Pelvi-ureteric Junction Obstruction Research Articles

Impact of Informative Videos on Proxy Consent by Parents for Pediatric Surgery: A Randomized Controlled Trial

ABSTRACT Background: Consent is never truly exercised in children as parents act as their proxy and often do not understand the advantages, disadvantages, risks, and benefits of the procedure. Their high anxiety state is mirrored in the child, leading to slower recovery. Hence, this study was designed to understand if an intervention can impact the effect of consent on parents’ anxiety, satisfaction, and knowledge. Methodology: A two-arm, parallel design, randomized controlled trial was conducted from March 2020 to March 2022 to analyze the effect of an educational video in comparison to an informational leaflet in parents giving proxy consents for various listed pediatric surgical procedures. Results: Parents in the video group had a higher mean knowledge score (6.97 vs. 6.77, P = 0.40), comparable satisfaction scores (27.45 vs. 27.58, P = 0.88), and statistically insignificant difference between anxiety scores. We noted highest score of satisfaction (28.5/40) in the parents educated up to High School level or less. Knowledge scores were lowest in pelvic-ureteric junction obstruction (5.1/10) patients. Conclusions: Our study done over a period of 2 years included a variety of diagnoses, and the videos and information leaflets were self-designed. It showed comparable anxiety, knowledge, or satisfaction in parents. Studies with more participants would be needed to take this research forward.

Outcome Analysis of Transperitoneal Laparoscopic Nephrectomy in Children: Experience from a Developing Country.

To analyse the outcome of laparoscopic nephrectomy among children. An observational study. Place and Duration of the Study: Department of Paediatric Urology, Children's Hospital Lahore, from September 2021 to June 2023. During the study, 100 children aged 5-180 months were included. Data regarding age, gender, cause, side, intraoperative and postoperative outcomes such as number of ports, estimated blood loss, need of transfusion, operative time, hospital stay, analgaesia requirement and complications were recorded. Data were collected through proforma which were entered and analysed through computer software SPSS version 16.0. Among 100 children, 58.0% were males and the mean age was 79.54 ±4.427 months while the major cause of laparoscopic nephrectomy was PUJO (Pelvi-Ureteric Junction Obstruction) for 47.0% of children. The mean blood loss was 19.93±65.556 ml and the majority of the children (96.0%) did not require blood transfusion. The mean operative time was 125.31 ± 43.365 minutes and the mean hospital stay was 2.82 ± 1.258 days. Most of the children (90.0%) had no complications while 3.0% had ileus which was managed conservatively and 3.0% were converted to open surgery while 4.0% had wound infection. Laparoscopic nephrectomy is an effective technique with regard to insignificant blood loss, operative time, hospital stay, and rate of complications. Laparoscopy, Nephrectomy, Children, Operative time, Hospital stay, Complications.

Outcome Analysis of Transperitoneal Laparoscopic Nephrectomy in Children: Experience from a Developing Country.

To analyse the outcome of laparoscopic nephrectomy among children. An observational study. Place and Duration of the Study: Department of Paediatric Urology, Children's Hospital Lahore, from September 2021 to June 2023. During the study, 100 children aged 5-180 months were included. Data regarding age, gender, cause, side, intraoperative and postoperative outcomes such as number of ports, estimated blood loss, need of transfusion, operative time, hospital stay, analgaesia requirement and complications were recorded. Data were collected through proforma which were entered and analysed through computer software SPSS version 16.0. Among 100 children, 58.0% were males and the mean age was 79.54 ±4.427 months while the major cause of laparoscopic nephrectomy was PUJO (Pelvi-Ureteric Junction Obstruction) for 47.0% of children. The mean blood loss was 19.93±65.556 ml and the majority of the children (96.0%) did not require blood transfusion. The mean operative time was 125.31 ± 43.365 minutes and the mean hospital stay was 2.82 ± 1.258 days. Most of the children (90.0%) had no complications while 3.0% had ileus which was managed conservatively and 3.0% were converted to open surgery while 4.0% had wound infection. Laparoscopic nephrectomy is an effective technique with regard to insignificant blood loss, operative time, hospital stay, and rate of complications. Laparoscopy, Nephrectomy, Children, Operative time, Hospital stay, Complications.

Assessment of post-pyeloplasty renal drainage in antenatally detected hydronephrosis by 99mTc-L, L-Ethylenedicysteine renal scintigraphy: the importance of delayed imaging.

