Transjugular Intrahepatic Portosystemic Shunt Placement in a Child Complicated by Perforated Roux-en-Y Portoenterostomy (1997)

Portal hypertension is one of the major complications of hepatic failure. Since the transjugular intrahepatic portosystemic shunt (TIPS) was pioneered for clinical use in 1982 by Colapinto et al. (1), it has been increasingly employed in advanced liver disease to relieve recurrent variceal bleeding or intractable ascites. TIPS has proved to be effective in reducing the portosystemic pressure gradient and thus in reducing incidence of variceal bleeding (2,3). However, complication rates can be high, depending on the technique used and degree of experience in the use of the TIPS at different institutions. A recent comprehensive review found that the TIPS carries a total complication rate ranging from 4% to 77% in adults (4). The TIPS has been predominantly used in adults, and review of the literature reveals few reports of successful placement in children. We report placement of a TIPS in a very young child with a unique life-threatening complication. CASE REPORT Case 1 The patient is a 21-month-old girl who was diagnosed with biliary atresia and malrotation soon after birth. At seven weeks of age, she underwent the Kasai portoenterostomy along with the Ladd's procedure in the Philippines. Subsequently, she had several episodes of ascending cholangitis and no significant improvement in bile flow. She moved to Hawaii at 20 months of age for medical treatment, where she continued to have significant ascites and three documented episodes of gastrointestinal bleeding. Her hemoglobin dropped to 5 g/dl, requiring multiple transfusions; her international normalized ratio was 1.6; and her ammonia levels reached a high of 131 μmol/l, although she was without clinical signs of encephalopathy. Her upper gastrointestinal bleeding was refractory to medications and repeated endoscopic sclerotherapy. She was subsequently transferred to our institution 1 month later for further management of her esophageal variceal bleeds and end-stage liver disease. Physical examination on admission revealed a thin, somnolent 21-month-old girl in no acute distress. Her temperature was 37.7°C, heart rate 131 bpm, blood pressure 92/57 mmHg, and respiratory rate 52/min. Her height was 81 cm (50th percentile), abdominal girth 60 cm, and weight 11.9 kg (25th-50th percentile). She had icteric sclera and jaundice. Her heartbeat was tachycardic with a regular rhythm, and she had a grade II-VI crescendo-decrescendo murmur, which was loudest in the left lower sternal border. Her lungs were clear to auscultation bilaterally. Her abdomen was markedly distended with ascites with a palpable liver 7 cm below the right costal margin in the midclavicular line, extending to 3 cm below the left costal margin in the midclavicular line. The spleen tip was not palpable. Examination of the extremities revealed clubbing without cyanosis or edema. Results of a neurologic examination were appropriate for the patient's age, without asterixis. Significant laboratory findings on admission include: white blood cell count 22,000/μl, hematocrit 32.2%, platelets 314,000/μl, total bilirubin 6.6 mg/dl, aspartate aminotransferase 144 IU/l, alanine aminotrasferase 112 IU/l, alkaline phosphatase 610 IU/l, γ-glutamyltransferase 392 IU/l, total protein 5.7 g/dl, albumin 2.2 g/dl, prothrombin time 13.9 seconds, partial thromboplastin time 24.1 seconds, total cholesterol 164 mg/dl, and triglycerides 78 mg/dl. On arrival, the patient was listed with the United Network for Organ Sharing after approval as a liver transplant candidate. While awaiting an available donor organ, she continued to bleed with guaiac-positive burgundy stools. Abdominal ultrasound revealed massive intrahepatic biliary ductal dilatation, inferior vena cava with good flow, and patent portal vein with hepatopetal flow. Size of portal veins was not mentioned. Endoscopy showed multiple, enlarged varices sized 3 to 4+, and a decision was made to place a TIPS as a bridge to the transplant to avoid recurrent gastrointestinal bleeding. On the fourth hospital day, a 10-mm × 68-mm Schneider Wallstent Schneider, Minneapolis, MN, U.S.A. was placed by interventional radiology. Under fluoroscopic guidance, the right internal jugular vein was accessed, the right hepatic vein was catheterized, and the wedged hepatic CO2 venogram performed. Difficulty was encountered accessing the portal vein because of extensive fibrosis in the region of the portahepatis, presumably related to the Kasai procedure. Several attempts were made