Congenital Ureteropelvic Junction Research Articles

Does thiol-disulphide balance in tissue and serum play a role in disease management in ureteropelvic junction stenosis?

The study aimed at evaluating the role of thiol-disulphide balance parameters [native thiol (SH), total thiol (SH+SS), disulphide (SS), disulphide/native thiol ratio (% SS/SH), disulphide/total thiol ratio (% SS/Total Thiol) and native thiol/total thiol ratio (%SH/Total Thiol)], which are important oxidative stress markers in the congenital ureteropelvic junction (UPJ) stenosis, in the diagnosis of the disease, and its role in determining the need for surgery and follow-up. This prospective study included 30 children diagnosed with congenital intrinsic ureteropelvic junction obstruction (UPJO) and a control group of 30 healthy children admitted to the pediatric clinic between January 2016 and February 2017. The children with UPJO underwent laparoscopic dismembered pyeloplasty. Thiol-disulphide balance parameters were assessed in both the peripheral blood and the excised tissue from the narrowed segment of the UPJ during surgery, as well as in the peripheral blood of the control group. Serum levels of native thiol (SH), total thiol (SH+SS), disulphide (SS), the disulphide/native thiol ratio (% SS/SH), the disulphide/total thiol ratio (% SS/Total Thiol), and the native thiol/total thiol ratio (% SH/Total Thiol) were subsequently compared between the two groups. In the UPJO cohort, correlation analyses were conducted to examine relationships between serum and tissue results for native thiol, total thiol, disulphide, % SS/SH, % SS/Total Thiol, and % SH/Total Thiol, alongside Tc(Technetium)-99m MAG-3 (mercaptoacetyltriglycine) differential renal function (DRF) (%), renal pelvic anterior-posterior (AP) diameter, renal parenchymal thickness, and plasma creatinine levels. The findings of this study indicated statistically significant differences in serum levels of native thiol, total thiol, disulphide, % SS/SH, % SS/Total Thiol, and % SH/Total Thiol between the UPJO and control groups. Specifically, the UPJO group exhibited higher values of serum disulphide, % SS/SH, and % SS/Total Thiol, while serum levels of native thiol, total thiol, and % SH/Total Thiol were significantly lower (p<0.05). Furthermore, no statistically significant correlations were observed in the UPJO group between tissue and serum results for native thiol, total thiol, disulphide, % SS/SH, % SS/Total Thiol, % SH/Total Thiol, and clinical parameters including MAG-3 differential renal function (DRF) (%), pelvic anterior-posterior (AP) diameter, renal parenchymal thickness, and plasma creatinine levels. The UPJO group displayed significantly elevated levels of serum disulphide, % SS/SH, and % SS/Total Thiol compared to the control group, while serum native thiol, total thiol, and % SH/Total Thiol were notably lower. Additionally, no correlations were found between serum and tissue thiol-disulphide balance parameters and clinical measures such as MAG-3 DRF (%), pelvic AP diameter, parenchymal thickness, and plasma creatinine levels. Further comprehensive studies are warranted to identify new biomarkers for monitoring UPJ stenosis.

Predictive Value of Initial Diagnostic Imaging for Congenital Ureteropelvic Junction Obstruction Requiring Pyeloplasty

Predictive Value of Initial Diagnostic Imaging for Congenital Ureteropelvic Junction Obstruction Requiring Pyeloplasty

Role of Interstitial Cells of Cajal in Congenital Ureteropelvic Junction Obstruction.

