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.
Main Subjects
License
This work is licensed under a Creative Commons Attribution 4.0 International license
References
- Jenny, B., Zérah, M., Swift, D., Tohic, L. A., Merzoug, V., Alvarez, H., Grangé, G., & Rilliet, B. (2010). Giant dural venous sinus ectasia in neonates. Journal of neurosurgery. Pediatrics, 5 5, 523-8. https://doi.org/10.3171/2009.12.peds0862
- Hanai, S., Muroi, A., Hitaka, D., Murakami, T., Sato, M., Matsumaru, Y., & Ishikawa, E. (2023). Remodeling of venous drainage after the treatment of dural sinus malformation with arteriovenous shunts in a neonate: a case report and literature review. Child's Nervous System, 39, 2245-2249. https://doi.org/10.1007/s00381-023-05961-w
- Demartini, Z., Maeda, A., Koppe, G., Da Rocha Guimarães, R. M., Gatto, L. M. M., & Cardoso-Demartini, A. (2022). Torcular Dural Sinus Malformation. Pediatric Neurosurgery, 57, 141 - 143. https://doi.org/10.1159/000521935
- Santos, R., Wing, S., & Martinez, M. L. (2025). Dural Sinus Malformation Involving a Persistent Transtentorial Sinus: A Rare Variant. Journal of Child Neurology, 41, 587 - 591. https://doi.org/10.1177/08830738251376737
- Zuniega, R. R. A., Santos, J. A., Galsim, R. J., & Elevazo, J. S. (2021). Neonatal giant dural sinus ectasia: a multimodality imaging approach. BMJ Case Reports, 14. https://doi.org/10.1136/bcr-2021-242439
- Kozłowska, Z., Komasińska, P., Steinborn, B., Toboła-Wróbel, K., Pietryga, M., Szymankiewicz-Bręborowicz, M., Szczapa, T., & Bekiesińska-Figatowska, M. (2022). Spontaneous Resolution of Congenital Dural Venous Sinus Ectasia Associated With Polymicrogyria—Case Report. Frontiers in Pediatrics, 10. https://doi.org/10.3389/fped.2022.822551
- Aziz, A. F. A., & Stritzke, A. (2018). Giant transient dural sinus dilatation with neonatal nuchal cord.. Pediatrics and neonatology, 60 3, 346-347. https://doi.org/10.1016/j.pedneo.2018.08.001
- Haan, T. D., Padberg, R., Hagebeuk, E., Aronica, E., Rijn, R. R., Majoie, C., & Kok, J. (2007). A case of neonatal dural sinus malformation: clinical symptoms, imaging and neuropathological investigations.. European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society, 12 1, 41-5. https://doi.org/10.1016/j.ejpn.2007.04.003
- Jones, J., Christie, J., Woodbury, S., & Mizia, K. (2021). Fetal dural sinus malformation: A case report and discussion of the literature. Australasian Journal of Ultrasound in Medicine, 24. https://doi.org/10.1002/ajum.12285
- Yankelevich, D. N. G., Tavares, L. S., Aragão, M., Pinho, R. S., & Marx, C. (2024). Fetal dural sinus thrombosis: case report and management review. Arquivos de NeuroPsiquiatria. https://doi.org/10.1055/s-0045-1807110
- Komiyama, M., Matsusaka, Y., Ishiguro, T., Kitano, S., & Sakamoto, H. (2004). Endovascular treatment of dural sinus malformation with arteriovenous shunt in a low birth weight neonate--case report.. Neurologia medico-chirurgica, 44 12, 655-9. https://doi.org/10.2176/nmc.44.655
- Barbosa, M., Mahadevan, J., Weon, Y., Yoshida, Y., Ozanne, A., Rodesch, G., Alvarez, H., & Lasjaunias, P. (2003). Dural Sinus Malformations (DSM) with Giant Lakes, in Neonates and Infants. Interventional Neuroradiology, 9, 407 - 424. https://doi.org/10.1177/159101990300900413
- Luther, E., Jamshidi, A., King, H., & Starke, R. (2021). Staged embolisation of a giant torcular dural sinus malformation in a neonate. BMJ Case Reports, 14. https://doi.org/10.1136/bcr-2021-244216
- Komiyama, M., Matsusaka, Y., Ishiguro, T., Kitano, S., & Sakamoto, H. (2004). Endovascular treatment of dural sinus malformation with arteriovenous shunt in a low birth weight neonate--case report.. Neurologia medico-chirurgica, 44 12, 655-9. https://doi.org/10.2176/nmc.44.655
- Requejo, F., Tcherbbis, V., González, M. L., Argañaraz, R., Marelli, J., & Mantese, B. (2019). Dural sinus malformation with giant pouch (DSMGP): symptoms and treatment. Child's Nervous System, 36, 343-348. https://doi.org/10.1007/s00381-019-04338-2
- Shah, M., Murthy, B., Khurana, A., Chatterjee, M., Jain, M., & Raut, A. (2021). Antenatal dural sinus malformation of torcular Herophili and straight sinus with unusual outcome of hyperdynamic circulation and cardiac failure. Ultrasound in Obstetrics & Gynecology, 58. https://doi.org/10.1002/uog.23597
- Crespo, M., Candela, R. C., Gopegui, R., Roldán, J., & Córdoba, O. (2019). EP08.07: Dural sinus malformation: case report. Ultrasound in Obstetrics & Gynecology, 54. https://doi.org/10.1002/uog.21259
- Saliou, G., Deiva, K., Möhlenbruch, M., & Lubicz, B. (2023). Anticoagulation helps shrink giant venous lakes and arteriovenous fistulas in dural sinus malformation. Journal of NeuroInterventional Surgery, 16, 183 - 186. https://doi.org/10.1136/jnis-2022-019923
- Peper, C., Iwanaga, J., Dumont, A., & Tubbs, R. (2021). Giant dural arteriovenous fistula in an infant. Anatomy & Cell Biology, 54, 518 - 521. https://doi.org/10.5115/acb.21.083