using the Rosch-Uchida and the Colapinto needle systems to push through the woody liver parenchyma before the right portal vein was accessed. The preprocedure portal vein pressure, which correlates with the hepatic wedge pressure, was 32 mmHg. A venogram performed before placement of the stent showed the portal venous anatomy (Fig. 1). The stent was then expanded to 8 mm across the parenchymal tract, bridging the right hepatic vein to the right portal vein (Figs. 2,3). A final portal venogram was performed, and atrial (6 mmHg) and portal (10 mmHg) pressures were obtained by placing a straight flush catheter within the splenic vein. The patient tolerated the procedure fairly well except for a 5-minute hypotensive episode during the procedure, which was presumably caused by variceal bleeding. This resolved with volume support and TIPS deployment. No other immediate complications were noted. Results of a portogram performed before deployment of the stent showed extravasation of contrast, presumably from coronary vein varices. Results of a portogram performed after the TIPS was deployed showed decompression of the coronary vein varix with adequate flow through the TIPS. Within 36 hours after the TIPS was placed, the patient developed a fever of 39.2°C, increasing abdominal girth, a white count of 26,400/μl, and a decrease in hematocrit from 26.2% to 22.1%. There were no further episodes of variceal bleeding, documented by nasogastric aspirates, but there was another source of bleeding evidenced by the patient's decreasing hematocrit. Findings in Doppler ultrasonography showed a large quantity of ascites and a patent TIPS shunt with good cephalad flow (Figs. 4,5). Results of abdominal x-rays taken in the upright and lateral-decubitus positions showed no free air but evidence of a distended small and large bowel consistent with an ileus. The patient had been receiving ceftriaxone for Staphylococcus aureus infection in the line since before placement of the TIPS. Her antibiotic coverage was changed at this time to ampicillin, gentamicin, and clindamycin. Blood cultures from the 2 days after placement of the TIPS became positive for Candida tropicalis, and she was given amphotericin B and flucytosine. On the patient's eighth hospital day, she was stable on antifungal drugs and successfully underwent an orthotopic liver transplant. The source of her fungemia was the end of the stent protruding through the anterior wall of the right portal vein into the lumen of her Roux-en-Y portoenterostomy. The stent was clotted and purulent material was found at the end, along with a moderate amount of bloody ascites. The patient's suprahepatic inferior vena cava was exposed without difficulty, but a congenital absence of the intrahepatic inferior vena cava was noticed. The larger-than-average common hepatic artery was aberrant in origin, arising directly from the aorta, superior to the usual position of the celiac trunk. Additional intraoperative findings include a preduodenal portal vein, bowel malrotation, and polysplenia. The patient received a liver from a 7-year-old donor, which was placed in the piggyback position. Her Roux-en-y was revised, and a biliary catheter was placed, from which golden brown bile flowed immediately after anastomosis. She began treatment with methylprednisolone, FK-506, dextran, heparin, and aspirin. Her liver enzymes peaked on postoperative day 2, then dropped progressively. She remained afebrile and received amphotericin B for 10 days. On postoperative day 3, her blood cultures were positive for Enterobacter, which was treated with ciprofloxacin and gentamicin. On postoperative day 7, her white cell count returned to normal and liver function test values continued to improve. She remained afebrile and was tolerating a clear liquid diet. Fifteen days after transplant, she returned to the operating room for lysis of adhesions that had caused a small bowel obstruction. Twenty-four days after transplant, she was tolerating a regular diet and was having regular bowel movements. She was discharged home in stable condition. The patient continues to do well with good graft function 6 months after her liver transplantation. DISCUSSION We have presented a unique case of the use of a TIPS in a young child, complicated by erosion of the stent through her portal vein into her Roux-en-Y portoenterostomy. There has been little experience with the TIPS in children, and there is no prior description of stent erosion through vessels or perforation of the Roux-en-Y by the TIPS. Most common complications with TIPS described in adults