The survival rate of patients with biliary atresia has been increasing since the introduction of Kasai (hepatic) portoenterostomy (1); however, because it is an auxiliary treatment unable to solve the progressive cholangiopathy, mortality and rates of complications, such as variceal bleeding, remain high. Miscellaneous modalities including endoscopy, intervention, and surgery have been introduced to treat variceal bleeding. Balloon-occluded retrograde transvenous obliteration (B-RTO) is now widely performed to treat gastric varices, and several reports have described the effectiveness of B-RTO in adults (2). Moreover, the hemodynamic alternations after B-RTO may improve hepatic function by resolving the problem of hepatic insufficiency (3). Children with biliary atresia have less severe hepatic dysfunction than adults with end-stage liver disease and are thus likely to show a better result, after B-RTO. However, there is no case report on the correlation between hepatic function and B-RTO in children. We report a case of successfully performed B-RTO in a 9-year-old girl with gastric variceal bleeding after a Kasai operation and its positive influence on hepatic function.
A 9-year-old girl was admitted via the emergency department with the first episode of profuse hematemesis and tarry stools. She was born at 40 weeks' gestation weighing 3150 g. The Kasai operation was performed at 60 days of life after diagnosis of biliary atresia (BA). She was treated 4 times with antibiotics for ascending cholangitis after the operation. At age 7 years, during regular follow-up, liver cirrhosis was observed by abdominal ultrasonography, and hepatic biochemistry increased.
Blood pressure on admission was 100/60 mmHg. Physical examination revealed icteric sclera and an enlarged spleen palpable 5 fingerbreadths below the left costal margin. Hemoglobin was 8.9 g/dL, white blood cell count 4150/mm3, and platelet 199,000/mm3. Bilirubin (total/direct) increased to 4.0/2.7 mg/dL, blood urea nitrogen/creatinine to 23/0.5 mg/dL, aspartate aminotransferase/alanine aminotransferase to 175/196 IU/L, and γ-glutamyl transpeptidase to 157 IU/L.
Isolated gastric varices without esophageal varices were observed on emergency gastroenteroscopy (Fig. 1). Strained red spots were seen on the gastric fundic mucosa. Computed tomography (CT) of the abdomen with contrast enhancement showed gastric varices, which were drained through the gastrorenal and gastrocaval shunts. We considered balloon-occluded retrograde transvenous obliteration (B-RTO) because the hemodynamic feature of the varices by CT indicated its appropriateness.
An informed consent was obtained before the procedure. A 5-F occlusion balloon catheter with an 8.5-mm diameter (Boston Scientific, Watertown, MA) was inserted into the gastrorenal shunt via the right femoral vein. Left adrenal venography with an inflated balloon showed an inferior phrenic vein and the gastrocaval shunt, but gastric varices were not opacified. After coil embolization of an inferior phrenic vein, gastric varices and posterior gastric vein were well opacified (Fig. 2). Then, a total of 12 mL of sclerosing agent was slowly injected until the gastric varices were completely filled with the sclerosing agent. The sclerosing agent used was a mixture of 5% ethanolamine oleate (Keukdong Pharm Co, Kangnam, Seoul, Korea) and iodized oil (Lipiodol; Guerbet Ltd, Roissy, Paris, France), mixed in a ratio of 5:1. The balloon remained inflated for 3 hours after injection of the sclerosing agent, and then as much sclerosing agent as possible was aspirated. Finally, the balloon was deflated and the catheter withdrawn. There was no intraoperative or immediate postoperative complication. After B-RTO, the reduction of tense gastric varices was confirmed by follow-up gastroendoscopy.
One month later, CT scan showed complete occlusion of the gastric varices with compact lipiodol uptake, and her hemoglobin increased to 13.1 g/dL, aspartate aminotransferase/alanine aminotransferase was 83/67 IU/L, and bilirubin (total/direct) 2.2/1.1 mg/dL. Endoscopic findings obtained 4 months later showed the complete eradication of the gastric varices without the appearance of esophageal varices. Her hepatic biochemistry has continued to improve during 7 months of follow-up (Table 1), the spleen decreased to 2 fingerbreadths, and peripheral blood bicytopenia improved.
