Isolated splenic vein thrombosis is a rare clinical syndrome that may lead to life threatening haemorrhage from isolated gastric varices. The splenic vein lies inferior to the splenic artery and posterior to the pancreas, hence the aetiology of splenic vein thrombosis is most often related to pancreatic disease [4].
Splenic vein thrombosis after splenectomy for trauma and iatrogenic causes such as partial gastrectomy, umbilical vein catheterisation, Warren-Zeppa shunt have been described in literature [4].
The pathogenesis of splenic vein thrombosis following trauma, is a result of venous stasis, vessel wall dysfunction and alteration in clotting mechanism in the form of hypercoagulability [5]. Immobility following trauma leads to venous stasis, reduced venous return and endothelial cell dysfunction because of decreased oxygen and nutrient delivery [5]. Neutrophil production and activation of cytokines leads to activation of platelets, intrinsic and extrinsic coagulation pathways [6]. Post trauma there is a decrease in circulating levels of plasma anti thrombin III [7, 8], protein C/S and plasmin [9]. The net result is of a pro coagulant state.
The longest delayed presentation of traumatic diaphragmatic hernia is 50 years from the time of original injury [10]. There are usually three phases of presentation for traumatic diaphragmatic rupture, namely acute phase, interval phase & obstructive phase [11]. Ball et al in their series observed that those who presented in the acute phase had usually left sided tears and those who had a delayed presentation had significant right sided tears causing herniation [12].
This is in contrast to our case where the penetrating injury was sustained in the left hypochondrium causing splenic herniation and leading him to present with upper gastrointestinal bleed secondary to splenic vein thrombosis 28 years later. We believe that the hypercoagulability was accentuated as a result of the aberrant position of the spleen causing venous stasis and sluggish flow through a kinked splenic vein.
Paramount to establishing the diagnosis of isolated splenic vein thrombosis is a high index of suspicion often aroused by a history of trauma in the vicinity of splenic vein [13].
The symptoms and signs most commonly associated with isolated splenic vein thrombosis is gastrointestinal bleeding and abdominal pain along with splenomegaly and normal liver function tests [14].
A thrombus in the splenic vein causes the venous out flow to return to the portal vein by the way of low pressure collaterals namely the short gastric veins, the veins of the upper half of the stomach, the coronary vein and the gastroepiploic veins [3]. Increased flow across the vessels creates a local form of extrahepatic portal hypertension sometimes referred to as left sided or sinistral portal hypertension [4]. The hypertensive short gastric veins cause increased pressure within the sub mucosal veins of the gastric fundus resulting in gastric varices [15].
It is difficult to diagnose isolated splenic vein thrombosis both endoscopically and radiologically [3]. Gastric varices may not be recognised with endoscopy [3]. Useful investigations are ultrasound examination, along with contrast enhanced CT, upper GI endoscopy and at times digital subtraction angiography [3]. One prospective study recommended the use of endoscopic ultrasonography, a technique quite sensitive for the determination of isolated gastric varices, in patients with high clinical suspicion [3].
Splenectomy is the procedure of choice in the management of haemorrhage due to isolated splenic vein thrombosis [3]. There is no consensus on the treatment of asymptomatic patients, as yet [3].