are stenosis and thrombosis, encephalopathy, and recurrent variceal bleeding. Of the 4% to 77% total complication rate described by Freedman et al., immediate complications were less than 10%, whereas late shunt occlusion occurred in 15% to 77%. Procedural mortality was found to be 0% to 2%, with death usually caused by intraoperative myocardial infarction or intraperitoneal hemorrhage. The thirty-day mortality rate ranged between 7% and 45%, with death usually caused by complications related to the patient's end-stage liver disease. Shunt occlusion was often the main cause of rebleeding, requiring dilatation of the shunt or placement of additional shunts (4). A popular theory for the etiology of occlusion is pseudointimal hyperplasia in the shunt (5,6). Hepatic encephalopathy is a potential complication, resulting from diversion of a significant amount of blood from hepatic metabolism. Incidence for hepatic encephalopathy varies from 5% to 35%. Fortunately, most respond well to medical therapy, only occasionally requiring treatment of an occluded shunt (4). Less common complications are thrombosis in the portal or jugular vein (0-9%) and acute thrombosis in the stent (0-10%). Shortening or incomplete placement of the shunt is reported in up to 10% of cases, and stent misplacement or migration occurs in 0% to 8%. There have been documented migrations of stents into the suprahepatic inferior vena cava, too far into the main portal vein, into the pulmonary artery, and into the right atrium (7,8). Poor positioning of the stent may cause problems with cross-clamping during liver transplantation, because clamping is done at the portion of the suprahepatic inferior vena cava below the diaphragm and across the portal vein (7). Transcapsular needle puncture occurs 5% to 30% of the time, mostly without any serious consequences. The puncturing of the liver capsule, the hepatic artery (0-1.5%), and kidney (0-1.5%) can cause hemoperitoneum. A potentially fatal complication is massive intraperitoneal hemorrhage from rupture of the portal vein, resulting from extrahepatic puncture and balloon dilation of the portal vein (0-1.3%). Occasionally the biliary system is punctured (0-10%) causing hemobilia. Additional reported complications in the use of a TIPS include acute renal failure caused by contrast (0-4%), infection-sepsis (0-10%), coagulopathy (0-4%), hemolysis (0-1.5%), progressive liver failure, and acute hepatic decompensation (4). Several factors predisposed the patient in this case to the potentially fatal complication, including her small size, history of Kasai procedure, and abnormalities in the perihepatic vessels. The stress placed on her small hypoplastic portal vessels by dilatation with a 10-mm stent was probably the major contributing factor to perforation of the vein. Is there an age below which the anatomy of the patient is too small for placement of a TIPS? The small diameter of vessels in children is an obstacle for insertion of the stent and increases the risk of shunt stenosis and thromboses. The size of stent used in the above case was that commonly used in adult patients. The Wallstent used may have been too wide and too long for this patient's portal vein, making it susceptible to erosion through her vein into the adjacent Roux-en-Y portoenterostomy. In addition, there has not been enough experience to know whether children as young as she have a more delicate venous anatomy. Berger et al. (9) used a miniaturized puncture set, with the maximum diameter of the introducer sheath at 7.5 French, for introducing the smallest Palmaz stents. Theoretically, shorter stents appropriate for the smaller child would work the same; however, there may be a minimum width for the stent to avoid unacceptably high rates of stenosis and thrombosis. The few pediatric cases involving placement of a TIPS in the literature are mostly in older children. Five cases were in children ages 10 to 13 (9,10). Two younger patients in the literature who successfully underwent placement of a TIPS were an 18-kg 4-year-old boy and a 13-kg 2.5-year-old boy. They both had bleeding stomal varices from cirrhosis secondary to cholestatic liver disease from hyperalimentation. The 4-year-old had an 8-mm × 40-mm Wallstent overlapping with an 8-mm × 20-mm Wallstent for additional length. He had no complications related to the TIPS for the 1 month he awaited liver transplantation. The 2.5-year-old had an 8-mm × 40-mm Wallstent, also with no complications for the 10 months of follow-up (11). We recently reported successful insertion of a TIPS using a smaller (5 mm × 