Purpose

To determine factors affecting mortality, and long-term patency of portal vein, in patients with pancreatic-portal vein fistula (PPVF).

Methods

Consecutive cases of PPVF at the University of Pittsburgh Medical Center from 2008 to 2020 were retrospectively identified. Clinical history, imaging studies, management strategies, complications, and long-term outcomes were analyzed.

Results

Fourteen patients, representing the largest PPVF cohort reported to date (mean age 58.6 years, 64.3% women, median follow-up 10 months [1–98 months]) were identified. Underlying chronic pancreatitis was seen in 9 (64.3%) patients, while 5 (35.7%) developed PPVF with first attack of acute pancreatitis. PPVF involved proximal main portal vein (MPV) in 10 (78.6%) patients. Of the 5 patients (35.7%) who died, all had occlusive (n=4) or near-occlusive (n=1) PPVF-associated filling defect (FD) in the MPV. Conversely, 7 of 9 survivors (87.5%) had subocclusive FD and patent MPV. In patients with sepsis (n=5), 1 underwent surgical necrosectomy and survived, while 3 of 4 (75%) patients without debridement died.

Conclusion

Occlusive/near-occlusive PPVF-associated MPV FD, and sepsis, are associated with high mortality rates, while subocclusive MPV FD is associated with survival and long-term MPV patency. PPVF is a potentially life-threatening, and possibly under-diagnosed, entity that warrants early clinical suspicion for timely diagnosis, to facilitate optimal management.

中文翻译：

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胰门静脉瘘：一种具有广泛并发症的罕见诊断——胰腺卓越中心 13 年经验

 目的

确定影响胰门静脉瘘（PPVF）患者死亡率和门静脉长期通畅的因素。

 方法

回顾性分析了 2008 年至 2020 年匹兹堡大学医学中心连续发生的 PPVF 病例。分析了临床病史、影像学研究、管理策略、并发症和长期结果。

 结果

确定了 14 名患者，代表迄今为止报告的最大 PPVF 队列（平均年龄 58.6 岁，64.3% 为女性，中位随访时间 10 个月 [1-98 个月]）。 9 名患者 (64.3%) 患有潜在的慢性胰腺炎，而 5 名患者 (35.7%) 因急性胰腺炎首次发作而出现 PPVF。 PPVF 累及近端门静脉主干 (MPV) 10 名 (78.6%) 患者。在死亡的 5 名患者 (35.7%) 中，所有患者均存在 MPV 闭塞 (n=4) 或接近闭塞 (n=1) PPVF 相关的充盈缺损 (FD)。相反，9 名幸存者中有 7 名 (87.5%) 患有亚闭塞性 FD 和明显的 MPV。在脓毒症患者 (n=5) 中，1 例接受了坏死组织切除术并存活，而 4 例未进行清创的患者中有 3 例 (75%) 死亡。

 结论

闭塞/近闭塞 PPVF 相关的 MPV FD 和脓毒症与高死亡率相关，而亚闭塞 MPV FD 与生存和长期 MPV 通畅相关。 PPVF 是一种潜在危及生命且可能诊断不足的实体，需要早期临床怀疑以便及时诊断，以促进最佳管理。