Dural sinus malformations (DSMs) are rare congenital malformations that can be midline or lateral in location. Midline DSMs have been reported to have a worse prognosis than lateral DSMs and have traditionally been more difficult to manage. The authors report 2 unusual manifestations of midline DSMs and their management with percutaneous transfontanelle embolization. The first patient (Case 1) presented at 21 days of life with a large midline DSM and multiple highflow dural and pial arteriovenous shunts. The child developed congestive cardiac failure and venous congestion with intracranial hemorrhage and seizures within a few weeks. The second patient (Case 2) presented with a large midline DSM found on prenatal imaging that was determined to be a purely venous malformation on postnatal evaluation. This large malformation resulted in consumptive coagulopathy and apneic episodes from brainstem compression. The patient in Case 1 was treated initially with endovascular embolization and eventually with curative percutaneous-transfontanelle embolization. The patient in Case 2 was treated with percutaneous transfontanelle embolization in combination with posterior fossa decompression and cranial expansion surgery.
Keywords: AV = arteriovenous; CCA = common carotid artery; DSA = digital subtraction angiography; DSM = dural sinus malformation; MMA = middle meningeal artery; MVP = midline venous pouch; NBCA = N-butyl cyanoacrylate; SSS = superior sagittal sinus; VA = vertebral artery; arteriovenous fistula; dural sinus malformation; pediatric arteriovenous malformation; percutaneous embolization; vascular disorders.