Diuretic renal scintigraphy is important in diagnosing pelvi-ureteric junction obstruction (PUJO) in antenatally detected hydronephrotic (ANH) kidneys. Early-phase dynamic renal scintigraphy has several pitfalls contributing to the false interpretation of obstructed drainage, especially after pyeloplasty. This study explores the utility of delayed imaging in 99mTc-L,L-Ethylenedicysteine (EC) diuretic renal scintigraphy to assess post-pyeloplasty renal drainage in ANH. Data from May 2019 to January 2024 was retrospectively studied to identify infants with ANH who underwent surgery for PUJO and pre-and post-surgical assessment of renal drainage using 99mTc-L,L-EC diuretic renal scintigraphy. Dynamic and post-void static images were analyzed to calculate percentage drainage till 3h. Pre- and post-operative quantitative parameters were compared. Receiver operator curve analysis was done to calculate the threshold drainage to detect renal obstruction on postvoid images. In a cohort of 104 patients with a mean age of 10.1 ± 6.5months (range 2-36months) at surgery, 106 renal units (bilateral involvement in two patients) were analyzed. There was no significant difference in the sum of obstructive and equivocal curve patterns (105 vs 79, p = 0.06) and T1/2 values (105 vs 82, p = 0.093) before and after surgery. However, there was a significant difference between the pre-and post-surgery median 3-h percentage drainage (26 vs 80%, p < 0.001). A drainage threshold of less than 59% at 3h had 93.4% sensitivity and 92.5% specificity to detect obstructive drainage. Delayed images in diuretic renal scintigraphy are crucial in accurately interpreting post-pyeloplasty drainage patterns and help overcome pitfalls of the dynamic study.

Congenital Anomalies of the Kidney and Urinary Tract in Patients with Hirschsprung Disease.

To determine the frequency and types of congenital anomalies of the kidney and urinary tract (CAKUT) in patients with Hirschsprung disease. An observational cross-sectional study. Place and Duration of the Study: Department of Paediatric Surgery, National Institute of Child Health, Karachi, Pakistan, from June to December 2022. All patients with biopsy-proven Hirschsprung disease were included. Ultrasound was done to find out the anatomical and structural anomalies of the kidney and urinary tract. Functional assessment was done by renal function tests and MAG3 radioisotope scan where indicated. Fisher's exact test was applied to find out the association. A p ≤0.05 was taken as significant. Out of a total of 83, 15 (18.0%) patients had CAKUT. The mean age of the study population was 5.5 ± 2.3 years. It included 61 (73.5%) males and 22 (26.5%) females. No significant association was found between the type of Hirschsprung disease and CAKUT (p = 0.7). The gender distribution between CAKUT patients was also insignificant (p = 0.7). Renal hypoplasia was the most common anomaly found in six patients followed by hydronephrosis due to pelvi-ureteric junction obstruction in four children. All of these children were asymptomatic. Five male patients had undescended testis. Nearly a fifth of the children with Hirschsprung disease had CAKUT of whom renal dysplasia was the most common anomaly. There was no gender predilection and patients were asymptomatic regarding the urinary system. A routine ultrasound abdomen is a good screening investigation for identifying CAKUT. Hirschsprung disease, Kidney and urinary tract, Congenital diseases of the urinary tract, Congenital anomalies.

Congenital Anomalies of the Kidney and Urinary Tract in Patients with Hirschsprung Disease.

To determine the frequency and types of congenital anomalies of the kidney and urinary tract (CAKUT) in patients with Hirschsprung disease. An observational cross-sectional study. Place and Duration of the Study: Department of Paediatric Surgery, National Institute of Child Health, Karachi, Pakistan, from June to December 2022. All patients with biopsy-proven Hirschsprung disease were included. Ultrasound was done to find out the anatomical and structural anomalies of the kidney and urinary tract. Functional assessment was done by renal function tests and MAG3 radioisotope scan where indicated. Fisher's exact test was applied to find out the association. A p ≤0.05 was taken as significant. Out of a total of 83, 15 (18.0%) patients had CAKUT. The mean age of the study population was 5.5 ± 2.3 years. It included 61 (73.5%) males and 22 (26.5%) females. No significant association was found between the type of Hirschsprung disease and CAKUT (p = 0.7). The gender distribution between CAKUT patients was also insignificant (p = 0.7). Renal hypoplasia was the most common anomaly found in six patients followed by hydronephrosis due to pelvi-ureteric junction obstruction in four children. All of these children were asymptomatic. Five male patients had undescended testis. Nearly a fifth of the children with Hirschsprung disease had CAKUT of whom renal dysplasia was the most common anomaly. There was no gender predilection and patients were asymptomatic regarding the urinary system. A routine ultrasound abdomen is a good screening investigation for identifying CAKUT. Hirschsprung disease, Kidney and urinary tract, Congenital diseases of the urinary tract, Congenital anomalies.

