Search

T1 Magnetization Prepared Two Rapid Acquisition Gradient Echo (MP2RAGE) with compress sensing (CS) has been proposed as an improvement of the standard MPRAGE sequence with multiple advantages including reduced acquisition time needed to provide a quantitative 3D anatomical image coupled with T1-map. Here we investigated the agreement between FreeSurfer-derived volume measurements obtained from MPRAGE and CS MP2RAGE acquisitions. MPRAGE and CS MP2RAGE images of 37 subjects (14 patients with neurodegenerative disorders and 23 healthy controls) were acquired on a 3 T MR scanner and grey matter volumes were extracted using standard FreeSurfer parcellation. Lin's concordance correlation coefficient (Lin's CCC), Bland-Altman analysis, Passing-Bablok regression and DICE similarity coefficient were calculated to assess the agreement between the two. We found a good correspondence for most of the regions examined, with 93.5 % of them showing a mean DICE index >0.70. Poorer results were found with Lin's CCC especially for subcortical labels across patients. The Bland-Altman analysis showed CS MP2RAGE tended to measure lower cortical volumes compared to MPRAGE but in most cases the difference wasn't statistically relevant. The Passing-Bablock regression indicated overall an absence of systematic constant and proportional bias when CS MP2RAGE was used instead of MPRAGE. We found a good concordance for volumes obtained from MPRAGE and CS MP2RAGE images using FreeSurfer, suggesting a possible role of CS MP2RAGE for structural analysis with significant advantages like shorter acquisition time and the possibility to simultaneously obtain quantitative T1-maps of the brain enriching the diagnostic power of this technique. [ABSTRACT FROM AUTHOR]

Cloacal exstrophy is a rare birth defect that affects both the genitourinary and gastrointestinal tract, with an estimated incidence between 1:200,000 and 1:400,000 live births. The exact mechanism that leads to this defect is still unknown. Our purpose is to report a case of omphalocele-exstrophy-imperforate anus-spinal defect (OEIS) complex with a rare variant to add to the literature. A 28-year-old female with no known medical history had a monochorionic diamniotic twin pregnancy. At 20 weeks of gestation a prenatal ultrasound demonstrated multiple fetal anomalies on twin B, including omphalocele, absent right kidney, non-visualization of bladder, and single umbilical artery. She had a scheduled Cesarean section at 34 weeks and 5 days of gestation. Twin B had a large omphalocele with an intact sac. Below the omphalocele, there was a cecal plate with a prolapsed ileum and a second small orifice corresponding to the hindgut. Two hemivaginas and hemiclitorises were appreciated inferior to the cecal plate but superior to a bladder plate bridge joining both halves. Each of the hemivaginas was connected to a hemiuterus with its own fallopian tube and ovary. The bladder was on both sides of the cecal plate and connected in the midline adopting a crescent shape with the concave side facing cephalad. Genetic testing confirmed an XX karyotype. On day-of-life 5 the patient underwent omphalocele repair and closure of the abdominal wall, tubularization of the cecal plate, and an end colostomy. Additionally, the hemivaginas and hemiclitorises were relocated to an orthotopic position and the bladder plates were joined together and used to cover part of the abdominal wall defect. She was discharged on day of life 42 with a weight of 2.72 kg. She continues to follow up outpatient. Her second stage procedure is still pending. The position of the genital tract superior to the bladder exstrophy observed in this case has not been described before and is not fully explained by the current theories on the development of OEIS. [ABSTRACT FROM AUTHOR]

The Rotterdam Prostate Cancer Risk calculator (RPCRC) has been validated in the past years. Recently a new version including multiparametric magnetic resonance imaging (mpMRI) data has been released. The aim of our study was to analyze the performance of the mpMRI RPCRC app. A series of men undergoing prostate biopsies were enrolled in eleven Italian centers. Indications for prostate biopsy included: abnormal Prostate specific antigen levels (PSA>4 ng/ml), abnormal DRE and abnormal mpMRI. Patients' characteristics were recorded. Prostate cancer (PCa) risk and high-grade PCa risk were assessed using the RPCRC app. The performance of the mpMRI RPCRC in the prediction of cancer and high-grade PCa was evaluated using receiver operator characteristics, calibration plots and decision curve analysis. Overall, 580 patients were enrolled: 404/580 (70%) presented PCa and out of them 224/404 (55%) presented high-grade PCa. In the prediction of cancer, the RC presented good discrimination (AUC = 0.74), poor calibration (p = 0.01) and a clinical net benefit in the range of probabilities between 50 and 90% for the prediction of PCa (Fig. 1). In the prediction of high-grade PCa, the RC presented good discrimination (AUC = 0.79), good calibration (p = 0.48) and a clinical net benefit in the range of probabilities between 20 and 80% (Fig. 1). The Rotterdam prostate cancer risk App accurately predicts the risk of PCa and particularly high-grade cancer. The clinical net benefit is wide for high-grade cancer and therefore its implementation in clinical practice should be encouraged. Further studies should assess its definitive role in clinical practice. [ABSTRACT FROM AUTHOR]

