Review of outcomes of using lower ethanol concentration (83%) in percutaneous ultrasound-guided renal cyst sclerotherapy in dogs.

BACKGROUND: Percutaneous renal cyst sclerotherapy (PRCS) as a treatment for renal cysts is usually performed with a high concentration of ethanol (≥ 90%). This study reviewed cases in which a lower concentration of ethanol (83%) was used for the procedure in dogs. METHODS: Records of cases of renal cysts treated by sclerotherapy using 83% ethanol in dogs were reviewed. Outcomes of the treatment were evaluated by comparing volumes of renal cysts before the procedure and the volumes after treatment, using ultrasound images with the volume reduction rates classified as follows: < 50% of initial volume (failed); ≥ 50% but < 80% of initial volume (partial success); ≥ 80% but < 95% of initial volume (great success); ≥ 95% of initial volume (complete success). RESULTS: Out of nine dog kidneys, renal cysts sclerotherapy with 83% ethanol achieved partial success in one kidney, great success in four, and complete success in the other four. No side effect was observed. The mean of the volume-reduction rates was 90.00 ± 11.00 while the minimum and maximum reduction rates were 65% and 100%, respectively. CONCLUSIONS: The lower ethanol concentration (83%) is good for disinfecting kidneys in PRCS.

Cystic kidney diseases in children.

Cystic kidney disease comprises a broad group of heterogeneous diseases, which differ greatly in age at onset, disease manifestation, systemic involvement, disease progression, and long-term prognosis. As our understanding of these diseases continues to evolve and new treatment strategies continue to emerge, correctly differentiating and diagnosing these diseases becomes increasingly important. In this review, we aim to highlight the key features of the most relevant cystic kidney diseases, underscore important diagnostic characteristics of each disease, and present specific management options if applicable.

Cystic Renal Masses: Old and New Paradigms.

Cystic renal masses describe a spectrum of lesions with benign and/or malignant features. Cystic renal masses are most often identified incidentally with the Bosniak classification system stratifying their malignant potential. Solid enhancing components most often represent clear cell renal cell carcinoma yet display an indolent natural history relative to pure solid renal masses. This has led to an increased adoption of active surveillance as a management strategy in those who are poor surgical candidates. This article provides a contemporary overview of historical and emerging clinical paradigms in the diagnosis and management of this distinct clinical entity.

Efficacy of single-session 99.5% ethanol sclerotherapy for incidentally found simple renal cysts.

Simple renal cysts are the most common masses in the kidney. Most are asymptomatic and are incidentally detected on imaging examinations performed for other reasons. This study aimed to compare the results of 40 and 120 minutes ethanol sclerotherapies that were performed in a single session to treat incidentally found simple renal cysts. We retrospectively reviewed 63 renal cysts in 62 patients treated by single session percutaneous ethanol sclerotherapy. Thirty-one patients with 32 cysts underwent a 40 minutes sclerotherapy (group A), and 31 patients with 31 cysts underwent a 120 minutes retention technique (group B). Under ultrasonographic and fluoroscopic guidance, cystic fluid was completely aspirated, and 50% of the aspirated volume was replaced with 99.5% ethanol (a maximum of 100 mL). Imaging follow-up of the patients was performed 3 months after sclerotherapy. The technical success rates were 100% in both groups. Eighteen patients (29.0%) were symptomatic (flank pain or discomfort). Indications of the other patients were large cysts (>5 cm; 46%) and an increment in the diameter on serial studies (25.4%). A significant difference between the 2 groups in terms of age, cyst diameter, volume of aspirated fluid, volume of injected ethanol, and percentage of reduction in cyst diameter (P > .05) was not found. After treatment, flank pain or discomfort resolved in 17 of 18 (94.4%) symptomatic patients. One patient complained of persistent flank pain; however, no significant abnormality was detected on post-procedural computed tomography images. There were no other complications after therapy in the 2 groups. Single session ethanol sclerotherapy with a 40 minutes retention technique is an effective, safe, and cost-effective method for the treatment of incidentally found simple renal cysts. Although the procedural time was reduced, there was no significant difference in therapeutic efficacy between the 40 and 120 minutes therapies.

Prediction of treatment response following ethanol sclerotherapy of renal cysts by computed tomography.

