Severe renovascular hypertension in an infant with congenital solitary pelvic kidney

Renal artery stenosis (RAS) is one of the most common causes of severe arterial hypertension in infants. Its management is very difficult, especially when present in a single kidney. We report a case of severe hypertension caused by RAS of congenital single pelvic kidney in a 4-month-old boy. The patient presented with cardiorespiratory insufficiency that was first treated as acute fulminate myocarditis. Medical treatment of arterial hypertension was disappointing, as it had to be balanced between congestive cardiac failure and acute renal failure. Percutaneous transluminal angioplasty (PTA) done by coronary balloon dilatation catheters through the left axillary access was successful. Following dilatation of the renal artery, blood pressure decreased and its good control was possible by only one drug. With improved medical blood pressure control and normal growth development, the reassessment of clinical therapy options adjusted to a larger vessel size would be possible. Renovascular hypertension due to RAS in infants with a solitary kidney is difficult to control by medical treatment alone. PTA should be considered as a viable option in infants with refractory hypertension due to renal artery stenosis in a solitary kidney, since it has the potential of improving hypertension while preserving renal function.     

Magnetic resonance urography: a new gold standard for the evaluation of solitary kidneys and renal buds?

PURPOSE: We evaluated the diagnostic potential of magnetic resonance urography (MRU) in pediatric patients with a solitary kidney and/or dysplastic orthotopic or ectopic renal buds. We present our evolving experience with this imaging procedure. MATERIALS AND METHODS: A total of 30 patients with a mean age of 43.8 months with a known or suspected solitary kidney with or without clinical symptoms underwent ultrasound of the urinary tract as well as voiding cystourethrogram, renal static dimercapto-succinic acid (DMSA) scintigraphy and MRU. The results of these diagnostic imaging studies were then compared with each other and set against the results of the final diagnosis established at surgery and by histology, if available. RESULTS: Dysplastic orthotopic or dystopic renal buds suspected on ultrasound in 11 of the 30 patients were confirmed in all 11 by MRU. While accompanying ectopic ureteral insertions were shown in only 3 patients by ultrasound, they were visualized in all 4 by MRU. The other 7 patients with dysplastic renal buds had orthotopic ureteral orifices. DMSA scintigraphy failed to detect 10 renal buds. CONCLUSIONS: The diagnostic value of MRU appears to be superior to that of other imaging tests, even to that of DMSA scintigraphy, which with voiding cystourethrography is currently considered the gold standard for evaluating sonographically suspected solitary kidneys with contralateral orthotopic or ectopic renal buds and ectopic ureteral insertion.     

A case of percutaneous transluminal angioplasty of renovascular hypertension in a solitary kidney

The patient was a 18-year-old woman. Past history included right nephrectomy for right congenital hydronephrosis when she was an infant of 40 days. On examination, her blood pressure was 220/140 mmHg, and plasma renin activity was 4.2 ng/ml/hr. The selective renal arteriogram showed fibromuscular dysplasia of the left main renal artery, and a diagnosis of renovascular hypertension in the solitary kidney with an aberrant artery was made. Treatment with orally-active inhibitor of angiotensin I converting enzyme, Captopril, was started. Her blood pressure became normal after oral administration of Captopril, but her renal function deteriorated. Therefore, percutaneous transluminal angioplasty was performed twice resulting in effective dilatation of the stenotic portion of the left main renal artery. Thereafter, her blood pressure fluctuated between 170/120 and 140/70 mmHg. Eight months later, her blood pressure is now being controlled with mild antihypertensive treatment.     

Angioplasty of a stenotic aorto-renal artery saphenous vein bypass graft to a single kidney

A 69-year-old white woman who 18 months previously had undergone a right nephrectomy and an aorto-renal artery saphenous vein bypass to her left kidney because of accelerated hypertension underwent angioplasty repair of the saphenous vein bypass, which had become stenotic and was causing accelerated hypertension and renal failure. Angioplasty resulted in improvement in BP control and renal function. This is the first report of a successful angioplasty of an aorto-renal saphenous vein bypass. This case also demonstrates the previously described captopril-induced acute renal failure seen in patients with compromised renal perfusion pressure.     

