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Portal vein thrombosis.

Portal vein thrombosis (PVT) is a complication of hepatic disease and a potentially lethal complication of splenectomy. The reported incidence of this complication is low (approximately 1%). However, its true incidence may have been underestimated due to difficulty in making the diagnosis. Herein we report the case of a 19 year-old woman who presented with a 2-year history of idiopathic thrombocytopenic purpura (ITP). Because she had become refractory to medical therapy, she underwent laparoscopic splenectomy. She was discharged on postoperative day 2 after an uncomplicated procedure. She did well, complaining only of mild backache, until postoperative day 21, when she presented with nausea, vomiting, and leukocytosis. CT showed PVT and superior mesenteric vein thrombosis. Despite heparin and fluid administration, her condition worsened. At laparotomy, she had diffuse small bowel edema and congestion. At a second-look procedure 24 h later, nearly all her jejunum and ileum were necrotic. After three procedures, she was left with 45 cm of proximal and 10 cm of distal small bowel. Bowel continuity was restored 8 weeks later. She continued on warfarin anticoagulation therapy for 1 year. Postsplenectomy PVT is most often seen following splenectomy for myeloproliferative disorders and almost never after trauma. The large splenic vein stump and the hypercoagulable state in patients with splenomegaly are thought to be contributory. The presentation of PVT is vague, without defining signs or symptoms. Color-flow Doppler and contrast-enhanced CT scans are the best methods for the nonoperative diagnosis of PVT. Aggressive thrombolysis offers the best hope for clot lysis and maintenance of bowel viability. Even vague symptoms must be considered seriously following splenectomy.

Adult↗

Videolaparoscopic cholecystectomy induces a hemostasis activation of lower grade than does open surgery.

BACKGROUND: Tissue injury after trauma and surgery may induce alterations in blood coagulation and fibrinolysis, and the hypercoagulable state observed after surgery can be associated with the risk of postoperative thromboembolic complications. Recently, videolaparoscopic (VLPS) cholecystectomy has been introduced, and its advantages over the open procedure seem related to the reduced surgical trauma. The aim of this study was to investigate hemostatic system alterations in patients who undergo open and VLPS cholecystectomy. METHODS: Fibrinogen, prothrombin fragment F1+2, D-dimer, and plasminogen activator inhibitor type-1 (PAI-1) activity was determined in 10 patients who underwent open (group A) and 10 patients who underwent VLPS cholecystectomy (group B), respectively. Blood samples were obtained the day of surgery in the morning (B1), after anesthesia (A1), 1 hour after the start of surgery (S1), then 30 min (E.05) and 24 h (E.24) after the surgery. RESULTS: No significant differences were observed in baseline values between groups A and B for the parameters investigated. At 24 h after surgery, fibrinogen increased significantly (p < 0.05) in group A and also was significantly higher than in group B (p < 0.05). In group A, a marked increase in F1+2 levels (p < 0.01) was observed in all the samples, with the maximum values on the first day after surgery (3.7 nmol/l; 1.2-6.0 nmol/l), whereas in group B, a slight but significant increase in F1+2 levels (2.1 nmol/l; 1.1-3.9 nmol/l; p < 0.01) was observed only 30 min after the end of surgery. In both groups A and B, D-dimer markedly increased after surgery, without statistical significant differences between the two groups at any time. The PAI-1 activity plasma levels remained in the normal range during and after surgery in both groups. CONCLUSIONS: These results indicate that VLPS surgery induces an activation of the clotting system that, although of low degree and short duration, can lead to a transient prothrombotic state.

Adult↗

Premature acute myocardial infarction in a child with nephrotic syndrome.

We report a case of acute myocardial infarction in a nephrotic child. A 7-year-old boy with a 4-year history of steroid-unresponsive nephrotic syndrome due to mesangial proliferation disease presented with acute vomiting and chest pain. An electrocardiogram showed ST elevation and pathological Q waves in leads consistent with anterior and septal myocardial infarction. Subsequent cardiac catheterization showed no evidence of atherosclerotic coronary artery disease, and thrombotic occlusion of the anterior descending coronary artery was the likely cause of the event. Myocardial scintigraphy showed extensive myocardial damage. The child had no long history of extreme hypercholesterolemia or hypertriglyceridemia. The case suggests that children with long-lasting nephrotic syndrome may be at increased risk for ischemic cardiovascular events, due to hyperlipidemia as well as a hypercoagulability state. The literature is reviewed regarding the relationship between nephrotic syndrome and the incidence of ischemic heart disease.

