Isolated jejunal varices.

SS Bhagwat, SS Borwankar, RH Ramadwar, AS Naik, GI Gajaree 
 Department of Paediatric Surgery, Seth G S Medical College, Parel, Mumbai.

Correspondence Address:
S S Bhagwat
Department of Paediatric Surgery, Seth G S Medical College, Parel, Mumbai.

Full Text

::   Introduction

The commonest cause of portal hypertension is portal vein thrombosis[1]. Esophagogastric varices are a common manifestations of portal hypertension. Ectopic varices in the duodenum, jejunum, ileum, colon, rectum, at the stoma of an enterostomy. and at areas of previous abdominal surgeries are known[2]. Isolated jejunal varices in the absence of oesophageal or gastric varices with no prior abdominal surgery has not been reported earlier. We describe a case of massive gastrointestinal bleeding due to jejunal varices in a 1 ½ year old boy. This case posed a diagnostic dilemma.

::   Case report

A 1 ½ year old boy with extra-hepatic portal hypertension was admitted with hematochezia and shock. The patient had six episodes of hematochezia and malena, the first one being at the age of four months. However, the patient had only one episode of hematemesis (about 510 cc only) at the age of one year. The patient was a home delivery and had suffered from umbilical sepsis. During each of the episodes, his liver function tests and coagulation profile were persistently normal. Upper gastrointestinal endoscopy and proctosigmoidoscopy were also normal. Ultrasound had demonstrated portal cavernoma with normal hepatic architecture and also splenic cavernoma. Splenoportogram [Figure:1] done at 14 months of age had shown splenomegaly, portal and splenic cavernoma with portal vein and splenic vein thrombosis. However, no oesophageal or gastric varices were seen. Meckel's scan was also normal. During prior episodes, bleeding had spontaneously subsided though the patient required multiple blood transfusions.

On admission, the patient was pale with a pulse ate of 142/min and blood pressure of 50/30 mm of Hg. The abdomen was non-tender. There was no hepatosplenomegaly or free fluid in the abdomen. His hematocrit was 18%. Coagulation profile and liver function tests were normal. The patient continued to pass bright red blood per rectally and required several transfusions to stabilise vital parameters. The nasogastric aspirate did not show blood. A repeat upper GI endoscopy was normal. 99m  Technetium labelled RBC scan did not reveal an active bleeding site. Abdominal aortography with selective celiac, superior mesenteric and inferior mesenteric angiographies did not reveal any active bleeding source. Splenic artery was seen arising from the superior mesenteric artery.

Since the patient continued to have uncontrolled life threatening haemorrhage, exploratory laparotomy was performed. The liver appeared to be normal. There was a portal cavernoma, the mesentery of the jejunum starting from the DJ junction and spread over a length of 40 cms of proximal jejunum showed dilated, tortuous vessels. There were no adhesions in this region. The small bowel was full of blood but the mesentery of the rest of the small bowel was normal. Meckel’s diverticulum was absent. Ligation of mesenteric vessels, segmental resection of the involved jejunum and a difficult duodenojejunal anastomosis were performed. The resected specimen showed large varices protruding into the intestinal lumen. Histo-pathology revealed dilated sub-mucosal and sub-serosal veins. The acute bleeding ceased after the surgery. The postoperative course was uneventful. The patient has not bled during a 8 month followup. Review of the venous phase of the superior mesenteric angiography, showed large varicoid mesenteric veins in the left upper quadrant [Figure:2]. The superior mesenteric vein was not visualised.

::   Discussion

The prevalence of symptomatic jejunal varices is low. Eleven cases of bleeding jejunal varices have been described so far, the youngest being in a 16 year old female[3]. Eight patients had cirrhosis[3],[4],[5],[6], two extrahepatic portal hypertension[7],[8] and one had Banti's syndrome[9]. Associated oesophageal varices were present in eight cases[4],[5],[6], Bleeding jejunal varices were closely associated with intraabdominal adhesions in these five cases[3],[4],[6],[7],[9] Our case is the youngest reported case. There were no associated varices. Our patient had not undergone any previous abdominal surgery. Cases reported so far presented with malena and hematochezia. However hematemesis was uncommon[3].

Gastrointestinal bleeding from jejunal varices occurs infrequently. Accurate preoperative diagnosis is often difficult. However venous phase of angiography is diagnostic for locating the jejunal varices. But extravasation of the dye into the intestinal lumen is rarely visualised. This is due to dilution of the dye in the venous phase. Also venous bleeding is slower than arterial bleeding .3 Small bowel enteroclysis is also useful in locating the bleeding site[10].

Vasopressin infusion via superior mesenteric artery is only temporarily effective. Surgical alternatives include portosystemic shunts, variceal ligation and resection of the involved bowel. Surgical approach depends on the severity of the liver disease[3]. In our case, on exploratory laparotomy, the liver was normal. There was a portal cavernoma. Localised jejunal varices starting from Ligament of Treitz extending on the 40 cms of jejunum were visualised. Localised resection of the involved bowel with duodenojejunal anastomosis was carried out. There was complete resolution of bleeding. In the literature two patients of bleeding jejunal varices were treated conservatively[4],[5]. Both had recurrent bleeding and one of them died. Four patients were treated with porto-caval shunt of which one patient had recurrent bleeding[4],[5],[6],[10],[11]. Variceal ligation was done in two patients one of whom died post-operatively. Jejunal resection was carried out in three patients. These patients did not have any recurrent bleeding. One of these patients had been followed up for three years. Our patient has not bled during an eight month followup period. He is under surveillance.

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