离体肾动脉瘤修补、肾动脉重建和自体肾移植治疗复杂性孤肾肾动脉瘤一例并文献复习

目的探讨离体肾动脉瘤修补、肾动脉重建和自体肾移植技术治疗复杂性孤肾肾动脉瘤的安全性和可行性。方法CT血管造影（CTA）确诊复杂性孤肾肾动脉瘤1例,病变位于肾动脉主干分叉部,累及节段分支动脉,深入肾门内。肾脏暂时性离体后,在低温和肾脏灌注液灌注保护肾脏的前提下,体外进行肾动脉瘤修补和自体大隐静脉肾动脉重建,然后将肾脏异位移植到右侧髂窝。结果手术成功,围手术期无严重并发症发生。术后血肌酐暂时性升高至约200μmol／L,半个月后逐渐恢复正常;术后2周复查CTA示右髂窝移植肾动脉及其分支血流通畅无狭窄,肾静脉回流通畅,输尿管无狭窄。结论该方法治疗复杂性孤肾肾动脉瘤安全可行,并为以后类似的复杂性肾脏疾病的处理提供了可行方法。     

Asymptomatic renal infarction, due to fibromuscular dysplasia, in a young woman with 11 years of follow-up

We report a 27-year-old woman with renovascular hypertension, renal infarction, and hepatic artery aneurysm due to fibromuscular dysplasia. The patient was first noted to have renal artery aneurysm and hepatic artery aneurysm at the age of 17. The renal infarction was asymptomatic and was incidentally detected by magnetic resonance imaging (MRI) examination. Because of the rather peripheral location of the aneurysms, percutaneous transluminal renal artery angioplasty was considered inappropriate. This case suggests the need for long-term and periodical follow-up of patients with fibromuscular dysplasia.     

Primary carcinoid tumor of the kidney: a case report

A 40-year-old male was referred to our hospital for further examination of a left kidney tumor. A left kidney tumor measuring 3 cm in diameter was incidentally found by abdominal ultrasound on physical checkup. Abdominal computed tomography and magnetic resonance imaging confirmed hypovascular tumor of the left kidney. Clinical diagnosis was left renal cell carcinoma and retroperitoneoscopic left nephrectomy was performed. However, pathological diagnosis was carcinoid tumor of the kidney. Primary carcinoid tumors of the kidney are uncommon. We present a case of primary carcioid tumor of the kidney and review the literature.     

Magnetic resonance imaging of the dysplastic renal moiety and ectopic ureter

PURPOSE: We determined the role of magnetic resonance imaging (MRI) in symptomatic children with clinically suspected and radiologically occult dysplastic renal moieties and ectopic ureters. MATERIALS AND METHODS: We reviewed clinical, imaging, cystoscopic, surgical and histological findings in 6 symptomatic children 1 to 15 years old with dysplastic renal moieties. RESULTS: After multiple conventional imaging studies failed to delineate urinary tract anatomy MRI provided detailed multiplanar images of dysplastic renal moieties that were diagnostic and predictive of subsequent intraoperative findings. Dysplastic upper pole moieties identified in 4 children were associated with ectopic ureters inserting into the vagina, prostatic urethra, bladder neck and bladder neck ureterocele in each. A solitary kidney with contralateral blind-ending ectopic ureters inserted into the bladder base in 2 cases. Pelvic cystic structures visualized by ultrasound in 3 patients were tortuous distal ureters on MRI. MRI specifically identified ureteral insertion sites that were not evident in 3 of the 5 patients who underwent cystoscopy. CONCLUSIONS: MRI may facilitate diagnosis, guide cystoscopy and aid in preoperative planning in children with poorly functioning renal moieties and ectopic ureters.     

