Case Report
Bleeding Stoma1 Varices: Treatment with a Transjugular Intrahepatic Portosystemic Shunt in Two Pediatric Patients1 Gregg D. Weinberg, MD Terence A. 5. Matalon, MD Michael C. Brunner, MD Suresh K. Patel, MD Richard Sandler. MD
Index terms: Inte~entionalprocedures, in infants and children, 957.453 Shunts, portosystemic, 957.453 Varices, 74.75
JVIR 1995; 6:233-236
PmENTs with enterostomies and coexistent portal hypertension occasionally develop varices a t the mucocutaneous junction of their stoma. These portosystemic collateral vessels are prone to recurrent episodes of bleeding despite local interventions including manual compression, sclerotherapy, percutaneous embolization, and stoma1 revision (1-6). Transjugular intrahepatic portosystemic shunt (TIPS) placement is currently under investigation for control of variceal bleeding a t the gastroesophageal junction and has recently been reported to be promising in treating intraabdominal intestinal varices (7,8). We report two pediatric cases in which bleeding stomal varices were successfully managed with TIPS placement.
CASE REPORTS
From the Departments of Diagnostic Radiology and Nuclear Medicine (G.D.W., T.A.S.M.,M.C.B.,S.K.P.)andthesection of Pediatric Gastroenterology, Department of Pediatrics (R.S.),Rush-Presbyterian-St. Luke's Medical Center, 1653 W Congress Pkwy,Chicago, IL 60610. Received July 15,1994; revision requested September 15; revision received October 21; accepted October 24. Address reprint requests to T.A.S.M. O SCVIR, 1995
Case 1.-The patient is a Cyearold, 18-kg boy who was born prematurely a t 28 weeks gestational age. His neonatal hospital course was complicated by severe necrotizing enterocolitis that resulted in an extensive small bowel resection leading to short bowel syndrome. He subsequently underwent several operations, which ultimately left him with a jejunostomy and a mucous fistula. Long-term hyperalimentation was complicated by cholestatic liver disease, cirrhosis, and portal hypertension. He presented to us with bleeding varices at his jejunostomy stoma that recurred despite local sclerotherapy and that had required multiple blood transfusions. General anesthetic was administered, and a Rosch-Uchida transjugular liver access set with a 0.038-inch stylet with accompanying 5-F Tefloncoated sheath (Cook, Bloomington, Ind) was used. A TIPS was created via the right internal jugular vein with
use of an 8 x 40-mm Wallstent (Schneider USA, Minneapolis, Minn). Standard TIPS technique was used. Portal venography performed before TIPS demonstrated hepatofugal flow to a single, large collateral vessel leading to the jejunostomy stoma. This large collateral vessel filled numerous peristomal varices as well as chest and abdominal wall collaterals. Also noted were several small esophageal varices filling via the coronary vein (Fig la). Mean portal venous and right atrial pressures were 31 and 14 mm Hg, respectively. The portosystemic gradient was 17 mm Hg. After TIPS, pressures were 26 and 20 mm Hg in the portal vein and right atrium, respectively, with a portosystemic gradient of 6 mm Hg. A portal venogram obtained after TIPS demonstrated hepatopetal flow through the portal vein and a patent shunt. Collateral vessels around the stoma were much less prominent, and the esophageal varices did not opacify. During the next 2 days, the patient bled intermittently from his stoma. Repeated portal venography via the right internal jugular vein demonstrated hepatopetal flow through a patent TIPS, although there was probably incomplete hepatic venous coverage of the parenchymal tract by the stent, which was not previously recognized. The stomal varices were barely evident, and the esophageal collaterals did not opacify. The portosystemic gradient across the stent now measured 10 mm Hg. An 8 x 20-mm Wallstent was placed at the cephalic end of the shunt, overlapping the original stent, which decreased the gradient across the stent to 4 mm Hg. The final angiographic appearance did not change after placement of the second stent (Fig lb). The patient experienced no further bleeding from his stoma. He did not develop signs of hepatic en-
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cephalopathy. Approximately 1month after the TIPS placement, the patient received a liver/small bowel transplant. The transplant surgeon encountered no technical difficulty due to the stent. Case 2.-The patient is a 2.5-yearold, 13-kg boy who underwent repair of gastroschisis immediately after birth. The procedure left him with a n ileostomy. Long-term hyperalimentation was complicated by cholestatic liver disease, cirrhosis, and portal hypertension. He presented with recurrent bleeding from stomal varices despite previous surgical ligation of the varices. As in the first case, these bleeding episodes had required multiple blood transfusions. General anesthetic was administered, and a Ring neonatal transjugular intrahepatic access set with a 18gauge transhepatic needle (Cook) was used. TIPS was performed via the left internal jugular vein because the right internal jugular vein was thrombosed. As in the first case, standard TIPS technique was utilized. A 8 x 40-mm Wallstent was used to create the shunt. Portal venous and right atrial pressures before TIPS were 14 and 2 mm Hg, respectively. The initial portosystemic gradient measured 12 mm Hg. Portal venography demonstrated hepatofugal flow filling a large collateral vessel leading to the stoma and filling multiple peristomal varices as well as chest and abdominal wall collateral vessels (Fig 2a, 2b). Portal venography performed after the procedure demonstrated hepatopetal flow through a patent TIPS with no filling of the stomal collaterals (Fig 2c). After TIPS, portal venous and right atrial pressures were 9 and 4 mm Hg, respectively. The portosystemic gradient was reduced to 5 mm Hg. Ten months after TIPS, the patient has had no recurrent bleeding from the stoma, nor has he developed hepatic encephalopathy. He is currently waiting for a suitable donor for transplantation.