Child↗

Nephrogenic fibrosing dermopathy in pediatric patients.

Nephrogenic fibrosing dermopathy (NFD) is a rare and recently recognized sclerosing skin disorder of unknown etiology. Reported cases have occurred in patients with chronic renal failure, with or without renal replacement therapy. All previous cases have been reported in older adult patients. We describe two pediatric patients who recently developed this condition and review the existing literature for NFD. Our patients included an 8-year-old boy on peritoneal dialysis with no prior renal transplant and a 19-year-old boy on hemodialysis with a history of previous failed renal transplants. We speculate that the recent emergence of this condition and occurrence in patients with chronic renal failure suggest an association with some newer pharmacological agent that has recently come into wide use. Since both our patients also had previously experienced large vessel thrombosis, hypercoagulable states may also be implicated.

Adult↗

Platelet-derived growth factor and platelet profiles in childhood nephrotic syndrome.

The aim of the study was to investigate (1) whether there are any changes in release of platelet-derived growth factor AA (PDGF AA) in children with nephrotic syndrome without clinical thromboembolic symptoms 2; (2) whether serum PDGF AA correlates with the platelet count (PLT) and platelet indices; (3) whether prednisone therapy affects the serum PDGF AA and the PLT; (4) whether PDGF AA is a useful predictor of disease activity. The study involved two groups of children: 33 with nephrotic syndrome (I) who were evaluated twice (A during relapse and B after 2 weeks of prednisone treatment) and 34 healthy children (II). The serum concentration of PDGF was measured by ELISA. In group I/A the PLT (P<0.01) and platelet distribution width (P<0.05) were elevated, the mean platelet volume (MPV) (P<0.05) was decreased and the plateletcrit (P>0.05) was normal. In group I/B, the PLT was decreased and MPV increased. The concentration of PDGF AA was still increased and correlated negatively with the albumin concentration. Hence in children with nephrotic syndrome an increase in PLT, a decrease in MPV, and a higher concentration of PDGF were observed. Treatment of nephrotic syndrome with prednisone for 2 weeks is not sufficient to normalize platelet parameters. Further studies are necessary to confirm the role of PDGF AA in the hypercoagulation state in children with nephrotic syndrome.

Blood Platelets↗

Portal and mesenteric vein thrombosis after portal vein embolization in a patient with protein S deficiency.

Portal vein embolization can be performed safely, and so far no major complications have been reported. We report an extremely rare complication of portal vein embolization, a case of portal and mesenteric thrombosis in a 65-year-old patient with protein S deficiency. Right portal vein embolization was carried out prior to extended right hepatectomy for advanced gallbladder carcinoma involving the hepatic hilus. Computed tomography 14 days after embolization revealed massive thrombosis of the portal and the superior mesenteric veins. A protein S deficiency was found by means of an extensive workup for hypercoagulable state. Portal vein embolization may have triggered a cascade of events that was expressed as portal and mesenteric vein thrombosis resulting from deficiency of protein S. It may be better to determine the concentrations of such coagulation regulators prior to portal vein embolization.

Aged↗

Treatment of varicose veins: an assessment of intraoperative and postoperative compression sclerotherapy.

Since thrombotic complications, such as superficial thrombophlebitis and subsequent skin pigmentation, are common after sclerotherapy, we conducted a study to evaluate whether combining sclerotherapy with ligation of varicose veins minimizes complications and what timing for sclerotherapy would be most beneficial-accompanying surgery or several weeks postsurgery. Surgical intervention and compression sclerotherapy were performed consecutively on 111 limbs (group A), and sclerotherapy was performed 28 days after surgical intervention on 87 limbs (group B). The volume of sclerosant used and the frequency of complications (thrombus formation and pigmentation) were analyzed. Plasma levels of thrombin-antithrombin III complex (TAT) and D-dimer (DD), as markers for activation of coagulation, were compared. In group A, the total volume of sclerosant used in patients with complications was significantly higher than that in patients without complications. The frequency of thrombus formation and of pigmentation was significantly lower (p <0.01) in group B (10% and 18%, respectively) than in group A (21% and 37%, respectively). The plasma levels of TAT 7 days after treatment were significantly lower in group B (3.4 +/- 1.2 mg/L) than in group A (4.9 +/- 1.1 mg/L). Performing compression sclerotherapy 28 days after surgical intervention is effective for reducing complications and a good alternative for patients with an underlying hypercoagulable state.

