fused kidney

融合肾
  • 文章类型: Case Reports
    背景:马蹄肾是最常见的肾脏融合异常,肾母细胞瘤是儿童最常见的肾脏恶性肿瘤。与马蹄肾相关的Wilms肿瘤的发生是罕见的异常。然而,畸形类型的出现,这是马蹄形肾的一种罕见的Wilms肿瘤,异常独特。
    方法:本报告介绍了一名5岁男性,其马蹄肾受累于一个巨大的异质性钙化肿块,在活检中被诊断为肾母细胞瘤占优势。根据肺部局部和区域的扩展和转移性肿瘤,患者接受了新辅助化疗,然后进行了手术.术后病理结果证实了畸胎瘤的诊断。
    结论:在临床环境中,与恶性肿瘤相关的肾脏异常的发生可能更为频繁。肾脏肿瘤和肿块有许多鉴别诊断,但是不应该否认异常的可能性,临床医生应该为这些场合做好准备。尽管研究表明化疗对畸胎瘤样肾母细胞瘤的影响很小,在进行前期肿瘤切除之前,必须评估肿瘤在非畸胎瘤区消退的可能性.
    BACKGROUND: Horseshoe kidney is the most common renal fusion anomaly, and Wilms tumor is the most frequent renal malignancy in children. The occurrence of Wilms tumor in association with horseshoe kidney is a scarce anomaly. However, the arising of a teratoid type, which is a rare variant of Wilms tumor in a horseshoe kidney, is exceptionally unique.
    METHODS: This report presents a 5-year-old male admitted with horseshoe kidney involved by a large heterogeneous calcified mass that was diagnose on biopsy as Wilms tumor blastemal dominant. According to the local and regional extension and metastatic tumor in the lungs, the patient underwent neoadjuvant chemotherapy and then surgery. Post-operative pathologic findings confirmed the diagnosis of teratoid Wilms tumor.
    CONCLUSIONS: The occurrence of renal anomalies associated with a malignancy might be more frequent in the clinical environment. There are numerous differential diagnoses for renal tumors and masses, but the possibility of exceptional anomalies should not be denied, and clinicians should be prepared for these occasions. Although studies propose that chemotherapy has a trivial effect on teratoid Wilms tumors, it is essential to evaluate the tumor for any possibility of regression in non-teratoid regions before proceeding to upfront tumoral resection.
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  • 文章类型: Case Reports
    背景:交叉融合肾异位(CFRE)是一种常见的先天性异常,其中一个肾脏异常位于中线的另一侧,经常与另一个肾脏融合。然而,单个输尿管引流交叉融合肾异位是罕见的。
    方法:这里,我们报告了一例有结石病史的46岁尼泊尔男性患者的交叉融合性肾异位伴单个输尿管。计算机断层扫描显示左肾位于右侧并与右肾融合。两个肾脏的肾盂融合,和一个输尿管,位于右侧,将两个肾脏排入膀胱。建议患者定期随访。
    结论:单输尿管交叉融合肾异位是一种罕见的肾脏异常。无症状患者通常可以保守治疗。建议定期随访以监测肾功能,微积分形成,感染,和恶性变化。
    BACKGROUND: Crossed fused renal ectopia (CFRE) is a common congenital anomaly where one kidney is positioned abnormally on the opposite side of the midline, often fused with the other kidney. However, single ureter draining crossed fused renal ectopia is a rare occurrence.
    METHODS: Here, we report a case of crossed fused renal ectopia with a single ureter in a 46-year-old Nepali male who presented with history of lithuria. Computed tomography revealed that the left kidney was situated on the right side and fused with the right kidney. The renal pelvises of both kidneys were fused, and a single ureter, located on the right side, was draining both kidneys into the bladder. The patient was advised to have regular follow-ups.
