Department of Pediatric Hematology, Emma Children's Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, the Netherlands.
Department of Pediatric Gastroenterology, Emma Children's Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, the Netherlands.
Thromb Res. 2023 Nov;231:44-49. doi: 10.1016/j.thromres.2023.09.015. Epub 2023 Sep 30.
Portal vein thrombosis (PVT) is a rare disease in children and may be complicated by portal hypertension (PH), hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) but their incidence and risk factors are unknown.
An observational, retrospective cohort study of all consecutive children (≤18 years) with PVT treated at the Emma Children's Hospital Amsterdam University Medical Centers between January 1996 and January 2022 was conducted to identify the incidence and risk factors of these post thrombotic complications (PTC) in pediatric patients.
In total 43/ 703 thrombosis patients had PVT (boys 72.1 %; mean age 1.3 ± 0.5 years). Overall, 51 % of patients developed PH (n = 22), complicated by PPHTN in one of them. In 16 of 22 patients, PVT presented with portal hypertension. Clinically relevant bleeding due to portal hypertension occurred in 13 (59.1 %) patients with PH. The mean age at the first clinically relevant bleeding was 5.1 ± 5.9 years. Risk factors for the development of PH were lack of complete thrombus resolution (OR 24.3, 95 % CI 1.2-7.0; p = 0.008) and unprovoked VTE (OR, 35.4; 95 % CI 1.4-6.3; p = 0.012). Median time from PVT to PH was 137 days (range: 0 days to 5.04 years).
We demonstrated that half of the patients develop PH after PVT, with a lack of thrombus resolution and unprovoked VTE as independent risk factors. This high incidence underlines the importance of long-term standardized follow-up of patients after PVT and standard screening in patients at risk of PTC.
门静脉血栓形成（PVT）在儿童中较为罕见，可能并发门静脉高压（PH）、肝肺综合征（HPS）和肺门静脉高压（PPHTN），但其发病率和危险因素尚不清楚。
对 1996 年 1 月至 2022 年 1 月期间在阿姆斯特丹大学医学中心艾玛儿童医院接受治疗的所有连续儿童（≤18 岁）进行了一项观察性、回顾性队列研究，以确定儿科患者发生这些血栓后并发症（PTC）的发病率和危险因素。
在 703 例血栓患者中，共有 43 例（72.1%为男性；平均年龄 1.3±0.5 岁）患有 PVT。总体而言，51%的患者出现 PH（n=22），其中 1 例并发 PPHTN。在 22 例患者中，有 16 例 PVT 表现为门静脉高压。PH 中有 13 例（59.1%）患者出现因门静脉高压导致的临床相关出血。首次出现临床相关出血的平均年龄为 5.1±5.9 岁。PH 发展的危险因素是不完全血栓溶解（OR 24.3，95%CI 1.2-7.0；p=0.008）和无诱因 VTE（OR 35.4；95%CI 1.4-6.3；p=0.012）。从 PVT 到 PH 的中位时间为 137 天（范围：0 天至 5.04 年）。
我们表明，一半的患者在发生 PVT 后会出现 PH，且血栓溶解不完全和无诱因 VTE 是独立的危险因素。如此高的发病率强调了对 PVT 后患者进行长期标准化随访以及对 PTC 高危患者进行标准筛查的重要性。