Adult↗

Mesenteric ischemia affects young adults with predisposition.

An 8-year retrospective review of Indiana University Hospital records consisting of any patient age 18 to 40 years old who presented with arterial mesenteric ischemia was performed. Three patients were identified that met our criteria. The first patient was discovered to have a protein C deficiency. The second patient was afflicted with afibrinoginemia, a protein C and an antithrombin III deficiency. The third patient had been previously diagnosed with Takayasu's arteritis and had an elevated ESR. Each patient had a protracted course of symptoms before mesenteric disease was considered, confirmed by angiography, and treated by arterial bypass with/without bowel resection. All patients survived and are currently asymptomatic at an average of 2 years postoperatively. Mesenteric ischemia in patients under the age of 40, especially in the absence of cocaine use, is rare and often causes a delay in diagnosis and appropriate treatment. The high incidence of hypercoagulable states in our study cases suggests the need for a search for such disorders and the possible need for long-term anticoagulation therapy as a deterrent to recurrence.

Adult↗

Homocysteine-associated acute mesenteric artery occlusion treated with thrombectomy and bowel resection.

Elevated plasma homocysteine is an acknowledged risk factor for arteriosclerotic occlusive disease, but little clinical evidence is available regarding its role in acute arterial thrombosis in the absence of an underlying lesion. A 45-year old man presented with an acute abdomen. A magnetic resonance arteriogram (MRA) showed occlusion of the superior mesenteric artery. At exploration, necrotic ileum was resected and the superior mesenteric artery was thrombectomized, restoring normal mesenteric flow. The plasma homocysteine level was 98.8 mmol/L, more than eight times the normal level. No embolic source was identified and an MRA and contrast arteriogram showed no residual occlusive disease in the superior mesenteric artery. Additional studies documented pernicious anemia, which was treated with cobalamin (vitamin B12) injections. This case provides further evidence of an association between hyperhomocysteinemia and acute arterial thrombosis. Hyperhomocysteinemia can result from easily correctible vitamin B12, B6, or dietary folate deficiencies. Plasma homocysteine levels should be determined in young individuals with acute arterial thrombosis whenever a hypercoagulable state is suspected.

Anastomosis, Surgical↗

Unusual case of mesenteric venous thrombosis associated with oral contraceptive use in an adolescent girl.

A 16-year-old girl presented with hematochezia and abdominal pain. The patient developed a small bowel obstruction that required a laparotomy, which showed a segment of grossly infarcted small bowel with 3 perforations, localized abscesses, and creeping fat. Resection and an end-to-end anastomosis were performed. Pathologic examination showed underlying mesenteric venous thrombosis with no evidence of Crohn's disease. Hematology workup showed no evidence of a congenitally inherent hypercoagulable state. The use of oral contraceptives was the only predisposing factor identified.

Adolescent↗

Effect of the factor VII R353Q missense mutation on plasma apolipoprotein B levels: impact of visceral obesity.

