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PRESENTING COMPLAINTS IN 250 PATIENTS WITH CONGENITAL PORTOSYSTEMIC SHUNTS

Valérie McLin 1, Valeria Casotti 2,3, Simona Korff 4, Julie Wacker 4, Khaled Mostaguir 4, Riccardo Superina 5, Laurent Savale 6, Anne-Laure Rougemont 4, Dominique Debray 7

Affiliations

1 Swiss Pediatric Liver Center, University Hospitals Geneva, Geneva, Switzerland

2 Hospital Papa Giovanni XXIII,, Bergamo, Italy

3 Hospital Papa Giovanni XXIII, Bergamo, Italy

4 University Hospitals Geneva, Geneva, Switzerland

5 Lurie Children's, Chicago, United States

6 Hopital Bicetre, Paris, France

7 Hopital Necker Enfants Malades, Paris, France

Summary

AI Generated

Registry data from 256 patients with congenital portosystemic shunts show most are asymptomatic at diagnosis, with intrahepatic shunts diagnosed earlier and more likely to close spontaneously than extrahepatic shunts.

Among 212 patients with known shunt type, 41.5% had intrahepatic shunts and 58.5% had extrahepatic shunts.
Most patients were asymptomatic at diagnosis: 72% of those with intrahepatic shunts and 68% of those with extrahepatic shunts.
Symptomatic intrahepatic shunt patients were diagnosed at a median age of 0.59 years, while symptomatic extrahepatic shunt patients were diagnosed later at a median age of 7.49 years.
Spontaneous closure occurred in 37.5% of closed intrahepatic shunts (all before age 1), but in only 2 extrahepatic shunt patients.
Closure methods varied by shunt type, with interventional radiology used in 52% of intrahepatic shunts and surgery in 54% of extrahepatic shunts, plus 7 liver transplants.
This descriptive analysis from the ESPGHAN-sponsored International Registry of Congenital Portosystemic Shunts may help clinicians recognize this rare disorder.

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This summary was generated by an AI large language model based on the content transcript. It is for informational purposes only and should not be considered a substitute for clinical judgment. Always rely on your professional expertise and the full clinical context when making clinical decisions.

Abstract

Introduction

Congenital portosystemic shunts (CPSS) are associated with an array of clinical signs and symptoms of varying severity in both adults and children. Yet, symptoms are often missed and ascribed to other etiologies. Understanding presenting complaints will increase awareness of this rare disorder. To this end the International Registry of Congenital Portosystemic shunts (IRCPSS) was created. Here we report an up-to-date description of the patients enrolled in the IRPCSS focusing on presenting complaints.

Aims & Methods

Descriptive analysis of the ESPGHAN sponsored IRCPSS described in (1)

Results

256 patients were enrolled - 125 females (49%). Type of CPSS was available for 212: 88 (41.5%) presented with intrahepatic (IHS) and 124 (58.5%) with extrahepatic (EHS) shunts. Clinical data were available for 71 with IHS and 106 with EHS. 51 (72 %) of IHS were asymptomatic. Of the 20 (28%) symptomatic, median age at diagnosis was 0.59 years (14 days-19.9 years). Presenting complaint included liver in 14, cardiopulmonary in 6, neurological in 2, other in 7. Of 56 IHS closed, 21 (37.5%) closed spontaneously before age 1. 29 (52%) were closed using interventional radiology. Among 106 patients with EHS, 72 (68%) were asymptomatic. Among symptomatic patients, median age at diagnosis was 7.49 years (14 days- 24.9 years). Presenting complaints included liver in 15, cardiopulmonary in 13, neurological in 13, and other in 17. 74 patients had closed shunts at last follow-up; they were closed at a median age of 2.7 years (18 days- 25.4 years). Spontaneous closure was reported in 2 patients, otherwise closure was performed by interventional radiology in 25 (34%), or surgery in 40 (54%) and 7 undergoing LT.

Conclusion

Most shunts were asymptomatic at diagnosis. Among symptomatic patients, the median age at diagnosis was higher in EHS than IHS. IHS and EHS were equally likely to be closed, with IHS more likely to close
spontaneously within the first year of life.

References

(1) Korff S, Mostaguir K, Beghetti M, D'Antiga L, Debray D, Franchi-Abella S, Gonzales E, Guerin F, Hachulla AL, Lambert V, Makrythanasis P, Roduit N, Savale L, Senat MV, Spaltenstein J, van Steenbeek F, Wildhaber BE, Zwahlen M, McLin VA. International registry of congenital porto-systemic shunts: a multi-centre, retrospective and prospective registry of neonates, children and adults with congenital porto-systemic shunts.Orphanet J Rare Dis. 2022 Jul 19;17(1):284.

Disclosure

V McLin consults for Mirum and Ipsen. L Savale consults for MSD, Janssen, and GSK. Neither

Conference

UEG Week Vienna 2024

Topics

Hepatobiliary

Submission format

Abstract

Session

LIVER (Posters)

Citation

United European Gastroenterology Journal 2024; 12 (Supplement 8)

Published

2024

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