Abstract
A newborn girl displayed sudden onset paresis of both legs at 4 weeks of age. An initial MRI of the spine revealed intramedullary bleeding at the level of the conus medullaris. Emergency surgery to evacuate the spinal cord hematoma was performed, and tortuous dilated intraspinal perimedullary vessels were noted. After surgery, the right leg weakness persisted, but the strength in the left leg improved. Initial catheter angiography workup at 3 months of age revealed a perimedullary arteriovenous fistula with associated varix at the level of the conus medullaris with radiculomedullary arterial feeders from the segmental arteries Th11 left and L4 left as well as a paraspinal arteriovenous malformation located at the lumbosacral junction with arterial feeders from the segmental arteries L4 right and left. Endovascular glue embolization (nBCA/ethiodized oil) of the perimedullary arteriovenous fistula was performed at 8 months (L4 left) and 9 months (Th11 left) of age with complete obliteration of the perimedullary arteriovenous shunt. The paraspinal AVM was successfully treated with glue embolization (nBCA/ethiodized oil) in two sessions at 15 and 18 months. On the last DSA follow-up at 18 months of age, there was no evidence for any recurrent intraspinal arteriovenous shunt, and the anterior spinal artery caliber had normalized. The child can now walk and run with no assistance. Neurogenic bladder issues have, however, been present since the spinal cord bleed. This case illustrates a rare combination of intra- and extraspinal vascular pathologies in a newborn. The clinical benefit of early identification and treatment of the intraspinal arteriovenous fistula at such an age – even after bleeding – is the main topic of this chapter.
References
Anson JA, Spetzler RF. Classification of spinal arteriovenous malformations and implications for treatment. BNI Q. 1992;8:2–8.
Bao YH, Ling F. Classification and therapeutic modalities of spinal vascular malformations in 80 patients. Neurosurgery. 1997;40(1):75–81. https://doi.org/10.1097/00006123-199701000-00017.
Barrow DL, Colohan AR, Dawson R. Intradural perimedullary arteriovenous fistulas (type IV spinal cord arteriovenous malformations). J Neurosurg. 1994;81(2):221–9. https://doi.org/10.3171/jns.1994.81.2.0221.
Brinjikji W, Nasr DM, Cloft HJ, Iyer VN, Lanzino G. Spinal arteriovenous fistulae in patients with hereditary hemorrhagic telangiectasia: a case report and systematic review of the literature. Interv Neuroradiol. 2016;22(3):354–61. https://doi.org/10.1177/1591019915623560.
Cullen S, Alvarez H, Rodesch G, Lasjaunias P. Spinal arteriovenous shunts presenting before 2 years of age: analysis of 13 cases. Childs Nerv Syst. 2006;22(9):1103–10. https://doi.org/10.1007/s00381-006-0075-x.
Djindjian R. Spinal vascular malformations. J Neurosurg. 1976;45(6):727–8. https://doi.org/10.3171/jns.1976.45.6.0727. PMID: 978247.
Djindjian M, Djindjian R, Rey A, Hurth M, Houdart R. Intradural extramedullary spinal arterio-venous malformations fed by the anterior spinal artery. Surg Neurol. 1977;8(2):85–93.
Gueguen B, Merland JJ, Riche MC, Rey A. Vascular malformations of the spinal cord: intrathecal perimedullary arteriovenous fistulas fed by medullary arteries. Neurology. 1987;37(6):969–79. https://doi.org/10.1212/wnl.37.6.969.
Heros RC, Debrun GM, Ojemann RG, Lasjaunias PL, Naessens PJ. Direct spinal arteriovenous fistula: a new type of spinal AVM. Case report. J Neurosurg. 1986;64(1):134–9. https://doi.org/10.3171/jns.1986.64.1.0134.
Kenning TJ, Deshaies EM, Adamo MA, Waldman JB, Boulos AS. Onyx embolization of a thoracolumbar perimedullary spinal arteriovenous fistula in an infant presenting with subarachnoid and intraventricular hemorrhage. J Neurosurg Pediatr. 2009;3(3):211–4. https://doi.org/10.3171/2008.12.PEDS0870.
