Tomosugi N, Mukai A, Yamaya H, Nakamura M, Asaka M, Ishii H, Takata K, Nakazawa T, Yuri T, Ishikawa I
Department of Nephrology, Kanazawa Medical University, Ishikawa, Japan.
Nihon Jinzo Gakkai Shi. 1995 Jul;37(7):410-6.
The management and screening of unruptured asymptomatic intracranial aneurysm (ICA) in patients with ADPKD and those with a family history of ICA remains a subject of considerable controversy. In recent years, it has been revealed that MRA (magnetic resonance angiography) can define the circle of Willis to allow detection of ICA as small as 3-4 mm. We report a case of a 63-year-old man with ADPKD and his family. No definite aneurysm was observed by angiography screening at 46 years of age, when he was referred for hemodialysis. For the past three years, his family history revealed that three relatives were suffering from subarachnoid hemorrhage at the ages of 32, 36 and 39 years, respectively, two of whom had ICA and one had arterio-venous malformation detected by angiography. Whether they had ADPKD was unknown, but two were suggestive of ADPKD. Therefore, our case underwent MRA as screening for ICA, which showed an ICA with a diameter of 5mm in the anterior communicating artery. The ICA was confirmed as being 6 mm in diameter by conventional angiography. His niece and her son, who had ADPKD, also underwent MRA, which showed a suspicious image of a 2 mm ICA in the latter case. These results suggest that prophylactic screening for ICA is important in an ICA clustering family. MRA is useful in screening for ICA and in the follow-up study on the natural course of ICA.
对于患有常染色体显性多囊肾病（ADPKD）的患者以及有颅内未破裂无症状动脉瘤（ICA）家族史的患者，其管理和筛查仍然是一个颇具争议的话题。近年来，研究发现磁共振血管造影（MRA）能够清晰显示Willis环，从而检测出小至3-4毫米的ICA。我们报告了一例63岁患有ADPKD的男性及其家族的病例。该患者46岁因需要血液透析前来就诊时，血管造影筛查未发现明确的动脉瘤。在过去三年里，其家族病史显示有三名亲属分别在32岁、36岁和39岁时发生蛛网膜下腔出血，其中两人经血管造影检测出患有ICA，一人患有动静脉畸形。他们是否患有ADPKD并不明确，但其中两人疑似患有ADPKD。因此，我们的病例接受了MRA检查以筛查ICA，结果显示前交通动脉有一个直径5毫米的ICA。经传统血管造影证实该ICA直径为6毫米。他患有ADPKD的侄女及其儿子也接受了MRA检查，后者显示有一个2毫米ICA的可疑影像。这些结果表明，在ICA聚集家族中对ICA进行预防性筛查很重要。MRA对于ICA筛查以及ICA自然病程的随访研究很有用。