OBJECTIVES: To investigate predictive factors of treatment response following ethanol sclerotherapy of large renal cysts via computed tomography (CT). METHODS: Retrospective study reviewed 71 patients (61.0 ± 13.2 years; M:F = 32:39) who underwent pretreatment CT and were treated with sclerotherapy of a large (> 5 cm) renal cyst (mean volume: 279.8 cc) using 99% ethanol from January 2010 to February 2019. Patients were followed up at least two times, short-term (defined as < 6 months, median 2.1 months) and long-term (defined as > 1 year, median 15.5 months), via ultrasound or CT. Suboptimal response was defined as the volume of residual cyst > 20 mL in each follow-up. Predictive variables of radiologic findings and radiomics features were analyzed using logistic regression analysis. RESULTS: Suboptimal response rates were 33.8% and 18.3% at short-term and long-term follow-ups, respectively. In radiologic findings, patients with suboptimal response in the short-term follow-up showed a more frequent estimated cyst volume ≥ 270 mL (OR 14.8, 95% CI 3.9-55.9, p < 0.001) and sinus protrusion (OR 7.0, 95% CI 1.7-28.5, p = 0.007). Cyst volume ≥ 270 mL was also associated with suboptimal response in the long-term follow-up (OR 4.6, 95% CI 1.3-16.9, p = 0.021). When radiomics features were combined, the area under the curve increased from 0.83 to 0.86 and from 0.68 to 0.82 to predict suboptimal response in short-term and long-term follow-ups, respectively. CONCLUSION: Greater estimated volume, sinus protrusion, and radiomics features of the cysts in pretreatment CT can help predict suboptimal response of renal cyst after sclerotherapy.

Type 2 calyceal diverticulum with an unusual appearance in the lower pole of the kidney.

A 45-year-old woman presented to our clinic with intermittent left flank pain. The family physician referred her for renal cystic mass with a calcified appearance. The non-contrast spiral abdominal computed tomographic (CT) scan demonstrated the mass-like cystic lesion with a densely calcified lesion in the lower pole of the kidney. A detailed history revealed that she underwent shock wave lithotripsy (SWL) for the lower pole renal stone one year ago. After SWL, the stone fragments migrated to the dependent diverticulum region and produced the misleading appearance of a Bosniak type III lesion. Contrast-enhanced computed tomography (CT) scan was done for further evaluation, and finally, the diagnosis of the calyceal diverticulum was confirmed in the lower pole of the kidney. Calyceal diverticula are the outpouching of the pyelocalyceal system lined by non-secretory transitional epithelium. It is a rare condition that occurs in less than 0.5% of the population. Most patients are asymptomatic and have been discovered incidentally in routine imaging modalities. As most of the patients are asymptomatic, many do not need intervention. However, in some instances, patients present with flank pain, hematuria, urinary tract infection, and stone formation in the diverticulum. They are in the differential diagnosis of renal cystic lesions such as simple renal cyst, renal cortical abscess, and parapelvic cyst. In renal cystic lesion besides of simple renal cyst or renal cystic mass, we should keep the differential diagnosis of the calyceal diverticulum type 2, especially in patients that underwent SWL for renal stones; the fragmented residual stone may have migrated to this dilated region and produce the deceptive appearance of a Bosniak type III lesion.

Percutaneous Internal Drainage in Symptomatic Renal Parapelvic Cyst Refractory to Sclerotherapy: A Case Report.

Percutaneous sclerotherapy is a safe and effective treatment for renal parapelvic cysts. However, if the cyst is in communication with the adjacent renal pelvocalyceal system, sclerotherapy is contraindicated and alternative treatment should be considered. Here, we report a case of a patient with a symptomatic renal parapelvic cyst that was treated using a novel technique involving percutaneous new tract formation between the cyst and renal pelvis.

Genetic Etiologies, Diagnosis, and Management of Neonatal Cystic Kidney Disease.

Fetal kidney development is a complex and carefully orchestrated process. The proper formation of kidney tissue involves many transcription factors and signaling pathways. Pathogenic variants in the genes that encodethese factors and proteins can result in neonatal cystic kidney disease. Advancements in genomic sequencing have allowed us to identify many of these variants and better understand the genetic underpinnings for an increasing number of presentations of childhood kidney disorders. This review discusses the genes essential in kidney development, particularly those involved in the structure and function of primary cilia, and implications of gene identification for prognostication and management of cystic kidney disorders.