The role of voiding cystourethrogram (VCUG) in evaluating vesicoureteral reflux (VUR) in patients with known ureteropelvic junction obstruction (UPJO) remains unclear. While VCUG is frequently performed, the incidence of concomitant VUR and UPJO is low, and VUR is often low-grade with high rates of spontaneous regression. To analyze the clinical relevance of VCUG in patients with UPJO by determining its incidence and studying the difference in clinical outcomes between patients with known, unknown, and negative VUR. Retrospective review of patients with UPJO who underwent pyeloplasty from 2012 to 2020 with <18 years-old, unilateral UPJO, postoperative follow-up of ≥2 months and had at least 1 renal ultrasound (US) after pyeloplasty. Results were compared among 3 groups: patients who underwent VCUG before pyeloplasty and were found to have VUR (group 1), patients who underwent VCUG before pyeloplasty without VUR (group 2), and patients who did not have a VCUG before pyeloplasty (group 3). A total of 275 patients met the inclusion criteria, of which 21 patients were classified in group 1, 166 patients in group 2, and 88 patients in group 3 (Table). The age at preoperative VCUG was 14.7 ± 32. 9 months in group 1 and 15.17 ± 35. 8 months in group 2 (p = 0.960). Overall, the incidence of concomitant UPJO and VUR was 11.2%. In group 1 the initial VUR grade was 5 in 2 patients, 4 in 3, 3 in 5, 2 in 7, and 1 in 4 patients. Of these, only 1 patient required ureteral reimplantation after pyeloplasty. Post-pyeloplasty, no significant differences were observed in complications (p = 0.7436), length of follow up (p = 0.3212), SFU grade 4 hydronephrosis (p = 0.2247), postoperative UTIs (p = 0.1047) and pyeloplasty success rate (p = 0.4206) among the 3 groups. Despite the use of antibiotic prophylaxis being significantly different amongst the three groups (p < 0.001), it was not associated with a lower incidence of postoperative UTIs (group 1 p = 0.068, group 2 p = 0.486, group 3 p = 1). In patients with reflux, an increase in age was associated with a decrease in the rates of complications (p = 0.019). We found no significant difference in the outcomes in patients who had a preoperative VCUG as compared to those who did not. The preoperative diagnosis of VUR by VCUG changed the management in less than 1% of the study population and thus its role in patients with UPJO should be reevaluated. Summary Table Comparison of clinical and postoperative outcomes between groups. Variable Group 1 VUR Group 2 VCUG, no VUR Group 3 No VCUG p -value Clinical characteristics Total (n) 21 (100%) 166 (100%) 88 (100%) – Male 16 (76.2%) 110 (66.3%) 64 (72.7%) 0.4358 Age at surgery (months) b 9.0 (5.0–34.0) 13.0 (4.0–50.0) 108.0 (75.0–173.0) <0.0001 Antibiotic prophylaxis 12 (57.1%) 32 (19.2%) 4 (4.5%) <0.0001 SFU Grade 4 hydronephrosis 17 (81.0%) 149 (89.8%) 66 (75.0%) 0.0078 Postoperative characteristics Complications excluding UTI 2 (9.5%) 16 (9.6%) 6 (6.8%) 0.7436 Post-operative UTI 2 (9.5%) 11 (6.6%) 1 (1.1%) 0.1047 Length of FU (months) a 17.0 ± 18.9 20.0 ± 20.9 17.0 ± 21.0 0.3212 Success 20 (95.2%) 148 (91.4%) 66 (84.6%) 0.4206 Categorial variables were represented as absolute numbers and percentages. Continuous variables were represented with median and interquartile range (IQR) or means and standard deviations (SD). a =Values as median and inter-quadrantile ranges. b =Values as mean and standard deviation. Comparisons among all groups were made using Wilcoxon Rank Sum for continuous variables. [ABSTRACT FROM AUTHOR]

• HER2 overexpression historically linked to poor prognosis in breast cancer. • However, the availability of active anti-HER2 compounds/combinations is evolving. • In HER2CLIMB, the tucatinib-containing combination improved overall survival. • The trial included patients with active brain metastases, an unmet need. • The review highlights tucatinib's journey from preclinical to clinical use. Approximately 20% of breast cancers (BCs) overexpress human epidermal growth factor receptor 2 (HER2), a transmembrane glycoprotein with tyrosine kinase activity, encoded by ERBB2 gene. Historically, HER2 overexpression has been linked with increased disease recurrence and a worse prognosis. However, the increasing availability of different anti-HER2 compounds and combinations is progressively improving HER2-positive BC outcome, thus requiring expertise to prioritize both overall survival (OS) prolongation and quality of life, without neglecting the accessibility to further treatment lines with a low attrition rate. In this context, tucatinib, an oral tyrosine kinase inhibitor, has recently been granted approval by regulatory agencies based on evidence from the HER2CLIMB, a clinical trial which randomized patients with metastatic BC to receive trastuzumab and capecitabine with either tucatinib or placebo. A distinctive feature of this study was the inclusion of patients with new or active brain metastases (BMs) at study entry, a population traditionally excluded from clinical trials. Thus, HER2CLIMB provides the first solid evidence of an OS benefit in patients with BC and BMs, addressing a long standing unmet medical need, especially given the high incidence of central nervous system metastatic spread in patients with HER2-positive disease. This review provides an overview of the molecular and clinical landscape of tucatinib for the treatment of advanced BC. It focuses on the technological journey that drove the development of this therapeutic innovation, from preclinical data to clinical practice. [ABSTRACT FROM AUTHOR]

