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The New Zealand Medical Journal

 Journal of the New Zealand Medical Association, 06-June-2008, Vol 121 No 1275

Ectopic varices leading to occult haemorrhage in portal hypertension: a surgical quandary
Syed I Andrabi, Jawad Ahmad, W Barry Clements
Abstract
When portal venous pressure exceeds 12 mmHg, portosystemic collaterals develop. Common sites include the oesophago-gastric junction, bare area of the liver, anterior abdominal wall, and the anorectal junction. Variceal haemorrhage from the oesophago-gastric junction is voluminous, overt, and usually easily accessible, however occult bleeding from ectopic varices presents the ultimate challenge to those managing gastrointestinal haemorrhage. In this article we describe two cases of ectopic varices with an outline of the management strategy for such patients.

Ectopic varices are those portosystemic collaterals occurring at sites other than the oesophago-gastric junction (OGJ).1 They account for 5–30% of variceal haemorrhage in patients with portal hypertension.1–3 Due to their relatively uncommon incidence, they are often overlooked by the investigating physician in patients with portal hypertension who are actively bleeding, despite a paucity of endoscopic stigmata.
It is extremely important to consider the diagnosis and have a clear algorithm for the investigation and management of patients with portal hypertension presenting with occult gastrointestinal haemorrhage.

Case 1

A 69-year-old man was admitted through accident and emergency with massive rectal bleeding. He was known to have portal hypertension on the basis of splenic vein thrombosis, and had presented initially with acute haematemesis secondary to oesophageal varices 30 years previously. At that time he entered the chronic sclerotherapy programme, but re-bled and subsequently underwent oesophageal transection with splenectomy. Two years prior to this presentation he had recurrent varices banded on two occasions.
On this admission he was shocked and required 6 units of packed cells. Gastroscopy was negative. On a mesenteric angiogram from the superior mesenteric run, an abnormal leash of vessels were demonstrated in the left upper quadrant, with active bleeding into the splenic flexure. A sub-total colectomy with an ileo-anal anastomosis was performed.
The postoperative recovery was unremarkable. Histological examination of the operative specimen revealed ectopic colonic varices (Figure 1).

Figure 1. Ectopic variceal vessel in the colonic wall


Case 2

A 68-year-old lady was admitted to hospital with anaemia and malaena. Her initial presentation had been 7 years previously with iron deficiency anaemia. At that time she had oesophageal varices and a duplex scan of the portal venous system demonstrated splenic vein thrombosis.
Chronic sclerotherapy was successful initially, however over the past 3 years she had been admitted to hospital on eight occasions with self-limiting malaena and anaemia requiring blood transfusion. Gastroscopy had repeatedly failed to show oesophageal or gastric varices but had revealed a marked portal gastropathy with generalised coarse folds and petechiae. Colonoscopy and small bowel imaging were normal.
Indium-labelled red cell scan demonstrated red cell pooling in the right iliac fossa. A selective superior mesenteric angiography revealed a conglomerate of actively bleeding abnormal varices in the mid-ileal region. Laparotomy was performed and 45 cm of terminal ileum was resected. Histology of the operative specimen described medium-sized thin-walled vessels in the submucosa of the specimen.
She remained well initially, however she developed recurrent occult gastrointestinal haemorrhage and had further admissions for blood transfusion. An angiogram confirmed the presence of splenic vein thrombosis with varices feeding the gastric fundus.
Splenectomy with devascularisation of the gastric fundus was performed and since that time she had no signs of recurrent gastrointestinal haemorrhage.