Although congenital ureteropelvic junction (UPJ) obstruction is the most common cause of neonatal hydronephrosis, aetiopathogenesis is still inconclusive. Recently, the paucity of interstitial cells of Cajal (ICC) at the narrow adynamic part of UPJ has been implicated as a causative factor. This prospective study was conducted between October 2019 and March 2022 to find out the density of ICC by the immunohistochemical method using CD117 (c-kit) antibody, in resected segments of UPJ in obstruction patients and in renal tumour patients as control. ICC/high power field (hpf) was also studied from the margins of the resected segment in the obstruction group. The pre-operative and post-operative sonographic and renal scintigraphic features were compared. The median age of patients in the study group (n = 25) was 36 months and in the control group was 39 months. The mean ICC/hpf at the stenotic part of UPJ in the study group was 3.56 ± 1.26 and in the control group was 12.56 ± 1.89 (P = 0.0001). ICC density from the proximal and distal margins of the resected segment was 11.12 ± 2.12 and 11.68 ± 1.62, respectively (P < 0.001). The post-operative antero-posterior diameter of the renal pelvis and differential renal function showed significant improvement in comparison to the pre-operative value (P = 0.0045 and 0.0005, respectively). The significant decrease in the density of ICC at the stenotic part of UPJ compared to controls suggests a pacemaker role of these cells in ureteral peristalsis and the aetiopathogenesis of UPJ obstruction. Histopathological analysis of ICC should not only be limited to the stenotic part of UPJ but also should focus on the anastomosed ends of the ureter, which reflects post-pyeloplasty outcome.

Determination of voiding pressure in infants with normal lower urinary tracts: Exploring the possible effect of sacral development.

To investigate the association between sacral underdevelopment, as defined by subnormal sacral ratio (SR) measurements, with increased maximum detrusor voiding pressure (P det. Max) in infants. In this 2007-2015 retrospective cohort study, the medical records of all infants who underwent a pyeloplasty due to congenital ureteropelvic junction obstruction were added. Their P det. Max was evaluated through the suprapubic catheter utilized for urinary drainage intraoperatively, without imposing any additional risk of urethral catheterization on the infant. SR was calculated via the plain kidney, ureter, and bladder (KUB) radiography film obtained during the voiding cystourethrogram (VCUG) evaluation before the surgery. Participants were categorized into SR < 0.74 or SR ≥ 0.74. P det. Max was subsequently compared between these two groups. A total of 45 patients were included in our analysis. Twenty-eight (62.2%) patients had a (SR < 0.74), while 17 (37.8%) had a (SR ≥ 0.74). P det. Max was shown to be significantly higher in the SR < 0.74 compared to the SR ≥ 0.74 group (167.5 ± 60.8 vs. 55.7 ± 17.9 cmH2O, p < 0.001). After adjusting for age and sex, SR remained a significant contributor to P det. Max (p < 0.001). Physiologic detrusor sphincter dyscoordination (PDSD) rate was significantly higher in the SR < 0.74 versus SR ≥ 0.74 group (100.0% vs. 70.6%, respectively; p = 0.005). Lumbosacral underdevelopment, as indicated by subnormal sacral ratios, is associated with sphincter-detrusor dyscoordination, which causes PDSD and can ultimately result in higher P det. Max in infants.

Renal Histopathological Changes in Children With Congenital Ureteropelvic Junction Obstruction

Renal histologic changes in congenital ureteropelvic junction obstruction (UPJO), although well documented, are sparsely studied in children. This study aims to establish a histological grading depending on the glomerular and tubulo-interstitial changes in hydronephrotic kidneys and determine correlation with age at surgery and impact on function post-pyeloplasty. A renal cortical wedge biopsy was obtained after pyeloplasty and histological changes were graded from 1 to 4. A total of 47 viable biopsies from 57 children showed grade 1 changes in 10, grade 2 in 7, grade 3 in 27, and grade 4 in 3. Age at surgery, histological grade, and changes in differential renal function (DRF) were statistically analyzed. Younger age, near normal histology, and those with changes in 1 domain had preserved DRF pre-operatively. Renal histology and overall grade showed positive correlation with pre-operative DRF. However, no statistically significant association was noted with age at surgery or change in post-operative DRF.

Surgical intervention and long-term renal outcomes of congenital ureteropelvic junction obstruction in a young adult cohort.