    CONCLUSIONS: Crossed fused renal ectopia with a single ureter represents a rare renal anomaly. Asymptomatic patients can typically be managed conservatively. Regular follow-up is recommended to monitor renal function, calculus formation, infections, and malignant changes.
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  • 文章类型: Case Reports
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    文章类型: Case Reports
    我们报道了1例乙状结肠癌伴马蹄肾的病例。一名79岁的男性患有下腹痛,并接受了结肠镜检查。结肠镜检查结果显示乙状结肠癌。术前计算机断层扫描显示马蹄肾。他接受了根治性腹腔镜手术。病理诊断为pStageⅡa(第9版)。手术后22个月,他没有复发。患有先天性尿路异常的结直肠癌的手术需要注意术中继发性损伤。因此,术前评估使用3D-CT是安全的有用工具。操作适当的解剖正常层将使安全的腹腔镜手术成为可能,而不会造成意外伤害。
    We reported a case of sigmoid colon cancer with horseshoe kidney. A 79-year-old man had lower abdominal pain and underwent colonoscopy. The results of colonoscopy revealed sigmoid cancer. Preoperative computed tomography revealed horseshoe kidney. He underwent radical laparoscopic surgery. The histopathological diagnosis was pStage Ⅱa(The 9th Edition). He has not recurred 22 months later after operation. Surgery for colorectal cancer with congenital anomalies of the urinary tract requires attention to intraoperative secondary injuries. Therefore, preoperative evaluation using 3D-CT is useful tool for safety. Operating the proper dissecting normal layer would make safe laparoscopic operation possible without unexpected injuries.
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  • 文章类型: Case Reports
    52岁,日本男子因肛门出血被送往医院,详细的检查结果诊断为局部晚期直肠癌。患者接受全新辅助治疗,然后进行短程放疗和巩固化疗,提供了部分回应。术前对比增强计算机断层扫描显示为马蹄形肾,机器人辅助,精确,腹腔镜,进行了低位前切除术,包括D3夹层和回肠造口术。肠系膜下动脉周围马蹄形肾峡部升高,左输尿管和精管在肾脏前面。腹下神经向腹侧移动到马蹄肾。有了机器人手术,在患有马蹄形肾的直肠癌患者中,由于良好的三维可视性和铰接式镊子操作,在识别血管和神经解剖结构的同时,可以进行更精确的手术.
    A 52-year-old, Japanese man presented to the hospital with a complaint of anal bleeding, and detailed examination resulted in a diagnosis of locally advanced rectal cancer. The patient underwent total neoadjuvant therapy followed by short-course radiation therapy and consolidation chemotherapy, which provided a partial response. After preoperative contrast-enhanced computed tomography showed a horseshoe kidney, robot-assisted, precise, laparoscopic, low anterior resection with D3 dissection and ileostomy construction was performed. The horseshoe renal isthmus was elevated surrounding the inferior mesenteric artery, and the left ureter and seminal vessels ran in front of the kidney. The hypogastric nerve traveled ventral to the horseshoe kidney. With robotic surgery, it was possible to perform more precise surgery while recognizing vascular and nerve anatomy in a rectal cancer patient with a horseshoe kidney due to good three-dimensional visibility and articulated forceps manipulation.
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  • 文章类型: Case Reports
    煎饼肾是一种肾脏融合异常,仅有少数报道的产前诊断。泌尿生殖系统以外的其他结构异常也可能相关。这项研究描述了产前检测到的双重异常病例,包括薄饼肾和空肠闭锁。产后腹部超声证实两个肾脏在主动脉分叉水平的中线融合,还有3b型空肠闭锁.根据关于煎饼肾的有限证据,在我们的病例中,根据6个月的随访,肾功能似乎仍基本保留且不受影响.然而,我们需要进一步研究,以探索与部分病例染色体和结构异常的潜在关联.
