台北での学会
４月６日から８日まで、台北であったアジア・オーストラリア脳神経血管内治療学会に参加してきました．正式学会名は、The 8th Meeting of the Asian-Australasian Federation of the Interventional and Therapeutic Neuroradiology (AAFITN2008)です．
私は、遺伝性出血性毛細血管拡張症（HHT）を発表してきました．下にそのproceedingを載せます．参加者は、日本にもこの疾患がこんなにあるんだ、といった印象を持ったようです．
発表の趣旨は、
１．日本にもHHTの患者さんが多くいる．
２．肺の動静脈瘻は、治療すべきであり、特に栄養動脈の径が3 mm以上あるとそうである．
３．脳の動静脈瘻・奇形には、micro-AVM, small AVM, AVFの３つのタイプがあるが、その治療適応にコンセンサスはない．
４．しかし、small AVMとAVFは是非、治療した方がいいであろう．
５． micro-AVMが問題であるが、ガンマナイフ等、定位放射線のリスクは低く、積極的に治療を行ってもいいであろう．
６．消化管出血や鼻血の重症例には、積極的な輸血治療が必要なことがある．
７．HHTでは、個々の病変を診るのではなく、この疾患を持つ患者自身を診る必要があるであろう．
８．脳神経血管内治療医の果たす役割は大きい．
Hereditary Hemorrhagic Telangiectasia (HHT): the Roles of Neuro-Interventionalists for Its Management
Masaki Komiyama, Tomoya Ishiguro
Department of Neurosurgery, Osaka City General Hospital, Osaka, Japan
Background: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with at least two known gene mutations, i.e., endoglin and ALK-1, which work on TGF-beta in angiogenesis. Clinical presentation includes nosebleed, muco-cutaneous telangiectases, GI bleeding, and visceral AVM/Fs including brain, spinal cord, lung, and liver. Neurological symptoms are caused by cerebral/spinal AVM/Fs, and paradoxical embolism through pulmonary AV shunts. We report our experiences in the management of the HHT patients
Materials and Methods: There were 8 males and 8 females (age 2 to 76) in 10 HHT families. All patient were diagnosed as definite HHT by Curacao criteria. Their symptoms included nosebleed (16), brain abscess (1), TIA or cerebral infarction (5), cerebral hemorrhage (3), spinal hemorrhage (1), respiratory failure (2), and GI bleeding (3). Cerebral AVF/Ms (5) were treated by surgery (2), embolization (3) and/or gamma knife (2). Pulmonary AVFs were actively embolized for prevention of neuro-complications in 10 or for control the respiratory failure in 2. Many other family members were interviewed and underwent brain MR and lung CT for screening, and were denied HHT, thus not included in this study.
Results: Acute brain hemorrhage (2) and brain abscess (1) required surgery. Two cerebral AVF/Ms were occluded by embolization. (Figure 1). Some cerebral micro-AVMs (< 1 cm) were occluded by gamma knife surgery. Pulmonary AVFs were occluded with coils as many as possible. (Figure 2). Spinal micro-AVM was conservatively managed. One patient with chronic GI bleeding (Hb < 7.0) was managed with repeated blood transfusion. All nosebleeds were managed conservatively without intervention. One neuro-complication occurred during embolization for pulmonary AVFs, resulting in 1/4 visual field defect.
Conclusion: Neuro-interventionalists should be familiar with many clinical aspects of HHT, and have to play important roles in its management.
せっかくですので、台北の写真を載せます．学会での写真を撮るのは忘れていました．
夜市という夜店 街角で: たこ紐での産毛取り
2008.4.11記