The vein of Galen malformation is caused by an abnormal shunting between choroidal arteries and the median prosencephalic vein during embryological development, leading to increased blood flow to the deep cerebral veins, intracranial damage, and systemic repercussions. Idiopathic spontaneous thrombosis of a vein of Galen malformation is rare, and its association with acute sinusitis has not been reported in the literature. We present the case of a girl with a postnatal diagnosis of a vein of Galen malformation at the age of 16 months, with secondary pulmonary hypertension that was adequately controlled with spironolactone. At 3 years old, while expecting elective endovascular treatment, the patient developed spontaneous thrombosis of the vein of Galen malformation, concomitant to an acute sinusitis episode, with complete resolution of the vascular malformation and secondary pulmonary hypertension. The patient continued with normal neurological development over a 5-year follow-up. We discuss the main pathophysiologic mechanisms that can explain spontaneous thrombosis of VOGMs and the patient’s outcome. Awareness of different mechanisms that can lead to spontaneous thrombosis can help in the decision-making process and prompt targeted approaches to individual patients with a vein of Galen malformation.
This is a preview of subscription content, access via your institution.
Buy single article
Instant access to the full article PDF.
Tax calculation will be finalised during checkout.
Subscribe to journal
Immediate online access to all issues from 2019. Subscription will auto renew annually.
Tax calculation will be finalised during checkout.
computerized tomography angiogram
magnetic resonance angiogram
magnetic resonance imaging
magnetic resonance venogram
vein of Galen malformation
Yan J, Wen J, Gopaul R, Zhang CY, Xiao SW (2015) Outcome and complications of endovascular embolization for vein of Galen malformations: a systematic review and meta-analysis. J Neurosurg 123:872–890. https://doi.org/10.3171/2014.12.JNS141249
Tufekci S, Ince Z, Yasa B, Bor M, Barburoglu M, Sencer S, Coban A (2017) Radiological and clinical features of vein of Galen aneurysmal malformation in newborn infants and, the results of endovascular interventional treatment: 10-years experience. Brain Disord Ther 06: https://doi.org/10.4172/2168-975x.1000234
Gopalan V, Rennie A, Robertson F, Kanagarajah L, Toolis C, Bhate S, Ganesan V (2018) Presentation, course, and outcome of postneonatal presentations of vein of Galen malformation: a large, single-institution case series. Dev Med Child Neurol 60:424–429. https://doi.org/10.1111/dmcn.13676
Duran D, Zeng X, Jin SC, et al (2019) Mutations in chromatin modifier and ephrin signaling genes in vein of Galen malformation. Neuron 101:429-443.e4. https://doi.org/10.1016/j.neuron.2018.11.041
Lasjaunias PL, Chng SM, Sachet M, Alvarez H, Rodesch G, Garcia-Monaco R (2006) The management of vein of Galen aneurysmal malformations. Neurosurgery 59:184–194. https://doi.org/10.1227/01.NEU.0000237445.39514.16
See AP, Orbach DB (2017) Vein of Galen arteriovenous malformations. Prim Cerebrovasc Dis Second Ed 482–487. https://doi.org/10.1016/B978-0-12-803058-5.00097-7
Kariyappa K, Krishnaswami M, Gnanaprakasam F et al (2016) Spontaneous thrombosis of vein of Galen malformation. J Pediatr Neurosci 11:264–266. https://doi.org/10.4103/1817-1745.193377
Recinos PF, Rahmathulla G, Pearl M, Recinos VR, Jallo GI, Gailloud P, Ahn ES (2012) Vein of Galen malformations: epidemiology, clinical presentations, management. Neurosurg Clin N Am 23:165–177. https://doi.org/10.1016/j.nec.2011.09.006
Wagner KM, Ghali MGZ, Srinivasan VM, Lam S, Johnson J, Chen S, Kan P (2019) Vein of Galen Malformations: The Texas Children’s Hospital experience in the modern endovascular era. Oper Neurosurg 17:286–292. https://doi.org/10.1093/ons/opy369
Yan J, Gopaul R, Wen J, Li XS, Tang JF (2017) The natural progression of VGAMs and the need for urgent medical attention: a systematic review and meta-analysis. J Neurointerv Surg 9:564–570. https://doi.org/10.1136/neurintsurg-2015-012212
Berenstein A, Paramasivam S, Sorscher M, Molofsky W, Meila D, Ghatan S (2019) Vein of Galen aneurysmal malformation: advances in management and endovascular treatment. Clin Neurosurg 84:469–478. https://doi.org/10.1093/neuros/nyy100
Hansen D, Kan PT, Reddy GD et al (2016) Pediatric knowledge update: approach to the management of vein of Galen aneurysmal malformations in neonates. Surg Neurol Int 7:S317–S321. https://doi.org/10.4103/2152-7806.182415