DISCUSSION Bleeding from portosystemic collateral vessels is a common complication
Figure 1. (a)Portal venogram obtained before TIPS demonstrates hepatofugal flow into a large varix (curved arrow) draining the jejunostomy stoma. There are many peristomal and anterior body wall portosystemic collaterals. Also note filling of small esophageal collaterals via the coronary vein (arrowhead). (b) Portal venogram obtained after placement of the second stent demonstrates hepatopetal flow through patent TIPS and no filling of collateral vessels.
of portal hypertension. These varices occur most commonly a t the gastroesophageal junction but have been reported throughout the entire gastrointestinal tract. In 1968, Resnick and coworkers initially described bleeding varices a t the ileostomy stoma in several patients treated with colectomy for hepatic encephalopathy (9). Since then, several authors have reported bleeding from ileal and colonic stomal varices in cirrhotic patients with coexistent inflammatory bowel disease, colorectal cancer, colonic obstruction, and ileal conduits for urinary diversion (1-6,101. These varices form a t the mucocutaneous junction of the stoma and are rarely associated with esophageal varices. Eade and coworkers suggested that these varices may act as a safety valve mechanism in delaying the development of esophageal varices (2). Mortality from bleeding esophageal varices may reach 40%; however, death from stomal hemorrhage is rare and approximated a t 3 % 4 % (3). Local therapeutic measures have included manual compression, direct variceal ligation, sclerotherapy, and stomal revision. Still, bleeding episodes are usually recurrent and often require
multiple transfusions (1-6). Additionally, these local measures could theoretically increase portal pressure by blocking routes for portal outflow. Cameron and Fone described a patient who developed bleeding esophageal varices several days after suture ligation of stomal varices (4). Adson and Fulton reported on two patients who developed esophagogastric varices subsequent to multiple local interventions (6). In 1989, Samaraweera and coworkers described four patients who underwent percutaneous transhepatic embolization of bleeding stomal varices (11).They successfully controlled the acute bleeding in all four of their patients. They concluded that transhepatic embolization may be a n effective temporary measure but is unlikely to be curative. Additionally, as with other local measures, the embolization could theoretically raise portal and stomal venous pressure and lead to esophageal varices. Haskal observed that excessive embolization could lead to stagnant portal flow and portal vein thrombosis, which has been reported in 5%-13% of patients undergoing embolization for esophageal varices (8). Several authors have reported good
Weinberg et a1
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Figure 2. (a) Early and (b) late images from portal venography performed before TIPS demonstrate hepatofugal flow into a large varix (sold arrow) leading to the stoma (open arrows in b) and numerous portosystemic collateral vessels. (c) Portal venogram obtained after TIPS demonstrates hepatopetal flow and absence of collateral flow.
results with operative portosystemic shunts for patients with bleeding stomal varices (1,4,6,10). However, operative mortality ranges from 4% to 83% depending on the urgency of the procedure and the extent of liver disease (12,13). This greatly exceeds the 3%-4% mortality of the bleeding stomal varices (3). Additionally, operative shunts may make eventual liver transplantation more technically difficult (14,15). Finally operative shunting has not been clearly shown to improve survival in this group of patients (15). For these reasons, operative portosystemic shunts may not be an attractive therapeutic alternative. TIPS placement is now well recognized as a n effective therapeutic alternative for bleeding gastroesophageal varices (7). Haskal and coworkers recently reported promising results with use of TIPS to primarily treat intestinal and colonic varices with coexistent portal hypertension (8). TIPS were placed in nine patients, six of whom were actively bleeding. Portosystemic pressure gradients were reduced from a mean of 26.8 to 8.8 mm Hg. Four of nine patients were alive a t 9-21 months after TIPS placement with no recurrent bleeding, with the exception of one patient who experienced recurrent bleeding 3 days after TIPS placement and underwent successful embo-
lization. Subsequent rebleeding did not occur. Two patients died within 5 days of TIPS. Our two, small pediatric patients underwent TIPS with no significant morbidity. Neither patient developed hepatic encephalopathy, which has been shown to develop in 5%-35% of all patients undergoing TIPS (16). The incidence of morbidity and mortality in pediatric patients undergoing TIPS is not yet known and will require further study. Our cases raise two interesting points. First, the initial patient rebled after his portosystemic gradient was lowered to 10 mm Hg. Adequate control of bleeding was only obtained after the gradient was lowered to 4 mm Hg via placement of the second stent. Variceal control in the second patient was obtained by decreasing the portosystemic gradient from 12 to 5 mm Hg. Perhaps pediatric patients need a portosystemic gradient below 5 mm Hg for control of variceal bleeding. This needs further scientific exploration. Second, the first case demonstrated the need for complete coverage of the hepatic parenchymal tract by the TIPS stent. Incomplete coverage may lead to deposition of blood clots and raise the portosystemic gradient. We make three conclusions on the basis of these two patients. First,
TIPS placement may be an effective, temporary, primary treatment for bleeding stomal varices. Compared with operative portosystemic shunts, morbidity and mortality with TIPS placement are considerably reduced. In addition to controlling the bleeding, TIPS placement may also prevent development of esophageal varices by decompressing portal venous pressures. Second, as demonstrated by our first patient, TIPS placement may be an effective bridge to liver transplantation in this group of patients. Because the shunt is intrahepatic, there is usually no technical difficulty encountered a t transplantation as may be encountered in patients who have had operative shunts. LaBerge et a1 described 22 patients who underwent liver transplantation subsequent to TIPS. Transplantation was not technically impeded by the TIPS (7). Because the portal system is decompressed, the transplantation may be facilitated, although this is a controversial topic and needs scientific confirmation. Finally, TIPS placement is technically feasible in very small children. References 1. Grundfest-Broniatowski S, Fazia B. Conservative treatment of bleeding stomal varices. Arch Surg 1983; 118:981-985.
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