Pelvic Dilatation Research Articles

Stentless pyeloplasty for ureteropelvic junction obstruction in children

Introduction Stenting after pyeloplasty is an established practice and helps in ensuring a patent anastomosis until healing has completed. Stents, however, may cause complications such as infection and displacement and increase the cost of management; therefore, stentless pyeloplasty is now considered as feasible alternative. Patients and methods From August 2008 to October 2010, we retrospectively analyzed the results of stentless surgery in patients with ureteropelvic junction (UPJ) obstruction. In all, 42 patients with UPJ obstruction were managed. Nine patients who were treated conservatively, one who underwent nephrectomy, and one pyelostomy for pyonephrosis in solitary kidney were excluded. Age range at surgery was 14 days–12 years with a mean age of 12.7 months. Results There were 23 male patients and eight female patients with a male-to-female ratio of 3 : 1. Fourteen patients had left, 12 right, and five had bilateral UPJ obstruction. A total of 34 pyeloplasties were performed in 31 patients. Of the five patients with bilateral UPJ obstruction, three underwent bilateral pyeloplasty and remaining two underwent unilateral pyeloplasty with conservative management on the opposite sides. Two patients underwent laparoscopic pyeloplasties and 32 pyeloplasties by open technique. Double J stent was placed at initial surgery in three patients and 31 stentless pyeloplasties were performed. The mean operative time was 75 min. The mean perinephric drain removal time was 2 days. None of the patients had persistent urinary leak. The mean hospital stay was 3.2 days. Reduction in anteroposterior diameter was noticed in 91% cases on 12 weeks follow-up scan. Complications included persistent or increase in hydronephrosis in three (9%) patients. In all the three patients, cystoscopic stenting was attempted. In two patients, size 4 Fr double J stent was passed easily into the renal pelvis. One patient improved, whereas other still has a dilated pelvis with static anteroposterior diameter after removal of stent at 6 weeks; patient is kept on close surveillance on regular ultrasonography. Re-exploration was performed in one patient, which showed kinking at the anastomosis site. Pyeloplasty was revised and patient improved. Other complications included lumbar hernia in one patient, which improved at 6-month follow-up, and stitch granuloma in one patient, which improved after removal of residual stitch. Conclusion Stentless surgery for UPJ obstruction is a safe and feasible technique; it reduces the cost of surgery and avoids multiple procedures. Keywords : management, pyeloplasty, stentless, ureteropelvic junction obstruction

Transposition des vaisseaux polaires pour syndrome de la jonction pyélo-urétérale : expérience préliminaire

Lower pole vessels are a classical cause of PUJO even in children. The "gold standard" in the management of PUJ obstructions remains a dismembered pyeloplasty as described by Anderson and Hynes. However, some authors have developed an alternative procedure to this approach with encouraging results. The aim of our study was to evaluate our preliminary results concerning laparoscopic vascular hitch for crossing vessels. We conducted a retrospective, monocentric study of all patients managed by this technique from January 2010 to December 2012. Eleven patients (7 boys, 4 girls) were managed by laparoscopy at a mean age of 10.7 years (5.4-17). They were referred to our center for clinical symptoms (intermittent pain 7, high blood pressure 1, UTI 1), antenatal diagnosis or accidental discovery. Obstruction was confirmed by MAG3 nephrogram and the presence of obstructive vessels by tomodensitometry or MRI. Mean operative time was 90.2minutes (48-184). Seven patients over 11 were strictly managed by laparoscopic transposition of lower pole vessels. Four required a classical video-assisted dismembered pyeloplasty due to a potential intraluminal stenosis. The latter were suspected by a distension test with furosemid in all four cases. Mean follow-up was 12.9±3 months. Nine patients over 11 were totally non symptomatic, whereas 2 still present mild intermittent pain. In all cases, ultrasound scans show an improvement of the pelvic dilatation. Laparoscopic transposition of lower pole vessels is a suitable and feasible alternative for the management of obstructive PUJ. Our preliminary experience emphasizes the need for a precise preoperative selection of patients along with a per operative evaluation of the obstruction. Further experience seems required to improve our criteria in this indication. 5.

