結節性硬化症伴隨著腎臟血管肌皮脂瘤破裂 所引起之致命性出血

Fatal Hemorrhage Due to Ruptured Renal Angiomyolipoma in Patients with Tuberous Sclerosis Complex

一位已被診斷為結節性硬化症的二十五歲男性病人伴隨著有多發性的臉部疣狀結節。孩童時期有多次的癲癇發作及智力發展遲緩。因右部腰痛及肉眼可見的血尿,被送進了急診部門。血液常規分析顯現出血紅素為6.2gm/dl的貧血，因此緊接地給予輸血。腹部電腦斷層顯現出於右側腎臟有一個約13x21x24公分的巨大血管肌皮脂瘤並伴隨著有數顆小血管肌皮脂瘤於左腎。在這巨大血管肌皮脂瘤下方可發現多處聚集的血塊及出血。右腎血管攝影顯現出彎曲及擴張的血管並伴隨局部的血管瘤及活動性的出血。雖經緊急的經動脈栓塞術，但個案病情在二小時內仍持續惡化。在經插置氣管內管後，家屬拒絕心肺復甦，個案於數小時後死亡。

A 25-year-old male had been diagnosed to have tuberous sclerosis complex (TSC) with multiple wart-like nodules on his face. He had previous episodes of seizure during childhood and was mentally retarded. He was admitted to our emergency room due to right flank pain and gross hematuria. Routine blood examination revealed anemia with a hemoglobin level of 6.2gm/dl, therefore blood transfusion was given. CT scan of the abdomen and pelvis showed huge angiomyolipoma (13 X 21 X 24 cm) on the right kidney and smaller lesions were depicted on the left side. Multiloculated blood clots and hemorrhages were noted on the inferior aspect of the huge AML. Selective right renal angiography revealed tortuous, dilated tumor vessels with focal aneurysm and evidence of active bleeding inferiorly. Transarterial embolization was carried out but his condition worsened two hours later and he was intubated. His family refused cardiopulmonary resuscitation and he expired a few hours later.