Proximal hypospadias and severe ventral chordee are often challenging to repair. To preserve penile length in chordee repair, Small Intestinal Submucosa (SIS) corporal grafting is often performed with potential long-term complications including recurrent curvature and erectile dysfunction (ED). There is a paucity of data evaluating sexual function in mid, late and post-pubertal patients. We aimed to assess long-term outcomes of genital self-perception and erectile function in mid, late and post-pubertal patients who underwent single-layer (1-ply) SIS corporal body grafting for correction of severe chordee. Patients with proximal hypospadias who underwent correction of severe chordee using SIS grafting between 2001 and 2015 were retrospectively identified. Patients were evaluated for erectile and sexual function using the modified erection hardness score (mEHS) and the modified sexual health inventory for men (mSHIM). Perceived function and straightness were measured with Hypospadias Objective Scoring Evaluation (HOSE). Penile self-perception was assessed using the Pediatric Penile Perception Score (PPPS). Results were compared to an age-matched healthy control group. Categorical variables were analyzed using Fisher's exact test, and continuous variables using paired and unpaired t-test and ANOVA. Nineteen patients with proximal hypospadias who underwent correction of severe chordee using SIS grafting and 18 controls participated in the study with a median age of 17 years for both groups. In the mEHS, 12 (63.2%) hypospadias-patients and 14 (87.5%) controls rated their erections as completely hard and very rigid. In the mSHIM, 1 (5.2%) hypospadias-patient was classified as having moderate ED. A total of 16 hypospadias-patients (84%) and 16 controls (88.9%) reported being very satisfied or satisfied with the straightness of their penis. No significant difference was observed in the mEHS, mSHIM and PPPS between groups (p < 0.05). The straightness of the erection was rated lower by participants, than by the pediatric urologist. In the HOSE, 12 (63.2%) hypospadias-patients and 16 (88.9%) controls obtained an acceptable score. Our findings indicate favorable long-term outcomes in ED and genital self-perception; only 5% of our population reported having a mild-moderate to moderate presentation of ED, and there were no reports of severe ED. The overall PPPS satisfaction rates were statistically similar for the control and hypospadias groups. The small sample population limits the significance of our findings. Corporal body grafting with 1-ply SIS suggests positive long-term outcomes in genital self-perception and erectile function, with mid, late and post-pubertal patients who underwent hypospadias repair having comparable results to age-matched healthy controls. Summary Table Comparison of outcomes from penile self-perception between the clinical and control group. Summary Table Very satisfied Satisfied Dissatisfied Very dissatisfied p-value Cases Controls Cases Controls Cases Controls Cases Controls Length of penis 4 (21%) 2 (11%) 11 (58%) 16 (89%) 2 (10%) 0 2 (10%) 0 0.16 Position and shape of urethral opening 9 (47%) 7 (39%) 9 (47%) 11 (61%) 0 0 1 (5%) 0 0.55 Shape of glans 9 (47%) 8 (44%) 9 (47%) 10 (55%) 0 0 1 (5%) 0 0.62 Shape of penile skin 7 (37%) 4 (22%) 11 (58%) 13 (72%) 0 1 (6%) 1 (5%) 0 0.43 Penile axis (straightness) 5 (26%) 6 (33%) 11 (58%) 10 (55%) 2 (10%) 2 (11%) 1 (5%) 0 0.76 General appearance 6 (31%) 6 (33%) 10 (53%) 11 (61%) 1 (5%) 1 (6%) 2 (10%) 0 0.59 [ABSTRACT FROM AUTHOR]

In patients with urinary continent channel (UCC) and Malone Antegrade Continent Enema (MACE) procedures, two separate abdominal stomas are needed. The umbilicus is a preferred site for single channel stomas given the ability to conceal the stoma. However, there are no studies describing outcomes of both stomas being created in the umbilicus. We aimed to describe our experience in patients who underwent UCC and MACE stomas both placed in the umbilicus. A retrospective review from 2009 to 2020 was performed in our institution for patients who underwent the creation of UCC and MACE stomas simultaneously in the umbilicus. The variation in the technique involves two V-skin shaped flaps in the umbilicus; the MACE and UCC stomas are delivered from both flaps and placed at the right and left side respectively. Patients with greater than 3 months of follow-up were included in the study. There were 17 patients identified with the median age of 13.5 years and a median follow-up of 32.8 months. The mean BMI percentile was 89.5%. Monti technique and split appendix with cecal extension were utilized in 8 (47.1%) and 7 (41.2%) patients respectively and 13 (76.5%) patients required concurrent urological procedures. All channel-related complications occurred within a mean time of 15.7 months. Skin-level stenosis in the MACE occurred in 5 (29.4%) events, and all were successfully managed by placing an indwelling catheter for up to two weeks. There were 2 (11.8%) complications related to UCC, which required subfascial minor surgical revision. The rate of patients with symptomatic UTI decreased 35.3% postoperatively, and no new onset of UTI occurred in patients without a prior history of UTI. During follow-up, all patients remained dry between CIC, however one had occasional leakage related to delay in catheterization. Total fecal continence was achieved in 14 (82.3%) patients. Additionally, 3 (16.6%) patients experienced improvement in fecal continence with sporadic soiling episodes. Placement of UCC and MACE stomas in the umbilicus demonstrate a percentage of complication of 7/34 (20.6%) with only 2 patients requiring surgical intervention, comparable to the standard. UTI rate decreased in patients with a prior history of UTI. We believe the patients' perspective and degree of satisfaction will fully determine the benefits of this technique. Simultaneous UCC and MACE stomas placed at the umbilicus showed good functional outcomes and similar complication rates to traditional approach where stomas were placed separately in the abdominal wall. [Display omitted] [ABSTRACT FROM AUTHOR]