Discussion

In both the cases presented the patients had presented with ectopic intestinal varices secondary to splenic vein thrombosis.
Ectopic varices are responsible for 5–30% of all variceal haemorrhage.1 They constitute 5% of bleeding episodes in patients with cirrhosis and 30% in patients with extrahepatic portal hypertension.2,4,5
Ectopic varices can occur anywhere along the length of the intestinal tract with a propensity for the duodenum, enterostomy sites, and in adhesions from previous surgery.6 In a review of 169 patients with ectopic varices1, 34% were located in the small bowel—the duodenum being affected twice as commonly as the remainder of the small bowel; 22% were located in the large bowel and 26% at the site of stomas.
Ectopic varices occur more frequently in patients who have had obliterative sclerotherapy. New collateral vessels develop at other sites of porto-systemic anastomoses in an attempt to decompress the portal venous system. The portal circulation in patients with ectopic varices is hyperkinetic and high portal pressures are maintained on account of an associated increase in the mean arterial pressure.7
Duodenal varices are more commonly seen in patients with cirrhosis whilst gastric varices are associated with splenic vein thrombosis.8 Small bowel varices are rare in patients without previous abdominal surgery. They are usually described in patients who have had abdominal surgery and either mesenteric or splenic vein thrombosis.3 Small bowel varices can be detected by enteroscopy and colour Doppler.9 Reverse blood flow in the mesenteric veins is strongly correlated with an increased risk of acute variceal haemorrhage.
In patients with occult upper gastrointestinal haemorrhage, Doppler studies should be employed at an early stage, as this investigation is sensitive in detection of ectopic small bowel varices which are associated with a high mortality (35%).10
Colonic varices have been described in the literature on over 70 previous occasions and are present in 0.07% of postmortem studies11 and 0.2% of colonoscopies.12 Colonic varices occur three times as commonly on the left side of the colon as on the right side. There have only been sporadic reports of varices affecting the transverse colon13 with 60% occurring around the splenic flexure. Sixty percent of patients with colonic varices have had prior sclerotherapy for oesophageal varices.14

Management of etopic varices

There are no clear guidelines for the management of ectopic varices as they are relatively uncommon and are difficult to diagnose. It is important to consider their existence in all patients with portal hypertension, especially those at increased risk.
Preliminary management involves resuscitation with crystalloids and blood products as required. As in all cases of gastrointestinal haemorrhage resuscitation should proceed simultaneously with investigation and treatment. Octreotide is effective during 48 hours in the treatment of acute variceal bleeding, probably by reducing variceal blood flow and pressure.15
Endoscopy is useful to exclude oesophageal and gastric varices as the source of bleeding. Colonoscopy in the acute setting may be unhelpful as blood or faecal residue may obscure the view. Video capsule endoscopy (VCE) can also be used to detect the origin of obscure gastrointestinal bleeding.16
Isotope-labelled red cell scanning is sensitive in identifying ectopic variceal haemorrhage, however it lacks specificity in that it only identifies pooled labelled blood. For patients in whom gastrointerestinal (GI) endoscopy has failed to establish a diagnosis, multi-section CT angiography17 is the most sensitive method for detecting actively bleeding varices.17
Embolisation (Figure 2A,2B) can be performed at the same time and is 95% successful in arresting haemorrhage from oesophageal varices, however its efficacy in arresting ectopic haemorrhage is not established as recurrent bleeding is frequent and reintervention is often required.18

Figure 2A. Bleeding ectopic varices


Figure 2B. Radiological embolisation performed


TIPSS (transjugular intrahepatic portosystemic shunt) is useful in controlling haemorrhage19 in cirrhotics where standard endoscopic measures are ineffective. It is a particularly useful technique in controlling gastric variceal haemorrhage at the expense of a more complex procedure and associated risk of encephalopathy.18
In patients with prehepatic portal hypertension there is a limited role for selective surgical shunting. Patients with sectorial portal hypertension can be successfully treated with splenectomy plus or minus a selective shunt. Originally it was assumed that to adequately decompress the portal system a non-selective shunt was necessary, however these shunts are associated with increased risk of encephalopathy.
The development of the 8 mm side-to-side portocaval shunt has resulted in a minor renaissance of the portocaval shunt. Selective shunts such as the lienorenal or mesocaval shunt remain the most efficacious surgical strategy in portal hypertension for their lower incidence of encephalopathy.
Resection of the involved part of intestine can also be performed but should be combined with operative or radiological shunt so as to prevent formation of new varices at another location or indeed the anastomotic site.

Conclusion

Ectopic variceal haemorrhage is more common than expected or reported. Ectopic varices should be considered in all patients who have portal hypertension who present with occult gastrointestinal haemorrhage. Whilst there are no standard protocols for the investigation and management treatment of ectopic varices, we suggest use of an algorithm, which provides an outline for the management of this challenging clinical condition (Figure 3).

Figure 3. Algorithm of management of ectopic variceal bleeding


Competing interests: None known.
Author information: Syed I Andrabi, Specialist Registrar in Surgery; Jawad Ahmad, Specialist Registrar in Surgery; W Barry Clements, Consultant Upper GI & Laparoscopic Surgeon; Department of Gastrointestinal Surgery, Royal Victoria Hospital, Belfast, Northern Ireland, UK
Correspondence: Syed I Andrabi, 73 Sicily Park, Belfast, BT10 0AN, UK. Email: imranandrabi@gmail.com
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