Robot-Assisted Pyeloureterostomy in the Treatment of the Lower Pole Ureteropelvic Junction Obstruction in an Incomplete Duplicated System.

PURPOSE: To describe the surgical treatment of a patient harboring a ureteropelvic junction obstruction in the lower unit of an incomplete duplicated collecting system. METHODS: A female patient with 32-year old presented a progressively increasing left lumbar pain for 1 year and urinary tract infections for the past 10 years. Computed tomography demonstrated a ureteropelvic junction obstruction in the lower unit of an incomplete duplicated urinary collecting system. RESULTS: The patient underwent a robot-assisted pyeloureterostomy. The surgical time was 118 minutes and there was no intraoperative complication. During surgery, anomalous vessels crossing to the lower pole of the kidney were identified. The bladder catheter and abdominal drain were removed at the first- and second postoperative days. The length of the hospital stay was 2 days and the postoperative course was uneventful. The double-J stent was retrieved 4 weeks after surgery. At 18 months of follow-up, the patient had complete pain resolution and no new episodes of urinary tract infections. The control image exam revealed minimal residual hydronephrosis. CONCLUSION: Robot-assisted pyeloureterostomy was an effective approach to the present case. Many times, the surgical strategy for patients with anatomic variants is determined intra-operatively. The surgeon must have a wide range of skills and be prepared for a timely decision.

Concomitant multiple anomalies of renal vessels and collecting system.

Although anomalies of renal vessels and collecting system are relatively frequent, their concomitant occurrence is a rare event. During dissection of a 75-year-old male formalin-embalmed cadaver, we found multiple variations in the renal vessels and renal collecting system. Both kidneys were normal in size and anteriorly malrotated, with duplex collecting system and duplex ureter. One ureter drained the upper part of the kidney and the second ureter drained the lower part of the kidney. Superior and inferior collecting systems were separated by renal parenchyma. The right kidney had two renal arteries, the first renal artery (main renal artery) originating from the abdominal aorta, passing behind the inferior vena cava (IVC) and entering the kidney through the superior and inferior renal hilum. The second artery was the inferior polar artery. In addition, the right kidney had two renal veins as well. Three renal tributaries emerged from the upper and lower portion of the right renal hilum, and they joined to form the main renal vein which drained into the IVC. The lower renal vein was the inferior polar vein. The left kidney had four renal arteries (two hilar arteries and two polar arteries). The main left renal vein emerged from both superior and inferior left renal hilum, passed in front of the abdominal aorta and drained into the IVC. The left kidney also had the inferior polar vein which was divided behind the aorta (retro aortic vein) into two venous trunks. These venous trunks drained separately into posteromedial aspect of the IVC. Finally, the right testicular vein was formed by two tributaries and drained into the IVC, whereas the two left testicular veins drained separately into the left main renal vein.

Uropatía obstructiva en paciente con doble sistema colector / Obstructive uropathy in a patient with double district ureter

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Endothelial marker-expressing stromal cells are critical for kidney formation.

Kidneys are highly vascularized and contain many distinct vascular beds. However, the origins of renal endothelial cells and roles of the developing endothelia in the formation of the kidney are unclear. We have shown that the Foxd1-positive renal stroma gives rise to endothelial marker-expressing progenitors that are incorporated within a subset of peritubular capillaries; however, the significance of these cells is unclear. The purpose of this study was to determine whether deletion of Flk1 in the Foxd1 stroma was important for renal development. To that end, we conditionally deleted Flk1 (critical for endothelial cell development) in the renal stroma by breeding-floxed Flk1 mice (Flk1fl/fl ) with Foxd1cre mice to generate Foxd1cre; Flk1fl/fl (Flk1ST-/- ) mice. We then performed FACsorting, histological, morphometric, and metabolic analyses of Flk1ST-/- vs. control mice. We confirmed decreased expression of endothelial markers in the renal stroma of Flk1ST-/- kidneys via flow sorting and immunostaining, and upon interrogation of embryonic and postnatal Flk1ST-/- mice, we found they had dilated peritubular capillaries. Three-dimensional reconstructions showed reduced ureteric branching and fewer nephrons in developing Flk1ST-/- kidneys vs. CONTROLS: Juvenile Flk1ST-/- kidneys displayed renal papillary hypoplasia and a paucity of collecting ducts. Twenty-four-hour urine collections revealed that postnatal Flk1ST-/- mice had urinary-concentrating defects. Thus, while lineage-tracing revealed that the renal cortical stroma gave rise to a small subset of endothelial progenitors, these Flk1-expressing stromal cells are critical for patterning the peritubular capillaries. Also, loss of Flk1 in the renal stroma leads to nonautonomous-patterning defects in ureteric lineages.

