy over two years. Gastric biopsies from corpus and antrum were initially tested for the presence of urease by a rapid urease test. Urease positive samples were followed by real-time PCR to confirm the presence of H. pylori and to detect point mutations. RESULTS: A total of one hundred and thirty patients were included in the study (72 women and 58 men). Resistance to clarithromycin was detected at 23.2 %. Neither gender nor age was independently correlated with resistance rate in our patient group. The most common mutations were A2142G and A2143G. CONCLUSIONS: A high rate of H. pylori resistance to clarithromycin was observed in our region, implicating that it should be addressed in accordance with the recommendations provided by national and international guidelines. Molecular testing should be considered an integral tool for effective monitoring in case of suspected antibiotic resistance. HIPPOKRATIA 2021, 25 (2):51-55.

Smoking habits and gallbladder disease: a systematic review and meta-analysis study.

BACKGROUND: It has been claimed that smoking is linked with an increased risk for gallbladder disease (GBD); however, related issues need further consolidation and clarification. The present systematic review and meta-analysis aimed to further investigate the potent correlation between GBD and smoking. METHODS: We conducted a comprehensive literature review to identify every study published from January 1989 to December 2019, reporting risk estimates regarding GBD and smoking. The random-effect, generic inverse variance method, according to description by DerSimonian and Laird, was used to compute pooled estimates. We used the Newcastle-Ottawa quality assessment scale to appraise the included studies' quality. RESULTS: Thirty published case-control, cross-sectional, and cohort studies including 4,623,435 individuals met the eligibility criteria and were considered for data synthesis. Compared to the non-smokers, ever smokers had 1.25 times higher odds of developing GBD [95 % confidence interval (CI): 1.09-1.44]; however, increased heterogeneity was observed (I2 =96 %, 95 % CI: 62-100 %, p <0.001). Publication bias was non-significant (Eggers' regression p =0.072). The main sources of heterogeneity, as detected by meta-regression analyzing study characteristics, biases and confounders, were non-adjustment for family history (p =0.007) and alcohol (p =0.020), respectively. Subgroup analysis indicated a comparable risk for GBD as far as current, former and ever smokers are concerned (p =0.520). Quantitative analysis suggested a dose-effect for current smoking and GBD (p =0.010). CONCLUSIONS: Non-smokers were demonstrated to be at a lower risk of presenting GBD when compared with ever smokers; all relevant risk estimates necessitate adjustment for family history and alcohol intake. HIPPOKRATIA 2020, 24(4): 147-156.

Recurrence of HCV infection in a sustained responder after chemotherapy for non-Hodgkin's lymphoma: successful retreatment.

It is known that sustained virological response (SVR) in patients with chronic hepatitis C is associated with sustained elimination of hepatitis C virus (HCV) and that late relapse after SVR in HCV patients is doubtful. A 47-year-old man with chronic hepatitis C genotype 3, achieved SVR after combination treatment with pegylated interferon and ribavirine for 6 months. Sixteen months later non-Hodgkin's lymphoma was diagnosed. After successful completion of chemotherapy for non-Hodgkin's lymphoma, he presented with HCV infection recurrence of the same genotype. Retreatment with the same schedule resulted in normalization of aminotransferases and disappearance of HCVRNA from the serum. This case suggests that recurrence of HCV infection in a sustained responder may be probable after immunosuppressive therapy. Prevention is currently impossible but retreatment may be successful.

Fatal acute acalculous cholecystitis as an early complication after radiation therapy..

Acute acalculous cholecystitis (AAC) is a life-threatening condition whose incidence is steadily increasing, although it is still very much lower than that of the corresponding calculus form. The severity of the disease is due to the rapid course towards gallbladder necrosis and biliary peritonitis. Traditionally, it has been thought that AAC is associated with recent trauma, overeating or major surgical procedures. We describe a patient who presented acute cholecystitis, two days after completion of radiation therapy for metastatic lymphadenopathy along the hepatoduodenal ligament and distal common bile duct. He underwent exploratory laparotomy but he died from uncontrolled sepsis three days later. Histological study of the resected gallbladder showed findings of acute acalculous cholecystitis.

Subcutaneous metastasis from a gastrointestinal stromal tumor of the stomach: a case report.