An atherogenic dyslipidemia, characterized by increased plasma triglyceride and apolipoprotein (apo) B levels, low HDL-cholesterol concentrations and the development of small, dense LDL particles has been associated with the presence of abdominal-visceral obesity. Visceral obesity is also associated with a hypercoagulate state and elevated concentrations of procoagulant factors such as factor VII. Moreover, it is known that some genetic variants in the gene encoding factor VII alter its activity and concentration, and consequently these variants may have an impact on atherosclerosis development. The objective of this study was to verify whether the factor VII R353Q polymorphism contributes to predict the risk of an atherogenic dyslipidemia in absence and in the presence of visceral obesity. A sample of 299 French-Canadian men, selected in order to cover a wide range of body fatness values, participated in this study. We observed that the R353 allele was more commonly observed among men characterized by apo B levels below 1.09 g/l than among men with apo B levels greater or above this threshold value (allele frequency of 92.1 vs 85.4%, chi(2)=6.18, P=0.01). Multivariate analyses further showed that the genotype R353/R353 was associated with a lower risk to exhibit atherogenic concentrations of total-apo B (>/=1.09 g/l) and LDL apo B (>/=0.95 g/l) before (odds ratio:0.47, 95%CI=0.27-0.90, P=0.02; odds ratio:0.46, 95%CI=0.25-0.85, P=0.01, respectively) and after adjustments for age and visceral AT (odds ratio:0.49, 95%CI=0.24-0.91, P=0.02; odds ratio:0.44, 95%CI=0.23-0.85, P=0.01, respectively). When the two genotype groups were further divided on the basis of visceral adipose tissue (AT) accumulation using a cutoff point of 130 cm(2), we observed that R353/R353 homozygotes with low visceral AT were characterized by a more favorable lipoprotein-lipid profile, mainly lower total-cholesterol, total-apo B, and LDL-apo B levels compared with R353/R353 homozygotes with high levels of visceral AT. In contrast, irrespective of obesity, plasma lipid levels among carriers of the Q353 allele were similar to those of viscerally obese men homozygous for the R353 allele. In conclusion, results of the present study suggest that the factor VII R353 allele is associated with lower concentrations of plasma apo B levels. However, the presence of visceral obesity abolishes this effect. Further studies will be necessary to confirm this association and the mechanism involved.

Adult↗

Behçet's disease associated with superior vena cava syndrome without thrombosis.

Behçet's disease is a multisystemic vasculitis of unknown etiology, which is characterized by recurrent urogenital ulceration, cutaneous eruptions, ocular manifestations, arthritis and vasculitis, and its diagnosis is based on clinical criteria. Superior vena cava (SVC) thrombosis is a rare but well-recognized manifestation of Behçet's disease, whereas SVC syndrome due to vasculopathy, without evidence of thrombosis, has not yet been described in the literature. The authors report the case of a patient with Behçet's disease, who presented SVC syndrome with reduction in the lumen of the SVC due to thickening of the vessel wall, without evidence of thrombosis upon computed tomography and magnetic angioresonance. The patient received early anticoagulant therapy, corticosteroid and monthly cyclophosphamide pulse therapy. Clinical control without recurrence was observed after 6 months of follow-up. Behçet's disease should be suspected in young patients presenting with SVC syndrome, in the absence of thrombosis or of a hypercoagulable state.

Adult↗

Postpartum cerebellar infarction and haemolysis, elevated liver enzymes, low platelet (HELLP) syndrome.

Pregnancy is considered to be a hypercoagulable state per se with an increased risk for cerebrovascular events, however cerebellar infarction has been rarely described in pregnant women. A nulliparous pre-eclamptic woman at 25 weeks' gestation was submitted to an echocardiographic exam that showed an impaired cardiac structure and function. After 2 h, the patient underwent caesarean section for diagnosis of haemolysis, elevated liver enzymes, low platelet (HELLP) syndrome. Afterwards her platelet count raised, and eight days later she developed nystagmus, ataxia, dysmetria and motor deficit in the right limbs and sensory impairment in the right side of the face and in the left limbs. Cerebral magnetic resonance imaging (MRI) demonstrated a right cerebellar and median posterior bulbar infarction. Colour-coded sonography of cerebral vessels showed an occlusion of the right vertebral artery. Coagulation pattern analysis evidenced double heterozygosis of the methylenetetrahydrofolate reductase (MTHFR) gene and single mutation of the prothrombin gene. This case report gives evidence of the importance of considering the different risk factors involved in stroke occurrence during pregnancy.

Adult↗

Plasma levels of coagulation and fibrinolysis markers in acute ischemic stroke patients with lone atrial fibrillation.

