大きなVenous Aneurysmを伴った幼児脊髄動静脈奇形の1例

An Infant Case of Spinal Arteriovenous Malformation with a large venous aneurysm

I．はじめに

　小児期あるいは乳幼児期の脊髄動静脈奇形は非常に稀であるとされている^11）．Raimondi^15）は彼の著書“Pediat—ric Neurosurgery”においてarteriovenous malforma—tions of the spinal cordの項を設けているが，その中でこの疾患は幼小児では大変少ないので手術法に関してはあえて言及しないと記している程である．われわれは最近急激に発症し大きなvenous aneurysmを伴う1歳8カ月男子の脊髄動静脈痩例を治療する機会得た．非常に珍しい症例であると考えられるのでここに報告する．

An 18-month-old boy was admitted to our hospital with suddenfonset of paraplegia, analgesia of the lower limbs, dysuria and constipation. His gestational and birth histories were unremarkable. Past history re-vealed he had lymphangioma in his left inguinal region, and had been treated in another hospital. Neurological examination revealed flaccid paraplegia, analgesia be-low Th12 dermatome and dysuria.

MRI revealed an intramedullary high intensity lesion surrounded by round low intensity areas located from Thl 1 to L2 vertebral levels, suggesting the existence of vascular tumor or spinal AVM. Spinal angiogram re-vealed arteriovenous fistula with large intramedullary aneurysmal vascular dilatation from T12 to L2 verte-bral level. The feeder was the Adamkiewicz artery which branched from the left Th12 intercostal artery. First, artificial embolization with thrombin gelf oam was performed successfully. However, follow-up MRI showed an image of flow void in the aneurysm again, indicating recanalization of the AVF. Therefore, an op-eration was undertaken on October 24th, 1988. The pa-tient was placed in prone position and osteoplastic lami-notomy from Th1O-L2 was performed. The thrombus and wall of the aneurysm were mostly removedthrough the lumbosacral midline myelotomy for decom-pression. Then, the feeder and drainers were ligated. Postoperative course was uneventful. 2.5 years after the operation, he still had flaccid paralysis at the ankle joints bilaterally, analgesia below 1-4 dermatome, neurogenic bladder and constipation. He is trying towalk with leg braces but, so far, he has not succeeded. Spinal AVMs are very rare in an infant less than 2 years of age and we collected only twelve such cases from past reports. Discussions were held on the pathophysiology and treatment of infant spinal AVMs.