Gastrointestinal stromal tumors (GISTs) are rare mesenchymal neoplasms of the stomach, which account for approximately 3.6% of all gastric tumors. They may or may not be malignant. Malignant GIST rarely metastasizes to distant organs. We report a case of a gastric GIST diagnosed in a 69- year-old woman presented with a synchronous subcutaneous paraumbilical metastasis. Computed tomography (CT) scan demonstrated a space-occupying lesion arising from the gastric wall with a second well-circumscribed lesion in the subcutaneous tissue which infiltrated the aponeurosis of the right rectus abdominis. The patient underwent total gastrectomy and resection of the subcutaneous mass. Pathologic examination of the gastric tumor and subcutaneous mass showed histological and immunohistochemical characteristics of a GIST. The patient succumbed on the 4th postoperative day. Gastric stromal tumor metastasis must be taken into consideration in the differential diagnosis of a palpable paraumbilical mass in a patient diagnosed with malignant GIST.

Improved survival of patients presenting with acute variceal bleeding. Prognostic indicators of short- and long-term mortality.

BACKGROUND AND AIM: Variceal bleeding is a severe complication of portal hypertension with a mortality rate between 30% and 60% in previous studies. During the last two decades the treatment of these patients has been improved. The aim of this study was to investigate the clinical outcome of patients after an episode of acute variceal bleeding and to identify risk factors for early and late mortality in these patients. MATERIALS AND METHODS: All patients with acute variceal bleeding hospitalised at two large hospitals between January 1, 1999 and June 30, 2004, were retrospectively enrolled in this study. After discharge, patients were followed until death or study closure date, on June 30, 2005. Bleeding related mortality, 6-week, 1-year and overall mortality were evaluated as well as factors related to early and late mortality. RESULTS: One hundred and forty one patients were included (114 men, 27 women) with a mean age of 60.5+/-13.5 years. In hospital, 6-week, 1-year and overall mortality were 12.1%, 18.4%, 32.6% and 48.2%, respectively. The mean length of hospitalisation was 11.4+/-9 days (1-55) and the mean packed red blood cell requirement was 3.9+/-3.7 (0-25). The rate of recurrent bleeding was 10.7% during initial hospitalisation. Being Child-Pugh C (p=0.003) and shock on admission (p=0.037) were independent predictors of 6-week mortality, while being Child-Pugh C (p=0.028), presence of hepatocellular carcinoma or other neoplasia (p=0.04) and partial thromboplastin time (p=0.021) during the initial admission were independent predictors for 1-year mortality. Mortality was not affected by the presence of active bleeding and/or white nipple at emergency endoscopy. Also presence of infection was not an adverse factor of clinical outcome in our patients. CONCLUSIONS: In conclusion, the clinical outcome of patients with acute variceal bleeding is better in comparison with previous studies. The severity of liver failure as well as the presence of neoplasia mainly affects the survival.

Safety and long-term follow-up of endoscopic snare excision of ampullary adenomas.

BACKGROUND: Adenomas of the duodenal papilla are rare. Because of their malignant potential, resection is mandatory. Options for resection include endoscopic resection techniques, transduodenal local excision, and pancreaticoduodenectomy. The aim of this retrospective study was to evaluate the safety and outcome of endoscopic snare resection of papillary adenomas in a Greek cohort of patients. METHODS: Fourteen patients (six women and eight men; age range, 42-76 years) were referred for endoscopic management of ampullary adenomas. A questionnaire was completed for each patient, which included preoperative and postoperative data points. Presenting symptoms were jaundice (n = 4), cholangitis (n = 1), and pain (n = 2). Seven patients were asymptomatic. If there was no common bile and main pancreatic duct invasion and the appearance suggested a benign lesion, biductal sphincterotomy onto normal duodenal tissue was performed. The adenomas were resected via a diathermy snare, along with the major papilla, after elevation of the lesion by epinephrine plus dextrose 50% (1:10,000) solution. At the discretion of the endoscopist, a biliary or pancreatic stent was inserted as a prophylactic procedure immediately after excision. RESULTS: Histopathologically, resected tissue included 11 adenomas and three adenomas with focal malignancy, referred for pancreaticoduodenectomy. Immediate complications were moderate bleeding (n = 1) and mild pancreatitis (n = 1). No procedure-related death occurred. Follow-up was available for 11 patients (mean, 28.36 months; range, 6-72). Pancreatic and biliary stents were placed in four and nine patients, respectively. Follow-up endoscopy revealed recurrent/residual adenomatous tissue in two patients (18%), which was resected endoscopically. CONCLUSION: Endoscopic snare resection of adenomas of the major duodenal papilla is a safe, well-tolerated alternative to surgical therapy. In expert hands, complications are mild and may be avoided by pre-resection biductal sphincterotomy, stent placement, and elevation of the lesion by epinephrine plus dextrose 50% solution injection.