Atrial fibrillation (AF) is a well-defined risk factor for ischemic stroke. Patients with lone AF represent a subgroup of AF patients with the lowest lifelong stroke risk. Nonvalvular atrial fibrillation (NVAF) confers a hypercoagulable state resulting in an increased risk of thromboembolism. This study was performed to determine the contributory role of alteration in the hemostatic markers of thrombin generation and fibrinolysis in patients with lone AF during acute ischemic stroke episode. We studied thrombin-antithrombin complexes (TAT), prothrombin fragments 1+2 (F1+2), tissue-type plasminogen activator (tPA) and plasminogen activator inhibitor type-1 (PAI-1) concentrations in patients with acute middle cerebral artery ischemic stroke due to atherosclerotic large artery disease (n=50), lone AF (n=24) and cardioembolism (n=21). The values were compared with those of age-matched control subjects with lone AF and sinus rhythm (n=21 and 15, respectively). The mean F1+2 concentration was higher in the control subjects with lone AF in comparison with those without AF (p=0.014). Patients with stroke due to possible cardioembolism, from lone AF or other causes, had higher TAT (and marginally higher F1+2) concentrations than those with atherosclerotic stroke (p<0.001). tPA concentrations were not different among groups (p=0.89). PAI-I levels were marginally higher in stroke patients with lone AF and atherothrombotic large artery disease compared to the controls without AF (p=0.05). These results suggest that in the acute period of ischemic stroke secondary to lone AF, enhancement of the coagulatory activity occurs as a result of increased thrombin generation, similar to other possible sources of cardioembolism. Observed hemostatic alterations in acute ischemic stroke associated with lone AF may indicate some therapeutic and prognostic implications.

Acute Disease↗

Aortic thrombosis after low anterior resection for rectal cancer: report of a case.

Pelvic surgery for malignant disease has been associated with numerous acute postoperative complications. These complications are primarily vascular or neurologic in origin. Several factors associated with the occurrence of these complications include the lithotomy position, the prolonged use of sequential compression devices, the use of certain types of stirrups, the presence of peripheral vascular disease, and the common hypercoagulable state of most cancer patients. We report for the first time a case of aortic thrombosis after elective low anterior resection for rectal cancer and discuss some factors that may have a role in the occurrence of this devastating complication.

Aged↗

Portal vein thrombosis after laparoscopic sigmoid colectomy for diverticulitis: report of a case.

Portal vein thrombosis is a very uncommon complication after laparoscopic surgery. Although only one case of portal vein thrombosis has been reported after laparoscopic colectomy, there are several reports of mesenteric vascular occlusion after other laparoscopic procedures. We present a case of portal vein thrombosis in a patient with no other demonstrable hypercoagulable states or risk factors, who underwent an uneventful laparoscopic sigmoid colectomy. Because alteration in coagulation may occur after establishing a pneumoperitoneum, we suggest that heparin prophylaxis may be advisable to avoid these kinds of complications, especially if a past history of coagulable disorders is present.

Aged↗

Antithrombotic therapy for stroke in young adults.

Stroke in young adults is a markedly heterogeneous disease, and remains an understudied phenomenon. While advances are being made in our understanding of the pathophysiology of its underlying conditions, treatment concerns are controversial, and clinical trials are sorely lacking. This review presents an overview of some of the relevant management issues in hypercoagulable states, migraine, patent foramen ovale, vascular dissection and venous sinus thrombosis.

Adolescent↗

Perioperative management of oral anticoagulation: when and how to bridge.

The management of patients on oral anticoagulation (OAC) who need to undergo surgery or invasive procedures is problematic. "Bridging" the subtherapeutic periods with either intravenous unfractionated heparin or subcutaneous treatment-dose low-molecular-weight heparin (LMWH) decreases the amount of time patients are not anticoagulated but may increase the risk of postoperative bleeding and is costly. The available literature does not provide sufficient information to allow clinicians to choose an optimal perioperative strategy. Recent studies primarily have examined the perioperative use of LMWH, and have found arterial thromboembolic rates of 0.4-1.5%. The observed incidence is greater than mathematically predicted, which may be due to a potential hypercoagulable state impacting the risk for arterial thromboembolic events. The literature suggests that major postoperative bleeding is low for invasive procedures but may be substantially higher for major surgery. Given the lack of definitive data or consensus, the decision must be based on estimates of the risks of thromboembolism and bleeding and the patient's preference. For most patients at low or moderate stroke risk, bridging will be unnecessary and may be harmful. Bridging is recommended for patients who have a high annual risk of stroke and thus have a more appreciable perioperative stroke risk. Postoperative anticoagulation must be used cautiously and patients monitored closely after major surgery due to the risk of postoperative major bleeding.

Administration, Oral↗