Torcular dural sinus malformation

The dural sinus malformation (DSM) are rare congenital vascular anomalies with variable anatomic features, clinical conditions, and outcomes. There are two forms of disease: a lateral subtype, affecting the jugular bulb with associated high flow arteriovenous fistula; and a midline subtype, called torcular DSM. The torcular subtype is more common and characterized by a giant dural sinus lake involving the confluens sinuum (torcular Herophili). We present a case of a 28-year-old woman with an ultrasound at 32 weeks’ gestation showing a fetal intracranial thrombosed lesion, measuring 6x4 cm. An elective c-section was performed at 38 weeks’ gestation, and a male neonate was born. He remained asymptomatic, and a brain MRI performed 9 days later showed a thrombosed midline mass due to a torcular DSM. The outcome with conservative treatment was favorable, and further development was unremarkable. The 2-year follow-up imaging demonstrated spontaneous involution of the clot. The torcular DSM is frequently diagnosed prenatally as an intraluminar lake thrombosis, and differential diagnosis include tumors, subdural collections, vein of Galen aneurysmal malformations, pial malformations, arachnoid and dermoid cysts. A literature review involving 126 patients with torcular DSM found an overall mortality of 22.1%. Torcular DSM seems to have better prognosis than those having dural arteriovenous shunts, and the worst outcomes were associated to brain damage and patent feeders. In addition, antenatal diagnosis and thrombosis of pouch have good prognosis, possibly because the clot may spontaneously obliterate the fistulas. Treatment options must be individualized, and include conservative, embolization, ventriculoperitoneal shunt and endoscopic third ventriculostomy. Although systemic anticoagulation is controverse in infants, it should be considered for patients with sinovenous thrombosis affecting outflow pathways, especially those aggravated by venous congestion. Intervention is recommended for grade III patients because they progress to grade IV, and endovascular treatment is the gold standard therapy due to the risk of fatal intraoperative exsanguination. Whenever possible, embolization should be prioritized over hydrocephalus treatment because it may avoid ventriculoperitoneal shunt. Close follow-up imaging is recommended to detect any changes, with lesion reduction indicating favorable outcome.

Staged embolisation of a giant torcular dural sinus malformation in a neonate

Torcular dural sinus malformations (tDSMs) represent a rare subset of paediatric cerebrovascular malformations and are often diagnosed antenatally via ultrasound. The management of these in utero lesions remains controversial as previous studies suggested elective termination of the pregnancy because of their presumably high mortality and severe long-term morbidity. However, more recent evaluations have suggested that the overall prognosis for infants harbouring these lesions may be much better than previously believed. As such, we present the case of a neonate with a giant tDSM, diagnosed in utero, who was treated postnatally via staged transarterial and transvenous embolisation to alleviate worsening obstructive hydrocephalus and brainstem compression. We provide details regarding the surgical approach and long-term neurological outcomes for this patient. To the best of our knowledge, this is one of the largest reported tDSM presented in the literature.

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

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.

Prenatal findings and postnatal follow-up of a midline dural sinus malformation

Dural sinus malformation is a rare condition. We describe a prenatally detected case followed by repeated ultrasound scans and a prenatal magnetic resonance imaging examination. A substantial spontaneous regression was observed, which is associated with a favorable outcome. We believe that our observations, including a long postnatal follow-up, will add to the present knowledge of prenatally detected cases, and thus improve management of the pregnancies as well as our possibilities to counsel the parents-to-be.

Direct percutaneous transcarotid approach for embolization of dural sinus malformation in a premature neonate: A case report

Dural sinus malformations (DSM) can manifest with high output cardiac failure in neonates. Interventional procedures reduce the effect of shunting on cardiac function. The umbilical artery access (UA) is the preferred route in such cases, but it is not always available. Although transfemoral arterial access (TFA) is feasible, it can be associated with complications including limb ischemia in neonates with low birth weight (LBW) and small size. The Transcarotid arterial (TCAA) approach has been successfully used in pediatric cardiac interventions, however, its use in Pediatric Neurointervention is rare. We report antegrade percutaneous TCAA for embolization of DSM in a premature neonate with BW of 1710 grams and prenatal diagnosis of DSM who was delivered at 32 weeks due to worsening cardiac failure. Due to unavailable UA, embolization was performed via (TFA) at day 2, which was complicated by significant limb ischemia. Recurrent clinical deterioration warranted additional treatment, including 2 further embolizations which were performed via percutaneous TCAA of the left and right common carotid arteries at day 59 and 71 with no immediate or intermediate complications. Conclusions Direct percutaneous TCAA can be a feasible access for intracranial interventions in neonates with LBW and unavailable UA access.