To evaluate the impact of surgical intervention on long-term renal outcomes for adult patients with congenital ureteropelvic junction obstruction (UPJO). We queried service members diagnosed with UPJO from the United States Military Health System electronic health records from 2005 to 2020. We assessed demographic, laboratory, radiology, surgical intervention, and outcome data. We evaluated the impact of surgical intervention on renal function based on the estimated glomerular filtration rate (eGFR), hypertension (HTN, defined as any prescription for blood pressure [BP] medication and/or average of two BP readings ≥ 130/80mmHg more than 2weeks apart), and changes in renal excretory function on radionuclide scans. We identified 108 individuals diagnosed with congenital UPJO; mean follow-up of 7years. Mean age at diagnosis was 25years; 95% male; 69% White, 15% Black. At diagnosis, median BP was 130/78mmHg and mean eGFR 93ml/min/1.73m2. Subsequently, 85% had pyeloplasty and 23% had stent placement. There were no significant differences in mean eGFR pre- and post-intervention (94 vs. 93ml/min/1.73m2, respectively; p = 0.15) and prevalence of defined HTN (59% vs. 61%, respectively; p = 0.20). Surgical intervention for right-sided UPJO significantly reduced the proportion of patients with delayed cortical excretion (54% pre vs. 35% post, p = 0.01) and T½ emptying time (35min vs. 19min, p = 0.009). Similar trends occurred with left-sided UPJO but were not significant. Surgical intervention was not associated with significant differences in the long-term outcomes of kidney function and HTN prevalence in our young adult cohort. However, renal excretory function improved on radionuclide scans.

Unique presentation of Congenital ureteropelvic junction obstruction in adult

Summary: A 34-year-old female, without comorbidities, presented to the emergency department with sudden right flank pain. Initial workup showed elevated WBC (14.5) and normal creatinine (0.81), but later developed fever spikes and rising CRP (59.50). Gentamicin treatment was initiated. Transferred for a Diuretic Renogram, confirming suboptimal function of the right kidney with obstruction at the renal pelvis/PUJ. Split functions indicated 32% for the right kidney and 68% for the left. Radiological workup included ultrasound, CT KUB with contrast, and CT Urography, revealing right renal acute hydronephrosis without evidence of stones. Management involved symptomatic treatment, but with persistent symptoms, Gentamicin prophylaxis was initiated. The patient was transferred for Renogram, detecting suboptimal right kidney function. A planned stent insertion followed by Robotic-assisted and laparoscopic pyeloplasty was scheduled. Discussion: Uretero Pelvic Junction Obstruction (UPJO) is a partial blockage where the ureter enters the kidney. Congenital UPJO can result from intrinsic narrowing, high ureter insertion, ureteral polyps, or accessory renal artery crossing vessels causing extrinsic compression. Most cases are detected on prenatal ultrasound, while postnatal presentations include UTI, abdominal mass, hematuria, and intermittent flank pain. Diagnosis involves CT urography and diuretic renogram, confirming suboptimal function (

Interstitial cells of Cajal in the ureteropelvic junction: does its expression have a role in the pathogenesis of congenital ureteropelvic junction obstruction?

Background One of the most frequent urological abnormalities in pediatric urology and the most frequent cause of hydronephrosis in the pediatric age group is uteropelvic &lt;ureteropelvic?&gt; junction obstruction (UPJO). Although its etiology has been the subject of several investigations, it is still unknown. Objective Comparing the quantitative changes in the interstitial cells of Cajal (ICC) between specimens with UPJO and specimens without hydronephrosis using light microscopy and correlating these changes to the etiology of the disease. Methods Patients with UPJO and patients without obstruction were compared immunohistochemically with c-kit (CD117) between February 2020 and February 2022 at Ain Shams University Hospital to quantify the ICC, which is situated close to the circular muscle layer and parallel to the muscle cells. Results Light microscopic analysis revealed that the control group’s Cajal cell density was much higher than the number of cells in the patients with UPJ blockage. Conclusion Our research found that the mean density of the ICC was lower in UPJO patients compared with controls, indicating that the absence of the ICC may contribute to the pathogenesis of UPJO.