原发性肾窦肿瘤三例报告

目的 总结原发性肾窦肿瘤的临床特点及治疗方法.方法 原发性肾窦肿瘤患者3例.女2例,男1例.平均年龄40(33～55)岁.临床表现为腰痛伴血尿1例,单纯腰部疼痛2例.肿瘤位于右侧2例,左侧1例.直径平均5.5(3.5～8.5)cm.3例均经行手术治疗. 结果行右肾窦肿瘤探查术1例,术中病理提示良性肿瘤,单纯切除肿瘤,病理报告为血管平滑肌瘤,随访3年未见复发.1例术中因肿瘤出血及肾盂破坏严重,行右肾切除,病理报告为血管平滑肌脂肪瘤,随访10个月未见复发.1例因肿瘤包绕肾动静脉,局部严重黏连,行左肾切除术,病理报告为脂肪瘤,随访4年未见肿瘤复发.结论 原发性肾窦肿瘤临床罕见,多为良性,易误诊为肾盂肿瘤,CT、MRI、IVU检查有助于鉴别;良性肿瘤直径<4 cm且无症状者可密切随访,有症状或直径>4 cm者可行肿瘤切除;肿瘤累及肾蒂并严重破坏肾盂者可行单纯肾切除;恶性肿瘤应行根治性肾切除.     

Pathologic Findings of Renal Biopsy Were a Helpful Diagnostic Clue of Stenosis of the Iliac Segment Proximal to the Transplant Renal Artery: A Case Report

Common iliac artery stenosis after renal transplantation is a rare complication; it can occur in the course of hypertension and renal dysfunction. We report a case of suspected renal allograft rejection with iliac artery stenosis proximal to a transplanted kidney. A 52-year-old man with a history of cadaveric kidney transplantation 26 years previously underwent a second cadaveric kidney transplantation in the left iliac fossa because of graft failure 3 years before. In June 2012, the patient had progressive renal dysfunction. In July, a percutaneous needle biopsy was taken, and it showed no rejection; however, his renal function continued to get worse through September. A percutaneous allograft renal biopsy was performed under ultrasound guidance and showed hyperplasia of the juxtaglomerular apparatus and renin granules. Magnetic resonance angiography was used to evaluate the arteries in the pelvis and showed left common iliac artery stenosis, and a stent was placed. After percutaneous intervention, the patient's ankle brachial pressure index was within the normal range and the allograft function had improved.     

Therapeutic embolization of renal angiomyolipoma: a case report

A case of bilateral renal angiomyolipoma without tuberous sclerosis is reported. A 49-year-old woman was admitted to the general practitioner with a sudden onset of severe left flank pain. An excretory urogram and ultrasonogram revealed an enlargement of the left kidney. She was subsequently referred to our clinic for further investigation and treatment. Computed tomographic scan and magnetic resonance imaging using Tl-weighted image showed several tumors with a fatty, dense area in the bilateral kidney. An arteriogram demonstrated a hypervascular renal mass with aneurysms in her left kidney. Diagnosis of bilateral renal angiomyolipoma was confirmed by percutaneous needle biopsy. Superselective embolization of the tumor was successfully performed, preserving normal renal tissue. Gelatin sponges containing Carboquone (CQ sponge) were used as embolic material. Angiomyolipoma has become relatively easy to diagnose by CT, ultrasound, MRI and so on. However, there are some cases of angiomyolipoma which are indistinguishable from renal cell carcinoma using these modes of testing. Therefore, in selecting a conservative management, we indicated that percutaneous biopsy or open biopsy should be done to confirm the results of the above procedures. Moreover, therapeutic embolization for angiomyolipoma was concluded to be very useful.     

Autotransplantation of solitary kidney with renal artery aneurysm treated with laparoscopic nephrectomy and ex vivo repair: a case report

Introduction

Renal artery aneurysms (RAA) are extremely rare clinical entities with associated morbidities including hypertension and rupture. Although most RAA can be treated with in vivo repair or endovascular techniques, these may not be possible in patients with complex RAA beyond the renal artery bifurcation. We report a case of RAA in a patient with a solitary kidney that we treated successfully by extracorporeal repair and autotransplantation and the 2-years follow-up.

