Current management of upper gastrointestinal bleeding.

Over a four-year period, 585 patients were hospitalized for massive upper gastrointestinal bleeding. Endoscopy diagnosed the cause of bleeding in 80% of 200 patients so studied. Selective angiography localized the bleeding site in 12 of 20 patients, and infusion of vasopressor stopped hemorrhage in six. Barium studies was 90% accurate in diagnosing ulcer disease but failed to detect gastritis. One hundred thirty (22%) patients were operated upon for medically uncontrolled bleeding. The proportion of patients requiring surgery fell from 33% in year one to 13% in year four. Benign ulcer disease caused bleeding in 51% of surgical patients, while gastritis was found in 20%, esophageal varices in 15% and stress ulcer in 8%. Overall operative mortality was 29%. Among 38 duodenal ulcer patients, mortality was 18%. Vagotomy and pyloroplasty were more effective than resection in this group. Resection for distal gastric ulcers in 22 patients resulted in a mortality of 14% and no rebleeding. While V&P controlled bleeding in 12 alcoholics with gastritis, five (42%) died postoperatively. Mortality among 20 patients with esophageal varices was 35%, although all five survived who had porto-caval shunts. Eight of 10 patients operated upon for stress ulcer bleeding died. Postoperative rebleeding occurred in 14 patients, eight of whom were again operated upon. In all but one a new lesion was found to be responsible for hemorrhage. Increasing use of gastroscopy and selective angiography can be expected to improve diagnostic capabilities in patients with upper gastrointestinal bleeding. Infusing vasopressor into selected arteries should reduce the need for surgical control of gastritis, variceal and stress ulcer bleeding, conditions poorly managed by current operative techniques.     

Severe thrombotic and bleeding complications in a baby with heterozygous factor V Leiden and acquired von Willebrand disease on ECMO

We aim to present the case of a 5-week-old girl with severe respiratory failure placed on veno-venous extracorporeal membrane oxygenation (ECMO) that was then switched to veno-arterial ECMO. She required up to 60 units/kg/hr of heparin to keep her heparin level within the target range at .3-.7 units/mL. During the ECMO course, substantial thrombus formation was observed within the venous site of the ECMO cannula, which led to two circuit changes on ECMO day 9 and day 20. On ECMO day 15, she was noticed to have purpuric lesions on her chest and her right hand with no obvious arterial or venous clot detected by Doppler ultrasound. She was also noted to have remarkable hemolysis as the plasma free hemoglobin levels were substantially elevated up to 700 mg/dL. She was noted to have continuous oozing from the catheter insertion sites despite adequate underlying coagulation status. Her subsequent platelet function analysis, the thromboelastography, and thromboelastography platelet mapping suggested substantial platelet dysfunction. Her von Willebrand panel revealed absence of high molecular weight multimers. Further coagulation workup was prompted which revealed heterozygosity for factor V Leiden. The patient developed severe pulmonary hemorrhages and ECMO was discontinued on day 40.     

Review article: unexpected bleeding in the operating room: the role of acquired von Willebrand disease

Acquired von Willebrand disease (AvWD) is a rare bleeding disorder that occurs in association with a variety of underlying disorders and can lead to unforeseen bleeding in surgical patients. Cardiovascular as well as malignant and immunological diseases may be associated with AvWD, and several pathophysiological mechanisms have been proposed. von Willebrand factor (vWF) is a plasma glycoprotein that mediates platelet adhesion to subendothelial collagen and causes platelet aggregation under high shear stress. Additionally, vWF acts as a specific carrier for coagulation factor VIII (FVIII) in the plasma. AvWD results from a reduced rate of vWF synthesis, an increased rate of vWF removal, or a final generation of lower-molecular-weight, less active subunits or multimers. In contrast to inherited von Willebrand disease patients, who are characterized by lifelong bleeding episodes, AvWD patients present with a sudden onset of bleeding symptoms, which can induce acute bleeding episodes during critical surgical procedures. Typically, no family history of bleeding is found. The clinical visualization of AvWD is similar to that of the hereditary form with mucocutaneous bleeding and increased perioperative bleeding, ranging from mild to severe bleeding. Laboratory evaluation of AvWD is mainly based on the measurement of vWF activity and antigens as well as on the multimeric analysis of vWF. A variety of therapeutic approaches have been used depending on the underlying disease and pathophysiological mechanisms. Treatment options to control acute hemorrhages or to prevent bleeding complications during surgery include desmopressin, FVIII/vWF concentrates, high-dose IV immunoglobulins, and plasma exchange. Because the half-life of vWF is reduced in AvWD, high doses of FVIII/vWF concentrates administered at frequent intervals may be necessary during bleeding episodes. In cases of unresponsiveness to standard therapy, recombinant activated factor VIIa may be an alternative option. However, the most effective therapy is the resolution of the underlying disease. In the present review, we focus on the current understanding of AvWD, outlining the associated disorders, underlying pathophysiological mechanisms, and possible treatment options.     