Obstrucción congénita de la unión ureteropiélica. Estudio de casos en un centro de segundo nivel

Objective: To evaluate the incidence, clinical characteristics, diagnosis, and treatment of congenital ureteropelvic junction obstruction in pediatric patients (CUPBO) at the Hospital Infantil de Morelia. Material and methods: This was a retrospective, observational, descriptive, and cross-sectional study of pediatric patients diagnosed with CUPBO and treated in the urology service of a second-level care medical center from January 2018 to December 2021. Results: We reviewed 53 patients with prenatal hydronephrosis who underwent postnatal follow-up, of whom only 6 patients diagnosed with congenital ureteropelvic junction obstruction (CUPBO) were identified prenatally. Seven patients were diagnosed with hydronephrosis postnatally and subsequently diagnosed with CUPBO, resulting in a total sample of 13 diagnosed patients. The incidence of CUPBO was 3.4 patients per 100 first visits to the Pediatric Urology Service. Of the cases, 92.31% were male, and the most frequent clinical manifestations were skin infections and localized pain. Surgery was performed in all cases, with Anderson-Hynes pyeloplasty being the most frequent procedure. Complications included urinary tract infections, wound infections, and fistula, but most patients had satisfactory results with a reduction of hydronephrosis after surgery. Findings or conclusions: Our study found a low rate of early detection of CUPBO in patients with prenatal hydronephrosis follow-up. We recommend an intentional search for urologic alterations in structural ultrasound scans of prenatal control. We emphasize the importance of an accurate diagnosis and timely treatment of CUPBO to prevent renal complications.

IMMUNOHISTOCHEMICAL EXPRESSION OF C-KIT AND S-100 MARKERS IN URETEROPELVIC JUNCTION OBSTRUCTION AND ITS CORRELATION WITH HISTOPATHOLOGY AND MASSON'S TRICHROME STAINING

Background: Congenital obstructive nephropathy is the principal cause of end stage renal disease (ESRD) in children ( Beneldet al. 2003). Obstructive nephropathy leads to Hydronephrosis, dened clinically by an enlargement of the kidney as a result of urine accumulation in the renal pelvis or calyces. [1,2] Ureteropelvic junction (UPJ) obstruction is the most common cause of hydronephrosis with an estimated incidence of 1 in 1000–1500 newborns ( Changet al. 2004). [2,3] Aims: To evaluate the pathogenesis of congenital ureteropelvic junction obstruction (UPJO) by histopathology, IHC markers CD117 &amp; S100 and special stain Masson's Trichrome. Methods And Materials: The study group consists of 50 Pediatric cases presenting with intrinsic ureteropelvic junction obstruction and 15 patients with Wilms tumor with no UPJO formed the control group. All the cases and controls were subjected to histopathological examination, immunohistochemical staining with markers CD117 &amp; S100 and special stain Masson's Trichrome. Mann – Whitney U test and Pearson's chisquare test were used for statistical analysis. The cases had reduce Results: d CD117 and S100 expression, Irregularity of muscle bers, increased wall thickness &amp; muscle thickness and submucosal collagen deposition compared to controls. Decreased ICC interpreted by Conclusions: decreased CD117, decreased neural innervation interpreted by decreased S100, structural changes - Irregularity of muscle bers arrangement, increased submucosal collagen deposition and increased smooth muscle thickness possibly cause disruption of the mobility of UPJ and lead functional obstruction.

Assessment of Cases of Congenital Obstructive Uropathy in Children

Background: The aim is to assess cases of congenital obstructive uropathy in children.Material &amp; Methods:Sixty- five children in age ranged 5-12 years of either gender with congenital obstructive uropathy were recruited for the study. In all cases, ultrasonography was done in all cases. Growth of the child was monitored and height standard deviation score (Ht-SDS) was calculated annually. Glomerular filtration rate (ml/min/1.73 m2) was calculated.Results:Out of 65 patients, males were 40 and females were 25. Etiology found to be ureteropelvic junction obstruction (PUJO) in 12, posterior urethral valve (PUV) in 14, PUJO + PUV in 30 and obstructive megaureter in 9 cases. Symptoms were fever in 34, pain abdomen in 20, burning micturition in 45, poor urinary stream in 21 and flank pain in 15 cases. Treatment given was pyeloplasty in 14, nephrectomy in 20, PUV fulguration in 11, ureterocele decompression in 12 and ureteric reimplantation in 8 cases.Conclusions:Most common etiologies for congenital obstructive uropathy in children were congenital uretero-pelvic junction obstruction and posterior urethral valve. Male preponderance was seen with burning micturition.