Case Report

A 64-year-old woman with a history of right nephrectomy for renal cell carcinoma presented with RAA found on routine computed tomography (CT). Preoperative workup demonstrated a 2.2 × 2.1 × 3-cm aneurysm in the distal left renal artery that was not amendable to in vivo or endovascular repair. The patient underwent a laparoscopic-assisted left nephrectomy, ex vivo renal artery aneurysm repair, and autotransplantation. She did well postoperatively and in clinic follow-up was found to have a creatinine of 1.2 mg/dL at the end of 2 years and stable blood pressure control.

Discussion

This patient with RAA in her solitary kidney was successfully treated with laparoscopic-assisted nephrectomy, ex vivo repair, and autotransplantation. Her creatinine was stable postoperatively despite absence of a second kidney.     

Degenerative (true) superficial temporal artery aneurysm: a case report with review of literature

This is a case report of a 72-year-old female who presented to our clinic with right temple mass that was found incidentally on a magnetic resonance imaging (MRI) of her head about 2 years ago, as part of a stroke workup. However, it was thought to be a sebaceous cyst and left as such. The patient then experienced a rapid increase in the size of the mass about 2 weeks prior to her presentation to us. On examination, she had a 2 × 2 cm pulsating aneurysmal mass over the right temporal fossa, which was felt to be an aneurysm of the superficial temporal artery and was confirmed by a color duplex ultrasound examination. Open surgical resection with primary ligation of both proximal and distal branches was achieved under local anesthesia. Pathology confirmed a true (degenerative) aneurysm of the superficial temporal artery. Her postoperative course was uneventful. This case report describes this rare entity along with a review of the literature.     

Renovascular hypertension complicated with VATER association

We report herein a case of a girl with renovascular hypertension associated with VATER association. Her plasma renin activity and aldosterone were high. The ultrasonic echogram and renogram revealed a right hypoplastic kidney without function and a normal-sized left kidney with normal function. Renal angiography revealed a small diameter right main renal artery and a normal left main renal artery with segmental stenosis of left branching renal arteries in the middle segment. Selective renal vein sampling indicated that renin secretion was primarily from the left kidney. This is the first report of renovascular hypertension complicated with VATER association.     

Efficacy of diffusion-weighted magnetic resonance imaging in detecting infected cysts in a case of polycystic kidney disease

Hepatic and/or renal cyst infection is a major complication in patients with polycystic kidney disease. In many cases, drainage of infected cysts is necessary, although accurate detection of infected cysts from among the numerous hepatic or renal cysts present is often difficult, because the findings of infected cysts on computed tomography and T1- and T2-weighted magnetic resonance imaging resemble those of normal cysts. We describe here a case of polycystic kidney disease complicated by hepatic cyst infection. On diffusion-weighted magnetic resonance imaging (DWMRI), which is occasionally used in the diagnosis of cerebral abscesses, infected hepatic cysts showed higher signal intensity than other cysts, facilitating differentiation of the cysts requiring drainage from numerous other cysts. Infected cysts showed a marked decrease of the apparent diffusion coefficient (ADC) values compared with those of normal cysts. DWMRI was very effective in detecting infected cysts in our patient and may be of value in other such cases with polycystic kidney disease.     

Unilateral renal angiodysplasia in a girl with hypertension

We report a case of very unusual renal vascular anomaly: angiodysplasia. The patient suffered from acute pyelonephritis immediately after birth. Renal ultrasonography, performed at age 1 day, revealed an enlarged left kidney with heterogeneously increased echogenicity, which involuted rapidly in 3 months. At age 10 years, she presented with severe hypertension during a course of acute pyelonephritis. Peripheral plasma renin activity was high. Computed tomographic angiogram revealed a very small but functioning left kidney. A single, narrow, left renal artery did not have focal stenosis. Pathology examination revealed dysplastic arterioles at the subcapsular area. After left nephrectomy, the blood pressure and the plasma renin activity were normalized. In conclusion, this is an unusual case of renal angiodysplasia, which induced renin-dependent hypertension.     