Li J, Li G, Bian L, Hong T, Yu J, Zhang H, Ling F. Concomitant lumbosacral perimedullary arteriovenous fistula and spinal dural arteriovenous fistula. World Neurosurg. 2017;105:1041.e7–1041.e14. https://doi.org/10.1016/j.wneu.2017.06.149.
Li J, Zeng G, Zhi X, Bian L, Yang F, Du J, Ling F, Zhang H. Pediatric perimedullary arteriovenous fistula: clinical features and endovascular treatments. J Neurointerv Surg. 2019;11(4):411–5. https://doi.org/10.1136/neurintsurg-2018-014184.
Matushita H, Caldas JG, Texeira MJ. Perimedullary arteriovenous fistulas in children: report on six cases. Childs Nerv Syst. 2012;28(2):253–64. https://doi.org/10.1007/s00381-011-1566-y. Epub 2011 Sep 6. PMID: 21894560.
Morgan MK, Chaseling R, Johnston I, de Silva M. Spinal arteriovenous malformation presenting at birth: case report. Neurosurgery. 1986;19(4):637–40. https://doi.org/10.1227/00006123-198610000-00024.
Mourier KL, Gobin YP, George B, Lot G, Merland JJ. Intradural perimedullary arteriovenous fistulae: results of surgical and endovascular treatment in a series of 35 cases. Neurosurgery. 1993;32(6):885–91; discussion 891. https://doi.org/10.1227/00006123-199306000-00001.
Park TS, Cail WS, Delashaw JB, Kattwinkel J. Spinal cord arteriovenous malformation in a neonate. Case report. J Neurosurg. 1986;64(2):322–4. https://doi.org/10.3171/jns.1986.64.2.0322.
Rodesch G, Hurth M, Alvarez H, Ducot B, Tadie M, Lasjaunias P. Angio-architecture of spinal cord arteriovenous shunts at presentation. Clinical correlations in adults and children. The Bicêtre experience on 155 consecutive patients seen between 1981-1999. Acta Neurochir. 2004;146(3):217–26; discussion 226-7. https://doi.org/10.1007/s00701-003-0192-1.
Sasamori T, Hida K, Yano S, Asano T, Iwasaki Y. Cervical perimedullary arteriovenous fistula in an infant presenting with subarachnoid hemorrhage—case report. Neurol Med Chir (Tokyo). 2008;48(9):409–13. https://doi.org/10.2176/nmc.48.409.
Spetzler RF, Detwiler PW, Riina HA, Porter RW. Modified classification of spinal cord vascular lesions. J Neurosurg. 2002;96(2 Suppl):145–56. https://doi.org/10.3171/spi.2002.96.2.0145.
Takahashi H, Morita A, Ishijima B, Kubota M, Nemoto S. An infant case of spinal arteriovenous malformation with a large venous aneurysm. No ShinkeiGeka. 1992;20(4):509–14.
Wendl CM, Aguilar Pérez M, Felber S, Stroszczynski C, Bäzner H, Henkes H. Paraspinal arteriovenous fistula: Stuttgart classification based on experience and a review of the literature. Br J Radiol. 2018;91(1088):20170337. https://doi.org/10.1259/bjr.20170337.
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El Mekabaty, A., Hellstern, V., Serna Candel, C., Schlaffer, S., Henkes, H. (2021). Perimedullary Arteriovenous Fistula: Ruptured Perimedullary Arteriovenous Fistula Associated with an Incidental Paraspinal Arteriovenous Malformation (AVM) in a New Born; Endovascular Cure by Staged nBCA Embolization. In: Henkes, H., Lylyk, P., Ganslandt, O. (eds) The Arteriovenous Malformations and Fistulas Casebook. Springer, Cham. https://doi.org/10.1007/978-3-030-51200-2_9-1
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