Non-surgical bleeding in patients with ventricular assist devices could be explained by acquired von Willebrand disease.

OBJECTIVE: Outcomes after ventricular assist device (VAD) implantation have significantly improved during the last decade. However, bleeding episodes remain a serious complication of VAD support. This cannot be explained by the individual anticoagulation regimen alone in several cases, but may be symptomatic of acquired von Willebrand disease (VWD). The leading finding in acquired VWD (AVWD) is the loss of large multimers which results in diminished binding to collagen and to the platelets. We, therefore, analysed patients with two VAD types for laboratory parameters of VWD and compared them with patients after heart transplantation (HTX). MATERIALS AND METHODS: Seven patients with a HeartMate II left-ventricular assist device and five patients who received a Thoratec biventricular assist device were included in this study. Eight HTX recipients served as controls. Analysis included international normalized ratio (INR), partial thromboplastin time (PTT), platelet count, von Willebrand factor (VWF) antigen, collagen binding capacity, ristocetin cofactor activity, the ratios of the latter two to the VWF antigen and presence of large VWF multimers. RESULTS: The VAD and HTX groups did not differ with regard to age or time-point of analysis after surgery. INR and number of platelets were comparable in both groups, PTT was prolonged in VAD patients. Both VAD and HTX patients had elevated but comparable amounts of VWF antigen. However, large multimers were missing in all of 10 tested VAD patients. In contrast, five of six tested HTX recipients displayed normal multimer pattern. Indeed, collagen binding capacity and ristocetin cofactor activity (which measures binding of VWF to platelets) were lower in VAD patients compared to HTX recipients. Impaired coagulation associated with VADs was also reflected by the diminished ratios of collagen binding capacity and ristocetin cofactor activity to VWF antigen. A pathologic collagen binding ratio was found in all 10 tested VAD patients and one of the eight HTX patients, a reduced ristocetin cofactor activity ratio in 10 of 12 VAD and one of eight HTX patients. CONCLUSION: Non-surgical postoperative bleeding after VAD implantation could be explained by an AVWD. Several pharmacologic treatment options (tranexamic acid, desmopressin, VWF-factor VIII concentrate, recombinant factor VIIa) may arise from our data. Improved VAD design could prevent this problem in the future.     

Perioperative management of a patient with an acquired von Willebrand syndrome

We report the case of a man suffering from a monoclonal gammapathy with an acquired von Willebrand disease in a perioperative context. This pathology is rare, but is important to diagnose because of the possible haemorrhagic complications encountered. We describe the main therapeutic options available today to prevent bleeding during major surgery.     

Recent advances in endovascular techniques for management of acute nonvariceal upper gastrointestinal bleeding

Over the past two decades,transcatheter arterial embolization has become the first-line therapy for the management of upper gastrointestinal bleeding that is refractory to endoscopic hemostasis.Advances in catheter-based techniques and newer embolic agents, as well as recognition of the effectiveness of minimally invasive treatment options,have expanded the role of interventional radiology in the management of hemorrhage for a variety of indications,such as peptic ulcerbleeding,malignant disease,hemorrhagic Dieulafoy lesions and iatrogenic or trauma bleeding.Transcatheter interventions include the following:selective embolization of the feeding artery,sandwich coil occlusion of the gastroduodenal artery,blind or empiric embolization of the supposed bleeding vessel based on endoscopic findings and coil pseudoaneurysm or aneurysm embolization by three-dimensional sac packing with preservation of the parent artery.Transcatheter embolization is a fast,safe and effective,minimally invasive alternative to surgery when endoscopic treatment fails to control bleeding from the upper gastrointestinal tract.This article reviews the various transcatheter endovascular techniques and devices that are used in a variety of clinical scenarios for the management of hemorrhagic gastrointestinal emergencies.     

Cause and management of upper gastrointestinal bleeding after distal splenorenal shunt.