Urinary Tract Infections in a Neonatal Intensive Care Unit

Our aim was to describe laboratory findings and imaging results for neonatal patients diagnosed with urinary tract infection (UTI). Medical records were reviewed for infants diagnosed with UTI in a single neonatal intensive care unit (NICU) over a 13-year period. Of the 8,241 patients admitted to the NICU during the study period, 137 infants were diagnosed with UTI. Imaging was reviewed for 101 patients. Renal pelvis dilation was found in 34% of patients and vesicoureteral reflux was found in 21%. Renal pelvis dilation was not associated with reflux (OR: 0.53 [95% CI: 0.18-1.5]). The sensitivity of urinalysis to detect a positive culture was 76%, and the specificity was 41%. Tests of cure for bacterial infections were uniformly negative. Renal pelvis dilation was common but not associated with reflux among NICU patients diagnosed with UTI. Diagnostic criteria in this population are not well defined, and guidelines are needed for diagnosis and management of UTIs in NICU patients.

Prevalence of upper urinary tract anomalies in hospitalized premature infants with urinary tract infection.

The 2011 American Academy of Pediatrics (AAP) guidelines address imaging after initial febrile urinary tract infection (UTI) in infants >2 months of age. We sought to determine the frequency of upper urinary tract anomalies (hydronephrosis and vesicoureteral reflux (VUR)) in hospitalized premature infants with UTI. We retrospectively reviewed the electronic medical records of neonatal intensive care unit (NICU) admissions at a tertiary care children's hospital between 1 January 2006 and 31 December 2010. We queried the records for UTI, renal ultrasound (US) and voiding cystourethrogram (VCUG). We identified 3518 unique admissions. UTI occurred in 118 infants (3%). Sixty-nine (60%) had a normal US. Renal dilation was predominantly renal pelvic dilation (12%) and isolated caliectasis (22%). VUR was identified in 15 (14%) infants evaluated with a VCUG. VUR was identified in nine (12%) infants without and in seven (16%) with an abnormality on US. Reflux was identified in 7% of male and 38% of female infants with a UTI. Anatomic abnormalities of the upper urinary tract are uncommon in premature infants with a UTI that occurs during neonatal hospitalization. In concordance with the AAP guidelines, a VCUG may not be required in all NICU infants under age 2 months after a single UTI.

Urgent percutaneous nephrolithotomy for acute kidney injury secondary to bilateral stones: is it safe and effective in infants?

To evaluate the efficacy and safety of urgent percutaneous nephrolithotomy (PNL) for acute kidney injury secondary to bilateral upper urinary stones in infants. A total of seven infants (five boys and two girls), aged from 5 to 12 months, underwent urgent PNL for calculus anuria from September 2011 to March 2013. The initial blood test revealed acute renal injury in all infants. After correcting electrolyte imbalance and acid-base status through medical treatment in a short time without dialysis, all urgent PNL procedures were performed with 16F percutaneous access and small-diameter nephroscopes designed specifically for pediatric surgery on the side with the more dilated pelvis. Stones were fragmented with a pneumatic lithotripter. Stones were completely removed from the operative side kidneys. The operating time ranged from 35 to 57 min. Blood BUN, serum creatinine, and electrolyte levels returned to normal on postoperative 36 h. There were no major perioperative or postoperative complications and deaths. When performed by experienced endourologists, urgent PNL is a safe and effective procedure in infants for acute kidney injury secondary to bilateral upper urinary stones.