Renal Cystic Echinococcosis: Long-Term Outcomes of Percutaneous Treatment.

PURPOSE: To evaluate the long-term outcomes of percutaneous treatment of renal cystic echinococcosis (CE) stratified by cyst stages according to the World Health Organization (WHO) classification. MATERIALS AND METHODS: Between January 1997 and February 2019, 34 patients with renal CE (18 women; mean age, 38 years) were treated with 3 different percutaneous techniques. According to the World Health Organization classification, the cysts were classified as CE1, CE2, CE3a, and CE3b. Puncture, aspiration, injection, reaspiration (PAIR) or standard catheterization was used for the dimension-based treatment of CE1 and CE3a cysts. Modified catheterization (MoCaT) was used to treat all CE2 and CE3b cysts. Technical and clinical success, complications, and reduction in cyst cavities were evaluated. RESULTS: The technical success rate was 100%. PAIR, standard catheterization, and MoCaT were used to treat 12, 9, and 13 cysts, respectively. The only severe adverse event was a bacterial superinfection that occured in the cyst cavity of a patient (3%) treated with MoCaT. Four patients (12%) experienced mild/moderate periprocedural allergic adverse events and were managed conservatively. The mean length of hospital stay was 1, 5, and 7 days for patients treated with PAIR, standard catheterization, and MoCaT, respectively. The clinical success rate was 97%. In 1 of 34 cysts (3%), recurrence was detected and the cyst was successfully re-treated. During the 10.5-year follow-up period, 95% volume reduction was achieved. The median final cyst volume was 10 mL. CONCLUSIONS: Renal CE can be successfully treated with minimum adverse events and recurrence rates using appropriate percutaneous techniques selected according to their stages as classified according to WHO.

Radiologically guided percutaneous aspiration and sclerotherapy of symptomatic simple renal cysts: a systematic review of outcomes.

Simple renal cysts are common benign lesions of the kidney with up to 4% of patients developing symptoms necessitating intervention including pain and haematuria. We conducted a systematic review of the literature to determine the efficacy and safety of aspiration-sclerotherapy of symptomatic simple renal cysts. A systematic review using Cochrane guidelines was conducted on published literature from 1990 to 2020. RCTs, cohort studies and case series meeting the inclusion criteria were reviewed and cumulative analysis of outcomes was performed. A total of 4071 patients from 57 studies underwent aspiration ± sclerotherapy for their simple renal cysts. 87.7% of patients who had aspiration with sclerotherapy demonstrated 'treatment success' with a >50% reduction in cyst size and complete resolution of symptoms. 453 minor, transient complications occurred (11.2%) whilst a major complication rate of less than 0.1% (4 patients) was reported. Pooled analysis of all available current literature demonstrates that aspiration-sclerotherapy is a safe and effective first-line therapy for symptomatic simple renal cysts. Although we are unable to compare those undergoing aspiration alone to aspiration-sclerotherapy, it is evident the use of a sclerosing agent is integral to treatment success; however, the optimum agent, volume, injection frequency, and dwelling time are yet to be defined. Cyst size should be considered when discussing treatment options; however, we conclude it reasonable for aspiration-sclerotherapy to be used in the first instance in all cases of symptomatic simple renal cysts. Furthermore, we propose definitions of treatment outcome measures in order to allow direct comparative analysis across future studies.

Diagnosis and Management of Renal Cystic Disease of the Newborn: Core Curriculum 2021.

Renal cystic disease encompasses a large variety of illnesses with various phenotypic expressions that can manifest in utero, in infancy, and in childhood. These diseases may be unilateral or bilateral and present with single or multiple cysts. Various cystic diseases may also progress to chronic kidney disease (CKD), including kidney failure, and hepatic disease, thus potentially being life threatening. The prevalence and serious complications of CKD in the pediatric population make it vital that health care providers detect these conditions early and provide effective management. This installment of AJKD's Core Curriculum in Nephrology discusses various genetic and sporadic kidney cystic diseases, including multicystic dysplastic kidney, nephronophthisis, cystic dysplasia, hepatocyte nuclear factor 1-ß (HNF1-ß) nephropathy, Bardet-Biedl syndrome, Meckel-Gruber syndrome, Zellweger syndrome, calyceal diverticulum, autosomal recessive polycystic kidney disease (ARPKD), and autosomal dominant polycystic kidney disease (ADPKD). This article discusses the epidemiology, genetics and pathophysiology, diagnosis, presentation, and management for each of these renal cystic diseases, with particular attention to prenatal care and pregnancy counseling.

