After a pancreatitis episode associated with hyperlipidemia, developing a thrombosis or occlusion in splenic vein may cause a left sided portal hypertension. Pancreatitis is
considered in hypertriglyceridemic patients with unclear abdominal pathology. Pancreatic enzymes are not always good predictor in these patients. In the differential diagnosis of a patient with upper gastrointestinal hemorrhage, splenic vein thrombosis or occlusion has an important role because it is rare and the diagnosis is difficult. Upper gastrointestinal
hemorrhage may be a unique sign of this series. Gastroscopy is very helpful with ultrasonography in emergency cases. Splenectomy is the appropriate management in sinistral portal hypertension when it is complicated.
A 20-years-old woman was admitted with findings massive upper gastrointestinal tract bleeding to the emergency
One and half years before admission, she was cared in ICU for 20 days because of acute pancreatitis. During the
pancreatitis attack, fasting glucose levels were up to 187 mg/dl, triglyceride was >1400 mg/dl, the amounts of the enzyme
amylase in serum were under 400 U/L but in ascites fluid was more than 5000 U/L. Meantime there were ischemic findings on ECG. After a few days fasting, triglyceride level has dropped under 500 mg/dl. Until her last episode, she hasn’t taken any medication for hypertriglyceridemia. Her mother and sister were taking medication for hyperlipidemia. There was no
history of alcohol consumption.
The patient with hematemesis and melena was evaluated in emergency room. Hemoglobin was 3.5 g/dl and hematocrit was 9.8%. The patient was transfused with total 21 unite
erythrocyte suspension and eight unite fresh frozen plasma for hemodynamic stability until operation time.
On endoscopic examination, major varicose veins with bleeding episodes were seen at sinistral gastric fundus
Abdominal computerized tomography (CT) was interpreted as: splenomegaly, splenic infarct, localized fluid collection medial to the spleen and suspected splenic vein thrombosis or nodular lesion on pancreas tail (Fig. 2).
Portal system doppler investigation has shown that splenic vein was six mm in diameter at portal confluence, but was 28.6 mm in diameter at splenic hilus. Between of these different calibrations, a hyperechogen nodular lesion 27.3 mm in diameter which restricted splenic venous flow has seen (Fig. 3 a,b).
After the last gastric bleeding episode which was
uncontrolled despite conventional methods, the patient was taken to operating room. Laparotomy confirmed dilated
collaterals along the grater curvature of the stomach, hypertrophic spleen which contained segmental infarct, 3 x 5 cm nodular lesion adhered to splenic vein and spleen between pancreatic tail and the splenic hilum.
We removed the spleen, resected the nodular lesion on splenic vein and ligated major dilated fundal gastric veins (Fig. 4a,b).
Histopathologic evaluation of hematoxylene-eosine (HE) stained slides of the nodular lesion 3 x 5 cm in size revealed partially fibrotic pancreatic tail, which adhered to the splenic vein and minimally canalized splenic vein with an organized thrombus. Chronic pancreatitis was seen on the nodular pancreatic tissue. Histiocyte aggregates were found on biopsy samples of spleen, hiler lymph nodes and liver, which were thought they related with hypertriglyceridemia (Fig. 5).
Hypertriglyceridemia may limit serum amylase elevation. Serum and urinary amylase levels maybe normal or minimally elevated in pancreatitis developed due to hypertriglyceridemia. Excess plasma lipids or an inhibitor factor may reduce
pancreatic enzymes activity. According to Warshow report, removal of excess lipids by ultracentrifugation does not
eliminate the inhibition of amylase activity. (1)
Hypertriglyceridemia may cause pancreatitis. Hypertrigly-ceridemia is defined as a triglyceride level > 150 mg/dl. If triglyceride levels are > 1000 mg/dl, the risk for pancreatitis and its squeals becomes clinically significant. (2)
The causes of hypertriglyceridemia are familial and
genetic syndromes, metabolic disease (diabetes, obesity, hypothyroidism, and nephrotic syndrome), drugs (thiazide diuretics or chlorthalidone, beta-adrenergic blocking agents, oral estrogen, tamoxifen, glucocorticoids, oral isotretinoin) and others (alcohol, high-carbohydrate diets, acute pancrea-titis, and pregnancy). Hypertriglyceridemia is correlated with an increased risk of cardiovascular disease.
The exact mechanism whereby hypertriglyceridemia causes pancreatitis is not clear. Presumably, hydrolysis of triglyceride in and around the pancreas by pancreatic lipase seeping out of the acinar cell leads to accumulation of free fatty acids in high concentrations. Unbound free fatty acids are toxic and could produce acinar cell or capillary injury. Increased concentration of chylomicrons in the pancreatic capillaries causes capillary plugging and leads to ischemia and acidosis, and in the acidotic environment, free fatty acids cause activation of trypsinogen and initiate acute
The reduction of triglyceride level to below 1000 mg/dL effectively prevents further episodes of pancreatitis. The
mainstay of treatment for the hypertriglyceridemia associated with pancreatitis includes dietary restriction of fat and
administration of lipid-lowering agents. Within 24 to 48 hours of the onset of pancreatitis, triglyceride levels fall rapidly as a result of fasting status. Benefits of plasmapheresis, lipid
pheresis, and extracorporeal lipid elimination are not clear. (4)
Pancreatitis may cause splenic vein thrombosis/obstruction. Because of its location, any type of pancreatic disease is likely to involve the splenic vein.