Although more than 1500 pediatric pyeloplasties are performed in the United States per year, there is a lack of consensus on the adequate imaging modality and timing of follow-up after surgery. The aim of this study is to evaluate if ultrasonographic stabilization in the grade of hydronephrosis after pyeloplasty is an adequate parameter to define the optimal length of follow-up after surgery and to detect failed procedures. Secondarily, we want to analyze clinical variables that could impact ultrasonographic stabilization. This is a retrospective chart review of patients who underwent pyeloplasty between 2012 and 2020. Patients were included if they were ≤21 years-old at the time of surgery, had pre and postoperative renal-ultrasounds, had ≥3 months of postoperative follow-up, and had met the criteria for ultrasonographic stabilization after surgery. Ultrasonographic stabilization was defined as 2 consecutive ultrasounds after pyeloplasty, a minimum of 1-month apart, with the same degree of hydronephrosis according to the Society for Fetal Urology (SFU) grading system. Data concerning clinical, surgical, and postoperative variables were collected. A total of 143 pyeloplasties met the inclusion criteria. Median age at surgery was 10.5 months with a median post-operative follow-up time of 29 months. Ultrasonographic stabilization was achieved in a mean time of 14.5 months post-operatively. Compared to the preoperative US SFU grade, at ultrasonographic stabilization 127 (88.9%) patients had an improvement of ≥1 grades of hydronephrosis. There were 9 pyeloplasty failures that were diagnosed and surgically corrected in a median of 7.5 months after the initial surgery. No clinical or surgical variables were associated with time to achieve ultrasonographic stabilization. There is great variability in the length of follow-up after pyeloplasty, ranging from 3-months to 7-years. In our cohort, the bast majority of patients had achieved ultrasonographic stabilization and displayed improvement in their initial grade of hydronephrosis by 14.5 months after surgery. Timeframe in which surgical failures were diagnosed and corrected. There were not clinical or surgical variables associated to time to achieve stabilization. Similar results have been reported in the literature; however, most of them have evaluated as their main outcome resolution and not stabilization of hydronephrosis. Ultrasonographic stabilization is a suitable and non-invasive parameter for determining the length of follow-up after unilateral pyeloplasty, as it is an adequate timeframe for identifying failed pyeloplasties, observing improvement or stabilization of hydronephrosis, and performing any additional procedure required after the primary repair. Summary table Clinical characteristics and its association with ultrasonographic stabilization. Summary table Variables US Stabilization time (mean) p -value Preoperative grade of hydronephrosis SFU 3 14.33 0.98 SFU 4 14.28 Diagnosis of hydronephrosis Prenatal 13.3 0.038 Postnatal 17.8 Vesicoureteral reflux Yes 10.8 0.22 No 14.7 Age at surgery < 6 months 13.2 0.57 ≥6 months to <1 year 16.7 ≥1 to <2 years 16.7 ≥2 to <6 years 12.1 ≥ 6 years 14.5 Surgical approach Open 14 0.91 Robotic-assisted 14.2 No 14.2 0.83 Yes 13.5 Redo pyeloplasty No 14.2 0.74 Yes 15.7 Complications (<90 days after surgery) No 14.2 0.09 Yes 8 ∗Only 141 renal units had preoperative MAG3% renal scan. [ABSTRACT FROM AUTHOR]

The objective of this laboratory study was to assess the effect of immediate dentin sealing (IDS) on the fracture strength of teeth restored with ultrathin occlusal veneers made of a polymer-infiltrated ceramic network (PICN – Vita Enamic) with different fissure/cusp thicknesses. Forty third molars were identically prepared in the dentin and allocated into four groups (n = 10) according to the fissure/cusp thickness of the occlusal veneers (0.3/0.6 mm or 0.6/0.9 mm) and the application or not of the IDS technique. Ten sound third molars were used as the control group. IDS was performed using a universal adhesive system (Single Bond Universal). The prepared teeth were scanned (Cerec software, version 4.1), and the occlusal veneers were shaped following the occlusal anatomy of a right second molar. Blocks of PICN were milled in the unit inLab MC XL. Following luting with Single Bond Universal and RelyX Ultimate, the fracture strength of the specimens was obtained with a universal testing machine. The fractures were classified as reparable or irreparable. Fracture strength values were analyzed with 2-way and 1-way ANOVA (α = 0.05) and Tukey's test. Only the IDS factor was significant (p = 0.001). The teeth restored with IDS (2875 ± 508 N) achieved significantly higher fracture strength than the teeth restored without IDS (2263 ± 354 N) (p < 0.05). The fracture strength of the specimens with IDS did not differ significantly from that of the sound teeth (3230 ± 620 N). The fractures were predominantly reparable. IDS improved the fracture strength of ultrathin occlusal veneers made of PICN. [ABSTRACT FROM AUTHOR]

