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IntroductionDespite evidence-based guidelines, exocrine pancreatic insufficiency is frequently underdiagnosed and undertreated in patients with chronic pancreatitis. Therefore, the aim of this study is to provide insight into the current opinion and clinical decision-making of international pancreatologists regarding the management of exocrine pancreatic insufficiency.MethodsAn online survey and case vignette study was sent to experts in chronic pancreatitis and members of various pancreatic associations: EPC, E-AHPBA and DPSG. Experts were selected based on publication record from the past 5 years.ResultsOverall, 252 pancreatologists participated of whom 44% had ≥ 15 years of experience and 35% treated ≥ 50 patients with chronic pancreatitis per year. Screening for exocrine pancreatic insufficiency as part of the diagnostic work-up for chronic pancreatitis is performed by 69% and repeated annually by 21%. About 74% considers nutritional assessment to be part of the standard work-up. Patients are most frequently screened for deficiencies of calcium (47%), iron (42%), vitamin D (61%) and albumin (59%). In case of clinically steatorrhea, 71% prescribes enzyme supplementation. Of all pancreatologists, 40% refers more than half of their patients to a dietician. Despite existing guidelines, 97% supports the need for more specific and tailored instructions regarding the management of exocrine pancreatic insufficiency.ConclusionThis survey identified a lack of consensus and substantial practice variation among international pancreatologists regarding guidelines pertaining the management of exocrine pancreatic insufficiency. These results highlight the need for further adaptation of these guidelines according to current expert opinion and the level of available scientific evidence. Introduction: Despite evidence-based guidelines, exocrine pancreatic insufficiency is frequently underdiagnosed and undertreated in patients with chronic pancreatitis. Therefore, the aim of this study is to provide insight into the current opinion and clinical decision-making of international pancreatologists regarding the management of exocrine pancreatic insufficiency. Methods: An online survey and case vignette study was sent to experts in chronic pancreatitis and members of various pancreatic associations: EPC, E-AHPBA and DPSG. Experts were selected based on publication record from the past 5 years. Results: Overall, 252 pancreatologists participated of whom 44% had ≥ 15 years of experience and 35% treated ≥ 50 patients with chronic pancreatitis per year. Screening for exocrine pancreatic insufficiency as part of the diagnostic work-up for chronic pancreatitis is performed by 69% and repeated annually by 21%. About 74% considers nutritional assessment to be part of the standard work-up. Patients are most frequently screened for deficiencies of calcium (47%), iron (42%), vitamin D (61%) and albumin (59%). In case of clinically steatorrhea, 71% prescribes enzyme supplementation. Of all pancreatologists, 40% refers more than half of their patients to a dietician. Despite existing guidelines, 97% supports the need for more specific and tailored instructions regarding the management of exocrine pancreatic insufficiency. Conclusion: This survey identified a lack of consensus and substantial practice variation among international pancreatologists regarding guidelines pertaining the management of exocrine pancreatic insufficiency. These results highlight the need for further adaptation of these guidelines according to current expert opinion and the level of available scientific evidence.

Introduction. Colorectal liver metastases have a poor prognosis and only 2% have an average 5-year survival if left untreated. In recent decades there has been a development in the diagnosis, treatment and palliative treatment of patients with colorectal liver metastases, and despite radical resection the average five-year survival is between 25% and 44%. Aim. To explore the experience of the Clinic in the treatment of colorectal liver metastases, comparing it with data from the literature and based on the comparison to determine the prognostic factors that affect survival after radical surgical treatment of patients. Methods. A retrospective study was conducted at the Clinic of General and Hepato-pancreatic Surgery at the University Hospital “Aleksandrovska”-Sofia. The study comprised the period between 01.01.2006 to 31.12.2015. It included a total of 239 cases, of whom: 179 patients underwent radical interventions, 5 palliative and 55 patients underwent explorative interventions due to liver metastases. Clinical and pathological materials were analyzed using SPSS-19 to determine the prognostic significance of a number of factors in relation to the survival: gender, age, type and localization of metastases, postoperative stage of the primary tumor, type and volume of liver resection, extrahepatic metastases, preoperative values of CEA, postoperative values (AST, ALT). Results. Factors that correlated with lower survival type: metastases (synchronous or metachronus), localization of metastases (uni-or bilobar), presence of the regional lymph node metastases and metastases to other distant organs and the impossibility of radical resection of liver were statistically significant with multivariant analysis. Elevated preoperative value of CEA, the value of hemoglobin and stage IV disease also affected the survival of patients. Conclusion. In patients with colorectal liver metastases only resection has potentially curative character. The surgical strategy for resection in context of increasing the percentage of patients with resectable potential is the only possible factor for long-term survival.

