Abstract
Background
Spinal arteriovenous metameric syndrome (SAMS) is a combination of more than two separate vascular malformations in the same embryonic metameres. This syndrome, also known as Cobb syndrome, is rare, especially in the neonate.
Case description
A neonatal girl with a birthmark in the occipital and posterior nuchal regions presented with severe heart failure on the day of birth. The large arteriovenous fistulas in the left hypoglossal canal and in the posterior nuchal region were embolized with detachable coils on the postnatal days 5 and 18, which improved heart failure markedly. The associated intramuscular arteriovenous malformation in the posterior neck was left untreated because large arteriovenous fistulas had been occluded. She grew up without any neurological deficits and developed with normal milestones until the latest follow-up of 8 years old.
Conclusion
To our knowledge, this is the first case with SAMS in a neonate presenting with congestive heart failure. Presence of a birthmark in a neonate presenting with congestive heart failure may suggest the possible underlying high-flow vascular malformations in the same metamere.
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References
Brant AJ, James HE, Tung H (1995) Cutaneomeningospinal angiomatosis (Cobb syndrome) with tethered cord. Pediatr Neurosurg 30:93–95
Cobb S (1915) Heamangioma of the spinal cord associated with skin naevi of the same metamere. Ann Surg 65:641–649
Ernst R, Bulas R, Tomsick T, Loveren HV, Aziz KA (1999) Three cases of dural arteriovenous fistula of the anterior condylar vein within the hypoglossal canal. AJNR Am J Neuroradiol 20:2016–2020
Jessen RT, Thompson S, Smith EB (1977) Cobb syndrome. Arch Dermatol 113:1587–1590
Kassarjian A, Zurakowski D, Dubois J, Paltiel HJ, Fishman SJ, Burrows PE (2004) Infantile hepatic hemangiomas: clinical and imaging findings and their correlation with therapy. AJR Am J Roentogenol 182:785–795
Kissel P, Dureux JB (1972) Cobb syndrome. Cutaneomeningospinal angiomatosis. In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology: the phacomatoses, vol 14. North-Holland, Amsterdam, pp 429–445
Lasjaunias PL, Alvarez H, Rodesch G, Garcia-Monaco R, Ter Brugge K, Burrows P, Taylor W (1996) Aneurysmal malformations of the vein of Galen. Follow-up of 120 children treated between 1984 and 1994. Interv Neuroradiol 2:15–26
Maramattom BV, Cohen-Gadol AA, Wijdicks EFM, Kallmes D (2005) Segmental cutaneous hemangioma and spinal arteriovenous malformation (Cobb syndrome). Case report and historical perspective. J Neurosurg Spine 3:249–252
Matsumaru Y, Pongpech S, Laothamas J, Alvarez H, Rodesch G, Lasjaunias P (1999) Multifocal and metameric spinal cord arteriovenous malformations. Review of 19 cases. Interv Neuroradiol 5:27–34
Miyatame S, Kikuchi H, Koide T, Yamagata S, Nagata I, Minami S, Asato R (1990) Cobb’s syndrome and its treatment with embolization. Case report. J Neurosurg 72:497–499
Mulliken JB, Glowacki J (1982) Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg 69:412–420
Niimi Y, Uchiyama N, Elijovich L, Berenstein A (2013) Spinal arteriovenous metameric syndrome: clinical manifestations and endovascular management. AJNR Am J Neuroradiol 34:457–463
Pascual-Castroviejo I, Frutos R, Viano J, Pascual-Pascual SI, Gonzalez P (2002) Cobb syndrome. J Child Neurol 17:847–849
Rodesch G, Hurth M, Alvarez H, Tadie M, Lasjaunias P (2002) Classification of spinal cord arteriovenous shunts: proposal for a reappraisal—the Bicetre experience with 155 consecutive patients treated between 1981 and 1999. Neurosurgery 51:374–380
Soeda A, Sakai N, Iihara K, Nagata I (2003) Cobb syndrome in an infant: treatment with endovascular embolization and corticosteroid therapy: case report. Neurosurgery 52:711–715
Wakabayashi Y, Isono M, Shimomura T, Tajima A, Terashi H, Asada Y, Izumi T, Hori S (2000) Neurocutaneous vascular hamartomas mimicking Cobb syndrome. Case report. J Neurosurg (Spine 1) 93:133–136
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Komiyama, M., Ishiguro, T., Terada, A. et al. Spinal arteriovenous metameric syndrome in a neonate presenting with congestive heart failure: case report. Childs Nerv Syst 30, 1607–1611 (2014). https://doi.org/10.1007/s00381-014-2439-y
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DOI: https://doi.org/10.1007/s00381-014-2439-y