动脉栓塞治疗肾血管平滑肌脂肪瘤破裂出血

目的评价选择性动脉栓塞治疗肾血管平滑肌脂肪瘤破裂出血的安全性和有效性。方法3例经B超、CT确诊的肾血管平滑肌脂肪瘤患者,左肾1例,右肾1例,双肾1例,突然出现腰腹痛、神志淡漠,血压下降等出血症状,急诊行选择性肾动脉造影和栓塞治疗,栓塞剂用无水酒精和弹簧圈。结果3例患者造影发现:肿瘤区血管紊乱并有动脉瘤形成,栓塞后造影显示:肿瘤血管血供减少,肿瘤内载有动脉瘤血供闭塞。无严重并发症出现。术后随访患者血压上升,临床症状改善。结论选择性动脉栓塞治疗肾血管平滑肌脂肪瘤破裂出血,能有效地控制出血并能最大限度保护正常肾组织,应作为急诊出血首选治疗方法。     

肾血管平滑肌脂肪瘤自发破裂出血的急诊处理(附24例报告)

目的 探讨肾血管平滑肌脂肪瘤自发破裂出血的急诊处理.方法 对24例肾血管平滑肌脂肪瘤自发破裂出血患者的临床资料及随访结果进行回顾性分析.术前均行B超和CT检查,22例诊断为肾血管平滑肌脂肪瘤自发破裂出血,2例未能排除肾癌出血.3例保守治疗,4例行选择性肾动脉栓塞术,17例急诊手术.结果 3例保守治疗患者中1例病情稳定,2例改行手术治疗.4例行肾动脉栓塞术患者栓塞成功,出血停止.4例行肾切除术,13例行保留肾单位手术,其中5例行后腹腔镜手术.术后病理诊断为肾血管平滑肌脂肪瘤并出血.随访3个月～6年,肿瘤无复发或转移.结论 B超和CT是诊断肾血管平滑肌脂肪瘤出血的重要手段.治疗可以选择保守治疗、肾动脉栓塞或者急诊手术.     

52例肾血管平滑肌脂肪瘤并自发破裂出血的诊治

目的探讨肾血管平滑肌脂肪瘤并自发破裂出血的诊治方法。方法回顾性分析52例手术治疗的肾血管平滑肌脂肪瘤并发自发破裂出血的诊治资料。术前均行B超和CT检查,46例诊断为肾血管平滑肌脂肪瘤并发自发破裂出血,6例未能排除肾细胞癌出血。6例保守治疗,11例行肾动脉栓塞术;35例急诊开放手术。结果6例经保守治疗成功无需手术,11例行肾动脉栓塞术中6例需开放手术;9例行保留肾单位肾切除手术,32例行全肾切除。全肾切除的原因:26例肿瘤广泛侵入肾脏,4例出血,2例未能排除肾细胞癌。术后病理诊断为血管平滑肌脂肪瘤并出血。结论B超和CT是诊断肾血管平滑肌脂肪瘤并出血的重要手段,治疗可以选择保守治疗、肾动脉栓塞或者急诊开放手术。     

肾血管平滑肌脂肪瘤的诊断与治疗（附32例报告）

目的：提高肾血管平滑肌脂肪瘤的诊断与治疗水平。方法：总结肾血管平滑肌脂肪瘤32例的诊断及治疗经验,根据肿瘤直径分为：小（＜4cm)、中（4－8cm)、大（＞8cm）三类,同时根据病变大小及症状分别采取观察、栓塞、肿瘤切除、肾部分切除及紧切除术。结果：B超诊断准确率为78．1％（25／32）,CT诊断准确率为87．1％（28／32）。对病变小、无症状者（5／32）采用定期观察,中等大小者（24／32）采用肿瘤切除（13／32）、肾部分切除（9／32）和肾切除（2／32）;病变大者（3／32）则采用肾切除术。32例患者全部获随访,均生存。结论：结合B超、CT以及HMB－45、HMB－50、CD63和细针穿刺细胞学检查明确诊断,根据病变大小和症状分别选择观察、栓塞、肿瘤切除、肾部分切除及肾切除术,治疗肾血管平滑肌脂肪瘤效果良好。     