Aquaporin-3 deletion in mice results in renal collecting duct abnormalities and worsens ischemia-reperfusion injury.

Aquaporin-3 (AQP3), a transporter of water, glycerol and H2O2, is expressed in basolateral membranes of principal cells in kidney collecting duct. Here, we report that AQP3 deletion in mice affects renal function and modulates renal injury. We found collecting duct hyperplasia and cell swelling in kidneys of adult AQP3 null mice. After mild renal ischemia-reperfusion (IR), AQP3 null mice had significantly greater blood urea nitrogen (57mg/dl) and creatinine (136µM) than wild-type mice (35mg/dl and 48µM, respectively), and showed renal morphological changes, including tubular dilatation, erythrocyte diapedesis and collecting duct incompletion. MPO, MDA and SOD following IR in AQP3 null mice were significantly different from that in wild-type mice (1.7U/g vs 0.8U/g, 3.9µM/g vs 2.4µM/g, 6.4U/mg vs 11U/mg, respectively). Following IR, AQP3 deletion inhibited activation of mitogen-activated protein kinase (MAPK) signaling and produced an increase in the ratios of Bax/Bcl-2, cleaved caspase-3/caspase-3 and p-p53/p53. Studies in transfected MDCK cells showed that AQP3 expression attenuated reduced cell viability following hypoxia-reoxygenation, with reduced apoptosis and increased MAPK signaling. Our results support a novel role for AQP3 in modulating renal injury and suggest the mechanisms involved in protection against hypoxic injury.

Robot-assisted laparoscopic lower pole partial nephrectomy in the pediatric patient.

INTRODUCTION: In pediatric urology, partial nephrectomy is used primarily to remove a non-functioning renal moiety in a duplicated system. There are few data on infants undergoing this procedure. As such, we present a robot-assisted laparoscopic lower pole partial nephrectomy in an infant. METHODS: Our patient was an 11-month-old (10.7 kg) male with a history of prenatal hydronephrosis, who was diagnosed postnatally with a duplicated right collecting system and severe hydroureteronephrosis of the right lower collecting system. A DMSA scan demonstrated no radiotracer uptake in the right lower pole. A robot-assisted laparoscopic lower pole partial nephrectomy was performed. RESULTS: A lower pole partial nephrectomy was accomplished. At 1 month postoperatively, an ultrasound demonstrated no hydronephrosis or perinephric fluid collection. CONCLUSIONS: Robotic partial nephrectomy is safe and feasible in pediatrics including both older children and infants. It is successful for both upper and lower pole partial nephrectomies.

Duplicated Renal Collecting System With Ectopic Ureter in Female Bladder Exstrophy: A Case Report.

Bladder exstrophy is a rare major birth defect which requires specialized surgical care. Associated genitourinary tract anomalies are extremely rare in this population, yet significantly impact surgical planning and outcomes. In this case, a full-term newborn girl with a prenatal diagnosis of classic bladder exstrophy was found to have a complete duplicated left collecting system with an ectopic ureter inserted to urethral plate. The patient underwent modern staged repair of exstrophy with bilateral anterior innominate osteotomies and concomitant ureteral reimplantation-the first reported case of ureteral reimplantation at the time of initial closure in a newborn.