The “flowerpot” sign: Inference of poor renal function in high grade vesicoureteral reflux by calyceal orientation

Modern radiographic advances have allowed for detailed and accurate imaging of not only urologic anatomy but also urologic function. The art of observational inference of subtle anatomic features and function from a static radiograph is being traded for new, more precise, and more expensive modalities. While the superiority of these methods cannot be denied, the total information provided in simpler tests should not be ignored. The relationship between high grade vesicoureteral reflux with the dilated calyces arranged cephalad to a dilated funnel-shaped renal pelvis on VCUG and reduced differential renal function has not been previously described, but has been anecdotally designated a "flowerpot" sign by our clinicians. We hypothesize that the appearance of a "flowerpot" kidney as described herein is an indicator of poor renal function in the setting of high grade VUR. IRB approval was obtained and 315 patients were identified from system-wide VCUG reports from 2004-2012 with diagnosed "high grade" or "severe" vesicoureteral reflux. Inclusion into the study required grade IV or V VUR on initial VCUG and an initial radionuclide study for determination of differential function. Patients with a solitary kidney, posterior urethral valve, multicystic dysplastic kidney, renal ectopia, or duplex collecting systems were excluded. Grade of reflux, angle of the inferior-superior calyceal axis relative to the lumbar spine, and differential uptake were recorded along with presence of the new "flowerpot" sign. Variables were analyzed using the Mann-Whitney U test to determine statistical significance. Fifty seven patients met inclusion criteria with 11 being designated as "flowerpot" kidneys. These "flowerpot" kidneys could be objectively differentiated from other kidneys with grade IV and/or grade V VUR both by inferior-superior calyceal axis (median angle, 52° [37-66] vs. 13° [2-37], respectively p < 0.001) and by differential renal uptake (median, 23% [5-49] vs. 45% [15-81], respectively p < 0.001). Likewise, there was no difference between either calyceal axis (median angle, 13° [3-20] vs. 13° [2-37]) or differential function (median, 48% [24-81] vs. 40% [15-66], p = 0.129) when comparing kidneys with grade IV and grade V VUR, respectively, that did not demonstrate the "flowerpot" sign. Grading of VUR is used to provide a common language for scientific discussion and determine prognosis for children with similar attributes. The dysmorphic calyceal system in the "flowerpot" kidneys supports the theory of abnormal renal blastema induction associated with abnormal differentiation of the ureteral bud. Even in the absence of urinary tract infections and/or pyelonephritis, renal abnormalities and decreased differential function can be observed on renal scintigraphy. This study also confirms the male predominance and functional similarities between grade 4 and 5 refluxing renal units. Recognizing this is a limited observational study based on imaging alone, the "flowerpot" sign is an indicator of the most severe form of grade 5 VUR but is only one factor in predicting long term overall renal prognosis. However, 14% (8/57) of our cohort had a relative uptake of less than 20% with 5 of these exhibiting the "flowerpot" sign. The "flowerpot" sign on VCUG can be used as indirect evidence of poor differential renal function and, therefore, useful in guiding parental expectations prior to formal functional imaging.

PO-0620 Antenatal Hydronephrosis – What Does It Mean Postnatally?

Background and aims There has been an increase in reporting of antenatal hydronephrosis with increasing use of antenatal sonography. It is known that renal pelvic dilatation (RPD) occurs in 1% of fetuses. This study was aimed to ascertain the postnatal outcome associated with antenatal RPD or other renal abnormality and to recommend a guideline for postnatal management. Methods We retrospectively analysed data of 50 infants born between August 2011 and July 2013. 49 infants had renal tract abnormality detected on antenatal sonography. Inclusion criteria were presence of renal tract anomaly or isolated RPD ≥7 mm on prenatal sonography, postnatal ultrasound and at least one follow up in designated Consultant clinic. All infants with RPD ≥7 mm were started on prophylactic Trimethoprim. Results Out of 50 infants, 16 (32%) had normal postnatal scan. 11 (22%) infants had transient/idiopathic hydronephrosis. The second most common diagnosis was Unilateral Pelvi ureteric Junction (PUJ) anomaly found in 9 (18%) infants. Out of these 9 infants, 5 (55.5%) had antenatal RPD ≥15 mm. Only 4 infants were noted to have Vesico Ureteric Reflux (VUR). In total, 11 out of 50 (22%) infants required urological intervention. None of 49 infants have had a culture positive urinary tract infection. Conclusion These data support that most cases of antenatal RPD, especially isolated unilateral RPD ≤10 mm resolve spontaneously. In contrast, infants with RPD ≥15 mm either unilateral or bilateral had significant uropathy requiring intervention. We also recommend that antibiotic prophylaxis be targeted to high risk infants (RPD ≥10 mm).