肾肿瘤自发性破裂所致的外科急症

肾肿瘤自发性破裂导致外科急诊是罕见的。肾血管平滑肌脂肪瘤是其中原因之一。病人和方法:1971-1990年间,收集该院肾肿瘤的外科手术、尸检及病理资料。结果:共368例原发性上尿路肿瘤,包括肾血管平滑肌脂瘤16例。其中6例为急诊外科手术均为血管平滑肌脂肪瘤。一些学者认为肾血管平滑肌脂肪瘤是一种罕见的肿瘤。自发性腹膜后出血,急腹症和低血容量休克提示肾肿瘤破裂的可能性,肾血管平滑肌脂肪瘤自发性破裂的机制是肿瘤侵犯了肾静脉所致。Chan等发现在他们医院过去20年中,肾血管平滑肌脂肪瘤是肾     

肾血管平滑肌脂肪瘤的诊断与治疗(附72例报告)

目的　探讨肾血管平滑肌脂肪瘤的诊治方法。　方法　总结分析 72例肾血管平滑肌脂肪瘤患者的临床资料。统计学方法采用 χ2 检验。　结果　 13例 (18.1% )术前误诊。诊断正确率B超为 73.6 % (5 3/ 72 ) ,CT为 81.9% (5 9/ 72 ) ,两者同时误诊者 10例 (13.9% )。直径 <4cm的肿瘤误诊率高于≥ 4cm者 ,P <0 .0 5。治疗以保留肾单位的手术为主 ,术前选择性动脉栓塞及术中暂时阻断肾动脉可显著减少出血。　结论　应提高对肾血管平滑肌脂肪瘤的术前诊断水平 ,采取各种措施保留肾单位。     

弹簧圈栓塞治疗颅内微小动脉瘤及中长期随访

目的评价弹簧圈栓塞治疗颅内微小动脉瘤（VSCAs）的有效性及稳定性。方法 19例患者（20枚VSCAs）接受血管内弹簧圈栓塞治疗。对其中9枚动脉瘤使用单纯弹簧圈栓塞,9枚使用支架辅助弹簧圈栓塞,2枚使用球囊辅助弹簧圈栓塞。根据DSA表现,将栓塞程度分为完全栓塞、次全栓塞和部分栓塞。术后进行中长期随访并收集DSA及临床结果。结果所有VSCAs均获成功栓塞。术后即刻DSA显示,20枚VSCAs中5枚为完全栓塞,9枚为次全栓塞,6枚为部分栓塞。1~2年随访DSA示20枚VSCAs均完全栓塞,未发现动脉瘤复发及弹簧圈脱出、移位。临床随访显示所有患者均未出现动脉瘤再次破裂出血及神经系统缺血症状。结论血管内弹簧圈栓塞治疗VSCAs有效、稳定;中长期随访证实次全及部分栓塞的患者可发展为完全栓塞。     

依维莫司在结节性硬化合并肾血管平滑肌脂肪瘤术前新辅助治疗中的应用

目的:探讨依维莫司在结节性硬化合并肾血管平滑肌脂肪瘤术前新辅助治疗中的疗效与安全性。方法:对1例结节性硬化合并双肾血管平滑肌脂肪瘤患者进行包括高选择性肾血管栓塞、肾部分切除术及依维莫司的综合治疗,对临床资料和随访情况进行回顾性分析并文献复习。结果:该患者接受了高选择性肾血管栓塞术加肾部分切除术治疗右肾血管平滑肌脂肪瘤,术后应用依维莫司治疗2个月,左肾肿瘤最大直径缩小60%(5.1cm),停药3个月后接受左肾部分切除术,痊愈出院,随访12个月,无肿瘤复发及进展。结论:结节性硬化症合并双肾血管平滑肌脂肪瘤的患者治疗必须考虑肾功能的保留问题,对于一些无法行保留肾单位手术的患者,可以考虑在术前行依维莫司新辅助治疗,使患者获得手术的机会。     