Management of complex renal cysts in Canada: results of a survey study.

BACKGROUND: Bosniak III and IV cysts have a high risk of malignancy and have traditionally been managed surgically. However, growing evidence suggests that many can be managed by active surveillance. The main objective of this study was to characterize the use of surveillance in the management of complex renal cysts. METHODS: A web-based survey was sent to all registered, active members of the Canadian Urological Association (N = 583) in October 2018. RESULTS: The survey response rate was 24.7%. Management of Bosniak III cysts varied considerably. A large proportion of respondents (33.1%) offered active surveillance in > 50% of cases. Only 13.7% of respondents reported never or rarely (< 5% of cases) offering surveillance. In contrast, for Bosniak IV cysts, 60.1% of urologists never or rarely offered surveillance, while only 10.1% offer it in > 50% of cases. A significantly greater proportion of academic urologists, compared to non-academic urologists, viewed surveillance as a management option for patients with a Bosniak III or IV cyst. The most commonly reported barriers to a greater adoption of surveillance were concerns regarding its oncologic safety, the lack of data to support surveillance in this population, and the lack of triggers for discontinuation of active surveillance and intervention. CONCLUSIONS: Despite active surveillance being included as a management option in guidelines, many Canadian urologists are reluctant to offer surveillance to patients with Bosniak III or IV cysts. Practice patterns are heterogeneous among those offering surveillance. High-quality studies are required to better define the benefits and risks of cystic renal mass surveillance.

What to Do With Renal Cysts in Children?

OBJECTIVE: To explore the current practice patterns for the management of renal cysts internationally among pediatric urologists and nephrologists. MATERIALS AND METHODS: A survey composed of 21 questions and 4 clinical scenarios was distributed to pediatric urologists and nephrologists. Survey questions evaluated optimal imaging modality, management, and follow-up period. Interspecialty comparisons were made using chi-square analysis where appropriate. RESULTS: A total of 183 respondents completed the survey (128 pediatric urologists, 37 pediatric nephrologists, and 19 other specialists). Most (57%) respondents agreed or strongly agreed with using an ultrasound based Bosniak classification to categorize renal cysts in children. The most commonly used follow-up intervals were 6-12 months for pediatric urologists and 1-2 years for pediatric nephrologists. Symptomatic mass effect (80.9%), gross hematuria (79.2%), or family history were the most common reasons for escalating surveillance. Pediatric nephrologists were more likely to increase follow-up with development of bilateral simple renal cysts (P = .008) or chronic kidney disease (P = .027) when compared to pediatric urologists. Laparoscopic marsupialization (39.4%) was the most common treatment for a simple renal cyst in a symptomatic child. Modified Bosniak III cysts had more heterogeneity in treatment based on the physician responses. CONCLUSION: There is currently no consensus on the optimal protocol for the surveillance, imaging, or treatment of renal cysts in children. Most respondents agree that using an ultrasound-based Bosniak classification is reasonable. A call to action is therefore necessary for the development of registries and guidelines on the management of pediatric renal cysts and their associated malignancies.

A Painless and Time-Saving Modified Technique for Simple Renal Cyst Treatment with Single-session Ethanol Sclerotherapy.

Percutaneous puncture ethanol sclerotherapy is a simple, effective, minimally invasive, and inexpensive procedure to manage symptomatic simple renal cysts. We modified specific technical aspects to balance certain potential intraprocedural factors for ensuring minimal procedural pain and duration as well as maximal clinical therapeutic effects and evaluated the safety and efficacy of this modified technique. A total of 84 eligible patients underwent computed tomography-guided single-session ethanol sclerotherapy using the conventional (group A) or modified (group B) technique. In group B, the puncture route was modified from tansparenchymal to direct for reducing renal injury, and preinjection of low-dose intracystic lidocaine was used to control distending pain caused by ethanol injections; therefore, greater ethanol volumes could be injected for improving the resistance and contact of ethanol with the cyst wall, precluding the need for patient repositioning multiple times to decrease procedural duration. Visual analog scale score for pain and procedural time were significantly higher in group A than in group B. The complication rate was slightly higher in group A than in group B, but the success rate was comparable between the two groups. These results suggest that the modified technique is painless, time-saving, and injury-reducing and can thus improve medical care.