    Pancake kidney is a renal fusion anomaly with only a few reported prenatal diagnoses. Other structural anomalies beyond the urogenital system may also be associated. This study describes a dual anomaly case detected prenatally, comprising of pancake kidney and jejunal atresia. A postnatal abdominal ultrasound confirmed both kidneys were fused in the midline at the aortic bifurcation level, along with a type 3b jejunal atresia. Based on the available limited evidence about pancake kidney, renal functions appear to remain largely preserved and unaffected as in our case according to 6 months of follow-up. However, further investigation is needed to explore any potential association with chromosomal and structural abnormalities in selected cases.
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  • 文章类型: Case Reports
    肾脏融合异常代表肾脏和泌尿道(CAKUT)的先天性异常的一部分。马蹄形肾脏(HSKs)是肾脏在其下部两极的融合。它是最常见的形式的CAKUT。症状通常很微妙。诊断通常是在儿童时期偶然做出的。HSK很少在以后的生活中出现症状。我们介绍了一名88岁的女性,有HSK病史,她到急诊科(ED)就诊,有三周的左侧腹部疼痛史,间歇性恶心,尿量减少.她的住院检查包括影像学检查显示HSK和双侧肾积水,左边更突出。在生命后期出现HSK症状极为罕见。
    Abnormalities in renal fusion represent a subset of congenital anomalies of the kidneys and urinary tract (CAKUT). Horseshoe kidneys (HSKs) are the fusion of kidneys at their lower poles. It is the most common form of CAKUT. Symptoms are usually subtle. The diagnosis is usually made incidentally during childhood. Rarely does an HSK become symptomatic later in life. We present the case of an 88-year-old female with a history of HSK who presented to the emergency department (ED) with a three-week history of left-sided flank pain, intermittent nausea, and reduced urine output. Her inpatient workup included imaging that revealed an HSK and bilateral hydronephrosis, which was more prominent on the left. The onset of symptoms for an HSK late in life is extremely rare.
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  • 文章类型: Case Reports
    2003年至2016年,我们在爱媛县中心医院对约200例患者进行了腹腔镜活体供肾切除术(LDN)。2016年,一名五十多岁的妇女是她丈夫的捐赠者候选人,通过对比增强计算机断层扫描显示患有马蹄形肾;其他LDN程序使用腹膜后方法,但是这种方法使用了经腹膜的方法,因为后一种方法允许更有利的视野。选择左肾,因为肾脏闪烁显像显示双侧肾功能相同,左侧肾动脉更简单。在峡部成功横切后切除肾脏,没有缺血。通过台式手术缝合了左肾中打开的花萼,肾脏被移植到受体身上.供体和受体的术后疗程均良好。
    We performed laparoscopic live donor nephrectomy (LDN) on approximately 200 patients in Ehime Prefectural Center Hospital between 2003 and 2016. In 2016, a fifty-something woman who was a donor candidate for her husband was revealed to have a horseshoe kidney through contrast-enhanced computed tomography; other LDN procedures used a retroperitoneal approach, but this one used a transperitoneal approach since the latter approach allowed for a more favorable visual field. The left kidney was selected since renal scintigraphy showed equal bilateral renal function and renal arteries are simpler on the left side. The kidney was removed after the isthmus was successfully transected without ischemia. The opened calyx in the left kidney was sutured via bench surgery, and the kidney was transplanted to the recipient. Postoperative courses of both donor and recipient were good.
