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Complications of congenital portosystemic shunts: liver tumors are affected by shunt severity, but pulmonary and neurocognitive associations are not

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Abstract

Background and purpose

Congenital portosystemic shunts (CPSS) are associated with multisystem complications, with the most common being liver tumors. The purpose of this study is to investigate the incidence of complications of CPSS, and to determine the natural history of liver tumors and their relationship with shunt closure.

Methods

Single-center retrospective cohort study of patients with CPSS referred from 1990 to 2020. Data on demographics, laboratory, radiological and histological investigations, clinical evolution, and surgery were reviewed. Mann–Whitney for continuous data and Fisher’s exact test for categorical data were used. A p value of 0.05 was considered significant.

Results

54 patients were investigated for CPSS with a median age of 1.1 years (IQR 0.2–11.8 years) at presentation—7 intrahepatic shunts resolved spontaneously and were excluded. Type 1 (without intrahepatic portal flow) had a higher rate of all hepatic tumors than Type 2 (partial intrahepatic portal flow) [18/22(82%) vs. 9/25(36%); p = 0.003); and malignant tumors (6/22(27%) vs 1/25(4%); (p = 0.04). Following shunt closure, 4/11(36%) of patients experienced partial and 3/11(27%) complete tumor regression. Pulmonary hypertension and hepatopulmonary syndrome affected 4(9%), and 3(6%) patients, respectively. Pulmonary complications affected 1 patient with Type 1 and 6 with Type 2 shunts (p = 0.1). Neurocognitive anomalies were identified in 16/47(34%) patients, 8/22(35%) with Type 1 shunts and 8/25(32%) with Type 2 shunts (p = 0.76). 9/47 (19%) required special needs schooling.

Conclusions

Severity of portal venous deprivation (Type 1 CPSS) increases the risk of hepatic tumors and surgical closure is associated with a reduction in size or complete resolution of benign tumors.

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No further data beyond what is in manuscript.

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Abbreviations

CPSS:

Congenital portosystemic shunt

NRH:

Nodular regenerative hyperplasia

FNH:

Focal nodular hyperplasia

HCA:

Hepatocellular adenoma

HCC:

Hepatocellular carcinoma

HB:

Hepatoblastoma

WDHN:

Well-differentiated hepatocellular neoplasm

LFTs:

Liver function tests

US:

Ultrasound

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Funding

This study was supported by a joint grant from the Children’s Liver Disease Foundation and the British Society of Paediatric Gastroenterology, Hepatology, and Nutrition. This grant supported only part of the research cost relating to the processing and staining of pathology specimens. Alberto Quaglia is supported by the National Institute for Health Research (NIHR) UCLH/UCL Biomedical Research Centre (BRC).

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Authors and Affiliations

  1. Department of Paediatric Surgery, King’s College Hospital, London, UK

    Athanasios Tyraskis & Mark Davenport

  2. Department of Radiology, King’s College Hospital, London, UK

    Annamaria Deganello, Maria Sellars & Pauline Kane

  3. Division of Clinical Pathology, Geneva University Hospital, Geneva, Switzerland

    Claudio De Vito

  4. Paediatric Hepatology, King’s College Hospital, London, UK

    Richard J. Thompson

  5. Institute of Liver Studies, King’s College Hospital, London, SE5 9RS, UK

    Richard J. Thompson & Nigel Heaton

  6. Department of Cellular Pathology, Royal Free Hospital, London, UK

    Alberto Quaglia

Authors
  1. Athanasios Tyraskis
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  2. Mark Davenport
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  3. Annamaria Deganello
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  4. Maria Sellars
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  5. Claudio De Vito
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  6. Pauline Kane
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  7. Richard J. Thompson
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  8. Alberto Quaglia
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  9. Nigel Heaton
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Corresponding author

Correspondence to Nigel Heaton.

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Conflicts of interest

Athanasios Tyraskis, Mark Davenport, Annamaria Deganello, Maria Sellars, Claudio De Vito, Pauline Kane, Richard J. Thompson, Alberto Quaglia and Nigel Heaton have No conflict of interest to declare.

Ethical approval

Ethical approval for this study was granted by the Heath Research Authority (16/EM/0342).

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As retrospective case study, none was required according to ethics board review.

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Tyraskis, A., Davenport, M., Deganello, A. et al. Complications of congenital portosystemic shunts: liver tumors are affected by shunt severity, but pulmonary and neurocognitive associations are not. Hepatol Int 16, 918–925 (2022). https://doi.org/10.1007/s12072-022-10328-5

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  • DOI: https://doi.org/10.1007/s12072-022-10328-5

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