Right lump kidney with varied vasculature and urinary system revealed by multidetector computed tomographic (MDCT) angiography.

Renal ectopia also known as ectopic kidney is an embryological renal anomaly characterised by abnormal anatomical location of one or both of the kidneys. This can occur in various forms such as (1) crossed fused renal ectopia, (2) ectopic thoracic kidney and (3) pelvic kidneys. The lump kidney is one of the six variations of crossed fused ectopic kidney. Throughout life, the patient may remain asymptomatic, however, symptomatic patients may present with minor traumatic injuries due to the abnormal location or normal kidney pathologies. During normal embryological development, there is cephalic migration during which the kidneys ascend to their normal retroperitoneal location; therefore, an ectopic location is as a result of arrested migration. During this embryological development the kidney has multiple aorto-illiac branches, which degenerate when the kidney reaches its normal location. Here they develop new renal branches from the aorta and during an arrested ascent the ectopic kidney tends to retain some of the older aorto-iliac vessels. Hence, the arterial supply and the veineux drainage are grossly abnormal, reflecting the metanephric malascent and the primitive vascular arrangement. The collecting systems also present with important anatomical variations. We present an extremely rare case of right lump kidney with six renal arteries, two renal veins and two duplicated pelvicalyceal systems.

Robot assisted heminephrectomy for duplicated renal collecting system: technique and outcomes.

BACKGROUND: The aim of this study was to describe experience with robotic assisted hemi-nephrectomy (RAHN) for the management of duplicated renal collecting system. METHODS: The authors' institutional database was queried to identify patients who underwent RAHN from 2007 to 2013 for the management of a duplicated system. Patient demographics, indications and surgical outcomes were reviewed and analyzed. RESULTS: Five patients were identified with a duplicated system of which all had a history of pain and recurrent infection. Three patients underwent hemi-nephrectomy for a poorly functioning lower pole and two for an abnormal upper pole moiety. All operations were completed successfully using a robotic approach. There were only two minor (Clavien grade 1-2) complications. CONCLUSIONS: RAHN is a viable option for the surgical treatment of an atrophic, chronically infected duplicated system. The unique features of robotic technology offer enhanced dexterity and vision facilitating the management of complex vascular and ureteral anatomy.

Step-by-Step robotic heminephrectomy for duplicated renal collecting system.

INTRODUCTION: A duplicated renal collecting system is a relatively common congenital anomaly rarely presenting in adults. AIM: In this video we demonstrate our step-by-step technique of Robotic heminephrectomy in a patient with non-functioning upper pole moiety. MATERIALS AND METHODS: Following cystoscopy and ureteral catheter insertion the patient was placed in 60° modified flank position with the ipsilateral arm positioned at the side of the patient. A straight-line, three arm robotic port configuration was employed. The robot was docked at a 90-degree angle, perpendicular to the patient. Following mobilization the colon and identifying both ureters of the duplicated system, the ureters were followed cephalically toward, hilar vessels where the hilar anatomy was identified. The nonfunctioning pole vasculature was ligated using hem-o-lok clips. The ureter was sharply divided and the proximal ureteral stump was passed posterior the renal hilum. Ureteral stump was used as for retraction and heminephrectomy is completed along the line demarcating the upper and lower pole moieties. Renorrhaphy was performed using 0-Vicryl suture with a CT-1 needle. The nonfunctioning pole ureter was then dissected caudally toward the bladder hiatus, ligated using clips, and transected. RESULTS: The operating time was 240 minutes and blood loss was 100 cc. There was no complication post-operatively. CONCLUSIONS: Wrist articulation and degree of freedom offered by robotic platform facilitates successful performance of minimally invasive heminephrectomy in the setting of an atrophic and symptomatic renal segment.

