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延遲診斷之脊髓動靜脈畸形及其泌尿系統併發症之處理:病例報告

Delayed Diagnosis of Spinal Arteriovenous Malformation and Management of Its Urological Complication: A Case Report

摘要


脊髓動靜脈畸形爲一罕見的疾病,其發現率依文獻報告推估應該每年每百萬人中僅有1人會被發現。因爲其表現多變,故即使依靠臨床症狀加上影像檢查,仍不易診斷。另外,脊髓動靜脈畸形亦可能造成神經性膀胱功能障礙,但僅有極少數病患會早期出現膀胱輸尿管逆流情形。 本篇報告一位女童,於12歲時因出現四肢完全無力及呼吸困難被診斷爲高位頸髓之脊髓動靜脈畸形。經檢查發現其解尿時膀胱內壓極高,且左邊腎臟短時間內就出現第二級膀胱輸尿管逆流,而這一般被認爲應該是脊髓損傷後的慢性併發症。回溯其病史,發現她在9歲時曾經有顱內蜘蛛膜下腔出血,但並未發現顱內血管構造異常,且當時並未有明確診斷。從當時起,就出現泌尿道症狀,並自12歲時症狀持續惡化。因爲當時並未針對此症狀作評估或治療,所以或許其已經有神經性膀胱及解尿時高膀胱內壓一段時間,進而導致輸尿管逆流。經放置留置性導尿管及給予抗膽鹼藥物後,追蹤發現其膀胱內壓明顯降低,且膀胱輸尿管逆流消失。 此病例報告呈現脊髓動靜脈畸形之臨床特徵,並希望能提醒醫療人員在發現病患有顱內蜘蛛膜下腔出血,且未發現顱內病灶時,應考慮脊髓動靜脈畸形的可能性。此外,我們亦針對脊髓動靜脈畸形後可能導致之神經性膀胱及其處理提出討論。

並列摘要


Spinal arteriovenous malformations (Spinal AVM) are a rare disorder with an estimated incidence of about 1 per million people per year. Because of the diversity of the presenting symptoms, this disorder is difficult to diagnose, despite new advances in neuronimaging. We report the case of a girl who was diagnosed at 12 years old to have spinal AVM at a high cervical level and presented with four limbs weakness and dyspnea. She was found to have extremely high voiding pressure and a rapid development of grade 2 vesicoureteral reflux (VUR), which was thought to be a chronic complication after spinal cord injury. Review of her history revealed that she had experienced a previous episode of intracranial subarachnoid hemorrhage at the age of 9 with negative intracranial angiography; no definite diagnosis was given. Urologic symptoms had been noted since then, and they became exacerbated one year before her definite diagnosis. No evaluation or treatment was given for this problem, and we speculate that she may have had neurogenic bladder with high voiding pressure for a long time, which resulted in VUR. We prescribed an indwelling catheter with an oral anticholinergic agent to relieve the high intravesical pressure. A subsequent videourodynamic study showed decreased intravesical pressure and the elimination of VUR. This article presents the clinical characteristics of spinal AVM in a single patient and seeks to remind physicians that spinal AVM should be included in the differential diagnosis for patients who have had intracranial subarachnoid hemorrhage and negative intracranial angiography. In addition, we discuss neurogenic bladder dysfunction after spinal AVM and its management.

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