Recommendations for the management of renal involvement in the tuberous sclerosis complex. / Recomendaciones de manejo de la afectación renal en el complejo esclerosis tuberosa.

Tuberous sclerosis complex (TSC) is a rare, hereditary, multisystemic disease with a broad phenotypic spectrum. Its management requires the collaboration of multiple specialists. Just as in the paediatric age, the paediatric neurologist takes on special importance; in adulthood, renal involvement is the cause of the greatest morbidity and mortality. There are several recommendations on the general management of patients with TSC but none that focuses on renal involvement. These recommendations respond to the need to provide guidelines to facilitate a better knowledge and diagnostic-therapeutic management of the renal involvement of TSC through a rational use of complementary tests and the correct use of available treatments. Their elaboration has been based on consensus within the hereditary renal diseases working group of the SEN/REDINREN (Spanish Society of Nephrology/Kidney Research Network). It has also counted on the participation of non-nephrologist specialists in TSC in order to expand the vision of the disease.

Healthcare recommendations for Joubert syndrome.

Joubert syndrome (JS) is a recessive neurodevelopmental disorder defined by a characteristic cerebellar and brainstem malformation recognizable on axial brain magnetic resonance imaging as the "Molar Tooth Sign". Although defined by the neurological features, JS is associated with clinical features affecting many other organ systems, particularly progressive involvement of the retina, kidney, and liver. JS is a rare condition; therefore, many affected individuals may not have easy access to subspecialty providers familiar with JS (e.g., geneticists, neurologists, developmental pediatricians, ophthalmologists, nephrologists, hepatologists, psychiatrists, therapists, and educators). Expert recommendations can enable practitioners of all types to provide quality care to individuals with JS and know when to refer for subspecialty care. This need will only increase as precision treatments targeting specific genetic causes of JS emerge. The goal of these recommendations is to provide a resource for general practitioners, subspecialists, and families to maximize the health of individuals with JS throughout the lifespan.

Pathologic characterization of renal epithelial neoplasms arising in nonfunctioning kidneys.

Nonfunctioning kidneys secondary to various etiologies display different histopathological features. Studies focused on incidence and types of renal neoplasms using the new World Health Organization and International Society of Urological Pathology classification system in various types of nonfunctioning kidneys are very limited. We identified 311 nephrectomies of nonfunctioning kidneys and categorized them into 5 categories: acquired cystic kidney disease (ACKD, n = 61); end-stage renal disease, nonspecific (ESRD, n = 63); adult polycystic kidney disease (APKD, n = 49); failed transplant kidney (FTK, n = 96); and those caused by obstructive conditions in the kidney (OCK, n = 42). ACKD (70%) and ESRD (43%) had higher cancer incidences than the other 3 groups (APKD = 2%, FTK = 0%, and OCK = 5%). Besides clear cell renal cell carcinoma (RCC) and papillary RCC, clear cell papillary RCC had a much higher incidence within ACKD patients (13/61) compared to other groups. ACKD-associated RCC was only identified in ACKD patients. ACKD patients had significantly longer dialysis duration compared to ESRD, APKD, and FTK. Although they had similar risk for clear cell RCC and papillary RCC, ACKD patients had a much higher risk for ACKD-associated RCC and clear cell papillary RCC than ESRD patients. Although most RCCs arising in these nonfunctioning kidneys were early pT1 stage, 6 ACKD patients and 3 ESRD patients had higher-stage diseases, which can be fatal if not treated appropriately. Therefore, precise clinicopathological classification of these nonfunctioning kidneys is important for predicting kidney cancer risk. These results indicate the need for active monitoring of the patients with high-risk nonfunctioning kidney diseases and appropriate surgical treatment when necessary.