Neonatal Giant Dural Sinus Ectasia: A Case Report and Review of the Literature

Abstract

Background: Neonatal giant dural sinus ectasia (DSE) is a rare congenital vascular malformation of the posterior major dural venous sinuses, often presenting as giant venous pouch. Subsequently, thrombosed lesions can progress spontaneously, and the lesions are accompanied by high flow dural arteriovenous fistulas (dAVFs) resulting in high rates of morbidity and mortality.Case Presentation: Our case about full term female neonate who developed respiratory distress, severe cyanosis, and high flow cardiac output failure immediately after birth, was initially mimicking vein of Galen malformation and Ebsten anomaly featured on cranial ultrasound and bedside echocardiogram.Neuroimaging MRI, MRA and MRV findings emphasize the complex giant DSE noted at the torcular herophili with high flow dAVFs, supplied by the middle meningeal and right occipital arteries, which was complicated with venous thrombosis.Then, on day 6 of life, transcranial endovascular embolization was done and intraoperative course and unfortunately was complicated by severe access site hemorrhages and 20 minutes cardiac arrest receiving urgent resuscitation. Then procedure resumed requiring coils and embolic liquid agents, demonstrating reduction in size of shunt by more than 50%. However, post intervention, the patient developed severe metabolic acidosis, and consumptive coagulopathy, bilateral high grade GMHs and stage III acute kidney injury, despite maximal quadruple inotropic support as well as peritoneal dialysis catheter placement. Eventually, on day 9 of life, she succumbed to refractory shock and multiorgan failure.Conclusion: Neonatal DSE with coexisting high flow dural AVFs represents a life-threatening entity competent of precipitating rapid, fatal multiorgan failure despite urgent intervention. Overall, prognosis determined by arteriovenous degree shunting, impairment of venous outflow, and secondary brain parenchyma injury.