PO-0780 Postnatal Follow-up Of Newborns With Prenatal Diagnosis Of Hydronephrosis

<h3>Background</h3> Prenatal Hydronephrosis is diagnosed in 1–5% of pregnancies worldwide. The diagnosis of antenatal hydronephrosis (ANH) causes stress to the parents and dilemmas to the paediatrician. <h3>Objectives</h3> To examine the correlation between the degree of the renal pelvic dilatation (RPD) detected by the first 2–5 days of life and the postnatal outcome. To investigate the correlation between bilateral hydronephrosis and the nephrologic outcome. To discuss the possibility of decreasing the postnatal examinations of these healthy babies. <h3>Methods</h3> During a period of two years, we enrolled 143 term newborns with ANH. These babies had an ultrasound at the age of 2–5 days and a second ultrasound at the age of 4–6 weeks. After the examinations they were referred to our nephrologist. <h3>Results</h3> Out of 8370 live-births, 143 infants had ANH. Six babies never completed the exam. At the first exam 69 babies were normal, 62 babies had mild, 3 babies had moderate and 3 babies had severe RPD. On their second ultrasound 76 had normal findings, 36 had mild, 8 had moderate and 12 had severe RPD. 132 babies completed both of the examinations. Bilateral hydronephrosis was detected in 33 cases during their first ultrasound while on the second only 27. There were 11 infants with UPJ obstruction 9 of them with severe RPD. We found 10 babies with VUR. Six babies needed surgical intervention. <h3>Conclusions</h3> There seems to be a correlation between the degree of RPD and the presence of postnatal pathology. Bilateral hydronephrosis probably carries increased risk for postnatal pathology.

Outcome of antenatal hydronephrosis in northeastern Thailand

Abstract Background: Antenatal hydronephrosis (ANH) is a condition characterized by fetal renal pelvic dilatation during pregnancy. It is detected in 1%-5% of all pregnancies. Most cases of ANH are physiological in nature, but some are pathological and can cause morbidity. Objective: To determine: (a) the causes of ANH; (b) the factors associated with complications; and, (c) the factors associated with surgical intervention. Methods: We reviewed the medical records of infants diagnosed with ANH; defined by a renal pelvic anteroposterior diameter ≥5 mm (based on antenatal ultrasonography) and being followed-up at Srinagarind Hospital. Results: Forty-six infants (32 boys and 14 girls) with ANH were identified. Over half (57%) were born in our hospital (in-hospital) with the condition. The two most common causes of ANH were ureteropelvic junction obstruction (32%) and transient hydronephrosis (22%). Of the 63 abnormal kidneys, 52% needed surgical intervention. Twenty-two patients (48%) had urinary tract infections and most had more than 1 episode. None of the 46 patients had end-stage renal disease, but one died because of lung hypoplasia during the neonatal period. The severity of ANH and time of first postnatal ultrasonography were related to medical complications, while bilateral ANH and more severe ANH were associated with the need for surgical intervention. A milder degree of ANH and postnatal ultrasound findings were significantly associated with transient hydronephrosis. Conclusion: Most cases of ANH were pathological and half required surgical intervention. Severe ANH and delayed investigation were associated with poor outcomes.

Retroperitoneal laparoscopic management of ureteropelvic junction obstruction in patients with horseshoe kidney.

To report our experience with retroperitoneal laparoscopic pyeloplasty for the management of ureteropelvic junction (UPJ) obstruction in patients with horseshoe kidneys (HSKs). Between March 2005 and May 2012, 2 boys, 5 men, and 1 woman (mean age, 23.9 years; range, 7-42 years) with HSKs underwent retroperitoneal laparoscopic dismembered pyeloplasties for UPJ obstructions with transections of the isthmuses. One patient had a renal calculus that was extracted during surgery. The follow-up included clinical evaluations, ultrasonography, intravenous urography, and diuretic renography. Retroperitoneal laparoscopic dismembered pyeloplasty was feasible in all cases. The median operative time was 192 minutes (range, 148-252 minutes), and there were no perioperative complications. The mean hospital stay was 5.8 days (range, 4-7 days). The mean follow-up time was 45 months (range, 18-96 months). All patients were asymptomatic and exhibited decreased pelvic dilation at follow-up. The retroperitoneal laparoscopic approach is feasible and safe for pyeloplasty in UPJ obstruction associated with HSK. Isthmectomy and a fully mobilized kidney offer better surgical exposure during the operation.