Factors predicting a positive capsule endoscopy in past overt obscure gastrointestinal bleeding: a multicenter retrospective study.

OBJECTIVES: Capsule endoscopy (CE) remains the examination of choice for the investigation of obscure gastrointestinal bleeding. Although the factors predicting positive CE findings in the overall obscure gastrointestinal bleeding have been investigated, the clinical characteristics that predict a positive CE in patients with past overt obscure gastrointestinal bleeding (OOGIB) have not been systematically studied. METHODS: Between September 2004 and December 2013, 262 patients underwent CE for evaluation of past OOGIB after negative upper and lower endoscopy, and other diagnostic modalities. Patients' records were retrospectively reviewed to assess the factors that could possibly predict positive CE findings. RESULTS: Two hundred and twenty four patients with a median age of 70 years (range: 17-87) were enrolled in the final analysis and were divided into two groups; those who had positive (group A: 118 patients) and those who had negative CE findings (group B: 106 patients). The overall diagnostic yield of CE was 52.68 %. Multivariate analysis demonstrated that age >65 years, anticoagulant use, antiplatelet use, and non-steroidal anti-inflammatory drugs use were independent predictive factors for positive findings on CE. Of the 118 patients with positive CE, therapeutic interventions were performed in 56 patients (47.46 %). Recurrence of bleeding presented in nine patients of group B compared with 39 patients of group A (p <0.001). CONCLUSIONS: Certain clinical characteristics predict a positive CE in patients with past OOGIB. Patients with OOGIB and negative CE had a considerably lower rebleeding rate, and further invasive investigational procedures may be adjourned or may not be required, though such recommendation warrants further validation. Hippokratia 2016, 20(2): 127-132.

Association of antiviral therapy with reduced disease progression in chronic Hepatitis B patients: Results from a nation-wide cohort study.

BACKGROUND AND AIMS: Although effective treatment in terms of inducing virological and biochemical response for chronic hepatitis B (CHB) is available, its effect on the clinical course of the disease has not yet been accurately estimated. Objective of this study was to evaluate the effect of antiviral therapy and its type [interferon +/- nucleos(t)ide analogs (NAs) vs. NAs] on the occurrence of a clinical event (liver decompensation, liver transplant, hepatocellular carcinoma and death from a liver-related cause) in CHB patients. METHODS: The study population was derived from the HEPNET-Greece, a nationwide cohort study aimed to evaluate the current epidemiological course of viral hepatitis. To account for time-dependent confounding, Cox marginal structural models were used to analyze data. RESULTS: Thirty out of 2,125 eligible patients experienced a clinical event during their follow-up. When comparing treated to untreated individuals, the hazard ratio (HR) for a clinical event was 0.39 (95% CI: 0.16-0.98; p =0.044) in the whole sample, whereas there were indications of a more intense effect in the subgroup of patients with cirrhosis at presentation (HR =0.16, 95% CI: 0.02-1.21; p =0.075). The effect of Interferon initiated treatment was not significantly different of that of NAs. There was some evidence, albeit not statistically significant, of a protective treatment effect on hepatocellular carcinoma development (HCC). CONCLUSIONS: Data from observational studies can provide useful inference, provided they are analyzed appropriately. The current study has shown that the available treatment options for CHB offer a significant clinical benefit to CHB infected individuals. Hippokratia 2016, 20(3): 214-221.

Non-invasive predictors of the presence of large oesophageal varices in patients with cirrhosis.

