Benign Tumours and Pseudotumours Within the Porta Hepatis Masquerading as Perihilar Cholangiocarcinoma.

BACKGROUND: Hilar cholangiocarcinoma (HC), also referred to as Altemeier-Klatskins tumour, is a lethal primary extrahepatic carcinoma of biliary epithelial origin, arising within 2cm of the hilar confluence. Radical surgical excision provides the best chance for a cure; however, the management of patients with HC is challenging not only because of the need for a high level of skill in biliary and hepatic resections, but also because of the difficulty in reaching an accurate diagnosis preoperatively. In fact, the differential diagnosis of HC is a diagnostic dilemma which is currently persisting, as modern, sophisticated diagnostic modalities are not always able to provide a definitive preoperative diagnosis. This difficulty is compounded by the fact that alternative entities that mimic HC may be present in up to 25% of patients with hilar obstruction. This makes precise preoperative characterisation of a hilar stricture extremely important by preventing unnecessary, high-risk, major surgical procedures. Therefore, alternative benign entities masquerading as Altemeier-Klatskins tumour deserve an important place in the differential diagnosis of hilar obstruction. PURPOSE: Considering the important clinical implications that a precise diagnosis of the aetiological cause of a biliary obstruction at the liver hilum would have, this paper will focus on the differentiation between HC and benign hilar obstructions and will review benign tumours and pseudotumours masquerading as HC along with their specific diagnostic features.

Adult congenital heart disease in Greece: Preliminary data from the CHALLENGE registry.

BACKGROUND: The majority of patients with congenital heart disease (CHD), nowadays, survives into adulthood and is faced with long-term complications. We aimed to study the basic demographic and clinical characteristics of adult patients with congenital heart disease (ACHD) in Greece. METHODS: A registry named CHALLENGE (Adult Congenital Heart Disease Registry. A registry from Hellenic Cardiology Society) was initiated in January 2012. Patients with structural CHD older than 16years old were enrolled by 16 specialized centers nationwide. RESULTS: Out of a population of 2115 patients with ACHD, who have been registered, (mean age 38years (SD 16), 52% women), 47% were classified as suffering from mild, 37% from moderate and 15% from severe ACHD. Atrial septal defect (ASD) was the most prevalent diagnosis (33%). The vast majority of ACHD patients (92%) was asymptomatic or mildly symptomatic (NYHA class I/II). The most symptomatic patients were suffering from an ASD, most often the elderly or those under targeted therapy for pulmonary arterial hypertension. Elderly patients (>60years old) accounted for 12% of the ACHD population. Half of patients had undergone at least one open-heart surgery, while 39% were under cardiac medications (15% under antiarrhythmic drugs, 16% under anticoagulants, 16% under medications for heart failure and 4% under targeted therapy for pulmonary arterial hypertension). CONCLUSIONS: ACHD patients are an emerging patient population and national prospective registries such as CHALLENGE are of unique importance in order to identify the ongoing needs of these patients and match them with the appropriate resource allocation.

Delayed duodenal stump blow-out following total gastrectomy for cancer: Heightened awareness for the continued presence of the surgical past in the present is the key to a successful duodenal stump disruption management. A case report.

INTRODUCTION: Duodenal stump disruption remains one of the most dreadful postgastrectomy complications, posing an overwhelming therapeutic challenge. PRESENTATION OF CASE: The present report describes the extremely rare occurrence of a delayed duodenal stump disruption following total gastrectomy with Roux-en-Y esophagojejunostomy for cancer, because of mechanical obstruction of the distal jejunum resulting in increased backpressure on afferent limp and duodenal stump. Surgical management included repair of distal jejunum obstruction, mobilization and re-stapling of the duodenum at the level of its intact second part and retrograde decompressing tube duodenostomy through the proximal jejunum. DISCUSSION: Several strategies have been proposed for the successful management post-gastrectomy duodenal stump disruption however; its treatment planning is absolutely determined by the presence or not of generalized peritonitis and hemodynamic instability with hostile abdomen. In such scenario, urgent reoperation is mandatory and the damage control principle should govern the operative treatment. CONCLUSION: Considering that scientific data about duodenal stump disruption have virtually disappeared from the current medical literature, this report by contradicting the anachronism of this complication aims to serve as a useful reminder for gastrointestinal surgeons to be familiar with the surgical techniques that provide the ability to properly manage this dreadful postoperative complication.