Ultrasound antenatal detection of urinary tract anomalies in the last decade: outcome and prognosis

Objective: This study has been undertaken to determine the frequency and pattern of urinary tract anomalies diagnosed by ultrasound, to correlate the prenatal with postnatal diagnoses and to identify prognostic factors.Methods: The Ultrasound Unit's database was reviewed for fetal urinary tract anomalies detected between January 2002 and June 2012. Prenatal diagnoses made by ultrasound were confirmed by postnatal ultrasound, as well as with surgical reports. Statistical analysis was performed using the Mann–Whitney U-test, Chi-square and Fisher’s exact tests. p values <0.05 were considered significant.Results: A total of 838 fetal malformations were prenatally diagnosed by ultrasound with a frequency of 21% of urinary tract anomalies (177/838). Renal pelvis dilatation and hydronephrosis accounted for more than half of the cases (52%). The most frequent postnatal diagnoses were also urinary tract dilatations. The prenatal diagnoses corresponded to the postnatal ones in 88.8% of cases. There was a highly significant association between anterior–posterior renal pelvis diameter above 10 mm in the last ultrasound performed before the birth and the need for surgery (p < 0.01).Conclusion: We emphasize the high degree of reliability of prenatal ultrasound in the establishment of diagnosis of urinary tract malformations and the prediction of postnatal outcomes.

Urinary Retention Accompanied by Retroperitoneal Urine Leakage in Schizophrenia

We presented a case of a 46-year-old man who attended the emergency department with right flank pain. Abdominal computed tomography showed dilated renal pelvis, ureter and distended bladder. Fluid collection was seen in retroperitoneal space and pelvic cavity. He had renal insufficiency. When a urethral catheter was inserted, 1,200 mL urine was drained. He was diagnosed with schizophrenia more than 20 years prior, and had been taking anti-psychotics. He had been living in a sanatorium. He had nocturnal enuresis and had worn an incontinence pad nightly for the last 10 years. His fluid consumption was 10 L/day. After placement of an indwelling urethral catheter for relieving voiding problems and restriction of fluid intake, fluid collection in retroperitoneal spaces and pelvic cavity was removed and renal insufficiency was recovered. After training of self-clean intermittent catheterization, he was discharged.

Our ultrasonographic experience in the management of symptomatic hydronephrosis during pregnancy.

The aim of the present study was to document the role of ultrasound in the diagnosis and management of symptomatic hydronephrosis during pregnancy. In this study, we reviewed 36 consecutive cases of pregnant women whose pregnancy was complicated by symptomatic hydronephrosis. In all patients, management was initially conservative (analgesics and fluids) after hospitalization. The following criteria were used to indicate double-J stent placement under US guidance rather than a conservative treatment: persistent pain with no improvement after conservative treatment, progressive hydronephrosis (>2cm dilatation of the renal pelvis) or presence of uterine contractions. The mean patient's age was 25years (17-35) and gestational age at clinical presentation was 24weeks (13-37). 81% of cases had a renal pelvis dilatation >2cm, while in only 19% of cases there was a hydronephrosis between 1 and 2cm in diameter; however, calculi were confirmed only in 25 patients. 28 patients required an invasive management with double-J stent insertion under US guidance. The sensitivity and specificity of US in the etiological diagnosis of hydronephrosis during pregnancy was 83 and 91%, respectively. Conservative management with medical therapy and observation should be the first-line treatment approach. In our opinion a rapid ureteral decompression with the insertion of a ureteral double-J stent, under US visualization, is the safest method in the treatment of pregnant women with obstructed renal systems.