Esophageal varices are prevented and treated with pharmacotherapy, endoscopic variceal band ligation or sclerotherapy, transjugular intrahepatic portosystemic shunts (TIPS), and surgery. However, these methods are not definitive for the treatment of gastric variceal bleeding.
Gastric varices are associated with less frequent bleeding but greater severity and a higher mortality due to the excess of blood flow than esophageal varices (4). Furthermore, endoscopic treatment is difficult because intravariceal blood flow is too fast to inject sufficient sclerosant through endoscopy (5). Endoscopic butyl cyanoacrylate (Histoacryl) sclerotherapy, recognized as the most effective way, is commonly applied in adults, but is rarely conducted and reported in children. Even in adults, its application needs some caution owing to the possibility of complications such as recurrent bleeding or systemic embolization (6).
If Histoacryl sclerotherapy cannot be performed, then TIPS may be an alternative. Periportal fibrosis is a crucial characteristic of BA and is likely to make TIPS more difficult, especially in small children. In addition, TIPS has several drawbacks in children. One is that it is technical challenging in children, and additional concerns are the longitudinal growth of liver parenchyma and portal vein over time and frequent rebleeding due to shunt occlusion (7). Moreover, the response rate of gastric varices to TIPS is lower than that of esophageal varices.
B-RTO has been recently performed for adults having gastric fundal varices with gastrorenal shunt in Japan (2). Furthermore, it has been reported to have a lower rebleeding rate and a higher survival rate than TIPS (8). However, accurate assessment of the hemodynamic alteration after B-RTO is necessary because it can elevate portal pressure in response to occlusion of the portosystemic shunt, unlike TIPS. There are 2 pediatric B-RTO cases after the Kasai procedure reported in the English-language literature. One was performed at the youngest age of 2 years (9), and the other was treated with B-RTO and dual balloon-occluded embolotherapy owing to the large size of the varices (10). These were conducted for prophylactic treatment. However, the correlation between B-RTO and hepatic function was not described in these cases. When it comes to adult B-RTO cases, it has been reported that in patients with cirrhotic portal hypertension showing hepatocellular insufficiency, the change of blood channel after B-RTO has a positive effect on hepatic function by increasing intrahepatic blood flow (3). In spite of the rise of blood flow, blood pressure is restored to the previous state and hepatic resistance is reduced. Of course, after endoscopic variceal band ligation or sclerosing therapy, portal flow may increase to some degree, but not enough to have a positive effect on liver because neither is likely to increase portal pressure as much for the improvement of intrahepatic flow as B-RTO (3,5).
Hepatic fibrosis in BA predominantly surrounds portal veins causing presinusoidal portal obstruction, mostly accompanied by nearly normal liver function. This positive alteration after B-RTO can be more apparently observed in pediatric patients with BA with minimal hepatic dysfunction. During 7-month follow-up of hepatic function, our case showed a rapid improvement in hepatic biochemistry and retained its normal range. This result supports the hypothesis that B-RTO is supposed to improve hepatic function in children as well as in adults, although the final conclusion can be made on the basis of inquiry into many cases similar to ours.
Our patient presented with isolated gastric varices after the Kasai operation and thus could be well adjusted to B-RTO. Nevertheless, complications such as the expansion of esophageal varices needed to be followed up. Fortunately, this patient showed rapid restoration of liver function without expansion of the esophageal varices.
We recommend the use of B-RTO in consideration of hemodynamic parameters in patients with BA who developed isolated gastric varices with gastrorenal shunt after the Kasai operation. Moreover, the improvement of hepatic function and peripheral blood bicytopenia with decreased size of splenomegaly after B-RTO can contribute to making a native liver endure longer through the delay of the cirrhotic process in the liver.
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