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Portal hypertension is a significant complication of cirrhosis and often leads to the development of abnormal collaterals between the portal and systemic circulation. These can occur anywhere in the gastrointestinal tract (GIT), but occur most commonly at the cardio-oesophageal junction. Collaterals developing at other sites have been broadly termed as ectopic varices. These can develop anywhere in the abdomen including the entire GIT, in the abdominal wall, sites of previous abdominal surgery, stomal sites, the falciform ligament and gall bladder. Figure 1 shows stomal varices supplied by the inferior mesenteric vein.
The prevalence of ectopic varices seems to depend on the cause of portal hypertension and the technique used to demonstrate the varices. Although these are a relatively common finding at endoscopy, they are an unusual cause of gastrointestinal haemorrhage and can account for up to 5% of variceal bleeding.1 In a review of 169 cases of bleeding ectopic varices, 17% occurred in the duodenum, 17% occurred in the jejunum or ileum, 14% in the colon, 8% in the rectum and 9% in the peritoneum. Twenty-six per cent bled from peristomal varices and a few from other infrequent sites.1
Management of bleeding from ectopic varices can be challenging both because of inaccessibility and initial difficulty in diagnosis and subsequent difficulty in treatment. It is difficult to determine the best treatment strategy for ectopic varices as most literature on this subject consists mainly of small series and case reports with no randomized trials of therapy.
Traditional conservative treatments include local ligation,2 cauterization, sclerotherapy3 or embolization of the feeding vessels. Described surgical options range from surgical excision, mucocutaneous detachment, stomal resiting or revision,4 to portosystemic shunting5, 6 and liver transplantation. High rebleeding rates are observed following local treatment of varices and both selective variceal embolization (VE) and portocaval surgery are associated with a high post-operative morbidity and mortality. More recently transjugular intrahepatic portosystemic stent shunts (TIPSS) have been used in management of bleeding ectopic varices.1
We report here experience from a single centre on the use of TIPSS to control bleeding from ectopic varices.
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Ectopic varices can occur at various sites in the abdomen including small bowel, rectum, stomas, falciform ligament, umbilicus, retroperitoneum, biliary tract, vagina and bladder. They are an unusual source of bleeding and account for up to 5% of all variceal bleeding.1 The bleeding from these sources can be difficult to manage because of an initial difficulty in determining the source of bleeding and subsequent difficulty in application of therapy because of inaccessibility of varices in some locations.
Various options have been tried for the treatment of ectopic varices, including medical therapies such as use of vasopressin analogues and octreotide,1 local therapies such as sclerotherapy,3, 10 thrombin injection,11 injection of tissue adhesives (histoacryl),10 ligation2 and application of argon plasma coagulation.12 Other treatments utilized include embolization,13, 14 surgical ligation, resection of a bowel segment with the varix and various local surgical measures for stomal varices.4, 15 Balloon-occluded retrograde transvenous obliteration16 and transileocaecal or percutaneous transhepatic obliteration17 of ectopic varices have also been tried and reported to be successful.
The evidence for efficacy of these procedures is mainly in the form of case reports and systematic evidence for the efficacy of these procedures is lacking. It is generally felt that these procedures have limited success in achieving initial haemostasis, and rebleeding rates following these procedures is high.1, 15, 18, 19 With all these treatments, the underlying portal hypertension remains uncorrected and thus the patients remain at a risk of rebleeding from ectopic varices because of continued formation of new feeding collateral vessels.
To treat the underlying portal hypertension, the options are either surgical creation of a portosystemic shunt5, 6, 20 or creation of TIPSS. Although surgically created portosystemic shunts may have a lower incidence of both rebleeding and need for additional procedures,20 these procedures are associated with high post-operative morbidity and mortality, especially in C–P class B or C patients and if performed in the acute setting. For reasons of the problems associated with portosystemic surgery, TIPSS is now the first line treatment for refractory variceal bleeding. In the literature, there have been several case reports and small series on the use of TIPSS in the management of bleeding ectopic varices at various sites. More recently, larger series of patients with ectopic varices treated with TIPSS have been reported.14, 21–23
In this study, we report the largest series of patients with bleeding ectopic varices treated with TIPSS at a single centre. TIPSS was successfully inserted in all but one patient and haemostasis in 67% (six of nine) patients was effectively achieved. This was achieved solely with TIPSS without concomitant VE in 21 of the 22 (95%) patients and in three of the five (60%) patients who had TIPSS and concomitant VE. Bleeding could not be controlled in three Child’s C category patients despite concomitant VE in two. Rebleeding from ectopic varices occurred in five (21%) patients. This was related to shunt dysfunction in two (40%) patients and responded to shunt-related interventions. In three patients rebleeding occurred despite a functioning shunt with low PPGs. The rate of procedure-related complications was low (3.7%).
Our observations regarding efficacy of TIPSS in achieving initial haemostasis are similar to the observations by Vangeli and Vidal (100%), Haskal (100%) and Shibata et al. (100%).