We present a video case report of a pediatric patient with previous robotic abdominal surgery who underwent robotic assisted Monti-Yang continent ileovesicostomy. This 10-year-old female had a history of spina bifida, with previous myelomeningocele repair and ventriculoperitoneal shunt as an infant and robotic-assisted Malone procedure and artificial urethral sphincter placed 4 years ago. After undergoing bilateral hip surgery, she presented with difficult urethral catheterization due to reduced leg mobility. A Monti-Yang ileovesicostomy procedure was planned. A 5 mm laparoscopic port placed through the umbilicus followed by four additional DaVinci XI working ports. Twenty centimeters proximal the ileocecal valve, a 3 cm segment of ileum was dissected, then transected, detubularized, and retubularized around a 10 Fr catheter. This conduit was reimplanted to the anterior bladder wall. The other edge was fixed to the umbilicus. A 10 Fr Foley catheter was left clamped in the conduit, and a urethral Foley was left to gravity. Total operative time was 3 h 56 min. The patient did well post-operatively. She was discharged home on POD 6. Starting three weeks after surgery, the patient began catheterizing through the umbilical stoma. At 3 month follow-up, the patient catheterizes through her stoma every 3 h without leakage between catheterizations. [ABSTRACT FROM AUTHOR]

Abstract: Objectives: Collagen disorganization is one of the main degradation patterns found in unsuccessful adhesive restorations. The hypothesis of this study was that pretreatment using natural collagen cross-linking agents rich in proanthocyanidin (PA) would improve mechanical properties and stability over time of the dentin collagen and, thus, confer a more resistant and lasting substrate for adhesive restorations. Methods: PA-based extracts, from grape seed (GSE), cocoa seed (CSE), cranberry (CRE), cinnamon (CNE) and açaí berry (ACE) were applied over the demineralized dentin. The apparent elastic modulus (E) of the treated dentin collagen was analyzed over a 12 month period. Specimens were immersed in the respective solution and E values were obtained by a micro-flexural test at baseline, 10, 30, 60, 120 and 240 min. Samples were stored in artificial saliva and re-tested after 3, 6 and 12 months. Data was analyzed using ANOVA and Tukey test. Results: GSE and CSE extracts showed a time-dependent effect and were able to improve [240 min (MPa): GSE=108.96±56.08;CSE=59.21±24.87] and stabilize the E of the organic matrix [12 months (MPa): GSE=40.91±19.69;CSE=42.11±13.46]. CRE and CNE extracts were able to maintain the E of collagen matrices constant over 12 months [CRE=11.17±7.22;CNE=9,96±6.11;MPa]. ACE (2.64±1.22 MPa) and control groups immersed in neat distilled water (1.37±0.69 MPa) and ethanol–water (0.95±0.33 MPa) showed no effect over dentin organic matrix and enable their degradation and reduction of mechanical properties. Significance: Some PA-based extracts were capable of improving and stabilizing collagen matrices through exogenous cross-links induction. [Copyright &y& Elsevier]

Abstract: Purpose: The literature on small intestinal submucosa for chordee correction in children is scarce. We reviewed our experience with 1 ply SIS for ventral corporal body grafting in cases of severe ventral penile curvature associated with proximal hypospadias in children. Materials and methods: From 04-2001 to 12-2007, 58 boys with proximal hypospadias and severe ventral curvature underwent single layered SIS graft to the corporal bodies to correct chordee. In 43 patients the surgery was done in the first stage of a planned 2-stage procedure. Fifteen patients underwent a 1-stage chordee correction with SIS and tubularized transverse preputial flap urethra. Results: Mean follow-up was 4.8 years. A straight phallus with good cosmesis was achieved in 57/58 patients. In 51/58 patients an artificial erection was performed in the operating room as part of a second stage procedure or for complications associated with the one stage urethroplasty. One patient needed a second procedure to correct the curvature (chordee was over-corrected and needed a ventral Nesbitt plication). In fifteen patients that underwent a 1-stage genital reconstruction, the neourethral meatus was left in the lower part of the glans in 8 patients and at the coronal sulcus in 7. Conclusions: Corporal body grafting with single layer SIS is a viable option for correction of severe chordee associated with corporal body disproportion. SIS is a material with reliable results, easy availability and no donor site associated morbidity. As a result of penile elongation with a graft, simultaneous island flap urethroplasty became difficult in many patients. [Copyright &y& Elsevier]