Mid common bile duct inflammatory pseudotumor mimicking cholangiocarcinoma. A case report and literature review.

INTRODUCTION: Biliary inflammatory pseudotumors (IPTs) represent an exceptional benign cause of obstructive jaundice. These lesions are often mistaken for cholangiocarcinomas and are treated with major resections, because their final diagnosis can be achieved only after formal pathological examination of the resected specimen. Consequently, biliary IPTs are usually managed with unnecessary major resections. PRESENTATION OF CASE: A 71-year-old female patient underwent an extra-hepatic bile duct resection en-bloc with the gallbladder and regional lymph nodes for an obstructing intraluminal growing tumor of the mid common bile duct (CBD). Limited resection was decided intraoperatively because of negative for malignancy fast frozen sections analysis in addition to the benign macroscopic features of the lesion. Histologically the tumor proved an IPT, arising from the bile duct epithelium, composed of inflammatory cells and reactive mesenchymal tissues. DISCUSSION: The present case underlines the value of intraoperative reassessment of patients undergoing surgical resection for histopathologically undiagnosed biliary occupying lesions, in order to optimize their surgical management. CONCLUSION: The probability of benign lesions mimicking cholangiocarcinoma should always be considered to avoid unnecessary major surgical resections, especially in fragile and/or elderly patients.

The role of open necrosectomy in the current management of acute necrotizing pancreatitis: a review article.

The optimal management of necrotizing pancreatitis continues to evolve. Currently, conservative intensive care treatment represents the primary therapy of acute severe necrotizing pancreatitis, aiming at prevention of organ failure. Following this mode of treatment most patients with sterile necroses can be managed successfully. Surgery might be considered as an option in the late phase of the disease for patients with proven infected pancreatic necroses and organ failure. For these patients surgical debridement is still considered the treatment of choice. However, even for this subgroup of patients, the concept of operative strategy has been recently challenged. Nowadays, it is generally accepted that necrotizing pancreatitis with proven infected necroses as well as septic complications directly caused by pancreatic infection are strong indications for surgical management. However, the question of the most appropriate surgical technique for the treatment of pancreatic necroses remains unsettled. At the same time, recent advances in radiological imaging, new developments in interventional radiology, and other minimal access interventions have revolutionised the management of necrotizing pancreatitis. In light of these controversies, the present paper will focus on the current role of surgery in terms of open necrosectomy in the management of severe acute necrotizing pancreatitis.

Double Duct to Mucosa Pancreaticojejunostomy for Bifid Pancreatic Duct following Pylorus Preserving Pancreaticoduodenectomy: A Case Report.

Bifid pancreatic duct represents a relatively rare anatomical variation of the pancreatic ductal system, in which the main pancreatic duct is bifurcated along its length. This paper describes the challenging surgical management of a 68-year-old male patient, with presumptive diagnosis of periampullary malignancy who underwent a successful double duct to mucosa pancreaticojejunostomy for bifid pancreatic duct. Following pylorus preserving pancreaticoduodenectomy, careful intraoperative inspection of the cut surface of the residual dorsal pancreas identified the main in addition to the secondary pancreatic duct orifice. Bifid duct anatomy was confirmed via intraoperative probing and direct visualization of the ductal orifices. A decision was made for the performance of an end-to-site double duct to mucosa pancreaticojejunostomy. Postoperative outcome was favorable without any complications. Although bifid pancreatic duct is relatively rare, pancreatic surgeons should be aware of this anatomical variation and be familiar with the surgical techniques for its successful management. Lack of knowledge and surgical expertise for dealing with this anatomical variant may lead to serious, life threatening postoperative complications following pancreatic resections.

Retroperitoneal extra-adrenal ganglioneuroma involving the infrahepatic inferior vena cava, celiac axis and superior mesenteric artery: A case report.