BACKGROUND/AIMS: The usual clinical practice is to screen all patients with established cirrhosis at the time of diagnosis by upper endoscopy for the presence of varices. Patients with large varices should be treated with non-selective beta blockers to reduce the incidence of first variceal bleeding. However, fewer than 50% of cirrhotic patients have varices at screening endoscopy and most have small sized varices, with a low risk of bleeding. The aim of the present study was to determine whether clinical or laboratory non-endoscopic parameters could predict the presence of large oesophageal varices. PATIENTS/METHODS: Seventeen variables considered relevant to the prevalence of oesophageal varices were tested in 184 patients with cirrhosis, who underwent screening endoscopy. Small varices were regarded as those which flatten with insufflation or slightly protrude into the lumen, while large varices are those which protrude into the lumen or touch each other. None of the patients was on beta blockers or other vasoactive drugs or had a history of variceal bleeding. RESULTS: Oesophageal varices were present in 92 patients (50%), and large varices in 33 patients (17.9%). Variables associated with the presence of large oesophageal varices on univariate analysis were the presence of ascites and splenomegaly either by clinical examination or by ultrasound (p < 0.01), the presence of spiders (p = 0.02), platelet count (p < 0.0001), and bilirubin (p = 0.01). Factors independently associated with the presence of large oesophageal varices on multivariate analysis were platelet count, size of spleen and presence of ascites by ultrasound. Using mean values as cut-off points, it is noteworthy that only five out of 39 patients (12.8%) with platelets > or = 18(x 10(9)/l), spleen length < or = 135 mm and no ascites had varices. Moreover, all these patients had small sized varices. On the other hand, 15 out of 18 patients (83.3%) with a platelet count < 118 x 10(9)/l, spleen length > 135 mm and ascites had varices. Moreover, five out of those 18 patients had large varices (28.3%). CONCLUSION: Thrombocytopenia, splenomegaly and ascites are independent predictors of large oesophageal varices in cirrhotic patients. We suggest that endoscopy could be avoided safely in cirrhotic patients with none of these predictive factors, as large varices are absent in this group of patients.

Needle-knife suprapapillary sphincterotomy avoids postprocedure pancreatitis in patients with sphincter of Oddi dysfunction of biliary type II: a report of three cases.

We report the cases of three patients who fulfilled the criteria for sphincter of Oddi dysfunction of biliary type II and underwent needle-knife suprapapillary sphincterotomy. These patients presented with episodes of biliary-type pain after cholecystectomy and significant elevation of liver enzymes. Ultrasonography and MRI cholangiography revealed dilatation of the common bile duct, without visible stones. The patients all underwent needle-knife suprapapillary sphincterotomy because free cannulation of the common bile duct could not be achieved. Needle-knife suprapapillary sphincterotomy enabled catheterization of the common bile duct. After clearing of the common bile duct with a balloon catheter, no stones, fragments of stones, or sludge were observed to exit from the sphincterotomy. None of our patients developed postprocedure pancreatitis. When needle-knife suprapillary sphincterotomy is performed by an experienced biliary endoscopist, it is a safe and effective procedure for patients with sphincter of Oddi dysfunction of biliary type II, who otherwise constitute a high-risk group for the development of postsphincterotomy pancreatitis.

Evaluation of contrast echocardiography and lung perfusion scan in detecting intrapulmonary vascular dilatation in normoxemic patients with early liver cirrhosis.

BACKGROUND/AIMS: Intrapulmonary vascular dilatations (IPVD) are extrahepatic complications occurring in liver transplant candidates, that can result in severe hypoxemia. The aim of this study was to compare the use of two diagnostic modalities, contrast echocardiography and lung perfusion scan, in detecting IPVD in normoxemic patients with early liver cirrhosis. METHODOLOGY: Fifty-six consecutive outpatients with biopsy-proven cirrhosis had contrast-echocardiography, a lung perfusion scan, pulmonary function tests, and arterial blood gas estimations. All patients were grade A or B according to the Child-Pugh classification. Patients with chronic intrinsic lung disease, heart failure or malignancy were excluded from the study. RESULTS: All patients had normal arterial blood-gas analyses. Eight out of 56 patients (14.3%) had a positive contrast echocardiogram, all with a decreased diffusion capacity (DLCO < 75% of the predicted value). An isolated DLCO impairment was observed in 40% of the patients with normal spirometry. None of the patients with echocardiography-proven IPVD had a positive lung perfusion scan (p<0.005). CONCLUSIONS: In normoxemic cirrhotic patients, subclinical pulmonary vasodilatation and gas-exchange abnormalities can occur. Contrast-enhanced echocardiography is the most valuable screening test in detecting IPVD in the early stages of hepatic insufficiency.

HCV infection in the general population of a Greek island: prevalence and risk factors.