Abstract: Biomodification of existing hard tissue structures, specifically tooth dentin, is an innovative approach proposed to improve the biomechanical and biochemical properties of tissue for potential preventive or reparative therapies. The objectives of the study were to systematically characterize dentin matrices biomodified by proanthocyanidin-rich grape seed extract (GSE) and glutaraldehyde (GD). Changes to the biochemistry and biomechanical properties were assessed by several assays to investigate the degree of interaction, biodegradation rates, proteoglycan interaction, and effect of collagen fibril orientation and environmental conditions on the tensile properties. The highest degree of agent–dentin interaction was observed with GSE, which exhibited the highest denaturation temperature, regardless of the agent concentration. Biodegradation rates decreased remarkably following biomodification of dentin matrices after 24h collagenase digestion. A significant decrease in the proteoglycan content of GSE-treated samples was observed using a micro-assay for glycosaminoglycans and histological electron microscopy, while no changes were observed for GD and the control. The tensile strength properties of GD-biomodified dentin matrices were affected by dentin tubule orientation, most likely due to the orientation of the collagen fibrils. Higher and/or increased stability of the tensile properties of GD- and GSE-treated samples were observed following exposure to collagenase and 8months water storage. Biomodification of dentin matrices using chemical agents not only affects the collagen biochemistry, but also involves interaction with proteoglycans. Tissue biomodifiers interact differently with dentin matrices and may provide the tissue with enhanced preventive and restorative/reparative abilities. [Copyright &y& Elsevier]

The present report describes an unusual case of an aneurysm of a right hepatic artery (RHA) branching from the superior mesenteric artery; the accessory RHA was looped to the left hepatic artery arising from the celiac axis (CA) and was associated with congenital atresia of the CA. The accessory RHA aneurysm was treated with the placement of a bare stent and detachable coils through the mesh of the stent. Complete and prompt exclusion of the aneurysm was achieved with blood flow preservation in the parent artery at midterm follow-up. [Copyright &y& Elsevier]

Abstract: Introduction: We report the comparison between ureteral replacements using a transverse tubularized colonic tube or ileal ureter in dogs. Materials and Methods: Ten canines were studied. Four underwent ureteral substitution with an ileal ureter; 5 with a single transverse tubularized colonic tube and 1 with a double colonic transverse tubularized tube. The animals were observed for a predetermined interval of 30 days. At 30 days, the animals underwent an intravenous pyelogram, cystogram, urine analysis, and serum electrolyte sampling, and the kidney, ureteral conduit, and bladder were examined grossly and histologically. Results: The pyelograms of both the ileal ureter and the transverse tubularized colonic tube showed prompt bilateral renal excretion. Grade 1 hydronephrosis was seen in 3 of 4 animals that received the ileal ureter and with 1 of 5 animals that received the colonic tube. Histological exam of the kidneys showed small focal areas of pyelonephritis in both groups without significant injury to the collecting tubules or glomeruli. Discussion: The transverse tubularized colonic tube is an effective form of ureteral replacement in the animal model. The advantages of using the colon include its proximity to the ureters, the minimal mobilization needed, position outside the radiation portals, and the short colonic segment necessary. [Copyright &y& Elsevier]

Summary Objective To describe robotic-assisted laparoscopic artificial urinary sphincter (RAL-AUS) placement and a Malone antegrade continent enema (MACE) procedure on a 6-year-old girl. Patients and results Our patient is a 6-year-old girl with myelomeningocele. She was totally incontinent of urine and chronic constipated despite aggressive bowel regimen. Preoperative workup included renal and bladder ultrasound showing normal findings. Video-urodynamics was also obtained showing low leak point pressure (25 cm H 2 O) with no reflux. RAL-AUS was performed totally intra-corporally with no complications. The total operative time was 5 h 42 min. Estimated blood loss was minimal. The patient was discharged on postoperative day 4 with the AUS deactivated. Follow-up cystoscopy and activation of the AUS was done 6 weeks later. At 3-month follow-up, the patient was fully recovered and remained completely dry between voids. Also her bowel movements improved significantly with the MACE. Both patient and family appeared comfortable in using the AUS. Conclusion To our knowledge, this is the first case described of RAL-AUS placement in the pediatric population. We believe this can be safely accomplished with good outcomes. The robotic approach provides an advantage in performing deep pelvic surgeries and facilitates concomitant intra-abdominal procedures. [ABSTRACT FROM AUTHOR]

Male epispadias is a rare congenital urogenital anomaly in which the meatus is ectopically located along the dorsal midline of the penile shaft. In cases associated with severe curvature, functional and cosmetic outcomes could be accomplished by lengthening the shorter dorsal surface with the use of corporal grafting. Various graft materials have been used in the past for hypospadias repair including tunica vaginalis, dermis, and small intestinal submucosa (SIS). The use of SIS grafting for corporoplasty during epispadias repair has rarely been described in the literature. To report the experience in the management of dorsal corporal body grafting using SIS in children with severe penile curvature due to epispadias. The authors retrospectively reviewed the charts of all patients with epispadias or bladder exstrophy/epispadias complex and severe dorsal chordee (>40°) who underwent epispadias repair with single-layer SIS for corporal body grafting. Clinical variables, surgical technique, and outcomes were analyzed. A total of nine consecutive patients underwent staged epispadias repair with dorsal corporal single-layer SIS grafting (summary figure). Of these, four (44.4%) had primary penopubic epispadias, one (11.1%) had mid-shaft epispadias, and four (44.4%) had bladder exstrophy/epispadias complex. The mean age at surgery was 13.4 ± 6 months. After phalloplasty with SIS grafting, there were no reported complications related to the graft during the post-operative period or follow-up visits. Although traditional techniques for epispadias repair allow some degree of corporal lengthening, they also result in abrupt medial rotation of the corporal bodies leading to torqueing and potential unsatisfactory cosmetic results. In contrast, the authors use single-layer SIS for corporal body grafting, and this study technique results in a more gradual inward rotation thus allowing more anatomical accuracy. Furthermore, an advantage of the use of SIS over other grafting materials is that there is no need to harvest an autologous graft such as tunica vaginalis or dermis. Epispadias repair using single-layer SIS corporal body grafting is an effective, safe, and feasible method, which provides satisfactory cosmesis and correction of dorsal curvature in congenital epispadias in children. Furthermore, a more normal penis appearance, without a decrease in the corporal length or diameter, is achieved with this technique. [ABSTRACT FROM AUTHOR]

