Background

Torcular dural sinus malformations (tDSMs) are rare vascular malformations that present in fetuses and infants. Existing data on prognostic imaging features, as well as the associated morbidity and mortality, are limited and variable. We therefore reviewed cases of tDSMs diagnosed on fetal magnetic resonance imaging (MRI) at our referral center to identify pre- and postnatal MRI imaging features associated with long-term outcomes.

Methods

We searched our imaging database for fetal and postnatal MRI reports of tDSM cases. The electronic medical record was then reviewed for pre- and postnatal clinical data, including follow-up imaging. Neurological outcomes were characterized using the previously reported scale based on the Bicêtre Score. Imaging features association with outcome scores were compared using the Fisher exact test.

Results

Sixteen cases of tDMS diagnosed by fetal MRI with postnatal clinical follow-up were identified, 11 of whom underwent postnatal MRI. The majority of cases of tDSM (73%) decreased in size or resolved on postnatal follow-up study without treatment. Restricted diffusion and parenchymal hemorrhage on fetal MRI were the only imaging features identified significantly associated with unfavorable neurological outcome or death, present in two patients with poor outcomes (two of two) and only one with a normal outcome (one of 14) (P = 0.025).

Conclusions

Findings of tDSM on fetal MRI most often regress and/or resolve with normal or mild neurological outcomes, with the most significant predictor of poor outcome being the presence of parenchymal injury on fetal MRI. In addition, a subset will present with venolymphatic malformations.

中文翻译：

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环形硬脑膜窦畸形：胎儿和产后影像学发现及其与临床结果的关联

背景

环形硬脑膜窦畸形 (tDSMs) 是胎儿和婴儿中罕见的血管畸形。关于预后成像特征以及相关的发病率和死亡率的现有数据是有限且多变的。因此，我们回顾了在我们的转诊中心通过胎儿磁共振成像 (MRI) 诊断的 tDSM 病例，以确定与长期结果相关的产前和产后 MRI 成像特征。

方法

我们在我们的影像数据库中搜索了 tDSM 病例的胎儿和产后 MRI 报告。然后审查电子病历以获取产前和产后临床数据，包括后续成像。使用先前报告的基于 Bicêtre 评分的量表来表征神经学结果。使用Fisher精确检验比较成像特征与结果评分的关联。

结果

确定了 16 例通过胎儿 MRI 诊断并进行产后临床随访的 tDMS，其中 11 例接受了产后 MRI。大多数 tDSM 病例（73%）在没有治疗的情况下在产后随访研究中缩小或消退。胎儿 MRI 上的弥散受限和实质出血是唯一与不利的神经系统结果或死亡显着相关的影像学特征，存在于两名预后不良的患者（两名中的两名）和一名预后正常的患者（14 名中的一名）（P  = 0.025)。

结论

胎儿 MRI 上 tDSM 的发现通常会随着正常或轻度的神经系统结果而消退和/或消退，最重要的不良结果预测因素是胎儿 MRI 上存在实质损伤。此外，一个子集会出现静脉淋巴畸形。