肾血管平滑肌脂肪瘤的保留肾单位手术

目的　评估保留肾单位手术在肾血管平滑肌脂肪瘤治疗中的作用。　方法　回顾性分析 49例行保留肾单位手术治疗的肾血管平滑肌脂肪瘤患者资料 ,观察术前及术后肾功能、手术并发症及预后等。肿瘤剜除术 3 1例 ,肾部分切除 15例 ,原位灌注肾部分切除术 3例 ;其中 3例患者术前行选择性肾动脉栓塞术。　结果　切除肿瘤平均直径 6.0cm (2 .5～ 16.0cm) ,3例原位灌注肾部分切除术肿块直径分别为 10 .5cm、12 .0cm和 15.0cm。 49例术中平均失血量 3 10ml (50～ 10 0 0ml)。无手术并发症发生。术前及术后 10d血肌酐值比较差异无显著性 [(89± 2 6)mmol/Lvs (98± 3 4 )mmol/L ,P>0 .0 5]。 45例平均随访 3 6个月 (1～ 76个月 ) ,无肿瘤复发或需血液透析治疗者。　结论　保留肾单位手术是外科治疗肾血管平滑肌脂肪瘤的首选方法     

肾血管平滑肌脂肪瘤破裂出血诊治体会(附20例报告)

目的总结肾血管平滑肌脂肪瘤(renalangiomyolipoma,RAML)出血的诊治经验。方法20例RAML出血患者,14例肿瘤直径>4cm出血难自止者经急诊手术治疗,其中行肾切除8例,肾部分切除和肿瘤剜除术各3例;肿瘤直径<4cm的6例先行抗休克治疗后行肾切除术、肿瘤剜除术各3例。结果术后病理检查均为RAML破裂出血。随访6～36月肿瘤无复发,行保肾手术(肾部分切除、肿瘤剜除术)的9例患肾功能正常。结论B超及CT对RAML出血的诊断具有重要意义,治疗原则常根据肿瘤大小及症状而定。     

Spontaneous rupture of renal angiomyolipoma

We report a case of renal angiomyolipoma with retroperitoneal hemorrhage treated by enucleation in a 47 year-old male. The mass in the anterior side of the left kidney, revealed by sonography and CT, was diagnosed as angiomyolipoma with a retroperitoneal hematoma caused by its spontaneous rupture. Removal of hematoma and enucleation of the tumor were performed after the diagnosis. Diagnosis and treatment of ruptured renal angiomyolipoma are discussed.     

Angiomyolipoma of the colon: Report of a case

Angiomyolipomas are benign mesenchymal tumors mostly arising from the kidney. Angiomyolipoma of the colon is extremely rare. Here we report the findings of a 51-year-old man who presented with a submucosal tumor covered with normal mucosa and hemorrhage in the descending colon. He underwent a partial resection of the descending colon. A histopathological examination showed that the tumor of 5.7 cm in diameter included smooth muscle (spindle cell type), mature adipose tissue, and vessels, and therefore a diagnosis of angiomyolipoma was made. A submucosal type of angiomyolipoma of the colon is extremely rare. If a renal angiomyolipoma is larger than 4 cm, there is a risk of rupture. When colonoscopy shows a submucosal tumor of the colon with hemorrhage, angiomyolipoma should be considered. If an angiomyolipoma of the colon is large, surgical resection should be considered as a treatment option due to the risk of hemorrhage.     

Bilateral renal angiomyolipoma coexistent with pulmonary lymphangioleiomyomatosis and tuberous sclerosis

A case of bilateral renal angiomyolipoma coexistent with pulmonary lymphangioleiomyomatosis and tuberous sclerosis was described, being in shock with massive hematuria. The left giant angiomyolipoma, filling the almost entire abdomen, was complicated with two large hump and moniliform aneurysms. This life-threatening hemorrhage was successfully treated by transcatheter arterial embolization; packing mechanically detachable coils into the aneurysms and embolization of trunk of the left renal artery. Lymphangioleiomyomatosis has been suggested to be an incomplete expression of tuberous sclerosis. Although coexisting renal and pulmonary involvement in tuberous sclerosis is rare, it is important to recognize lymphangioleiomyomatosis as a pulmonary involvement of angiomyolipoma with tuberous sclerosis.     