Summary Background Vesicoamniotic shunting (VAS) and other bladder drainage techniques for fetal lower urinary tract obstruction (LUTO) have been proven to ameliorate pulmonary hypoplasia and increase survival in patients with an initial poor prognosis. Currently there are limited prognostic tools available during gestation to evaluate and predict postnatal renal function. Objective The aim was to describe the prenatal growth of the renal parenchymal area (RPA) in patients with LUTO and determine its application as a predictor of renal function at one year of life. Study design The study population comprised a retrospective cohort of all infants who survived the fetal VAS to birth. Renal growth and size were measured using imageJ software to calculate the RPA in sequential prenatal ultrasounds. The parenchymal area was measured from the image of each kidney with the greatest longitudinal length. These measurements were further correlated and analyzed as a predictor of end-stage renal disease (ESRD) within the first year of life. Results Etiologies of LUTO in the 15 male fetuses included eight posterior urethral valves, four Eagle–Barrett/prune belly syndrome, two urethral atresia, and one megacystis microcolon intestinal hypoperistalsis syndrome. All patients had patent shunts, in place, at birth. Furthermore, ultrasonographic parameters such as oligohydramnios, keyhole sign, and bladder wall thickness showed no statistical difference between groups. Renal parenchymal growth correlated with postnatal renal function in both the ESRD (r = 0.409, p = 0.018) and the non-ESRD (r = 0.657, p < 0.001) groups. Most notably, RPA during the 3rd trimester predicted ESRD with the best cut-off point determined to be 8 cm2 (sensitivity, 0.714; specificity, 0.882; and positive likelihood ratio, 6.071) (Table). Discussion Despite definitive VAS for LUTO, postnatal morbidity and mortality remain high, emphasizing the role of renal dysplasia in postnatal renal failure, in spite of urinary diversion. Renal growth statistically differs between groups in the 3rd trimester of gestation; RPA development appears stagnant in patients that developed ESRD within the first year of life. In contrast, patients that did not develop ESRD continued to have renal parenchymal growth in a linear fashion. This suggests that prenatal RPA may be predictive of postnatal ESRD. Conclusions RPA measurement during the prenatal period could play an important role as a non-invasive tool to predict postnatal renal function and to anticipate postnatal clinical interventions. Table Prenatal total renal parenchymal growth in children with and without ESRD. Table ESRD Non-ESRD p 2nd Trimester, cm2 4.76 (3.56–6.15) 4.97 (2.95–8.38) 0.831 3rd Trimester, cm2 7.07 (6.36–8.95) 10.97 (8.78–14.58) 0.002 [ABSTRACT FROM AUTHOR]

Summary Background Successful primary bladder closure is the most crucial element for urinary continence in patients with classic bladder exstrophy (CBE). In the newborn period, bladder closure can be performed in the first 48 h without pelvic osteotomy or external fixation, but requires postoperative lower extremity immobilization (i.e., spica cast, Bryant's or Buck's traction). Objective To present a novel surgical approach for primary bladder closure for CBE using two-pin external fixation without pelvic osteotomy, and without postoperative lower extremity immobilization. Study design A retrospective chart review of patients with CBE was performed at the current institution from 2000 to 2016, including all primary bladder closures with external fixation and without osteotomy or lower extremity immobilization. Patients were discharged with the external fixator in place, which was later removed in clinic. Baseline clinical and demographic variables, and follow-up data were recorded. Results Thirteen patients were analyzed; eight (61.5%) were male. Pre-operative intersymphysial distance was 3.68 ± 1.0 cm (2.0–5.0). Mean follow-up was 56.8 ± 40.3 months (10–131). One patient had a partial bladder neck dehiscence, due to pin displacement on postoperative day 1: he had the lowest gestational age of 34 weeks ( Summary table ). Discussion This approach used external fixation to bring the pubic bones together intra-operatively, and to decrease the tension in closing the pelvic ring and abdominal wall without osteotomy. External fixation with osteotomy and long-term immobilization, or using a spica cast without osteotomy offered the added advantage of improved wound care, due to lack of lower limb immobilization, less patient discomfort, and facilitation of mother/caregiver and newborn bonding. Conclusion The two-pin external fixator without osteotomy as an adjunct to primary bladder closure in CBE patients was technically feasible. At the current institution this approach had an equivalent success rate to previous reports in the literature for primary bladder closure, decreased the length of hospital stay, and precluded the need for lower extremity immobilization. Early data for bladder capacity were encouraging. Summary table Baseline characteristics and follow-up outcomes of the participants. Clinical variables Mean ± SD (minimum–maximum) Gestational age (months) 38.6 ± 1.9 (34–41.2) Age at surgery (days) 2.6 ± 1.5 (1–5) Hospitalization length (days) 30 ± 15.5 (13–65) Expected bladder capacity for age (%) 55.5 ± 32.4 (11.51–108.47) Successful primary closure (%) 12 (92.3) SD, standard deviation. [ABSTRACT FROM AUTHOR]