Background A variety of effective methods for treatment of hemorrhoids has been proposed. In recent years, there has been an increasing number of studies comparing transanal hemorrhoidal dearterilization (THD) and conventional hemorrhoidectomy (CH), but the focus of most studies has been about the early postoperative results. The data about long-term outcomes is still limited. We aimed to compare Doppler-guided THD and CH with regard to early and long-term postoperative results. Methods The conducted prospective research included 287 patients who underwent CH (167 cases) or Doppler-guided THD with mycopexy (120 patients) between November 2010 and December 2015. Information on hemorrhoidal stage, demographic data, presenting symptoms, complications, duration of hospital stay, postoperative pain, patients’ satisfaction and follow-up were obtained. Statistical tests were performed by SPSS 19.0. Results There was no significant difference between the studied groups according to gender, mean age, preoperative prolapse, pain and pruritus, hemorrhoidal stage and postoperative complications. Preoperative bleeding was more frequent in THD group (p = 0,002). The mean visual analog scale (VAS) pain scores in CH and THD groups on days 1, 2 and 7 were 7.01 vs 5.03, 5.07 vs 2.98, 2.39 vs 0,57 (p = 0,000). Practically, there was no difference in VAS on day 30 and patients’ satisfaction at the 18th month. Mean hospital stay was 5,13 (CH) and 3,38 days (THD), p = 0,000. The postoperative follow-up was between 18 and 78 months (mean 46 ± 16 months). During this stage, 5 patients (2,99%) in CH group required surgery for recurrence. In THD group, 3 patients (2,5%), all with 4th-degree hemorrhoids underwent additional procedures (p 0,802). Conclusions Doppler-guided THD seems to be an efficient and safe option for treatment of hemorrhoids, related to lower postoperative pain and excellent, similar long-term outcomes compared to CH. For advanced grades of hemorrhoids, Doppler-guided THD could be a valuable alternative, but there is a need for patients’ selection. Trial registration (retrospectively registered) researchregistry3090.

Introduction. Massive blood loss while performing resections of the liver continues to be a serious problem with potentially lethal outcome. Therefore in the last 2-3 decades there has been a significant developement of techniques for vascular control during liver resections. Methods. In the period from 01.01.2006 to 31.12.2015 in KOCPH UMBAL "Aleksnadrovska" a total of 239 patients with colorectal liver metastases underwent surgery of whom: 179 patients were radicaly operated on and 57 patients were subjected to Pringle maneuver. Using the statistical software SPSS-19 we analyzed various factors that may affect the early postoperative results. Results. In resections of colorectal liver metastases there was a significant difference in the postoperative functional parameters (AST, ALT), which correlated with the degree of liver damage, in patients with Pringle and without Pringle maneuver 265.32 vs. 448 (p = 0.001), and 300.53 vs. 481.91 (p = 0.002),- respectively. There was no significant difference in the postoperative results in comparisson of resections < 15 minutes, performed without Pringle and with Pringle maneuver. The blood loss is another factor that affects the postoperative complications (p = 0.048), and it was lowest in the Pringle group

Highlights • Familial multiple lipomatosis (FML) is a rare hereditary benign disease. • The origin of extra-gastrointestinal stromal tumors (EGISTs) remains controversial. • EGISTs and cells of Cajal have similar features—the expression of CD117 and CD34. • Surgical resection is the standard treatment of EGISTs. • Targeted medical therapy of EGISTs by tyrosine kinase inhibitors can be useful., Introduction Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Rarely, GISTs can be located in mesentery, retroperitoneal space, omentum or pancreas. In these cases, the neoplasm is defined as “extra-gastrointestinal stromal tumors” (EGISTs). Presentation of case We reported a case of a 63-year-old male patient diagnosed by computer tomography with large intraabdominal tumor with vague origin, postoperatively determined as an EGIST. The diagnosis was confirmed by immunohistochemical study. The patient had multiple, subcutaneous, painless lipomas localized in the arms, forearms, thighs, abdomen and thorax. Because of the family history and the clinical presentation the disease was determined as familial multiple lipomatosis (FML). We performed radical tumor resection with distal pancreatectomy and splenectomy, and abdominoplasty, removing redundant skin and underlying subcutaneous fat tissue with multiple lipomas. Discussion FML is a rare hereditary benign disease. On the other hand, only few cases with familial GIST have been reported. In cases with extensive abdominal involvement, the primary origin of EGIST may be impossible to determine so the differential diagnosis is very difficult. Conclusion Although we could not prove correlation between the observed diseases, they are extremely rare and their combination is unusual which makes the presented case valuable and interesting.

