BACKGROUND Deep medullary vein (DMV) thrombosis is a rare cause of brain damage in both preterm and full-term neonates. In this study, we aimed to collect data on clinical and radiological presentation, treatment, and outcome of neonatal DMV thrombosis. METHODS Systematic literature review on neonatal DMV thrombosis was carried out in PubMed, ClinicalTrial.gov, Scopus, and Web of Science up to December 2022. RESULTS Seventy-five published cases of DMV thrombosis were identified and analysed (preterm newborns were 46%). Neonatal distress, respiratory resuscitation, or need for inotropes were present in 34/75 (45%) of patients. Signs and symptoms at presentation included seizures (38/75, 48%), apnoea (27/75, 36%), lethargy or irritability (26/75, 35%). At magnetic resonance imaging (MRI), fan-shaped linear T2 hypointense lesions were documented in all cases. All had ischaemic injuries, most often involving the frontal (62/74, 84%) and parietal lobes (56/74, 76%). Signs of haemorrhagic infarction were present in 53/54 (98%). Antithrombotic treatment was not mentioned in any of the studies included. Although mortality was low (2/75, 2.6%), a large proportion of patients developed neurological sequelae (intellectual disability in 19/51 [37%] and epilepsy in 9/51 [18%] cases). CONCLUSIONS DMV thrombosis is rarely identified in the literature, even if it is possibly under-recognized or under-reported. Presentation in neonatal age is with seizures and non-specific systemic signs/symptoms that often cause diagnostic delay, despite the pathognomonic MRI picture. The high rate of morbidity, which determines significant social and health costs, requires further in-depth studies aimed at earlier diagnosis and evidence-based prevention and therapeutic strategies.

中文翻译：

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新生儿深髓静脉血栓形成：系统文献综述。

背景深髓静脉（DMV）血栓形成是早产儿和足月新生儿脑损伤的罕见原因。在本研究中，我们旨在收集有关新生儿 DMV 血栓形成的临床和放射学表现、治疗和结果的数据。方法 截至 2022 年 12 月，在 PubMed、ClinicalTrial.gov、Scopus 和 Web of Science 上对新生儿 DMV 血栓形成进行系统文献综述。 结果 对 75 例已发表的 DMV 血栓病例进行了识别和分析（早产儿占 46%）。34/75 (45%) 的患者存在新生儿窘迫、呼吸复苏或需要正性肌力药物的情况。就诊时的体征和症状包括癫痫发作（38/75，48%）、呼吸暂停（27/75，36%）、嗜睡或易怒（26/75，35%）。在磁共振成像 (MRI) 中，所有病例均记录到扇形线性 T2 低信号病变。所有人都有缺血性损伤，最常见的是额叶（62/74，84％）和顶叶（56/74，76％）。53/54 (98%) 存在出血性梗塞迹象。所有纳入的研究均未提及抗血栓治疗。尽管死亡率较低（2/75，2.6%），但很大一部分患者出现神经系统后遗症（19/51 [37%] 病例出现智力障碍，9/51 [18%] 病例出现癫痫）。结论 DMV 血栓形成在文献中很少被识别，即使它可能未被充分认识或报道不足。新生儿时期的表现是癫痫发作和非特异性全身体征/症状，尽管有特征性的 MRI 图片，但往往会导致诊断延误。高发病率决定了巨大的社会和健康成本，需要进一步深入研究，以实现早期诊断和循证预防和治疗策略。