Abstract: Congenital midureteral stricture is an uncommon cause of prenatal hydronephrosis, and it is generally associated with proximal hydroureteronephrosis . We report a unique case of a congenital severe midureteral dilatation associated with mild proximal ureteral dilatation and normal distal ureteral diameter. The dilated midureter was excised, and an open end-to-end ureteroureterostomy was performed. Follow-up 6 months after surgical correction revealed marked improvement in the left-sided hydronephrosis and complete resolution of the hydroureter. [Copyright &y& Elsevier]

Objective: To study genotype–phenotype correlation of ring chromosome 18 [r(18)] in 9 patients with 46,XN karyotype. Study design: In 9 patients with a de novo 46,XN,r(18) karyotype (7 females, 2 males), we performed high-resolution single-nucleotide polymorphism array analysis (Illumina Human Omni1-QuadV1 array in 6 patients, Affymetrix 6.0 array in 3 patients), investigation of parental origin, and genotype–phenotype correlation. Results: No breakpoint was recurrent. Single metaphases with loss of the ring, double rings, or secondarily rearranged rings were found in some cases, but true mosaicism was present in none of these cases. In 3 patients, additional duplications in 18p (of 1.4 Mb, 2 Mb, and 5.8 Mb) were detected. In 1 patient, an additional deletion of 472 kb in Xp22.33, including the SHOX gene, was found. Parental origin of r(18) was maternal in 2 patients and paternal in 4 patients, and formation was most likely meiotic. Karyotype was normal in all investigated parents (n = 15). At birth, mean maternal age was 30 years (n = 9) and mean paternal age was 34.4 years (n = 9). Conclusion: Genotype–phenotype correlation revealed extensive clinical variability but no characteristic r(18) phenotype. Severity of clinical signs were generally correlated with the size of the deletion. Patients with large deletions in 18p and small deletions in 18q exhibited mainly symptoms related to 18p-, whereas those with large deletions in 18q and small deletions in 18p had symptoms of 18q-. [Copyright &y& Elsevier]

Abstract: The extrarenal calyx (ERC) is a rare congenital anomaly, associated with various other abnormalities of the urogenital system. We report a unique case of ERC in a solitary functioning kidney with a massively dilated pelvis that developed spontaneous rupture. A dismembered pyeloplasty was performed at the time of rupture. The patient did well post-operatively with a stable creatinine and stable SFU grade 2 hydronephrosis. [Copyright &y& Elsevier]

Purpose: Growing evidence suggests that laparoscopic surgery can be successfully performed in pediatric population. The purpose of this study was to review and report our experience with laparoscopic heminephrectomy in patients less than 1 year of age. Material and Methods: A total of 28 unilateral laparoscopic heminephrectomies for non-functioning duplicated renal moiety were performed on 21 girls and 7 boys less than 1 year of age (45 days - <12 months). The procedures consisted of 26 upper pole and 2 lower pole heminephrectomies. The non functioning renal moiety was associated with ectopic ureteral insertion in 14 patients (50%), ureterocele in 12 patients (42%) and high grade vesico-ureteral reflux into lower pole in 2 patients (8%). The initial approach was transperitoneal in 25 patients while 3 patients were started with retroperitoneal approach. Concomitant ancillary procedures were performed in 14 patients (50%) including 3 open procedures. Results: All procedures were completed laparoscopically. Two procedures started retroperitoneally were completed transperitoneally due to development of pneumoperitoneum. The mean operative time was 135minutes (95-300min) with longer duration observed in patients who also had ancillary procedure performed. The average blood loss was 10ml. and feeds were resumed within 24hours in all patients. Complications were observed in 4 patients (14%). One patient (3%) had intraoperative pneumothorax, managed with chest tube drainage. Two patients (6%) had post operative collection which was managed conservatively. One patient developed hypertension (3%) following an upper pole excision requiring anti-hypertensives. The mean duration of hospital stay was 2.3 days. Conclusions: Laparoscopic procedures can be safely performed for benign renal disease in infants with minimal morbidity. Transperitoneal approach is the preferable due to limited retroperitoneal space. Complications rates are low and other open procedures can be easily preformed concomitantly with good results. [Copyright &y& Elsevier]

Abstract: A 2-month-old child presented for evaluation of prenatal hydronephrosis. Imaging studies were consistent with a right duplex system with a dysplastic, nonfunctioning upper pole and lower pole ureteropelvic junction obstruction. We proceeded with removal of the upper pole and pyeloplasty and were surprised to find a single collecting system with a cystic, dysplastic upper pole segment and the absence of an upper pole pelvis or ureter. The rare diagnosis of a segmental multicystic dysplastic kidney with ipsilateral ureteropelvic junction obstruction was made. We present a review of the case and of previous literature on this topic. [Copyright &y& Elsevier]