FC025: Physiological Role of KV Channel in Ureter Smooth Muscle Cell Investigated Quantitatively by Electrophysiological Modeling

Abstract BACKGROUND AND AIMS Spontaneous peristaltic contraction disorder of the ureter smooth muscle (USM) is an important etiology of congenital giant ureter, congenital ureteropelvic junction deformity and non-obstructive hydronephrosis [1]. The USM cell action potential (AP) plays a key role in commencing USM contraction by allowing influx of Ca2+ from the extra cellular space [2]. Therefore, an in-depth quantitative investigation of the USM cell AP generation will assist in developing better pharmacological therapies for the USM abnormal contraction. The purpose of this in-silico study is to unfold the physiological role of Kv channel in modulating the USM AP generation. METHOD The model consists voltage- dependent Na+ currents (INa), hyperpolarization-activated cyclic nucleotide- gated current (Ih), voltage- dependent Ca2+ currents (ICa), Ca2+ dependent potassium channel (IBKCa) and voltage-dependent potassium channels (IKv) as the components of active electrophysiological characteristics [3]. All ion channels are represented by the Hodgkin–Huxley formalism. The maximum conductance of the is varied to investigate the physiological role of the Kv channel in modulating the USM AP generation. RESULTS The whole cell IKv current is generated by applying the voltage clamp protocol, where the membrane potential is stepped from a holding potential of –80 mV–60 mV for a duration of 500 ms. The half activation potential, half inactivation potential, activation slope factor and inactivation slope factors are: 43 mV, –76.1 mV, 15.4 mV and 6.5 mV, respectively, to fit the Boltzmann function equation. The model reveals that fast activation and inactivation properties are the essential feature of IKv for the USM cell. The resting membrane potential (RMP) is set at –50 mV. The AP was induced in the whole cell model by applying an external stimulus current (10–30 pA), as a brief square pulse of 500 ms duration. It is demonstrated that IKv opposes the Ca2+ current during the first 50 ms of the AP duration and then inactivated. It doesn't contribute to the repolarizing phase of the AP. CONCLUSION The plateau phase of the USM AP shows that the total AP duration much longer than that in other smooth muscle cells. It strongly supports the phenomenon is due to the activation of Ca2+-dependent K+ channel and inactivation of L-type Ca2+ channel. In ureter cells, the IKv does not contribute significantly to the repolarization of the AP, but it may regulate membrane excitability by opposing Ca2+ current activated around the threshold of the AP. Therefore, the agonist and antagonist of the IKv might be useful partially as new pharmacological targets for the USM abnormal contraction.

Case of congenital ureteropelvic junction obstructive nephrolithiasis with acute urinary retention-X-ray abdomen (KUB view)

 A 36-year-old male patient was brought to casualty with complaints of colicky left sided flank pain, nausea, hematuria and difficulty in urination since 3 months. The patient came with no passage of urine since last 12 hours and sudden onset of pain. Per abdomen examination reveals tenderness at costovertebral angle with tachycardia and tachypnea. Patient was advised for X-ray abdomen KUB. Large left calculus in lower calyx with hydronephrosis as shown in (A) with mild tenderness over left flank. Catheterization was done. The patient was further advised for routine blood investigations, renal ultrasound, urine examination. The emergency surgery decision was taken on next day. Open pyelolithotomy with anderson-hynes pyeloplasty under general anesthesia was done. Post operatively patient was managed by Inj. Ceftriaxone 700 mg IV 12 hourly, Inj. Gentamycin 60 mg IV 8 hourly, Inj. Pantoprazole 40 mg IV 12 hourly and Inj. Neomol 1g IV 8 hourly. Liquid tolerated diet was advised to patient on post-operative day 2. Patient suggested to take very low calcium diet. After 7 days patient vitals was normal and shifted to normal diet and oral medication. The patient was advised to increase liquid diet and water intake. The final diagnosis was congenital ureteropelvic junction obstructive large (2.6 cm) nephrolithiasis with acute urinary retention, while the differentials included renal cell carcinoma, renal vein thrombosis, polycystic kidney disease.