Case of renal artery stenosis in an elderly patient after nephrectomy diagnosed by ultrasound sonography, showing improvement of blood pressure and renal dysfunction after renal artery stenting

Arteriosclerotic renal artery stenosis is one of the increasingly common diseases that affects many aged patients. There are various non-invasive methods to diagnose renal artery stenosis, such as contrast enhanced CT or MRI. However, these methods are not appropriate for patients with renal dysfunction. Ultrasound sonography is becoming one of the promising methods to diagnose artery stenosis because of photographic improvements. In this case, a 72-year-old woman was hospitalized 7 months after nephrectomy because of severe hypertension, heart failure and kidney dysfunction. The heart failure was quite uncontrollable in spite of massive administration of diuretics, and finally, hemodialysis was started to control her volume status. In consideration of her past history and abdominal bruit, we evaluated the renal artery stenosis by ultrasound sonography and confirmed the diagnosis by renal angiography. To improve hypertension control, we performed renal artery stenting, which resulted in an impressive improvement of her blood pressure and renal function. We recognized the importance of careful causal evaluation of renal dysfunction, even though it is difficult to apply invasive therapy to patients after nephrectomy.     

Bellini duct carcinoma of the kidney: a case report

A case of Bellini duct carcinoma is reported. A 70-year-old man visited our hospital because of gross hematuria and left flank pain. Although no abnormality was found on ultrasonography, drip infusion pyecography, computed tomographic scan and cystoscopy. However class IV was suspected based on urinary cytology. Magnetic resonance imaging showed an irregular pattern in the left upper kidney. Ureterscopic biopsy revealed transitional cell carcinoma and class V was suspected on the urinary cytology of the left renal pelvis. Under the preoperative diagnosis of a left renal pelvic tumor, left nephroureterectomy was performed. The histopathological diagnosis with immunohistostaining was Bellini duct carcinoma. No evidence of recurrence or metastasis was noted 9 months after surgery without any adjuvant therapy.     

Renal artery stenosis and nephrotic syndrome: a rare combination in an infant

We describe an uncommon pediatric finding of unilateral renal artery stenosis, which presented as nephrotic syndrome, hypertension, failure to thrive, and hyponatremia. The child was a previously well 8-month-old male who looked well but had mild periorbital edema with severe hypertension. After 3 days of captopril therapy, the nephrotic-range proteinuria significantly improved. However, the hypertension persisted. Renal imaging revealed a small left kidney with reduced parenchymal uptake and no significant excretion. A renal angiogram demonstrated left renal artery stenosis with increased left renal vein renin activity. The hypertension resolved within 24 h of a left nephrectomy, but non-nephrotic-range proteinuria persisted for 8 months post operatively. Pathology of the left kidney was consistent with fibromuscular dysplasia. Although a few glomeruli (1%) had changes consistent with focal segmental glomerulosclerosis, such a few abnormal glomeruli were unlikely to account for the nephrotic syndrome. Hypertension-induced changes in the unaffected right kidney probably caused the nephrotic-range proteinuria.     

Functional assessment of the kidneys in a 10 month‐old child with renal artery stenosis by intravoxel incoherent motion

Renovascular stenosis is an important cause for arterial hypertension in childhood. We report a 10‐month‐old girl with arterial hypertension caused by right‐sided renal artery stenosis detected by Doppler ultrasound. Magnetic resonance imaging (MRI) was performed before renal artery angioplasty to depict vascular anatomy in detail and to retrieve additional functional information of the kidneys by analysis of intravoxel incoherent motion (IVIM). The value of quantitative diffusion weighted imaging of the kidneys prior to percutaneous transluminal renal angioplasty (PTRA) is discussed.