Torcular dural sinus malformations: a single-center case series and a review of literature

Paediatric intracranial dural arterio-venous shunts have clinical presentations and evolutions, with angiographic characteristics that differ from those described in adults. We report our experience concerning their therapeutic management, emphasizing the relevance of early diagnosis and appropriate treatment for satisfactory neurocognitive development. Using a prospective database, we reviewed the clinical and radiological data of all children with dural arteriovenous shunts managed between 2002 and 2020. Dural shunts were categorized into three types: dural sinus malformations with arteriovenous shunts; infantile dural arteriovenous shunts; adult-type dural arteriovenous shunts. Therapeutic strategies and outcomes were analyzed depending on lesional subtypes. Modified Rankin Scale for the paediatric population was assessed pre-treatment and at last follow-up. Twenty-eight patients (16 girls [57.1%]; 12 boys [42.9%]) were included: 17 dural sinus malformation (10 boys [58.8%]; seven girls [41.2%]), three infantile shunts (three girls [100%]), eight adult-type shunts (four girls [50%]; four boys [50%]), with a mean age of 19.2±36.6 months at presentation. Twelve (42.9%) had a modified Rankin Scale score of 0-2, four (14.3%) a score of 3, three (10.7%) a score of 4 and eight (28.6%) a score of 5. Embolization was performed in 22 children (78.6%; 12 girls [54.5%]; 10 boys [45.5%]). Fifteen patients could be cured (68.2%): 11 dural sinus malformations (73.3%), four adult-type lesions (100%), but no infantile shunt. Mean post-treatment follow-up was 39.5 months (max. 139 months): 14 patients (63.6%) presented a modified Rankin Scale score 0-2 and eight (36.4%) a score ≥3. In the dural sinus malformation group, the modified Rankin Scale score was improved in 11 patients (73.3%) and unchanged in three (20%). Only one patient with infantile subtype (33.3%) improved clinically. In the adult-subtype group, all children (100%) improved. Of six untreated patients (four girls [66.7%]; two boys [33.3%]), four with adult-subtype shunts showed uneventful evolutions, one with dural sinus malformation died, and therapeutic abortion was conducted in an antenatally diagnosed dural sinus malformation. Pediatric dural fistulas comprise different subtypes with variable clinical courses. Proper diagnosis is mandatory for optimal therapeutic strategies within appropriate therapeutic windows.

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“…Furthermore, endoscopic third ventriculostomy should be proposed when embolization has failed to correctly master hydrocephaly. 32 , 33 …”

Section: Discussionmentioning

confidence: 99%

Paediatric intracranial dural arteriovenous shunts: types, clinical presentation and therapeutic management

Smajda

Söderman

Dorfmüller

et al. 2022

Brain Communications

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“…The overall mortality rate is 15%, with a favourable outcome seen in 87% of the surviving patients [11]. Factors in favour of a good neurological outcome were lateral type of DSM and antenatally detected thrombosis which either showed progressive decrease in size or spontaneous resolution [7,[11][12][13] .Presence of parenchymal infarctions, haemorrhage, hydrocephalus, congestive cardiac failure and non-resolution of thrombus or postnatal onset of thrombosis all contributed to poor prognosis resulting in either fetal/postnatal demise or a poor neurological outcome in the neonate [7,11].…”

Section: Discussionmentioning

confidence: 99%

Fetal imaging of a rare case of dural sinus malformation: a case report

Kaushik

Acharya

Ananthasivan

et al. 2021

Egypt J Radiol Nucl Med

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Background Dural sinus malformations (DSM) are rare congenital anomalies, accounting for less than 2% of all intracranial vascular malformations. Fetal MRI plays an important role in the confirmation of the diagnosis, prognostication, and planning of treatment strategies. Here, we present a rare case of dural sinus malformation without thrombosis, diagnosed by prenatal ultrasound and fetal MRI. In addition to this, fetal intracranial 3D gradient recalled echo Dixon-based MRA was done which, to the best of our knowledge, is a first. Case presentation A 24-year-old multigravida with no known comorbidities underwent a routine second trimester anomaly scan in which an unusual posterior interhemispheric cyst was diagnosed. Further evaluation with fetal MRI revealed dilated posterior sinuses and torcula, normal internal jugular vein, and maintained flow voids with no mass effect. Fetal Dixon-based MRA and correlated Doppler revealed supply to the lesion by bilateral occipital arteries and posterior cerebral arteries. With these imaging features, a midline dural sinus malformation was diagnosed. Conclusion Dural sinus malformations should be considered in the differential diagnosis of unusual posterior fossa cystic lesions detected on antenatal ultrasound coupled with a colour Doppler examination. Prompt fetal MRI is essential to establish the diagnosis, identify intracranial complications and decide the postnatal treatment strategy, thereby possibly improving the postnatal outcome.

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“…On the other hand, the partial or complete dural partition of the superior sagittal sinus allows that, while one channel is obstructed, another one drains correctly (5,12,14) . On the other hand, arteriovenous shunts in this malformation are generally of low velocity and rarely cause systemic hemodynamic complications (15) . Prognosis depends on the absence of predisposing conditions and normal MRI evaluation of the brain parenchyma (7) .…”

Section: Discussionmentioning

confidence: 99%

Diagnóstico prenatal de trombosis de la tórcula de Herófilo

Torres-Cepeda¹,

Rondón-Tapía

2021

Rev peru ginecol obstet.

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La trombosis de la tórcula de Herófilo es una enfermedad rara de etiología desconocida. Hasta ahora se han descrito alrededor de 50 casos en la literatura. La ecografía es un método diagnóstico eficaz y es útil para el seguimiento. Los hallazgos ecográficos característicos incluyen una lesión anecoica triangular bien definida en la región occipital con una estructura ecogénica en su interior que es el trombo. La ecografía Doppler puede ayudar a establecer el diagnóstico en forma más precisa. El pronóstico es variable, ya que la mayoría de los casos tienen un curso clínico irrelevante debido a la resolución espontánea del trombo, mientras que otros casos pueden experimentar atrofia cerebral y muerte. Se presenta un caso de diagnóstico prenatal de trombosis de la tórcula de Herófilo.

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Torcular dural sinus malformations: a single-center case series and a review of literature

Cited by 17 publications

References 17 publications

Paediatric intracranial dural arteriovenous shunts: types, clinical presentation and therapeutic management

Paediatric intracranial dural arteriovenous shunts: types, clinical presentation and therapeutic management

Fetal imaging of a rare case of dural sinus malformation: a case report

Diagnóstico prenatal de trombosis de la tórcula de Herófilo

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