Wünderlich Syndrome in Renal Transplant Recipients: A Case Report and Literature Review

BackgroundWünderlich syndrome, or spontaneous atraumatic renal hemorrhage, is a clinical entity rarely described in the native kidney of patients who have undergone renal transplant. Although its manifestation is quite similar in reported cases, it may present few symptoms, from bleeding of unidentified etiology to dramatic pictures associated with hypovolemic shock. There are few reports of spontaneous hemorrhage of a native kidney after kidney transplantation.Case reportWe present a 38-year-old male patient who developed hemorrhage of a ruptured native kidney after a late renal transplantation. We analyze what has been reported in the literature and highlight the possibility of this complication after kidney transplantation. Imaging exams and surgical specimen demonstrated the presence of ruptured angiomyolipoma in the patient's native right kidney. The postoperative period was not complicated, with a benign clinical evolution.ConclusionAlthough rare in patients who have undergone renal transplant, it is justified to suggest Wünderlich syndrome in the presence of acute flank pain, abdominal tenderness, and signs of internal bleeding (Lenk's triad), with unexplained hemoglobin drop.     

RENAL ANGIOMYOLIPOMA IN CHILDREN: DIAGNOSTIC DIFFICULTY IN 3 PATIENTS

Purpose

Because angiomyolipoma is less common in children than in adults, its diagnosis can be difficult. We present 3 cases of pediatric angiomyolipoma in which diagnostic problems resulted due to the presenting characteristics.

Materials and Methods

We report on 3 children with unilateral renal angiomyolipoma. Computerized tomography (CT) and ultrasonography revealed 3 large renal masses, 20, 7 and 8 cm. in diameter, respectively. A correct diagnosis was not made preoperatively in any case by CT, ultrasound or fine needle biopsy. Wilms tumor was suspected in the first patient who received preoperative chemotherapy. Imaging was inconclusive in the other 2 cases.

Results

All patients underwent surgical exploration and subsequent nephrectomy due to the large size of the tumor. At followup 33, 23 and 13 months postoperatively all children were well without signs of recurrence.

Conclusions

It has been reported that the demonstration of fat on renal ultrasound and CT can diagnose angiomyolipoma in 95% of the cases. Most radiologists rely solely on CT demonstration of lipid density in the renal mass to diagnose angiomyolipoma but the identification at imaging of lipid tissue may be difficult in small tumors. In our cases the fat content of the tumors was less than 10% despite the large size. This low fat content results in misdiagnosis, since fatty tissue is also present in other renal tumors, such as lipoma, liposarcoma, teratoma and Wilms tumors. We recommend conservative surgery when tumor size permits in pediatric patients with angiomyolipoma to avoid chemotherapy.     

经导管选择性动脉栓塞治疗创伤性假性动脉瘤

目的探讨经导管选择性动脉栓塞治疗损伤性假性动脉瘤的疗效。方法 16例损伤性出血患者,其中肾脏出血5例,肝脏出血3例,剖宫产后出血4例,切口妊娠流产1例,宫颈癌放疗后出血1例,骨外伤术后出血2例。采用经股动脉穿刺选择性插管进行靶动脉造影及栓塞治疗。采用明胶海绵和（或）弹簧钢圈栓塞瘤腔及供血动脉。结果全部患者动脉造影均见假性动脉瘤,其中2例伴有动静脉瘘。栓塞成功率100%,栓塞后即刻造影示假性动脉瘤消失,止血成功率93.75%。术后均未发生严重并发症,随防6个月均无复发。结论经导管选择性动脉栓塞治疗损伤性假性动脉瘤性出血安全有效、创伤小、并发症少,是可靠的治疗方法 。     