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  • 文章类型: Review
    背景:在此案例报告中,我们展示了我们的后腹腔镜下马蹄肾T1b右肺门肿瘤切除术的技术。
    方法:一名77岁的越南妇女因右侧腹部疼痛被送往医院。在介绍时,她的血清肌酐为0.86mg/dL,肾小球滤过率为65.2mL/min/1.73m2.根据她的肾闪烁显像,右侧和左侧部分的肾小球滤过率分别为24.2和35.5mL/分钟,分别。计算机断层扫描成像显示5.5×5.0cm实性肺门肿块,cT1bN0M0肿瘤分期正确。经过讨论,患者选择了微创手术来治疗她的恶性肿瘤。患者被置于侧腹位置。我们使用高尔的气球技术创造了腹膜后工作空间,计划使用四个套管针端口。解剖了三条动脉,包括两条供血正确部分的动脉,一条供血于峡部的动脉,和一条静脉,被Hem-o-lok剪裁和分割。使用内窥镜进行峡部切除术。因此,输尿管被夹住并分开。最后,马蹄肾的整个右段被动员起来,并通过侧腹切口取出。
    结果:总手术时间为250分钟,估计失血量为200mL。患者术后血清肌酐为1.08mg/dL,肾小球滤过率为49.47mL/min/1.73m2。患者在术后第4天出院,无并发症。肿瘤标本的最终病理检查显示FuhrmanII级透明细胞肾细胞癌,囊侵犯,手术切缘阴性。经过三个月的随访,血清肌酐为0.95mg/dL,肾小球滤过率为57.7mL/min/1.73m2。随访计算机断层扫描成像未发现局部复发或转移。
    结论:腹膜后腹腔镜肾细胞癌切除术对于马蹄形肾细胞癌患者是一种安全可行的技术,在没有使用机器人技术的低收入环境中可能特别有用。
    BACKGROUND: In this case report, we demonstrate our technique of a retroperitoneal laparoscopic heminephrectomy for a T1b right hilar tumor in a horseshoe kidney.
    METHODS: A 77-year-old Vietnamese woman presented to the hospital because of right flank pain. On presentation, her serum creatinine was 0.86 mg/dL and glomerular filtration rate was 65.2 mL/minute/1.73 m2. According to her renal scintigraphy, glomerular filtration rates of the right and left moieties were 24.2 and 35.5 mL/minute, respectively. Computed tomography imaging demonstrated a 5.5 × 5.0 cm solid hilar mass with a cT1bN0M0 tumor stage was in the right moiety. After discussion, the patient elected a minimally invasive surgery to treat her malignancy. The patient was placed in a flank position. We used Gaur\'s balloon technique to create the retroperitoneal working space, and four trocar ports were planned for operation. Three arteries were dissected, including two arteries feeding the right moiety, one artery feeding the isthmus, and one vein, which was clipped and divided by Hem-o-lok. The isthmusectomy was performed with an Endostapler. Consequently, the ureter was clipped and divided. Finally, the whole right segment of the horseshoe kidney was mobilized and taken out via the flank incision.
    RESULTS: The total operative time was 250 min with an estimated blood loss of 200 mL. The patient\'s serum creatinine after surgery was 1.08 mg/dL, and glomerular filtration rate was 49.47 mL/minute/1.73 m2. The patient was discharged on postoperative day #4 without complication. Final pathologic examination of the tumor specimen revealed a Fuhrman grade II clear cell renal cell carcinoma, capsular invasion, with negative surgical margins. After a three-month follow-up, the serum creatinine was 0.95 mg/dL, and glomerular filtration rate was 57.7 mL/minute/1.73 m2. Local recurrence or metastasis was not detected by follow-up computed tomography imaging.
    CONCLUSIONS: Retroperitoneal laparoscopic heminephrectomy is a safe and feasible technique for patients with renal cell carcinoma in a horseshoe kidney and may be particularly useful in low income settings without access to robotic technology.