Comparison of Drainage Methods After Pyeloplasty in Children: A 14-Year Study

Objective: To summarize our experiences with drainage methods after laparoscopic pyeloplasty with a 14-year study.Methods: We reviewed the data of the 838 children operated on for hydronephrosis due to congenital ureteropelvic junction obstruction (UPJO) between July 2007 and July 2020. Patients' demographics, perioperative details, postoperative drainage stents [including double-J stent, percutaneous trans-anastomotic (PU) stent, and trans-uretero-cystic external urethral stent (TEUS)], complications, hospital stay, and long-term follow-up outcomes were analyzed. Long-term follow-up was performed by outpatient visits and telephone follow-up. Moreover, we reviewed the details of nine cases of recurrence after laparoscopic pyeloplasty.Results: Comparison of preoperative general data among the three groups indicated that there was no statistical difference in age, gender, and surgical side of the three groups. Statistical differences were found in the incidence of postoperative complications from the three postoperative drainage method groups, especially the incidence of reoperations (p < 0.01): there were six cases (3.19%) of recurrences in the TEUS group, two cases (0.36%) in the DJ group, and one case (0.93%) in the PU group. In the six recurrent cases from the TEUS group, four cases (44.4%) were found to have stenosis, and two cases (22.2%) have iatrogenic valvular formation.Conclusion: Not all three types of drainage methods are suitable for drainage after pyeloplasty. Based on our findings, TEUS is not recommended.

Management of ureteropelvic junction obstruction in an era of minimally invasive surgery

IntroductionIn an era of minimally invasive surgery, laparoscopic pyeloplasty has emerged as an alternative to conventional open pyeloplasty. Although the open method boasts a success rate of over 90%, laparoscopic pyeloplasty far exceeds patient satisfaction due to its low perioperative morbidity. The aim of this study was to evaluate the outcome of patients who underwent laparoscopic pyeloplasty due to congenital ureteropelvic junction obstruction (UPJO). Material and Methods A retrospective observational study was conducted at Sri Jayawardenapura general hospital from March 2014 to December 2020. All patients were subjected to CT urogram and diuretic renogram (DTPA) scan. Clinical, imaging and biochemical findings were recorded. Patients included were those who underwent laparoscopic pyeloplasty based solely on the clinical severity of the disease, those who underwent lap-nephrectomy or open surgery was excluded. Follow up was based on clinical and DTPA scan findings. Improvement in symptomatology, split renal function(SRF) by 5% and/or diuretic curve pattern were considered successful outcomes. Results A total of 40 patients who underwent LP were evaluated with a mean age of 32 years and slight male preponderance. The majority (82.5%) were symptomatic with pain and/or infection. All were diagnosed with hydronephrosis with impaired DTPA. An SRF &lt; 40% in the affected kidney was observed in 22(55%) of patients. Concomitant pyelolithotomy was performed in 7(17.5%) patients. Mean hospital stay was 3.5 days. At mean follow up of 22-months, improvement in symptoms were noted in 35(87.5%) patients and SRF improvement (&gt;5%) were noted in 11(27.5%); the function remained stable in 27(67.5%) and deteriorated in 2(5%) of patients. Discussion Although LP is technically demanding, it offers excellent symptomatic relief and renal preservation. It offers less morbidity, better aesthetic and post-operative convalescence: a promising viable option replacing open surgery.

Minimally invasive management of massive giant hydronephrosis in tertiary care centre Northern Sri Lanka: A case report.

Introduction & importanceGiant hydronephrosis (GH) is defined as a kidney containing greater than 1000 ml of urine in its collecting system. It is a rarely presented condition in an adult. Common aetiology for GH is due to congenital ureteropelvic junction obstruction. We present a case of a late presentation of GH was being managed by a minimally invasive laparoscopic technique.Case presentationA 63-year-old Sri Lankan male presented with worsening of generalized abdominal swelling with vague abdominal symptoms for a six-month duration, found to have a GH of the left kidney on USS. The Contrast-enhanced computerized tomography (CECT) scan confirmed the diagnosis with 12l of fluid and possible ureteropelvic junction obstruction (UPJO). CT did not show any excretion of the left kidney. Hydronephrosis was drained one day before the surgery by a percutaneous nephrostomy tube. Laparoscopic transperitoneal nephrectomy was performed. The patient had a smooth and fast recovery.DiscussionA neglected congenital UPJO can present with GH in adults. It causes vague abdominal symptoms like abdominal distension, dyspepsia, and fatigue. CECT will give the diagnosis and identify the aetiology of GH. Non-functional GH kidneys can be treated with nephrectomy by open or laparoscopic surgical technique. Laparoscopic nephrectomy shoes less blood loss during surgery, less postoperative pain and early recovery after surgery. However, in GH, surgeons need the experience to overcome the challenges like adhesions and working in a small space.ConclusionsThe percutaneous nephrostomy decompression followed by laparoscopic transperitoneal nephrectomy is feasible for a massive GH.