Jain NK, Swaika S, Thomas B, Kesavadas C, Kapilamoorthy TR (2015) Varying clinical and imaging outcomes in patients with spontaneous thrombosis of vein of Galen malformation—a report of two cases. Child’s Nerv Syst 31:809–813. https://doi.org/10.1007/s00381-014-2612-3
Collins JJ, Fisher WS (1990) Vein of galen aneurysm presenting with recurrent aseptic meningitis and subsequent spontaneous thrombosis. Surg Neurol 33:325–328. https://doi.org/10.1016/0090-3019(90)90200-9
Kuzeyli K, Çakir E, Karaarslan G, Ahmetoğlu A, Peksoylu B, Yazar U, Baykal S (2004) Spontaneous thrombosis of vein of Galen aneurysmal malformation after ventriculoperitoneal shunting. J Clin Neurosci 11:439–442. https://doi.org/10.1016/j.jocn.2003.03.008
Irfan M, Lohman B, McKinney AM (2009) Confirmation of T1-bright vein of Galen aneurysm spontaneous thrombosis by subtraction magnetic resonance venography. Acta radiol 50:812–815. https://doi.org/10.1080/02841850903055611
Shah QA, Qureshi AI (2011) Spontaneous thrombosis of a large vein of Galen malformation. J Neuroimaging 21:87–88. https://doi.org/10.1111/j.1552-6569.2009.00397.x
Konus OL, Ilgit ET, Ozdemir A, Onal B (2000) Spontaneous thrombosis of a vein of Galen aneurysmal malformation: possible effects of contrast media. Eur Radiol. 10:1456–1458. https://doi.org/10.1007/s003300000353
Abrão GP, Barbosa LA, Sá-Junior AT, Caldas JGM (2010) Spontaneous thrombosis of a malformation of the vein of Galen. Arq Neuropsiquiatr 68:648–650. https://doi.org/10.1590/s0004-282×2010000400032
Konovalov AN, Pitskhelauri DI, Arutiounov NV (2002) Surgical treatment of the thrombosed vein of Galen aneurysm. Acta Neurochir (Wien) 144:909–915. https://doi.org/10.1007/s00701-002-0976-8
Nikas DC, Proctor MR, Scott RM (1999) Galen aneurysmal malformation. 02115:33–39
Mohanty C, Srinivas D, Sampath S (2016) Spontaneous thrombosis of a vein of galen malformation. Asian J Neurosurg 11:74. https://doi.org/10.4103/1793-5482.145166
Geng B, Wu X, Malhotra A (2020) Septic cavernous sinus thrombosis — case series and review of the literature
Szyfter W, Bartochowska A, Borucki Ł, Maciejewski A, Kruk-Zagajewska A (2018) Simultaneous treatment of intracranial complications of paranasal sinusitis. Eur Arch Oto-Rhino-Laryngology 0:0. https://doi.org/10.1007/s00405-018-4932-5
Chen MC, Ho YH, Chong PN, Chen JH (2019) A rare case of septic cavernous sinus thrombosis as a complication of sphenoid sinusitis. Tzu Chi Med J 31:63–65. https://doi.org/10.4103/tcmj.tcmj_1_18
Ziegler A, Patadia M, Stankiewicz J (2018) Neurological complications of acute and chronic sinusitis. 1–8
Beristain-Covarrubias N, Perez-Toledo M, Thomas MR, Henderson IR, Watson SP, Cunningham AF (2019) Understanding infection-induced thrombosis: lessons learned from animal models. Front Immunol 10:. https://doi.org/10.3389/fimmu.2019.02569
Moftakhar P, Danielpour M, Maya M, Alexander MJ (2009) Spontaneous thrombosis of neonatal vein of Galen malformation. Neurosurg Focus 27:2–7. https://doi.org/10.3171/2009.8.FOCUS09162
Mahmoodi R, Habibi Z, Heidari V, Nejat F (2016) Spontaneous regression and complete disappearance of the vein of Galen aneurysmal malformation. Child’s Nerv Syst 32:593–598. https://doi.org/10.1007/s00381-016-3033-2
Lasjaunias P, Ter Brugge KG, Berenstein A (2006) Surgical neuroangiography: Clinical and interventional aspects in children
Brinjikji W, Krings T, Murad MH, Rouchaud A, Meila D (2017) Endovascular treatment of vein of Galen malformations: a systematic review and meta-Analysis. Am J Neuroradiol 38:2308–2314. https://doi.org/10.3174/ajnr.A5403
Lecce F, Robertson F, Rennie A, Heuchan AM, Lister P, Bhate S, Bhattacharya J, Brew S, Kanagarajah L, Kuczynski A, Peters MJ, Ridout D, Schmitt A, Toolis C, Vargha-Khadem F, Ganesan V (2018) Cross-sectional study of a United Kingdom cohort of neonatal vein of galen malformation. Ann Neurol 84:547–555. https://doi.org/10.1002/ana.25316
The patient’s legal guardians granted written informed consent for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Conflict of interest
The authors declare no competing conflicts of interest
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
About this article
Cite this article
Pulido, L., Murcia Salazar, D., Gómez Amarillo, D. et al. Spontaneous thrombosis of a vein of Galen malformation associated with acute sinusitis: a case report. Childs Nerv Syst (2021). https://doi.org/10.1007/s00381-021-05072-4
- Vein of Galen malformation
- Arteriovenous malformation
- Spontaneous thrombosis
- Acute sinusitis
- Pulmonary hypertension
- Case report