BACKGROUND. The aims of this study were to determine the causes of recurrent upper gastrointestinal hemorrhage (UGH) after distal splenorenal shunting (DSRS) and to summarize our experience in the prevention and management of this complication. METHODS. This study is based on a retrospective review of 145 consecutive patients undergoing DSRS from 1978 through 1991. RESULTS. Recurrent UGH developed in 19 patients (13%), most frequently secondary to residual portal hypertension (84%). Eight patients had shunt thrombosis and 11 had patent shunts. The incidence of shunt thrombosis was significantly greater in patients whose splenic vein was less than or equal to 8 mm in diameter (44%) than those whose splenic vein was greater than 8 mm (7%, p less than 0.001). The frequency of shunt failure from 1985 through 1991 was significantly lower (2%) than from 1978 through 1984 (10%, p less than 0.05). Five patients, all with occluded shunts, underwent surgical treatment for recurrent UGH and three died (60%). Fourteen patients were managed nonoperatively, with a mortality rate of 38%. CONCLUSIONS. Recurrent UGH after DSRS occurs in patients with patent shunts and in those with occluded shunts; DSRS thrombosis is more frequent when the splenic vein diameter is less than or equal to 8 mm; DSRS thrombosis decreases with operative experience; and the mortality rate for this complication is high with both operative and nonoperative management.     

Endoscopic Nd:YAG laser therapy in upper gastrointestinal bleeding

Between 1983 and 1986 thirty-seven patients with upper gastrointestinal bleeding or stigmata of acute bleeding were treated at the Second Department of Surgery with endoscopic laser therapy. The non-contact method was used. The cause of bleeding was gastric ulcer in 13 cases and duodenal ulcer in 5. Two patients had anastomotic ulcer, 6 had a simple ulcer, 7 had telangiectases (Mb. Osler), 2 had Mallory Weiss tears and 2 bled after gastric biopsy. Twenty-one patients bled during endoscopy and 16 had signs of recent bleeding. During acute bleeding laser treatment was effective in 95% (1, 3). However, 41% of all patients (15/37) rebled within a week after laser therapy and in 30% (11/37) an emergency operation was necessary. The overall mortality rate was 10.8% (4/37). Endoscopic laser coagulation is successful in the initial treatment of acute upper GI-bleeding. However, there is a considerable risk of rebleeding. Acute laser therapy may change an emergency operation into an elective one, provided that the group at risk of rebleeding can be anticipated at first endoscopy.     

Plasma therapy in von Willebrand factor protease deficiency

We report a patient with relapsing hereditary hemolytic uremic syndrome (HUS) that began in the neonatal period with life-threatening jaundice and hemolytic anemia. He progressed to end-stage renal failure at 14 years of age and had a cerebrovascular accident while on dialysis. The cause of HUS was a constitutional deficiency in the von Willebrand factor cleaving protease. Hematological features of HUS significantly improved following bilateral nephrectomy. After renal transplantation, he had an early recurrence of HUS associated with two episodes of retinal and cerebral ischemia. Long-term treatment with fresh-frozen plasma exchanges prevented recurrence of HUS, cerebrovascular attacks, and early loss of the graft.     

Endoscopic injection sclerotherapy in non-variceal upper gastrointestinal bleeding

Summary To date several agents have been used to achieve haemostasis in patients with non-variceal upper gastrointestinal bleeding using endoscopic sclerotherapy techniques. Polidocanol has been widely used but local complications have been reported after treatment. We have compared the efficacy and safety of thrombin and polidocanol in 82 consecutive patients with ongoing or recent bleeding from duodenal, gastric, or anastomotic ulcers. Primary control of haemostasis from spurting vessels was achieved in 90% of cases using polidocanol and in 86.6% using thrombin. Definitive haemostasis was obtained in 80% of patients in both groups. When a non-bleeding vessel was visible, injection of polidocanol or thrombin effectively prevented rebleeding in 90.9% and 85.7% of cases, respectively. When a non-bleeding sentinel clot was present, injection of polidocanol or thrombin provided definitive haemostasis in 100% and 92.8% of cases, respectively. No statistically significant difference was evident between the two agents. In the polidocanol group, one local haemorrhagic complication was noted. No general or local complications were recorded in the thrombin group.     

Emergency diagnosis of upper gastrointestinal bleeding by fiberoptic endoscopy.

Emergency esophagogastroduodenoscopy has been performed in 192 consecutive patients admitted with massive gastrointestinal bleeding. Accurate endoscopic diagnosis was made in 184 or 96%; 58 patients underwent emergency operations to control bleeding with an overall operative mortality of 26%. Excluding 16 patients who underwent emergency portacaval shunting, the operative mortality was 7%. In 6 patients, the bleeding was controlled by endoscopic electrocoagulation. There were no complications. Emergency endoscopy should be done routinely as the primary diagnostic approach in the diagnosis of upper gastrointestinal bleeding.