Infant Robot-assisted Laparoscopic Pyeloplasty: Outcomes at a Single Institution, and Tips for Safety and Success

BackgroundSince its first description, multiple reports proved efficacy and safety of the robotic platform. Further progress has been made allowing for the application of robotic surgery to smaller patients, including infants. Despite the early favorable results, the use of robot surgery in infants is still controversial and more studies are needed to confirm its benefits. ObjectiveTo our knowledge, we present the largest single-institution case series of robot-assisted laparoscopic pyeloplasty (RAL-P) in infants, aiming to contribute to the current literature with a guide for key technical steps and safety tips for infant RAL-P. Design, setting, and participantsWe performed a retrospective review of a prospectively maintained database. The study protocol was approved by the institutional review board. Surgical procedureOnly infants (≤12 mo of age) with a diagnosis of congenital ureteropelvic junction obstruction (UPJO) undergoing primary robotic dismembered pyeloplasty were included in the study. MeasurementsWe critically reviewed the clinical outcomes, described the main steps of the operation, and shared tips for a safe approach. Results and limitationsFrom January 2012 to August 2019, 44 infants underwent RAL-P for UPJO––33 (75%) males and 11 (25%) females. All robotic cases were completed successfully, with no laparotomic conversions. The median age and weight were 4 (1–12) mo and 6.8 (3.8–10.5) kg, respectively. The mean operative time was 142 (±25) min. The mean estimated blood loss was 7 (±3.6) ml, and no intraoperative complications occurred. The mean length of hospital stay (LOS) was 1.4 (±0.7) d. Seven (15.6%) patients had postoperative complications—one (2%) ileus (Clavien-Dindo grade [CDG] I), four (9%) urinary tract infections (CDG II), and two (4.5%) port-site hernias (CDG III). At a median follow-up of 19 mo, the success rate was 100%. ConclusionsGiven the successful outcomes, benefits of decreased LOS, and improved cosmesis, RAL-P is an appealing management option for UPJO in infants. Market release of new systems, further miniaturization of instruments, and more affordable costs will hopefully be shedding light on more complex applications. Patient summaryInfants (≤12 mo of age) diagnosed with ureteropelvic junction obstruction undergoing primary robotic dismembered pyeloplasty were selected and included in this study. No intraoperative complications or conversion to an open approach occurred. Seven patients (16%) developed postoperative complications—one (2%) postoperative ileus, four (9%) urinary tract infections, and two (4.5%) port-site hernias. At a median follow-up of 19 (7–66) mo, the success rate was 100%.

Giant infected hydronephrosis: A kidney bag filled with 6300 cc pus

Giant hydronephrosis has been variably defined in literature as that one that contains more than 1 liter of fluid or comprising >1.6% of the body weight. It is also described as that occupying a hemiabdomen, crossing the midline and occupying more than five vertebral heights. Congenital uretero-pelvic junction obstruction is the most common cause of giant hydronephrosis in children and young adults. Other important causes include vesico-ureteric junction obstruction, ureteral atresia and primary obstructed megaureter. We herein report a rare case of giant hydronephrosis secondary to pelvi-ureteric junction obstruction presenting as an acute abdomen in a young female.