Intraperitoneal laparoscopic technique in trendelenburg position: an effective surgical method for pyelolithotomy, pyeloplasty, and heminephrectomy in patients with horseshoe kidneys

PurposeTo evaluate the safety and feasibility of intraperitoneal laparoscopic surgery in Trendelenburg position (ILSTP) for pyelolithotomy, pyeloplasty, and heminephrectomy in patients with horseshoe kidneys (HSKs).MethodsBetween March 2021 and March 2024, three patients with HSKs underwent ILSSP. Of these three patients, two with pelvi-ureteric junction obstruction with recurrent kidney stones underwent pyelolithotomy and pyeloplasty, one with symptomatic nonfunctioning left moiety of a HSK was managed with heminephrectomy.ResultsMean operating time was 114 ± 64.8 (44–172) min, and estimated blood loss was 63.3 ± 51.3 (20–120) ml. The mean hospital stay was 3.3 ± 1.5 (2–5) days. There were no major intra- or post-operative complications.ConclusionsILSTP is a feasible and effective technique for performing pyelolithotomy, pyeloplasty, and heminephrectomy in patients with HSKs.

Factors Affecting The Short Term Outcome Of Pyeloplasty In Congenital Primary Unilateral Pelvi-Ureteric Junction Obstruction In Infants and Toddlers: A Single Center trial

Factors Affecting The Short Term Outcome Of Pyeloplasty In Congenital Primary Unilateral Pelvi-Ureteric Junction Obstruction In Infants and Toddlers: A Single Center trial

Postpercutaneous nephrolithotomy pseudoaneurysm embolization – Immediate endovascular coil migration into the pelvicalyceal system causing obstruction

ABSTRACT Bleeding is the most dreaded complication after percutaneous nephrolithotomy (PCNL). We report a case of post PCNL hematuria managed with selective angioembolization of the pseudoaneurysm. During the embolization, the emolization coil along with the glue migrated into the pelvicalyceal system from the pseudoaneurysmal cavity. The pseudoaneurysm was again embolized using cyanoacrylate glue and a screening computed tomography was immediately performed which showed the migrated coil along with the glue into the pelvicalyceal system resulting in pelviureteric junction obstruction. The coil was retrieved endoscopically, after the patient was stabilized. To our knowledge, this is the first reported case of an immediate migration of the coil and the urologists should be aware of it.

Pelvi-ureteric junction obstruction in a case of horseshoe kidneys with lower pole crossing vessels:a rare case and its management

Pelvi-ureteric junction obstruction is one of the most common urological conditions found in children. Horseshoe kidneys are a predisposing factor and to find a vascular anomaly causing the obstruction is rare. A 2-year-old girl with two episodes of culture-positive febrile urinary tract infections (UTI), was found to have right sided hydronephrosis. Renal scan revealed decreased functioning of right kidney with scars. A contrast CT scan showed a horseshoe kidney with pelvi-ureteric junction obstruction on right side with dilatation of renal pelvis. During laparoscopic pyeloplasty, lower pole crossing vessels were detected, not reported on CT scan. A dismembered pyeloplasty with transposition of uretero-pelvic anastomosis anterior to lower pole vessels was done. Anomalous vasculature of kidney should be kept in mind during surgery in such cases. A dismembered pyeloplasty along with transposition of anastomosis is necessary to prevent recurrence.

Comparison of outcomes of open, laparoscopic and robot-assisted laparoscopic pyeloplasty in children with ureteropelvic junction obstruction: protocol for a systematic review and meta-analysis

IntroductionThe treatment of children with pelviureteric junction obstruction (PUJO) has naturally progressed from open, to minimally invasive approaches, including laparoscopic pyeloplasty and robot-assisted laparoscopic pyeloplasty (RALP). The RALP is now...

Assessment of ureteric jets as a supportive diagnostic modality for unilateral pelvi-ureteric junction obstruction and its utility in follow-up: A pilot study

Pelvi-Ureteric Junction Obstruction (PUJO) is a common cause of hydronephrosis (HDN) in children. While ultrasonography (USG) is useful for initial assessment and grading of hydronephrosis, it cannot differentiate obstructive from non-obstructive cases. Renal Dynamic Scintigraphy (RDS) confirms the diagnosis but involves ionizing radiation exposure. Ureteric jets using colour Doppler USG have been proposed for diagnosing obstructive HDN. Our study aimed to evaluate Ureteric Jet Frequency (UJF) and Relative Jet Frequency (RJF) in unilateral PUJO before and after furosemide (Lasix) administration, assessing their diagnostic and post-operative utility. Children (<14 years) with unilateral HDN underwent USG and RDS for PUJO diagnosis. Pyeloplasty was performed based on standard criteria. UJF and RJF were assessed before and after furosemide administration (0.5mg/kg) by colour Doppler USG. The non-obstructed side was taken as the control. Follow-up included repeat RDS and ureteric jet assessment. Fifty-two cases were included. UJF (pre- and post-Lasix) was significantly lower in the obstructed side compared to the non-obstructed side at baseline and post-pyeloplasty (p<0.0001). However, the baseline UJF difference between cases and controls was not significant (p>0.05). UJF and RJF (pre- and post-Lasix) increased postoperatively. The UJF difference decreased postoperatively (p<0.05). (attached Table) CONCLUSION: UJF and RJF are useful for diagnosing and monitoring unilateral PUJO. The effect of furosemide on UJF needs to be assessed using additional studies with larger sample sizes to understand if it can affect UJF in a way similar to that noted in diuretic scintigraphy.