PFM.40 Postnatal Outcome of the Antenatal Diagnosis of Hydronephrosis

ObjectivesTo determine the outcome of antenatally diagnosed hydronephrosis, including the analysis of the progression of the disease entity in utero, and ex- utero postnatal scans and surgical intervention.MethodsA retrospective audit...

Evaluation and predictive factors of renal function progression using cystatin C and creatinine in neonates born with CAKUT

Congenital anomalies of the kidney and urinary tract (CAKUT) is a main cause leading to endstage renal disease (ESRD) during childhood occurring at a frequency of 1 in every 500 pregnancies. No early predictive markers of long-term renal function (RF) are validated in these neonates. The aim of this study was to compare CysC and creatinine (creat) as markers of RF from birth to 2 years and to identify factors of RF progression. The 56 patients included in this study were followed for a median of 235 days (137 - 739). Repeated measures of CysC and creat during 2 years of RF evaluation were taken in 28 patients. Changes in RF with age were analyzed. Potential risk factors for RF progression were analyzed for: type of kidney disease (KD), bilateralism of KD, prenatal pelvic dilatation, reflux and initial relative RF (RRF) asymmetry obtained by scan. With age, a rapid decrease of CysC (16.3%, p < 0.001), and creat (68.6%, p < 0.001) was observed at 1 month. Between 1 month and 1 year, CysC decreased 4% per month (p < 0.001) and creatinine stabilized (+ 1.9%/m, p = 0.11). After 1 year, both CysC and creat stabilized. In the multivariate model, CysC significantly increased in patients with bilateralism (p = 0.004) or asymmetric RRF (p = 0.03). Creat was not significant. CysC was a better marker than creat to follow RF in neonates with CAKUT. Using CysC, bilateralism, and RRF asymmetry were significantly associated with RF progression.

Use of 3-Dimensional Printing Technology and Silicone Modeling in Surgical Simulation: Development and Face Validation in Pediatric Laparoscopic Pyeloplasty

Pediatric laparoscopy poses unique training challenges owing to smaller workspaces, finer sutures used, and potentially more delicate tissues that require increased surgical dexterity when compared with adult analogs. We describe the development and face validation of a pediatric pyeloplasty simulator using a low-cost laparoscopic dry-laboratory model developed with 3-dimensional (3D) printing and silicone modeling. The organs (the kidney, renal pelvis, and ureter) were created in a 3-step process where molds were created with 3D modeling software, printed with a Spectrum Z510 3D printer, and cast with Dragon Skin 30 silicone rubber. The model was secured in a laparoscopy box trainer. A pilot study was conducted at a Canadian Urological Association meeting. A total of 24 pediatric urology fellows and 3 experienced faculty members then assessed our skills module during a minimally invasive surgery training course. Participants had 60 minutes to perform a right-side pyeloplasty using laparoscopic tools and 5-0 VICRYL suture. Face validity was demonstrated on a 5-point Likert scale. The dry-laboratory model consists of a kidney, a replaceable dilated renal pelvis and ureter with an obstructed ureteropelvic junction, and an overlying peritoneum with an inscribed fundamentals of laparoscopic surgery pattern-cutting exercise. During initial validation at the Canadian Urological Association, participants rated (out of 5) 4.75 ± 0.29 for overall impression, 4.50 ± 0.41 for realism, and 4.38 ± 0.48 for handling. During the minimally invasive surgery course, 22 of 24 fellows and all the faculty members completed the scoring. Usability was rated 4 or 5 by 14 participants (overall, 3.6 ± 1.22 by novices and 3.7 ± 0.58 by experts), indicating that they would use the model in their own training and teaching. Esthetically, the model was rated 3.5 ± 0.74 (novices) and 3.3 ± 0.58 (experts). We developed a pediatric pyeloplasty simulator by applying a low-cost reusable model for laparoscopic training and skills acquisition. The model's usability, realism, and feel are good, it can be imaged under common modalities, and it shows promise as an educational tool.