40 mm) Wallstent in a 9-kg 10-month-old infant, who died 36 hours later because of sepsis that was present before the placement procedure was performed (12). These cases provide encouragement that placing a TIPS is technically possible in small children, using the smallest of available stents. Many children who may need a TIPS will have biliary atresia, which can greatly complicate the placement procedure. The major cause of hepatic failure in children is biliary atresia, which is a disease associated with progressive liver failure and worsening portal hypertension. Findings in a prospective sonographic study indicate that the caliber of portal vein may progressively decrease in patients with biliary atresia and poor hepatobiliary function (13). Roux-en-Y portoenterostomy is the preferred method of treatment for children with biliary atresia, as in the patient in the case reported. This procedure brings a loop of the small intestines flush against the liver-a procedure that, theoretically, would increase the likelihood of bowel perforation if the liver capsule is punctured by the needle or by the stent. The extensive fibrosis of the surrounding liver caused by the procedure can also make cannulation of the portal vein during placement of a TIPS extremely difficult. This introduces the question of whether a history of portoenterostomy should be a contraindication for use of a TIPS. Very important is a special awareness that a child with biliary atresia may have Ivemark's syndrome (polysplenia). Results of studies show that more than 10% of children with biliary atresia have polysplenia, along with the associated abnormalities in perihepatic vessels. Our patient had all of these perihepatic abnormalities, including azygous continuation of the inferior vena cava and preduodenal portal vein and anomalous origin of the hepatic artery. They also usually have bowel malrotation, bilaterally bilobed lungs, and congenital heart disease (14,15). Most relevant to the placement of a TIPS is the hypoplastic preduodenal portal vein, which can lead to a greater chance of missed cannulation or perforation. These perihepatic vascular abnormalities have caused complications in liver transplantations in the past (16,17). When a child with end-stage liver disease must receive an intervening procedure (e.g., TIPS) as a bridge to transplant, what can be done to decrease the likelihood of complications? We offer the following recommendations: fully assess the anatomy of the child, especially possible perihepatic vascular anomalies; optimize the fit between size of the child's venous anatomy and the size of stent used; consider the use of additional imaging studies, which may be helpful in the prevention or rapid diagnosis of stent malposition. Real-time sonography in addition to fluoroscopy may be helpful during placement of a TIPS, and sonography in the early postprocedural period can aid in early diagnosis of complications. In addition, should a complication similar to that in the reported case occur, warning signs may resemble those described in the case report: namely, high white cell count, fever, fungemia, and a drop in hematocrit. Other potential signs that were not evident in our patient are peritonitis, free air in the abdomen detected by abdominal x-ray, bacteremia, and abnormal findings in abdominal ultrasonography. With growing experience and awareness of potential complications, we hope to better understand the indications and limitations for use of the TIPS and to improve on the efficacy and safety of the TIPS for the management of portal hypertension in young children.FIG. 1.: Wedged right hepatic CO2 venogram demonstrates portal venous anatomy before placement of a transjugular intrahepatic portosystemic shunt.FIG. 2.: Angioplasty of parenchymal tract between the hepatic and portal veins during creation of a transjugular intrahepatic portosystemic shunt shows extrinsic waist-like impression on the balloon during dilation of fibrotic tissue.FIG. 3.: Appearance of the 10-mm diameter × 68-mm long Wall-stent between the right hepatic vein and the portal vein. The stent extends well into the extrahepatic portal vein.FIG. 4.: Longitudinal ultrasonographic image after a procedure to insert a transjugular intrahepatic portosystemic shunt shows the echogenic stent (arrowheads) extending below liver margins (arrows) into the extrahepatic portal vein.FIG. 5.: Duplex ultrasonography obtained 1 day after placement of a transjugular intrahepatic portosystemic shunt demonstrates significant unidirectional blood flow within the shunt in its midsegment.