The most common pathologies resulting in splenic vein thrombosis or obstruction are chronic pancreatitis, pancreatic pseudocysts and pancreatic neoplasms. (5,6)
In the diagnosis of splenic vein deformation spleno-portography, angiography and ultrasonography are helpful. The studies with splenoportography have shown that partial splenic vein deformation was obtained 9-85% in the patients with pancreatitis and that complete occlusion was found up to 24%. (7)
Thrombosis of the splenic vein develops by intrinsic or extrinsic damage. Intrinsic damage to the intima of the splenic vein occurs as a result of inflammatory and neoplastic disease or surgical manipulation. Extrinsic damage occurs by compression of the vein secondary to edema, cellular infiltration, and fibrosis. The result in either case is stasis of blood flow and eventually partial to complete occlusion.(6)
Sinistral portal hypertension may occur as a result of splenic vein thrombosis/obstruction. After splenic vein thrombosis occurs, pressure is transmitted through its anastomoses with the short gastric and gastroepiploic veins and sub-sequently via the coronary vein into the portal system. Increased flow across the short gastric veins creates a local form of extrahepatic portal hypertension, referred to as left-sided or "sinistral" portal hypertension. This phenomenon is distinct from the common phenomenon of generalized portal hypertension. Eventually, gastric varices develop along the greater curvature and fundus of the stomach. (6)
The radiological characteristics of localized gastric varices in the fundus have been well described. Gastric series with barium and gastroscopy are helpful in diagnosis. When outlined by barium, they appear as thick, tortuous mucosal folds, filling defects, or distorted mucosal configurations over the greater curvature extending toward the
cardia. (8) Fundal varicose folds can be seen in upper gastro-intestinal endoscopic studies.
The most frequent complication associated with splenic vein occlusion is gastrointestinal hemorrhage. Recurrent hematemesis, melena, and anemia are manifestations of the significant blood loss from bleeding gastric varices. Gastric varices and hemorrhage are late signs of splenic vein
Management of this condition involves splenectomy and surgical removal of the primary cause. (5) Splenectomy decreases the arterial inflow into the left portal system. The success rate of the splenectomy to prevent of another episode of gastrointestinal blood loss is up to 92%. (9,10) Prophylactic splenectomy may not be necessary in all patients with sinistral portal hypertension. Conservative approach may be better in those with more minor bleeds or in asymptomatic patients. Embolization of the splenic artery has been suggested as an alternative to splenectomy in unstable patients. After embolization, splenic abscess may occur.
1. WARSHAW, A.L., BELLINI, C.A., LESSER, P.B., - Inhibition of serum and urine amylase activity in pancreatitis with hyperlipemia. Ann. Surg., 1975, 182:72.
2. Executive Summary of the third report of the national cholesterol education program expert panel on detection, evaluation, and treatment of high blood cholesterol in adults. JAMA, 2001, 285:2486.
3. HAVEL, R.J. - Pathogenesis, differentiation and management of hypertriglyceridemia. Adv. Intern. Med., 1969, 15:117.
4. ISKANDAR, S.B., OLIVE, K.E. - Plasmapheresis as an Adjuvant Therapy for Hypertriglyceridemia-Induced Pancreatitis. Am. J. Med. Sci., 2004, 328:290.
5. SAKORAFAS, G.H., SARR, M.G., FARLEY, D.R., FARNELL M.B. - The significance of sinistral portal hypertension
complicating chronic pancreatitis. Am. J. Surg, 2000, 179:129.
6. THOMPSON, R.J., TAYLOR, M.A., MCKIE, L.D.,
DIAMOND, T. - Sinistral portal hypertension. Ulster. Med. J., 2006, 75:175.
7. LEGER, L., LENROIT, J., LAMAIGRE, G. - Hypertension portale segmentaire des pancreatities. Aspect angiographiques. J. Chir. (Paris), 1968, 95:599.
8. MARSHALL, J.P., SMITH, P.D., HOYUMPA, A.M. J.R., - Gastric varices: Problem in diagnosis. Am. J. Dig. Dis., 1977, 22:947.
9. MOOSSA, A.R., GADD, M.A. - Isolated Splenic Vein Thrombosis. World J. Surg., 1985, 9:384.
10. WEBER, S.M., RIKKERS, L.F. - Splenic vein thrombosis and gastrointestinal bleeding in chronic pancreatitis. World J. Surg., 2003, 27:1271.