INTRODUCTION: Ganglioneuromas are rare benign neoplasms arising from the sympathetic neuroendocrine system. These tumors usually occur in the abdomen and tend to grow around major blood vessels making often their complete excision challenging and demanding. PRESENTATION OF CASE: The authors present the challenging surgical management of a sizable retroperitoneal extra-adrenal ganglioneuroma involving the infrahepatic inferior vena cava, portal triad, celiac axis and superior mesenteric artery in a 23-year-old female patient. The tumor was safely and completely excised in toto with preservation of all neighboring vital anatomical structures using a midi laparotomy access. DISCUSSION: Ganglioneuromas should be included in the differential diagnosis of any retroperitoneal mass. Their management involves total surgical excision however, in some instances; it can be challenging and demanding because of their tendency to engage neighboring vital anatomical structures. CONCLUSION: A surgical strategy including meticulous operative dissection guided by the quality principles of surgical oncology although challenging and demanding can result to a safe and complete tumor excision, which is directly correlated with an improved patients' postoperative outcome and excellent prognosis.

Alanine-glutamine dipeptide pretreatment protects rat renal function from small intestine ischemia-reperfusion injury.

AIM: Oxidative injury can cause renal function impairment and failure. Glutathione, a free radical scavenger, plays in the kidney a central role in oxidant-related events. The aim of this study was to investigate the potential beneficial effect of glutamine, a precursor of glutathione in the form of alanine-glutamine dipeptide (AGD) on small intestine ischemia/ reperfusion (I/R)-induced oxidant renal damage in rats. METHODS: Wistar rats were subjected to intestinal I/R for 30 min, induced by occlusion of the superior mesenteric artery, followed by 60 min reperfusion. AGD pretreatment was given 48 and 24 hours before I/R. At the end of the experimental procedure the left kidney was excised and a thin tissue slice was obtained for electron microscopy study. Kidney biopsies were obtained for malonyl dialdehyde, myeloperoxidase, and glutathione assays. RESULTS: Intestinal I/R caused significant oxidative injury in rat renal parenchyma consisted of severe alterations observed in subcellular renal structures associated with a significant increase in renal malonyl dialdehyde levels and a significant decrease in renal glutathione levels. Changes regarding subcellular renal structures were ameliorated in AGD pre-treated animals in which renal glutathione levels did not decreased significantly. CONCLUSION: Glutamine pretreatment in the form of AGD can prevent small bowel I/R-induced oxidant renal damage in rats.

Splenic artery aneurysm in a cardiac transplant patient: a case report.

We present the case of a 61-year-old woman who underwent repair of a symptomatic 4-cm splenic artery aneurysm. This patient had received heart transplantation for hypertrophic congestive cardiomyopathy 5 years before this event. She was under immunosuppression therapy with cyclosporine, prednisone and azathioprine. The aneurysm was ligated both proximally and distally without removal of the spleen. To our knowledge, this is the first reported case of an isolated splenic artery aneurysm in the heart transplant population. We discuss the potential role of immunosuppressive agents and hormonal factors in the development of this extremely rare occurrence.

Systematic review: endoscopic and imaging-based techniques in the assessment of portal haemodynamics and the risk of variceal bleeding.

BACKGROUND: Invasive measurement of the hepatic venous pressure gradient (HVPG) is regarded as the gold standard for risk stratification and the evaluation of pharmaceutical agents in patients with portal hypertension. AIM: To review the techniques for endoscopic and imaging-based assessment of portal haemodynamics, with particular emphasis on trials where the results were compared with HVPG or direct portal pressure measurement. METHODS: Systematic search of the MEDLINE electronic database with keywords: portal hypertension, variceal bleeding, variceal pressure, endoscopic ultrasound, Doppler ultrasonography, magnetic resonance angiography, CT angiography, hepatic venous pressure gradient. RESULTS: Computed tomography angiography and endoscopic ultrasound (EUS) have been both employed for the diagnosis of complications of portal hypertension and for the evaluation of the efficacy of endoscopic therapy. Colour Doppler ultrasonography and magnetic resonance angiography has given discrepant results. Endoscopic variceal pressure measurements either alone or combined with simultaneous EUS, correlate well with HVPG and risk of variceal bleeding and have a low interobserver variability. CONCLUSIONS: Endoscopic and imaging-based measurements of portal haemodynamics provide an alternate means for the assessment of complications of portal hypertension. Further studies are required to validate their use in risk stratification and the evaluation of drug therapies in patients with portal hypertension.