无水乙醇碘油混合液栓塞治疗肾血管平滑肌脂肪瘤的疗效分析

目的探讨无水乙醇碘油混合液介入栓塞治疗肾血管平滑肌脂肪瘤的价值。方法回顾性分析我院采用无水乙醇碘油混合液栓塞治疗肾血管平滑肌脂肪瘤患者24例的资料,分析介入手术成功率,术后症状缓解、术后并发症及栓塞前后瘤体大小情况。结果 24例患者共30个病灶,介入栓塞治疗29个病灶,另1个病灶较小,择期处理,介入栓塞手术成功率100%。患者腰部疼痛、血尿症状术后均逐渐减轻、消失;患者栓塞术后出现不同程度的栓塞后综合征,其中发热12例(12/24,50%)、腹痛腹胀10例(10/24,41.67%)、恶心呕吐10例(10/24,41.67%),无严重并发症发生。随访6~12个月,瘤体最大径术前为(8.13±3.51)cm,术后为(5.29±2.18)cm,差异有统计学意义(P0.05)。结论无水乙醇碘油混合液介入栓塞治疗肾血管平滑肌脂肪瘤疗效确切,安全有效,创伤小,值得临床推广应用。     

超声引导下兔体内直接性门腔静脉分流支架植入术的可行性与安全性

目的探讨超声引导下经皮穿刺门静脉右支(RPV)与肝后段下腔静脉(RHSIVC),行直接性门腔分流支架植入术的可行性与安全性。方法超声引导下对10只实验兔经皮穿刺RPV,调整穿刺针角度后,穿刺RHSIVC,送入导丝并沿导丝放入支架,建立直接性门腔分流通道,观察实验兔支架内血流充盈情况,并测量血流速度。术后30min解剖实验兔,观察支架位置及是否存在肝包膜下出血、腹腔大血管及周围脏器损伤等并发症。结果对9只实验兔成功实施超声引导下直接性门腔静脉分流支架植入术,另1只因术中下腔静脉出血过多致死。超声所测平均RPV内径为(0.23±0.02)cm,RHSIVC内径为(0.38±0.05)cm,RPV与RHSIVC之间平均角度为(22.38±1.60)°;平均穿刺时间(11.4±3.69)min,穿刺RPV、RHSIVC平均次数分别为(1.30±0.48)次和(1.50±0.71)次,平均支架长度(1.06±0.09)cm,支架内平均血流速度(8.14±0.89)cm/s。术后大体解剖结果显示,除1只死亡外,9只实验兔支架均位于RPV与RHSIVC之间的肝实质内,且未出现肝包膜下出血、腹腔大血管及周围脏器损伤等并发症。结论超声引导下兔体内直接性门腔静脉分流支架植入术具有一定的安全性及可行性。     

Nonoperative treatment for a ruptured pseudoaneurysm of the celiac trunk: Report of a case

We report the case of a 67-year-old man in whom hemorrhage from a ruptured celiac trunk pseudoaneurysm, which occurred as a consequence of leakage at the site of gastroduodenostomy, was successfully controlled by transcatheter arterial embolization (TAE) with stainless steel coils and N-butyl cyanoacrylate (NBCA). The occurrence of a pseudoaneurysm of the celiac trunk associated with anastomotic leakage is etiologically rare. We compiled reports from the literature on TAE for ruptured aneurysms of the celiac trunk, and compared its therapeutic value with that of surgical treatment. Operative death occurred in 4 of a series of 43 patients with aneurysms of the celiac trunk that were surgically treated (9.3%). In 5 patients with ruptured aneurysms, the operative mortality rate was 40% (2/5). Conversely, while the unsuccessful rate of TAE therapy was 17% (1/6), the mortality rate was nil. The patient whose case is presented here was affected by methicillin-resistant staphylococcus aureus (MRSA) at the site of leakage and in the lung. Under septic conditions such as hemorrhage secondary to pancreatitis, the mortality rate of surgical therapy was 23%–29%, whereas the success rate of TAE therapy was 79% and the mortality rate was 4%. Based on these findings, it is suggested that TAE therapy is a viable alternative to surgery for patients even with ruptured pseudoaneurysms of the celiac trunk.