Outcome of Percutaneous Nephrostomy in Children With Ureteropelvic Junction Obstruction

Introduction and objectives: Congenital ureteropelvic junction obstruction (UPJO) is the most common cause of hydronephrosis. Management protocols are based on the presence of symptoms and when the patient is asymptomatic the function of the affected kidney determines the line of treatment. Percutaneous nephrostomy (PCN) became a widely accepted procedure in children in the 1990s. The aim of the study was to evaluate the results of performing percutaneous nephrostomy (PCN) in all patients with UPJO and split renal function (SRF) of less than 10% in the affected kidney, because the management of such cases is still under debate. Methods:This prospective clinical trial was carried out at Dhaka Medical College Hospital from January 2014 to December 2016. Eighteen consecutive patients who underwent PCN for the treatment of unilateral UPJO were evaluated prospectively. In these children, ultrasonography was used for puncture and catheter insertion. Local anesthesia with sedation or general anesthesia was used for puncture. Pig tail catheters were employed. The PCN remained in situ for at least 4 weeks, during which patients received low-dose cephalosporin prophylaxis. Repeat renography was done after 4 weeks. When there was no significant improvement in split renal function (10% or greater) and PCN drainage (greater than 200 ml per day) then nephrectomies were performed otherwise pyeloplasties were performed. The patients were followed up after pyeloplasty with renograms at 3 months and 6 months post operatively. Results: All the patients had severe hydronephrosis during diagnosis and 14 patients with unilateral UPJO were improved after PCN drainage and underwent pyeloplasty. The rest four patients that did not show improvement in the SRF and total volume of urine output underwent nephrectomy. In the patients with unilateral UPJO who improved after PCN drainage, the SRF was increased to 26.4% ±8.6% (mean± SD) after four weeks and pyeloplasty was performed. At three and six months follow-up, SRF value was 29.2% ±8.5% and 30.8.2% ±8.8% respectively. Conclusion: Before planning of nephrectomy in poorly functioning kidneys (SRF &lt; 10%) due to congenital UPJO, PCN drainage should be done to asses improvement of renal function. Bangladesh Journal of Urology, Vol. 20, No. 2, July 2017 p.61-64

Report of a de novo c.2605C > T (p.Pro869Ser) change in the MED13L gene and review of the literature for MED13L-related intellectual disability

BackgroundMED13L-related intellectual disability is a new syndrome that is characterized by intellectual disability (ID), motor developmental delay, speech impairment, hypotonia and facial dysmorphism. Both the MED13L haploinsufficiency mutation and missense mutation were reported to be causative. It has also been reported that patients carrying missense mutations have more frequent epilepsy and show a more severe phenotype.Case presentationWe report a child with ID, speech impairment, severe motor developmental delay, facial deformity, hypotonia, muscular atrophy, scoliosis, odontoprisis, abnormal electroencephalogram (EEG), and congenital ureteropelvic junction obstruction (UPJO) combined with high ureter attachment. We used whole-exome sequencing (WES) to detect the genetic aberration of the child and found a de novo mutation, c.2605C > T (p.Pro869Ser), in the MED13L gene. Neither of her parents carried the mutation. Additionally, we review the literature and summarize the phenotypes and features of reported missense mutations. After reviewing the literature, approximately 17 missense mutations in 20 patients have been reported thus far. For 18 patients (including our case) whose clinical manifestations were provided, 100% of the patients had ID or developmental delay (DD). A total of 88.9, 83.3 and 66.7% of the patients had speech impairment, delayed milestones and hypotonia, respectively. A total of 83.3% of the patients exhibited craniofacial deformity or other dysmorphic features. Behavioral difficulties and autistic features were observed in 55.6% of the patients. Cardiac anomalies were seen in only 27.8% of the patients. Of these patients, 44.4% had epileptic seizures. Of the 17 mutations, 2 were located in the N-terminal domain, 8 were located in the C-terminal domain, and 1 was located in an α-helical sequence stretch. One of them was located in the MID domain of the MedPIWI module.ConclusionsWe report a new patient with a reported missense mutation, c.2605C > T (p.Pro869Ser), who exhibited some infrequent manifestations except common phenotypes, which may broaden the known clinical spectrum. Additionally, by reviewing the literature, we also found that patients with missense mutations have a higher incidence of seizures, MRI abnormalities, autistic features and cardiac anomalies. They also have more severe ID and hypotonia. Our case further demonstrates that Pro869Ser is a hotspot mutation of the MED13L gene.