Early endograft collapse following endovascular treatment of a traumatic thoracic aortic disruption: a case report.

The authors present the case of a 39-year-old man who underwent endovascular repair of a thoracic aortic disruption. Implantation of a Gore TAG stent-graft achieved total exclusion of the traumatic lesion with no contrast extravasation. However, on the third postoperative day, the patient developed complete anuria necessitating continuous venovenous hemofiltration. On the IV postoperative day there were no palpable femoral pulses and the pressure gradient between the lower and upper limbs was -80 mmHg. Given the development of severe intestinal and peripheral hypoperfusion status a possible endograft collapse was suspected. Urgent computed tomography (CT) angiography demonstrated central subtotal collapse of the device and proper distal sealing. A second TAG stent-graft was deployed successfully within the collapsed device with no contrast extravasation and good apposition of the stent-graft to the aortic wall. At 6 months, there is no sign of graft collapse or endoleak. Endovascular reintervention succeeded re-expansion of the collapsed endoprosthesis and resolution of the initial symptoms.

Appendectomy for an adult with cyanotic congenital heart disease.

Few patients with cyanotic congenital heart disease reach adulthood without a cardiac operation. The prognosis for ''unrepaired'' pulmonary atresia with ventricular septal defect is approximately 8% in the 1st decade of age. Consequently, the number of adults with this particular heart disease (unrepaired) who are expected to need a non-cardiac surgery is extremely low. General anesthesia may aggravate the preexisting right to left shunt and lead to persistent severe hypoxemia. The goal of anesthetic management should be to maintain intravascular volume. Systemic and pulmonary vascular resistance changes, such as might occur due to acidosis, hypothermia, hypercarbia or excessive airway pressures, should be avoided. Maintenance of preload, contractility and sinus rhythm is of major importance. The complex pathophysiology of such heart disease, in addition to the circumstances of emergency operation, exacerbate the total anesthetic risk. We present here a rare case of an acute appendectomy with successful outcome in an adult with pulmonary atresia and ventricular septal defect.

Profunda femoris as an access site vessel for a hybrid approach to the treatment of a para-anastomotic common iliac artery aneurysm and intermittent claudication, occurring after previous abdominal aortic thbe graft repair and femoro-femoral bypass graft. A case report.

A true para-anastomotic right common iliac artery aneurysm and intermittent claudication developed in a 76-year-old man 5 years after open abdominal aortic aneurysm repair with a Dacron tube graft. Following the initial operation the patient developed acute left iliac occlusive disease necessitating an immediate right-to-left femoro-femoral crossover bypass graft. The patient was a poor open surgical candidate because of multiple medical comorbidities. Therefore, a hybrid approach was used consisting of exposure and catheterization of the right profunda femoris artery, which was used as the access site vessel for the deployment of a covered stent graft extending from the ostium of the common iliac artery into the external iliac artery. Simultaneously, the right profunda femoris provided inflow for an open above-knee profunda femoro-popliteal bypass graft to perfuse the right lower extremity. Postoperative angiography demonstrated primary technical success, with exclusion of the aneurysm and no endoleak. The patient is doing well 34 months postoperatively, with a patent endograft and no sign of intermittent claudication. Profunda femoris proved to be an excellent alternative to the common femoral artery for the application of a hybrid technique in a high-risk patient with complicated anatomy.

Submucosal lipoma of the ascending colon as a source of massive lower gastro-intestinal bleeding: a case report.

Colonic lipomas are relatively uncommon lesions. They have been documented as the source of massive low gastro-intestinal bleeding in only five previous reports in the English language literature. We report an extremely rare case of massive haemorrhage caused by an ascending colon submucosal lipoma and review the pathophysiology, diagnosis and management.

