Abstract Number: OC 20.4
Meeting: ISTH 2021 Congress
Background: PVT is an unusual disorder but it is associated with significant morbidity. Data on neonatal and pediatric PVT is scarce.
Aims: To describe clinical characteristics, diagnosis, thrombophilia testing(TT), management and outcomes of PVT in neonates(N-PVT) and pediatric(P-PVT) patients in a tertiary pediatric care center in Argentina.
To investigate the effect of antithrombotic treatment(ATT) in achieving recanalization.
Methods: From Jan-2010 to Dec-2020, consecutive children under 18 years of age with confirmed PVT were prospectively followed. Demographic data, imaging findings, TT, ATT and outcomes were registered. N-PVT unrelated to umbilical vein catheter(UVC) and P-PVT unrelated to liver transplant(LT) were considered eligible for TT. Patients with and without ATT were compared using Fischer´s exact test.
Results: Forty-seven pts were studied. N-PVT:20pts, incidence: 5,2 per 1000 ICU admissions/yr. P-PVT:27pts, 17pts(63%) associated with LT(LT-PVT). The prevalence of PVT among total LT recipients was 4,8%(95%CI:2.8-7.6). Clinical characteristics and ATT are described in Table1. TT was performed in 3/5 N-PVT and 5/10 P-PVT. Outcomes relative to occlusive versus non-occlusive PVT and treatment are shown in Table2. PVT resolution was observed in 25/35pts(71.4%) and 1/12pts(8.3%) with and without ATT respectively (p=0.0001, OR=27.5, 95%CI=[3.0-1229]). Neither progression nor bleeding complications were observed. Cavernous transformation was diagnosed in 11/22pts with non-recanalized PVT. All-cause mortality was 17% and no mortality attributable to PVT was observed.
N-PVT (n = 20pts) |
P-PVT (n = 27pts) |
|
Gender (M/F) | 9/11 | 14/13 |
Median age, (range) |
17 days (6-28) |
1,03 years (0,4-16,6) |
Local risk factors | n = 20 UVC (15) abdominal infection (6) surgery (7) intestinal malformation (4) intestinal oclusion (1) |
n = 26 liver transplant (17) abdominal infection (7) abdominal surgery (other than LT) (11) local malignant tumors (3) Crohn´s disease (2) congenital portosystemic shunt (1) |
Systemic risk factors | n = 9 systemic infection (7) congenital heart disease (9) Down syndrome (2) |
n = 3 systemic infection(2) acute lymphoblastic leukemia (1) antiphospholipid syndrome (2) |
Diagnostic Imaging | ultrasound (18) computed tomography (3) |
ultrasound (24) computed tomography (16) |
PVT Location | left branch (17) right branch (1) common branch (3) |
left branch (7) right branch (5) common branch (24) complete PVT (1) |
Symptoms | abdominal colateral circulation (1) | ascitis and abdominal distension (3) |
Anticoagulant Therapy | unfractionated Heparin (0) enoxaparine (12) vitamin K antagonists (1) |
unfractionated Heparin (9) enoxaparine (23) vitamin K antagonists (13) |
Median follow-up, years (range) |
4,4 (0,15 – 8,8) |
2,7 (0,2-10,32) |
PVT | n | Thrombophilia | Anticoagulation therapy (PVT resolution) |
No Anticoagulation therapy (PVT resolution) |
Pharmacomechanical Thrombectomy (PVT resolution) |
Portal hypertension | Cavernous transformation | Deaths | |
Neonatal | occlusive | 16 | Heterozygous FV Leiden, 1 | 11 ( 6 ) | 5 ( 1 ) | 0 | 3 | 3 | 3 |
non occlusive | 4 | – | 1 ( 1 ) | 3 ( 0 ) | 0 | 0 | 1 | 1 | |
Pediatric LT associated PVT | occlusive | 11 | not done | 9 ( 8 ) | 2 ( 0 ) | 8 ( 7 ) | 3 | 3 | 2 |
non occlusive | 6 | not done | 6 ( 5 ) | 0 | 3 ( 3 ) | 1 | 0 | 0 | |
Pediatric no LT associated PVT | occlusive | 5 | persistent aPL, 1 | 3 ( 1 ) | 2 ( 0 ) | 0 | 3 | 3 | 1 |
non occlusive | 5 | persistent aPL, 1 Heterozygous PTG20210A, 1 |
5 ( 4 ) | 0 | 1 ( 1 ) | 0 | 1 | 1 |
Conclusions: The incidence of N-PVT and LT-PVT was similar to other reports. Idiopathic PVT was rare; UVC and LT were the predominant risk factors in N-PVT and P-PVT respectively. Since PVT was incidentally diagnosed in most pts, screening patients at risk should be considered.
High-risk hereditary thrombophilia was not detected; the two patients with Crohn’s disease presented persistently positive antiphospholipid antibodies(aPL). Contribution of thrombophilia to PVT remains unclear. Recanalization was associated with antithrombotic treatment. Since cavernous transformation developed in unresolved PVT patients, antithrombotic treatment may also decrease this long-term complication.
To cite this abstract in AMA style:
Cervio C, Hepner M, Bianco B, Pieroni G, Annetta E, Frontroth JP, Sciuccati G. Portal Vein Thrombosis(PVT) in Neonates and Children: A Ten-year Prospective Registry of a Tertiary Care Single-centre in Argentina [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/portal-vein-thrombosispvt-in-neonates-and-children-a-ten-year-prospective-registry-of-a-tertiary-care-single-centre-in-argentina/. Accessed March 21, 2024.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/portal-vein-thrombosispvt-in-neonates-and-children-a-ten-year-prospective-registry-of-a-tertiary-care-single-centre-in-argentina/