Komiyama Masaki, Ishiguro Tomoya, Terada Aiko, Watanabe Yusuke, Nakajima Hideki, Ohata Yuuki, Matsusaka Yasuhiro
Department of Neuro-Intervention, Osaka City General Hospital, 2-13-22, Miyakojima-Hondori, Miyakojima, Osaka, 534-0021, Japan,
Childs Nerv Syst. 2014 Sep;30(9):1607-11. doi: 10.1007/s00381-014-2439-y. Epub 2014 May 21.
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.
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.
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.
脊髓动静脉节段性综合征(SAMS)是指在同一胚胎节段中存在两个以上独立的血管畸形。该综合征也称为科布综合征,较为罕见,尤其是在新生儿中。
一名枕部和项后部有胎记的新生儿女孩在出生当天出现严重心力衰竭。在出生后第5天和第18天,分别用可脱卸弹簧圈栓塞了左侧舌下神经管和项后部的大型动静脉瘘,心力衰竭明显改善。由于大型动静脉瘘已被闭塞,后颈部相关的肌肉内动静脉畸形未予治疗。她成长过程中没有任何神经功能缺损,发育正常,直至8岁的最新随访。
据我们所知,这是首例患有SAMS且表现为充血性心力衰竭的新生儿病例。患有充血性心力衰竭的新生儿出现胎记可能提示同一节段存在潜在的高流量血管畸形。