BACKGROUND/AIMS: Although HCV seroprevalence in blood donors in Greece is low (0.2-0.4%) epidemiologic characteristics of HCV infection in the general population have not been studied enough. The objective of this study was to examine the seroprevalence of HCV infection and associated risk factors in the general population of Zakinthos, a Greek island with a well-defined mixed (urban and rural) population. METHODOLOGY: A household health survey was carried out in a randomly selected sample of 718 adults. A questionnaire was completed and a blood sample was obtained from all participants. Serum samples were tested for anti-HCV antibodies by third generation enzyme-linked immunosorbent assay and supplemental test. The influence of sociodemographic characteristics and possible associated risk factors on the HCV seroprevalence was investigated by logistic regression analysis. RESULTS: The overall anti-HCV prevalence was 1.25%. A well-defined rural area with a significant higher prevalence (6.8% vs. 0.62%; P < 0.001) was identified. There was a trend of increasing prevalence with age, with a significant difference (P < 0.027) between the age groups 15-44 (0%) and over 45 (2.15%). The logistic regression analysis confirmed a significant association between anti- HCV positivity and: increasing age (P < 0.001), history of blood transfusion (0.0001), intramuscular injections (P < 0.04). CONCLUSIONS: The results of this field-survey in a well-defined general population, indicates that HCV seroprevalence (1.25%) is much higher than that of blood donors in the same area. The increasing prevalence with age and the association with parenteral exposure indicates that HCV infection can mainly be attributed to parenteral techniques in the past. The identification of a concrete rural area with particularly high seroprevalence needs further study of the whole population of the area.

Needle-knife papillotomy: a safe and effective technique in experienced hands.

BACKGROUND/AIMS: Results from studies evaluating needle-knife papillotomy are conflicting. The aim of this retrospective study was to assess the safety and efficacy of needle-knife papillotomy as a precut procedure to achieve biliary access during ERCP. METHODOLOGY: During a period of seven years, ERCP was performed 938 times. During this time, needle-knife papillotomy was carried out in 68 patients, with complete follow-up obtained in all patients. The follow-up concentrated on the safety and efficacy of the procedure and short-term complications. RESULTS: Cannulation of the common bile duct was successful immediately after needle-knife papillotomy in 44 patients (66%), during a second ERCP in 18 patients (26%), and in a third ERCP in 2 patients (3%) achieving a total cannulation rate of 94%. There were no needle-knife papillotomy related deaths. Complications included bleeding in 5 patients (7%), and pancreatitis in 3 patients (4%). All complications were managed conservatively. CONCLUSIONS: Our experience indicates that needle-knife papillotomy is a versatile, effective and safe technique of gaining biliary access in patients in whom deep cannulation proves impossible and biliary access is considered essential.

Predictive value of risk factors in patients with obstructive jaundice.

Clinical and laboratory parameters that may affect post-operative mortality and morbidity were studied in 215 patients operated on for obstructive jaundice. The cause of the bile duct obstruction was benign disease in 56.7% of patients and malignant disease in 43.3%. Overall postoperative mortality and morbidity rates were 21.9% and 33.5%, respectively. Eight risk factors were shown to be associated with increased mortality: age > 70 years; malignancy; leucocytosis > 15 000 cells/microl and fever > 38.5 degrees C; haematocrit < or = 30%; creatinine > 1.3 mg/dl; albumin < or = 3 g/dl; bilirubin > 20 mg/dl; and serum alkaline phosphatase > 100 IU/l. The simultaneous presence of less than three risk factors was always associated with post-operative survival, but concomitant existence of seven or more risk factors indicated 100% mortality. Understanding the factors related to post-operative morbidity and mortality in patients with obstructive jaundice will better guide appropriate surgical or non-surgical management and lead to improved survival.

Intussusception of the small bowel due to Peutz-Jeghers syndrome: a case report.

The Peutz-Jeghers syndrome is an autosomal dominant disorder characterized by hamartomatous polyposis of the gastrointestinal tract, melanin pigmentation of the skin and mucous membranes, and an increased risk for cancer. The incidence of surgical complications in these patients is relatively rare, and correlates with the size and location of the polyps. Herein we report the case of a 27-year-old woman presented with episodes of abdominal pain, abdominal distention and intermittent vomiting. Moreover, multiple pigmentation of the mouth was also noted. A preoperative diagnosis of a double jejunal intussusception and jejunal occlusion was based on the findings of small bowel enema and computed tomography. The diagnosis was confirmed at laparotomy.