TIPS for Kids

See “Long-term Outcome of Transjugular Intrahepatic Portosystemic Shunt in Children With Portal Hypertension” by Di Giorgio et al on page 615. The evidence base to support decision making in the clinical care of children with portal hypertension has developed only slowly over many decades. Current pediatric clinical practice relies heavily on extrapolating to children the results of the extensive adult literature. In this way, clinical experience has grown to recognize that routine therapies to treat acute variceal bleeding (such as intravenous octreotide and endoscopic ligation or sclerotherapy) and to treat cirrhotic ascites (sodium restriction, diuresis with spironolactone, and furosemide) are generally effective. There is, however, still uncertainty about the optimal approach to the treatment of children who are refractory to routine treatment. These complicated clinical scenarios are relatively rare in pediatrics, and their scarcity causes difficulty in conducting high-quality research to generate clinical guidance. In adult hepatology, evidence-based indications for transjugular intrahepatic portosystemic shunts (TIPS) are firmly established as standard-of-care in specific situations (1), and a few publications have reported the use of TIPS for children in similar circumstances (Table 1). Many pediatric hepatology centers will consider TIPS as an option for acute management of challenging cases refractory to standard therapy, to minimize ongoing portal hypertensive complications during the wait for the more definitive treatment of liver transplantation. The use of TIPS has also been reported in children with noncirrhotic liver diseases that cause portal hypertension and its complications, but which usually do not need liver transplantation, such as congenital hepatic fibrosis and portal vein thrombosis.TABLE 1: Evidence-based indications for transjugular intrahepatic portosystemic shunts in adults, and corresponding pediatric case reports or case series (1–5)In this edition of JPGN, Di Giorgio et al (2) report their experience with TIPS maintained as long-term treatment in children with portal hypertension. In 29 children with various causes of portal hypertension both before and after liver transplantation, TIPS were placed to control recurrent variceal bleeding (n = 18) or refractory ascites (n = 11). TIPS patency was maintained in 100% during a mean follow-up period of 2.8 years (range 0.1–8.1 years). Although only 8 patients were followed for 4 years or more, the actuarial 4-year “primary shunt patency” was 46% (without revisions or interventions to treat shunt occlusion or stenosis). Eight patients each required 1 intervention to treat shunt dysfunction. Perhaps reflecting the small and clinically heterogeneous cohort, predictors of shunt dysfunction, or primary patency were not identified. Previous reports of long-term follow-up of TIPS in small cohorts of children are scarce, and have demonstrated a range of reintervention rates (45%–68%) and a similarly high rate of overall patency (71%–100%) after mean follow-up periods of 22 months to 5 years (3–5). The need for reintervention is much less frequent in more recent reports compared to the early pediatric experience from 20 years ago. Have we now reached a tipping point, such that all pediatric centers should embrace the use of TIPS to address appropriately problematic portal hypertensive challenges? Are Di Giorgio et al correct in suggesting that TIPS should be “considered in every patient with portal vein cavernoma having an unfavorable anatomy for mesoportal bypass” and that it “should not be regarded only as a bridge to transplantation, but also as an effective and less invasive alternative to surgical vascular shunts?” To support this argument, they compare the outcomes in their patients receiving TIPS with those of a historical, unmatched cohort of 31 children with portal vein thrombosis who underwent surgical portosystemic shunts or mesoportal bypass procedures at the same institution over a period of 30 years. The shunt or bypass patency rate at last follow-up for these 31 children was 82% after a median follow-up of approximately 2.5 years, compared to 100% in the TIPS group after 2.8 years mean follow-up in their current study. Such comparisons between studies are problematic. The mix of patients’ primary diagnoses in each group is clearly different, we have inadequate knowledge of the intensity of monitoring and indications for intervention to maintain patency of the surgical shunts and bypasses compared to the TIPS, and we are unable to control for the possible differential impact between the 2 groups of the improving outcomes in recent years. Many other questions also remain unanswered, including the generalizability of these single-center results, and the longer-term outcomes beyond the first few years, when children's growth may result in the TIPS becoming too small or too short to maintain effective function. Di Giorgio et al are to be congratulated on their excellent technical success rate with TIPS insertion, and the positive clinical outcomes experienced by their patients. Their results are an important addition to our understanding, and provide encouragement to clinicians who may consider TIPS to rescue a patient from treatment-refractory complications of portal hypertension but fear a poor longer-term sustainability of the clinical benefit. Referral of such patients to expert centers for consideration of TIPS can be associated with excellent outcomes, where the local expertise in TIPS insertion and vascular surgical interventions, and the local availability and success rates with liver transplantation, can be weighed in the risk/benefit balance when choosing the best intervention for each individual case. For the future, researchers must develop effective collaborations to answer these important clinical questions using multicenter research studies to identify adequate patient numbers. The feasibility, efficacy, and safety of TIPS will most likely vary according to the patients’ primary diagnoses and other clinical circumstances, and larger numbers of patients will be needed to tease out these confounding factors. Eight years ago, an editorial in this Journal accompanied a previous publication about TIPS by Di Giorgio et al, and concluded with similar comments (6). Let us hope that in another 8 years from now, more powerful evidence will be available to guide our clinical decisions for these children.

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