Rates of rebleeding from ectopic varices (21%) in our series are similar to those reported by Vidal et al. (25%) and Shibata et al. (16.5%), but lower than those reported by Vangeli et al. (37%), although it is not clear from the latter’s paper whether TIPSS effectively achieved initial haemostasis in all the patients. Like Vidal and Shibata et al., we also found that rebleeding can be related to shunt dysfunction and responds to shunt interventions. This was also observed to occur in one of the patients in Vangeli’s series. None of the factors such as components of C–P score, Child’s grade, pre-TIPSS PPG or the type of stent used at TIPSS (covered vs. uncovered) were significantly associated with either risk of recurrence of bleeding from ectopic varices or death in our series. This may, however, be due to the small number of patients.
An interesting observation in our and previous series is that patients with ectopic varices may rebleed after TIPSS despite initial achievement of haemodynamic targets and presence of a patent functioning shunt in situ. In the series by Vangeli et al., this occurred more commonly in those who had TIPSS alone without concomitant VE cf. those who had concomitant VE (48% vs. 28%). Furthermore, they also found that the rebleeding in the majority responded to subsequent VE. On the basis of this, Vangeli et al. recommended TIPSS with concomitant VE for control of ectopic variceal bleeding. However, in the series by Vidal et al.,23 the frequency of rebleeding with a patent TIPSS was found to be low and occurred in only two (8.3%) patients. These patients did not have concomitant VE with TIPSS to achieve initial haemostasis and in both the cases, the rebleeding responded to subsequent VE. In the series by Shibata et al. where again TIPSS alone was used to achieve initial haemostasis, none of the patients had rebleeding with a functional shunt in situ. In our series, we found rebleeding from ectopic varices despite a patent functional shunt occurred in three patients. This responded to thrombin injection in one, but did not respond to thrombin injection in the other patient. In the latter patient, unfortunately, VE was not technically possible because of inaccessibility of the vessel feeding the varices.
Rebleeding from ectopic varices in the presence of a patent functional shunt raises some important issues. First, it is important to recognize that it can occur. It is difficult to say why this occurs. A historical comparison with a previous series from our unit revealed that far fewer patients rebled, despite a patent shunt where the indication for TIPSS was bleeding oesophageal varices (15% cf. 60% for our series).24 This difference in rebleeding rates at the two sites may relate to anatomical differences between ectopic varices and varices at other sites. Variceal bleeding occurs when an expanding force within the varix exceeds the maximum wall tension. The wall tension, calculated by the Laplace equation, is directly proportional to the transmural pressure difference and the radius of the varix but is inversely proportional to the wall thickness of the varix.25 Ectopic varices, unlike oesophageal varices are true veins and are likely to have larger diameters resulting in greater wall tension25, 26 and this may be responsible for higher rates of rebleeding.
The second important issue is regarding the prevention of rebleeding in the presence of a patent TIPSS. To minimize its occurrence, Vangeli et al. have recommended concomitant VE at the time of index TIPSS. Although their data are persuasive, observations from our study and others’ series21, 23 suggest that the overall likelihood of rebleeding with a functional shunt is low (three of 24 patients who had a patent stent rebled in our series). Also, VE at the initial procedure may not be effective in achieving initial haemostasis as two of the three patients in our series where bleeding could not be controlled had concomitant VE at index TIPSS. Technically, it is not always possible to achieve VE as ectopic varices are frequently numerous, tortuous and complex and access to them may be challenging. Even when accessible, complete obliteration of ectopic varices may not be possible because of the presence of other communications with the systemic or mesenteric venous system. These anatomical differences may underlie differences in rebleeding rates seen in the various series, which have or have not used concomitant VE with TIPSS. Concomitant VE, in addition to prolonging the procedure, is invasive, has its own inherent set of complications, such as propagative thrombus or paradoxical systemic embolization.27 We therefore feel that concomitant VE should not be routinely performed at the initial procedure. In the event of rebleeding, if the varices are accessible, local treatments may be tried first. If these measures are ineffective or not possible and if varices are accessible radiologically, VE may be tried.
In the event of uncontrolled bleeding despite TIPSS with or without concomitant VE, options are very limited especially if the patients have advanced liver disease and hence are extremely poor operative candidates. Bleeding could not be controlled in three patients (all Child’s C) and sadly all these three patients developed progressive liver failure and died within 5 days of the initial procedure indicating the dismal prognosis of these patients. Further treatment options in such patients should be explored.
Cumulative primary shunt patency rates are higher in this series compared with previous series.28 This may be related to a greater proportion of patients who had covered stents at time of TIPSS. It is notable that no episodes of shunt insufficiency occurred in patients who had covered stents in situ. The incidence of post TIPSS HE in our series is comparable to the other series in the literature.29
As in previously reported series, the incidence of procedure-related complications in our series was low (3.6%) and these are usually manageable by further radiological interventions.
In conclusion, we feel, TIPSS alone is safe and effective both in achieving initial haemostasis and in preventing rebleeding from ectopic varices with a low risk of complications and an acceptable rate of post TIPSS HE. It should therefore be the first line of treatment in the management of bleeding from ectopic varices. Rebleeding may be related to shunt dysfunction, which responds to shunt interventions, and therefore shunt should be investigated first in the event of rebleeding. It is important to be aware that a significant proportion of patients rebleed, despite functioning shunts. Adjunctive measures such as thrombin injection, or VE should be reserved for patients for such patients.