Influence of the stable prostacyclin analog iloprost on the healing of colonic anastomosis in rats.

AIM: The aim of this study was to investigate whether iloprost injected intraperitoneally immediately after colon resection can improve anastomotic healing on the fifth and eighth postoperative days. METHODS: Forty Wistar rats were randomised into 2 equal groups. After the resection of a 1 cm segment of transverse colon, an end to end sutured anastomosis was generated. From the day of the operation, group 1 (control) received intraperitoneal 3 cc saline solution once daily until sacrifice, while group 2 (iloprost) received iloprost in a dose of 2 mg/kg body weight intraperitoneally once daily until sacrifice. Each group was further randomly divided into 2 equal subgroups and animals were sacrificed on the fifth (subgroup A), and eighth (subgroup B) postoperative days. After sacrifice, anastomoses were examined macroscopically and were measured for bursting pressures and tissue hydroxyproline levels while anastomotic healing process was evaluated histopathologically. RESULTS: None of the rats exhibited any clinical evidence of leakage and there were no instances of peri-anastomotic abscess or peritonitis. Bursting pressure on the fifth postoperative day was significantly higher in the iloprost group than in the control group (P<0.001), while on the eighth postoperative day, bursting pressure was higher in the iloprost group but not significantly different (P=0.165). On both the fifth and eighth postoperative days rats in the iloprost group developed significantly more marked neo-angiogenesis and, in parallel with this, there was a trend showing a higher inflammatory cell infiltration. CONCLUSION: The intraperitoneal administration of iloprost promoted neo-angiogenesis and enhanced colonic healing on the fifth postoperative day.

Effect of iloprost on impaired anastomotic healing caused by 5-fluorouracil plus leucovorin.

PURPOSE: This experimental study was designed to investigate whether iloprost can reverse impaired colonic healing caused by immediate postoperative intraperitoneal administration of 5-fluorouracil plus leucovorin. METHODS: Eighty Wistar rats were randomized into four groups. After resection of a 1-cm segment of transverse colon, an end-to-end sutured anastomosis was generated. Rats received saline solution (Group 1), 5-fluorouracil plus leucovorin (Group 2), iloprost (Group 3), and 5-fluorouracil plus leucovorin plus iloprost (Group 4) intraperitoneally from the day of operation and once daily until killing. Each group was further randomized into two subgroups. Subjects were killed on the fifth (Subgroup a) and eighth (Subgroup b) postoperative days. After killing, anastomoses were examined macroscopically and graded histologically. Rats were measured for anastomotic bursting pressures and tissue hydroxyproline levels. RESULTS: The leakage rate of the anastomoses was significantly higher in the 5-fluorouracil plus leucovorin group compared with the other groups (P = 0.049). Bursting pressure was significantly lower in 2a subgroup (5-fluorouracil plus leucovorin, postoperative Day 5) than in 4a (5-fluorouracil plus leucovorin plus iloprost, postoperative Day 5; P < 0.001). Adhesion formation was significantly higher in all b subgroups compared with the Control b subgroup. Neoangiogenesis was significantly higher in iloprost and iloprost plus 5-fluorouracil plus leucovorin subgroups compared with the 5-fluorouracil plus leucovorin subgroups. Hydroxyproline levels, collagen deposition, fibroblasts, and white cell count were significantly higher in the iloprost plus 5-fluorouracil plus leucovorin b subgroup (postoperative Day 8) compared with the 5-fluorouracil plus leucovorin b subgroup (postoperative Day 8). CONCLUSIONS: The immediate postoperative, intraperitoneal administration of iloprost counteracts and reverses the negative effects of 5-fluorouracil plus leucovorin chemotherapy and protects colonic healing in rats.

Recurrent arterial thromboses in a woman with heparin induced thrombocytopenia, successfully managed with iloprost followed by clopidogrel. An alternative therapeutic option for heparin induced thrombocytopenia type II syndrome.