Step-by-Step robotic heminephrectomy for duplicated renal collecting system

Introduction A duplicated renal collecting system is a relatively common congenital anomaly rarely presenting in adults. Aim In this video we demonstrate our step-by-step technique of Robotic heminephrectomy in a patient with non-functioning upper pole moiety. Materials and Methods Following cystoscopy and ureteral catheter insertion the patient was placed in 600 modified flank position with the ipsilateral arm positioned at the side of the patient. A straight-line, three arm robotic port configuration was employed. The robot was docked at a 90-degree angle, perpendicular to the patient. Following mobilization the colon and identifying both ureters of the duplicated system, the ureters were followed cephalically toward, hilar vessels where the hilar anatomy was identified. The nonfunctioning pole vasculature was ligated using hem-o-lok clips. The ureter was sharply divided and the proximal ureteral stump was passed posterior the renal hilum. Ureteral stump was used as for retraction and heminephrectomy is completed along the line demarcating the upper and lower pole moieties. Renorrhaphy was performed using 0-Vicryl suture with a CT-1 needle. The nonfunctioning pole ureter was then dissected caudally toward the bladder hiatus, ligated using clips, and transected. Results The operating time was 240 minutes and blood loss was 100 cc. There was no complication post-operatively. Conclusions Wrist articulation and degree of freedom offered by robotic platform facilitates successful performance of minimally invasive heminephrectomy in the setting of an atrophic and symptomatic renal segment. .

Robotic-assisted partial nephrectomy in duplicated collecting systems in the pediatric population: techniques and outcomes.

OBJECTIVE: We present our experience with pediatric robotic-assisted laparoscopic partial nephrectomy of a nonfunctioning moiety in a duplicated system (RALPN), comparing techniques and outcomes with those previously reported in the literature. To our knowledge, this is the largest series of this surgical procedure to date. PATIENTS AND METHODS: We retrospectively reviewed all pediatric patients at our institution that had undergone RALPN from 2006 to 2012. RESULTS: Twenty-one patients underwent RALPN between 2006 and 2012. Mean patient age was 4.1 years. Mean operative time was 301 min. Mean estimated blood loss was 36 ml. Mean length of stay was 38 h. The majority of cases were performed with three laparoscopic ports. At initial follow-up ultrasound 6/21 (29%) demonstrated a fluid collection. The majority of these collections occurred in cases where the resection defect was not closed intraoperatively (42% of cases vs. 11% of cases). All fluid collections were asymptomatic and managed conservatively. CONCLUSION: RALPN is associated with low complication rates. The robotic system allows for the use of only two small robotic working ports in most cases. Postoperative fluid collections may be prevented by formal closure of the polar defect, but fluid collections that do occur can be followed conservatively.

Pediatric robotic-assisted laparoscopic ipsilateral ureteroureterostomy in a duplicated collecting system.

INTRODUCTION AND OBJECTIVE: Duplex renal collecting system is a common congenital anomaly in the pediatric population. The superior moiety ureter is often associated with an ectopic insertion. A common site of insertion is the urethra, which results in continuous incontinence. A novel repair for this abnormality is a robotic assisted laparoscopic ipsilateral ureteroureterostomy with end to side anastomosis. This teaching video will demonstrate positioning and operative technique for this procedure. PATIENT AND METHODS: This is the case of a 5-year-old girl who presented with lifelong continuous urinary incontinence. Workup revealed a duplicated collecting system with a functional superior moiety and ectopic ureter inserting into the urethra. A laparoscopic ipsilateral ureteroureterostomy was performed using a robotic surgical system. RESULTS: After docking the robotic surgical system, the duplicated collecting system was identified and an ipsilateral ureteroureterostomy was performed. The patient tolerated the procedure well and the ultrasound on follow up revealed no residual hydronephrosis. CONCLUSION: The accompanying teaching video shows appropriate positioning and operative technique for a robotic-assisted laparoscopic ureteroureterostomy. This innovative and minimally invasive technique is a safe and feasible approach to repair a duplex collecting system with an ectopic ureter in the pediatric population.

