Paediatric Neurology and Neurophysiology Unit, Department of Women's and Children's Health, University Hospital of Padua, Padua, Italy.
Neuroimmunology Group, Paediatric Research Institute "Città della Speranza,", Padua, Italy.
Neonatology. 2023;120(5):539-547. doi: 10.1159/000530647. Epub 2023 Jun 28.
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.
Systematic literature review on neonatal DMV thrombosis was carried out in PubMed, ClinicalTrial.gov, Scopus, and Web of Science up to December 2022.
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).
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.
深髓静脉（DMV）血栓形成是早产儿和足月儿脑损伤的罕见原因。在本研究中，我们旨在收集关于新生儿 DMV 血栓形成的临床和影像学表现、治疗和结局的数据。
在 PubMed、ClinicalTrials.gov、Scopus 和 Web of Science 上对新生儿 DMV 血栓形成进行了系统的文献回顾，检索时间截至 2022 年 12 月。
共确定并分析了 75 例 DMV 血栓形成病例（早产儿占 46%）。34/75（45%）例患者存在新生儿窘迫、呼吸复苏或需要正性肌力药物。发病时的症状和体征包括癫痫发作（38/75，48%）、呼吸暂停（27/75，36%）、嗜睡或易激惹（26/75，35%）。在磁共振成像（MRI）上，所有病例均记录到扇状线性 T2 低信号病变。所有病例均有缺血性损伤，最常见于额叶（62/74，84%）和顶叶（56/74，76%）。53/54（98%）例有出血性梗死的迹象。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了神经后遗症（51 例中有 19 例智力残疾[37%]和 9 例癫痫[18%]）。
尽管 DMV 血栓形成的文献报道很少，但即使它可能被低估或漏报，在新生儿期的表现也与癫痫发作和非特异性全身症状/体征有关，这些症状/体征经常导致诊断延迟，尽管 MRI 具有特征性表现。发病率高会导致较高的发病率，从而带来重大的社会和健康成本，因此需要进一步深入研究，以实现更早的诊断和基于证据的预防和治疗策略。
