{
  "abstract": "Porto-sinusoidal vascular disease (PSVD) is a condition which affects the portal venules and sinusoids in the absence of cirrhosis. 1 Knowledge about PSVD natural history is still limited.2–4 We aimed to describe the characteristics of children affected with PSVD, as well as their histological findings and clinical outcomes. We performed a retrospective review of children with native liver biopsy samples suggestive of PSVD from January 2019 to October 2024.Forty-eight patients fit the diagnostic criteria of PSVD as described by Gottardi et al.1 with 64.6% (n=31) having associated portal hypertension (PHT) and 35.4% (n=17) without PHT. The most common reason for referral to hepatology was isolated elevated transaminases in the non-PHT group, hypersplenism and portal hypertensive bleed in the PHT group (table 1). There were no significant differences between the histological features found on both groups, apart from a higher prevalence of perisinusoidal fibrosis in those with PHT (p=0.018).The non-PHT group had a higher median ALT of 61 (IQR 35, 213) at presentation when compared to the PHT group (p=0.021). During the follow up period no significant adverse events were observed in the non-PHT group, while 2 children developed hepatopulmonary syndrome and 3 underwent liver transplantation in the PHT group. Of those with PHT, 16 had evidence of portal vein thrombosis (PVT) with cavernous transformation at presentation and 2 developed PVT during the follow up period.In summary, PSVD is a condition with a variable phenotypic presentation and disease course. Those without PHT, mostly presenting with isolated elevated transaminases, appear to have a milder disease course, while those with PHT can have poor outcomes. Prospective studies are required to improve our understanding of the disease course and identify those at risk of progressing to PHT and developing adverse outcomes.Abstract OC86 Table 1Description of baseline characteristics and outcomes of patients with PSVD with and without associated portal hypertension (PHT) Baseline characteristics and outcomes of children with PSVD Total Non-PHT PHT Number of patients, n (%) 48 (100) 17 (35.4) 31 (64.6) Gender: male, n (%) 30 (62.5) 9 (52.9) 21 (67.7) Median age in years at presentation (IQR) 5.0 (2.1, 11.6) 4.3 (1.0, 11.7) 6.0 (2.6, 11.1) Median follow up in years (IQR) 2.4 (1.6, 5.3) 2.1 (1.6, 2.4) 3.6 (1.9, 5.6) Associated conditions, n (%) 35 (72.9) 13 (76.5) 22 (71.0) Reason for referral to hepatology, n (%) Isolated elevation in transaminases 12 (25.0) 12 (70.6) 0 (0) Splenomegaly 4 (8.3) 0 (0) 4 (12.9) Hypersplenism 12 (25.5) 0 (0) 12 (38.7) Portal Hypertensive bleed 12 (25.5) 0 (0) 12 (38.7) Other 8 (16.7) 5 (29.4) 3 (9.7) Liver elastography, median in Kpa (IQR) 7.7 (6.0, 10.1) 6.9 (5.1, 7.7) 8.6 (6.1, 10.9) Management, n (%) Conservative 17 (35.4) 17 (100) 0 (0) Endoscopic surveillance 22 (45.8) 0 (0) 22 (71.0) Shunt surgery 6 (12.5) 0 (0) 6 (19.4) Liver transplant 3 (6.3) 0 (0) 3 (9.7) Hepatopulmonary syndrome, n (%) 2 (4.2) 0 (0) 2 (6.5) Death, n (%) 1 (2.1) 0 (0) 1 (3.2) References De Gottardi A, Rautou P, Schouten J, et al. Porto-sinusoidal vascular disease: proposal and description of a novel entity. The Lancet Gastroenterology & Hepatology 2019 May;4(5):399–411.Magaz M, Heloïse Giudicelli-Lett, Abraldes JG, et al. Porto-sinusoidal vascular liver disorder with portal hypertension: natural history and long-term outcome. Journal of Hepatology 2024 Aug 1.Di Giorgio A, Matarazzo L, Sonzogni A, et al. Paediatric porto-sinusoidal vascular disease: two different clinical phenotypes with subtle histological differences. Liver International 2023 May 8;43(7):1523–36.De Gottardi A, Sempoux C, Berzigotti A. Porto-sinusoidal vascular disorder. Journal of Hepatology 2022 Oct;77(4):1124–35.",
  "authors": [
    {
      "affiliations": [
        "Leeds Children’s hospital"
      ],
      "name": "Sandra Fernandes Lucas"
    },
    {
      "affiliations": [
        "Leeds Children’s hospital"
      ],
      "name": "Kavitha Jayaprakash"
    },
    {
      "affiliations": [
        "Leeds Children’s hospital"
      ],
      "name": "Marumbo Mtegha"
    },
    {
      "affiliations": [
        "Leeds Children’s hospital"
      ],
      "name": "Sanjay Rajwal"
    },
    {
      "affiliations": [
        "Leeds Children’s hospital"
      ],
      "name": "Palaniswamy Karthikeyan"
    }
  ],
  "title": "OC86 The clinical spectrum of porto-sinusoidal vascular disease in paediatrics: a single centre review",
  "uid": "de6b0026-58d3-5d62-a552-ff9563b3023d"
}