Abstract

Background: There are very limited high-quality data from which to derive therapeutic approaches to portal hypertension (PHT) in children. Management of varices, in particular, is quite controversial in pediatrics. IMPPHR was developed to derive large-scale international data, thereby enhancing our knowledge of PHT. The three major foci of data collection in IMPPHR are, 1) morbidity and mortality of first variceal hemorrhage, 2) feasibility and safety of primary prophylaxis of varices, 3) approaches to secondary prophylaxis of variceal hemorrhage. Subject level data collection is on-going in IMPPHR (n= 241 cases as of 4.27.23) and will be reported in the future. This report provides center-specific data relevant to the management of varices.

Methods: Each site submitted institutional resources and clinical activity accrued over 2 years between January 1, 2018 and December 31, 2019 to present a snapshot of resources and approaches available in clinical practice.

Results: 23 centers (11 countries, 4 continents) serving an aggregate population of >100,000,000 with 5970 hospital beds and 1024 ICU beds provided site specific data. Overall 600 liver transplants were performed at the sites for indications that included but were not limited to PHT ([median per center] 19: [25-75%ile] 6-34) of which 112 (1: 0-6) were living donor and 222 (5: 0-10) were technical variant grafts. In aggregate, 885 (23: 15-38) endoscopic variceal ligations were performed by 99 (4:2-6) individuals, while 266 (3:0–10) endoscopic sclerotherapy sessions were performed by 46 (2: 0-3) individuals. Potential two year endoscopic practitioner caseload varied significantly by site (variceal ligation 7: 2.8-13.8, sclerotherapy 1.5: 0.0-5.0). Nontransplant nonendoscopic interventions for PHT included 55 (range per center 1–20) portosystemic shunts (12/23 centers), 21 (range 1–5) TIPS (8/23 centers) and 30 (range 1–8) MesoRex bypass procedures (11/23 centers). 8 centers, Group A, performed at least 3 of at least one of these nontransplant nonendoscopic procedures; their center characteristics differed from the remaining 15 centers, Group B (Table).

Conclusion: A multi-center registry focused on pediatric esophageal varices, has been developed with ongoing patient data entry. Site specific data reveals marked variability in approaches. Many pediatric centers perform only small numbers of endoscopic procedures for PHT, often divided among several proceduralists. There is also variable and limited use of nonendoscopic nontransplant interventions for PHT. IMPPHR will permit analysis of the impact of differences in approach on outcomes, helping to inform optimal treatment decisions and program planning.

Supported by the Spain Family and an ESPGHAN Networking Grant.