Robot-assisted upper pole nephrectomy in adult patients with duplicated renal collecting systems.

Duplicated renal collecting systems are a common congenital anomaly, with patients usually presenting in childhood, rarely in adult life. To our knowledge, only one case of robot-assisted heminephrectomy in adults has been described. We reviewed the medical records of four adult patients with symptomatic unilateral duplicated collecting systems and nonfunctioning upper pole renal units (NFUPRU) who underwent robot-assisted laparoscopic heminephrectomy at our institutions. Heminephrectomy for NFUPRU should be approached differently than partial nephrectomy, because of complex vascular anatomy. Patients who are undergoing this procedure have brief hospital stays, minimal morbidity, preservation of renal function, and resolution of symptoms. Robot-assisted laparoscopy is well suited for this procedure because it allows improved visualization during dissection of the unique vascular anatomy as well as scaling of surgeon movements and improved ergonomics.

Renal collecting system growth and function depend upon embryonic γ1 laminin expression.

In order to understand the functions of laminins in the renal collecting system, the Lamc1 gene was inactivated in the developing mouse ureteric bud (UB). Embryos bearing null alleles exhibited laminin deficiency prior to mesenchymal tubular induction and either failed to develop a UB with involution of the mesenchyme, or developed small kidneys with decreased proliferation and branching, delayed renal vesicle formation and postnatal emergence of a water transport deficit. Embryonic day 12.5 kidneys revealed an almost complete absence of basement membrane proteins and reduced levels of α6 integrin and FGF2. mRNA levels for fibroblast growth factor 2 (FGF2) and mediators of the GDNF/RET and WNT11 signaling pathway were also decreased. Furthermore, collecting duct cells derived from laminin-deficient kidneys and grown in collagen gels were found to proliferate and branch slowly. The laminin-deficient cells exhibited decreased activation of growth factor- and integrin-dependent pathways, whereas heparin lyase-treated and ß1 integrin-null cells exhibited more selective decreases. Collectively, these data support a requirement of γ1 laminins for assembly of the collecting duct system basement membrane, in which immobilized ligands act as solid-phase agonists to promote branching morphogenesis, growth and water transport functions.

[Hybrid-NOTES transvaginal hemi-nephrectomy for duplicated renal collecting system in the adult patient]. / Heminefrectomía NOTES-híbrido transvaginal por duplicidad del sistema colector renal en el paciente adulto.

INTRODUCTION: Duplication of the ureter and renal pelvis is the most common anomaly of the upper urinary tract. Upper pole heminephrectomy is a treatment option when duplication anomalies are associated with ureteral ectopia or ureterocele with an associated nonfunctioning or infected upper pole moiety. MATERIAL AND METHOD: We describe a NOTES hybrid transvaginal upper pole heminephrectomy in a 24 year old with recurrent infections in a poorly functioning right upper pole moiety. The procedure was performed with a bariatric trocar in the vagina, and a multichannel single-port device (Triport, Olympus Surgical) in the umbilicus. An ultrasonic scalpel was used for the heminephrectomy. The specimen was retrieved through the vagina. RESULTS: Operative time was 150 minutes and blood loss 50 cc. One week later the patient developed urinoma at the surgical site and was re-explored laparoscopically. The cut edge of the heminephrectomy defect was fulgurated and a drain placed. The patient recovered uneventfully following re-exploration. CONCLUSIONS: We describe the technique for transvaginal Hybrid-NOTES heminephrectomy. This approach requires further development with respect to instrumentation, and surgical expertise. The combined umbilical and vaginal approached restored triangulation and facilitates dissection, but more experience is required to determine safety, efficacy and reproducibility.