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  • 文章类型: Case Reports
    背景:嗜铬细胞瘤是一种神经内分泌肿瘤,其治疗依赖于手术切除。由于横截面成像的广泛可用性,嗜铬细胞瘤通常被认为是小于10厘米的小肿瘤,并且大多采用微创手术治疗。它们与马蹄形肾或其他解剖和血管异常同时存在是罕见的。在这里,我们介绍了一个手术复杂的巨大嗜铬细胞瘤病例,该病例接受了左肾上腺开放根治术。
    方法:一名41岁的西班牙裔女性,表现为左12×8厘米的高血管肾上腺肿块,骨盆马蹄肾,和严重扩张的大左主动脉后肾静脉分支成左肾上腺静脉,左卵巢静脉充血,离开子宫丛.她接受胰岛素和二甲双胍治疗A1c水平为9%的未控制糖尿病,接受多沙唑嗪治疗持续性高血压。嗜铬细胞瘤的临床诊断通过尿液和血清中的偏肾上腺素和去甲肾上腺素升高得到证实。术前ACTH在正常范围内,地塞米松抑制试验和24小时尿游离皮质醇正常。通过开放方法对高度侵袭性肾上腺肿块进行肾上腺切除术,以获得足够的手术暴露。由于肿瘤的体积较大,并且其与多个相邻结构的显著受累,为了获得成功的患者结局,包括保留骨盆马蹄肾的血液供应,需要与多个手术团队进行协调,并通过麻醉学进行密切的血流动力学监测.组织病理学诊断为嗜铬细胞瘤,手术切缘阴性。术后1、4、12和24周随访患者。她的术后eGFR正常,并能够在四周停止抗高血压和抗糖尿病药物治疗。她有短暂性肾上腺功能不全,在五个月后解决。马蹄形肾脏完好无损,除了由于梗塞导致的左上肾部分最小区域增强不足,这在六个月时显著改善。
    结论:我们的患者有一个巨大的嗜铬细胞瘤,其解剖结构的变异使已经具有挑战性的手术复杂化。尽管如此,通过多个提供商的协作,详细的术前手术计划,细致的围手术期监测,巨大嗜铬细胞瘤的根治性切除术是安全可行的,术后转归成功.
    BACKGROUND: Pheochromocytoma is a neuroendocrine tumor, and its treatment is dependent on surgical resection. Due to the wide availability of cross-sectional imaging, pheochromocytomas are commonly seen as small tumors less than 10 cm in size and are mostly treated with minimally invasive surgery. Their concomitant presence with horseshoe kidney or other anatomical and vascular anomalies is rare. Herein, we present a surgically complex giant pheochromocytoma case who underwent an open left radical adrenalectomy.
    METHODS: A 41-year-old Hispanic female presented with a 12 × 8 cm left hypervascular adrenal mass, pelvic horseshoe kidney, and severely dilated large left retro-aortic renal vein which branched into a left adrenal vein, congested left ovarian vein, and left uterine plexus. She was managed with insulin and metformin for uncontrolled diabetes with an A1c level of 9% and doxazosin for persistent hypertension. Clinical diagnosis of pheochromocytoma was confirmed with elevated urine and serum metanephrine and normetanephrine. The pre-operative ACTH was within normal range with a normal dexamethasone suppression test and 24-hour urine free cortisol. The adrenalectomy of the highly aggressive adrenal mass was performed via open approach to obtain adequate surgical exposure. Due to the large size of the tumor and its significant involvement with multiple adjacent structures, coordination with multiple surgical teams and close hemodynamic monitoring by anesthesiology was required for successful patient outcomes including preservation of blood supply to the pelvic horseshoe kidney. The histopathological diagnosis was pheochromocytoma with negative surgical margins. The patient was followed at 1, 4, 12, and 24 weeks postoperatively. She had a normal postoperative eGFR and was able to discontinue antihypertensive and antidiabetic medications at four weeks. She had transient adrenal insufficiency, which resolved at five months. The horseshoe kidney was intact except for a minimal area of hypo-enhancement in the left superior renal moiety due to infarction, which was significantly improved at six months.
    CONCLUSIONS: Our patient had a giant pheochromocytoma with anatomical variations complicating an already surgically challenging procedure. Nonetheless, with multiple provider collaboration, detailed pre-operative surgical planning, and meticulous perioperative monitoring, radical resection of the giant pheochromocytoma was safe and feasible with successful postoperative outcomes.
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