In its more severe form heparin induced thrombocytopenia (HIT) is a rare immune mediated complication of heparin administration that potentially has catastrophic results, and significant mortality. In view of the severity of this condition it is important for the clinician to maintain a high index of suspicion and get alerted to the HIT syndrome by the precocity of platelet count decrease in any patient group, and especially in those previously exposed to heparin. We report on a 72-year-old woman who developed HIT syndrome that was complicated by recurrent arterial thromboses after receiving postoperative antithrombotic prophylaxis with tinzaparin, a low molecular weight heparin. The patient was successfully treated with iloprost (Ilomedin, iloprost tromethamine, Schering) a stable prostacyclin analogue, at the acute phase of the syndrome, followed by long-term treatment with clopidogrel (Plavix, clopidogrel bisulfate, Sanofi) an inhibitor of adenosine diphosphate (ADP) receptor. Although direct thrombin inhibitors have been proven to be effective for the treatment of HIT thrombosis, they do not completely eliminate the morbidity and mortality of this disorder. Our case report suggests that antithrombotic treatment by targeting of the activated platelets with a potent platelet inhibitor during the acute phase of type II HIT syndrome followed by long-term administration of oral anticoagulation may be an additional, safe and effective therapeutic alternative that merits to be systematically studied.

Sebaceous gland tumors and internal malignancy in the context of Muir-Torre syndrome. A case report and review of the literature.

BACKGROUND: The Muir-Torre syndrome is a rare autosomal dominant condition and is currently considered a subtype of the more common hereditary nonpolyposis colorectal cancer syndrome, in which multiple primary malignancies occur together with sebaceous gland tumors. CASE PRESENTATION: We describe a case of a 62-year-old woman with three primary colorectal tumors, genital tumor, and sebaceous adenomas and present her family history of three generations. Our case represents the first case reported from Greece in the international literature. CONCLUSION: Recognition of the syndrome in patients with sebaceous gland tumors should facilitate early detection of subsequent malignancies if the patient is entered into appropriate screening programs.

Thoracic splenosis twenty-nine years after traumatic splenectomy mimicking intrathoracic neoplasm.

Thoracic splenosis refers to a condition of ectopic splenic tissue in the thoracic cavity. It is usually a consequence of splenic tissue seeding in the pleural cavity after thoracoabdominal trauma. A rare case of thoracic splenosis, in a 62-year-old man who had had a traumatic splenectomy due to thoracoabdominal trauma 29 years earlier, is reported. The patient, a heavy smoker, was admitted for evaluation of a left-side thoracic lesion discovered on a plain chest film. Bronchoscopy, CT scan and needle biopsy proved inconclusive for the diagnosis. Exploratory thoracotomy was necessary to establish the diagnosis. During the operation, a thoracic splenosis was confirmed. To date, only 28 cases of thoracic splenosis have been reported in the literature. The purpose of this report is to present a new case of splenosis of the thoracic cavity simulating intrathoracic neoplasm.

Endoscopic management of retained bile stones with an indwelling T-tube.

BACKGROUND: Endoscopic sphincterotomy (ES) is widely used for the treatment of residual bile duct stones in patients who had common bile duct (CBD) exploration and T-tube insertion. METHODS: In a 4-year period 45 patients were referred for endoscopic removal of residual bile duct stones. All patients had been operated 7-15 days earlier for choledocholithiasis and had a T-tube in the common bile duct (CBD). RESULTS: Four patients were excluded. Three patients had a periampullary carcinoma and the fourth patient had no residual stone seen at cholangiography. All patients had a successful ES, conventional in 34, precut-knife in 3, and with the rendezvous technique in 4 patients. In 24 patients, all having stones distal to the T-tube, complete clearance of the CBD was achieved during one session and the T-tube was removed after 48 h. In the remaining 17 patients (15 having stones proximal to the T-tube), the T-tube had to be removed first and following stone extraction, a plastic stent was inserted in the CBD. Complete bile duct clearance and stent removal was achieved in a second session 3-4 weeks later. There were no serious complications or biliary related symptoms after the procedures and after a mean follow-up period of 18 months. CONCLUSION: The endoscopic technique is safe and efficient for the treatment of residual stones after CBD exploration with a T-tube insertion, offering immediate cure compared to the percutaneous techniques. It is also an ideal method for the diagnosis of periampullary carcinomas.