Giant Hydronephrosis Due to Congenital Uretropelvic Junction Obstruction, a Report of 2 cases

Ureteropelvic junction (UPJ) stenosis occurs in 13% of children. Antenatally, the diagnosis is possible by showing renal pelvis dilatation by obstetric ultrasonography. Giant hydronephrosis (GH) is a rare condition. There are different etiologies for GH which are mostly congenital and occur during infancy and childhood. GH was confirmed by complementary evaluations in a 2-month-old female infant with poor feeding, lethargy, and vomiting together with an antenatal sonography in favor of hydronephrosis, and a 5-year-old boy with an abdominal mass diagnosed by sonography following 2 weeks of progressive abdominal distention. Pyeloplasty was associated with an excellent result in both of them. Keywords : Hydronephrosis; Ureteral Obstruction; Congenital; Urologic Diseases.

Embryofetal Development Study of Vismodegib, a Hedgehog Pathway Inhibitor, in Rats

Vismodegib (Erivedge) is a first-in-class small-molecule hedgehog pathway inhibitor for the treatment of adults with advanced basal-cell carcinoma. Because this pathway is known to play key roles in patterning and growth during vertebrate development, vismodegib was anticipated to be embryotoxic. To support marketing applications, an embryofetal development study was completed in which a limited number of pregnant rats (n = 6/group) was administered vismodegib by oral gavage on gestation days 6 to 17. When vismodegib was administered at ≥60 mg/kg/day, doses associated with evidence of pharmacologic activity in previous rat toxicity studies, all conceptuses were resorbed at an early embryonic stage in the absence of significant maternal toxicity. When administered at 10 mg/kg/day, corresponding to an exposure (AUC0-24h ) approximately 15% of the median in patients at steady state, a variety of malformations were observed, including absent/fused digits in the hindlimb of multiple fetuses, multiple craniofacial abnormalities in one fetus, and an anorectal defect in one fetus. In addition, the incidence of variations, including dilated renal pelvis or ureter and incompletely or unossified skeletal elements, was significantly greater when compared with the controls. These results confirmed that vismodegib is likely to be embryotoxic at clinically relevant maternal exposures, and doses ≥60 mg/kg/day resulted in a 100% incidence of embryolethality that likely resulted from severe defects in early embryonic development. In contrast, craniofacial defects typically associated with hedgehog pathway inhibition were only observed in one fetus at the low dose of 10 mg/kg/day, which likely reflected minimal or intermittent pathway inhibition at low exposures.

Laparoscopic management of obstructed partial duplex with Y ureteric configuration

An 11-year-old girl underwent a laparoscopic upper-to-lower moiety ureteropyelostomy for lower moiety pelviureteric junction obstruction associated with Y ureteric duplication. She presented with left flank pain and was noted to be hypertensive with microscopic haematuria on admission. An abdominal ultrasound scan revealed a duplex left kidney with gross lower moiety pelvicalyceal dilatation. A subsequent MAG-3 scan demonstrated reduced differential function in the left lower moiety. A laparoscopic transmesocolic approach confirmed a dilated lower moiety pelvis and revealed the Y ureteric duplication. An upper to lower moiety ureteropyelostomy and insertion of a JJ stent is described in the accompanying video. The child was discharged home on the second day post operation. Postoperative imaging showed good decompression and improved differential function. The child has been completely well and normotensive at 2 years' follow-up.

Laparoscopic Redo Pyeloplasty: Point of Technique and Our Experience

Abstract Introduction: Open dismembered pyeloplasty is a gold standard for managing ureteropelvic junction (UPJ) obstruction resulting in a success rate >90%.1 The conventional open surgery for failed pyeloplasty is very challenging because of the fibrosis and scarring at the previous surgical site. The current options for managing the failed pyeloplasty are endopyelotomy or redo pyeloplasty, which can be an open, laparoscopic or robot-assisted approach.2 The ureterocalicostomy is considered to salvage the kidney when there are no other options. The redo pyeloplasty is equally difficult with both the open and laparoscopic approach. We present a video demonstration of laparoscopic redo pyeloplasty. The port positions are depicted in the illustration. After achieving the pneumoperitoneum, the colon was reflected along the line of toldt. The affected renal unit and dilated renal pelvis were exposed after incising Gerota's fascia. The normal ureter was identified and dissection was continued proximally toward...