Highlights • Rosai–Dorfman diseasе (RDD) is a rare histiocytic disorder оf unclear etiology. • RDD is characterized by emperipolesis and presence of S-100 positive histiocytes. • RDD usually presents as a massive, bilateral enlargement of the neck lymph nodes. • Localization of RDD in the liver and gallbladder is extremely rare. • Treatment is recommended in cases with symptomatic localized or extended RDD., Introduction Rosai–Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic disorder of unclear etiology. Lymphadenopathy commonly affects the lymph nodes of the neck and the head, but extranodal involvement has been observed too. Presentation of case We describe an unusual case of RDD with involvement of the gallbladder, liver and cystic lymph node, clinically manifested as an exacerbated cholecystitis. The patient underwent a cholecystectomy with atypical liver resection because the finding was macroscopically suspected of gallbladder cancer. The diagnosis of RDD was confirmed by immunohistochemical study. Discussion Histological features of RDD include histiocytic proliferation, emperipolesis and positive immunostaining for S-100 protein and typically negative for CD1a. Gastrointestinal localization of RDD, especially in the liver and pancreas, is extremely rare. Typically, the liver is affected as a part of systematic spread of RDD. We have not found reports for involvement of the gallbladder. Conclusion The presented case is valuable due to its rarity and difficulty in differential diagnosis.

Background IgG4-related disease (IgG4-RD) is a newly recognized disorder, characterized by massive IgG4+ lymphocyte and plasma cell infiltration, storiform fibrosis, causing enlargement, nodules or thickening of the various organs, simultaneously or metachronously. Involvement of the gastrointestinal tract is very rare and can be presented as a diffuse wall thickening or polyp or mass-like lesion. Up to now, there have been reported only a few cases of isolated gastric IgG4-RD. Case presentation We present an unusual case of IgG4-RD of the stomach with involvement of the regional lymph nodes, clinically manifested as a gastric cancer with related pyloric stenosis. The patient underwent distal gastrectomy, omentectomy and lymph node dissection. The postoperative serum IgG4 level was increased. The diagnosis was confirmed by immunohistochemical study. Conclusions In the most of the reported cases there was not sufficient data about the regional lymph nodal status, although the majority of the patients had been operated with presumptive diagnosis of gastric neoplasm. Our case is rare and valuable because it presents a gastric IgG4-related lesion larger than all previously reported in literature, and IgG4-related lymphadenopathy, confirmed histologically, which contributes to better knowledge of the disease.

Introduction: Metastatic lesions account for approximately 8% of all ovarian neoplasms. About 12% of clinically apparent metastases from non-gynecological primary site originate from breast carcinomas with a variable period of manifestation and detection. Report of Case: We present a case of 58-year-old woman who underwent bilateral adnexectomy with small bowel resection during urgent operation for an incarcerated umbilical hernia. There was a slight enlargement in both ovaries and presence of cystic formation with some papillary structures in the right one. Histologically, bilateral infiltration of tumor cells with moderate cytological and nuclear atypia and tubular, nested and cystic growth pattern was observed. The patient had a long list of co-morbidities: liver cirrhosis, ascites, isolated cytology with presence of tumor cells, elevated level of CA125 and quadrantectomy of the right breast, performed 15 years ago. Diagnostic examination continued in differing primary ovarian carcinoma from metastatic process using immunohistochemical markers – Gross cystic disease fluid protein-15 (GCDFP-15), Mammaglobin, Wilms tumor protein (WT1), p53, Ki67 and hormone receptors for estrogen (ER), progesterone (PR) and human epidermal growth factor (HER2). Considering the clinical data and the results from the pathological examination we concluded that the patient had bilateral ovarian metastases from breast carcinoma. We share our stepwise approach to the diagnosis discussing some of the difficulties emerged in the process and some of the pitfalls reaching the final conclusion.

Diverticulosis of lower gastrointestinal tract is a common disease with potentially lethal complications. Recent studies demonstrated that the number of attacks of uncomplicated diverticulitis is not necessarily an overriding factor in defining the appropriateness of surgery so the approach has to be more individualized. On the other hand, the complicated diverticular disease requires surgical treatment, often matter of urgency, and depends on localization and severity of the process. There is not sufficient evidence supporting the aggressive approach to treatment in younger patients. The available evidence suggests that surgery should be indicated after one attack of uncomplicated disease in immunocompromised individuals.