Clinical findings, diagnosis and therapy of patent ductus venosus in children: a case series.

Clinical findings, diagnosis and therapy of patent ductus venosus in children: a case series.
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DOI:
10.21037/cdt-22-179
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发表时间:
2022-10
影响因子:
2.4
通讯作者:
Gan, Qing
Gan, Qing
中科院分区:
医学4区
文献类型:
--
作者:
Xiang, Yonghua;Jin, Ke;Cai, Qifang;Peng, Yinghui;Gan, Qing

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静脉导管未闭(PDV)是一种罕见的先天性门体分流。由于临床症状的多样性和对这种情况的了解不足,临床医生经常没有进行有针对性的检查,导致漏诊和误诊。本文总结了PDV的临床、放射学表现及外科治疗方法,旨在提高对PDV的早期诊断和指导治疗。对PDV患者的临床、实验室和放射学资料进行了回顾性分析。共有9名PDV患者纳入研究(男性7例,女性2例;年龄中位数1.6岁,年龄16天至16.5岁)。对所有9例PDV患者的资料进行复习。最常见的最初临床表现是黄疸和呼吸道症状。实验室检查发现低氧血症5例,高氨血症2例,高胆红素血症7例,凝血功能异常6例,心肌酶异常4例,肝功能障碍8例,肾功能障碍3例。PDV的直接影像征象是门静脉左支与下腔静脉连接的血管结构。所有9例患者的继发影像表现均为右心、肺动脉和LPV扩张,门静脉右支萎缩。门静脉主干扩张8例,缩小1例,肝增大8例,肝右叶低灌注者3例。脾增大8例,缩小1例,肾脏影像异常2例。肝性脑病4例,PDV合并其他畸形7例,以先天性心脏病和血管畸形最多见,3例成功结扎PDV。PDV可导致多系统损坏。PDV的二次放射学征象对其早期诊断和术前评估有重要意义。并发症和合并畸形是常见的,在术前评估时不应遗漏。建议对PDV尽早手术闭合。
Patent ductus venosus (PDV) is a rare form of congenital portosystemic shunt. Because of the diversity of clinical symptoms and insufficient knowledge of this condition, clinicians often fail to perform targeted examinations, resulting in missed diagnoses and misdiagnoses. This study summarized the clinical and radiological findings, as well as surgical methods, of PDV with the aim of improving early diagnosis and guiding treatment. Clinical, laboratory, and radiologic data of patients with PDV were analyzed retrospectively. In all, 9 patients with PDV were included in the study (7 male, 2 female; median age 1.6 years, age range 16 days to 16.5 years). Data for all 9 patients with PDV were reviewed. The most common initial clinical presentations were jaundice and respiratory symptoms. Laboratory data revealed hypoxemia in 5 patients, hyperammonemia in 2, hyperbilirubinemia in 7, abnormal coagulation function in 6, abnormal myocardial enzymes in 4, hepatic dysfunction in 8, and renal dysfunction in 3. The direct imaging sign of PDV was a vascular structure connecting the left branch of the portal vein (LPV) to the inferior vena cava. Secondary imaging findings observed in all 9 patients were dilated right heart, pulmonary artery, and LPV, and an atrophic right branch of the portal vein. The main portal vein was dilated in 8 patients and shrunk in 1. Moreover, 8 patients had enlarged livers, and 3 presented with hypoperfusion in the right lobe of the liver. The spleen was enlarged in 8 patients but shrunk in 1. Renal imaging was abnormal in 2 patients. Hepatic encephalopathy was found in 4 patients; 7 patients had PDV combined with other malformations, with congenital heart disease and vascular abnormalities being the most common; 3 patients successfully underwent surgical ligation of PDV. PDV can lead to multisystem damage. Secondary radiological signs of PDV play an important role in early diagnosis and preoperative evaluation. Complications and coexisting malformations were common and should not be missed during preoperative evaluation. Early surgical closure for PDV is recommended.
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