Urinary Tract Aspergillosis in a Child with Pelviureteric Junction Obstruction

Abstract Aspergillus spp. are fungi which are mostly nonpathogenic, but some species such as Aspergillus fumigatus, Aspergillus flavus, and Aspergillus niger can cause aspergillosis in humans. Aspergillosis can manifest as pulmonary or extrapulmonary infections, with respiratory involvement being common but infections in other body parts being rare. Urinary aspergillosis, an uncommon manifestation of Aspergillus infection, presents unique challenges in diagnosis and management due to its rarity and varied clinical presentations. It is seen in immunocompromised individuals, patients with underlying structural urinary tract abnormalities, or those undergoing invasive procedures. Here, we present a case of an 8-month-old child who presented with intermittent fever and increased urination since birth. The child underwent bilateral open pyeloplasty with double-J (DJ) stenting. A month later, the child returned with anuria, fever, and abdominal mass, leading to infected DJ stent removal and bilateral percutaneous nephrostomy. Despite antibiotic treatment, fever persisted. A fungal culture was done which showed growth of A. flavus. Liposomal amphotericin B was started for the patient after which he became afebrile, and all the fungal cultures done, thereafter, were negative. Urinary tract aspergillosis is rare, particularly among individuals with a strong immune system. Persistence of urinary tract symptoms despite antibiotic and medical therapy may raise the possibility of fungal infection such as urinary tract aspergillosis.

Analysis of outcomes of robot-assisted laparoscopic pyeloplasty in children from a tertiary pediatric center in South India.

This study aims to analyze the outcomes of robot-assisted laparoscopic pyeloplasty (RALP) in children with pelvi-ureteric junction obstruction (PUJO) over a 10-year period at a tertiary care center in South India. This study provides a detailed analysis of prospectively acquired data from 2013 to 2023 of all children who underwent RALP at our institution. Pre- and post-operative renal ultrasound and isotope renography were used to assess outcomes. Detailed information on patient demographics, procedural duration, post-operative pain relief, operation steps, and post-surgical follow-up protocols has been provided. The analysis included all patients who completed a 1-year follow-up. Between 2013 and 2023, 201 children underwent RALP. Of these, 185 children completed at least 1 year of follow-up and were included in the analysis. The mean age of the cohort was 4.9 years (1 month to 17 years), with males comprising the majority (77.3%). Twenty-five children (13.5%) were younger than 1 year of age. Left-sided PUJO was found to be more common. The mean console time was 76.5 min (40-180 min), and the average hospital stay was 2.8 days (2-5). After surgery, the mean reduction in antero-posterior diameter of the renal pelvis was more than 50% of its pre-operative value and statistically significant (3.3 ± 0.3 to 1.9 ± 0.9 cm). At the end of 1 year, the overall reduction in renal size was also significant (9.7 ± 2.3 cm pre-operative to 8.9 ± 1.8 cm post-operative). The pre-operative Society of Fetal Urology (SFU) grade of hydronephrosis was compared to the post-operative SFU grade, and the improvement (resolution/downgrading) was found to be statistically significant. The median split renal function in this series was 39% pre-operative and 43% post-operative, and the overall functional improvement after RALP was significant. A successful outcome was observed in 181 children (97.8%). Four children experienced persistent severe hydronephrosis and underwent redo stenting and/or redo pyeloplasty (2.1% failure rate). Post-operative complications, according to the Clavien-Dindo classification, were classified as type 1 in three children and type 3b in two children. There were no conversions to open surgery in the series. RALP emerges as the minimally invasive procedure of choice for children with PUJO at our institution. It is safe, delivering consistently excellent results and minimal complications. Our outcomes are comparable to those of previously published series. We trust that our experience will serve as a roadmap for those centers (especially in South Asia) embarking on a pediatric robotic program.

Protean Drainage Patterns of the Left Renal Vein: A Cadaveric and Retrospective Clinical Study on the Surgical Implications and Technical Feasibility.

The diverse drainage patterns of the left renal vein (LRV), often with asymptomatic congenital anomalies, present considerable challenges in renal and retroperitoneal surgical contexts. The potential for significant bleeding and subsequent renal compromise upon vascular injury highlights the need for increased surgical awareness. This study investigates the LRV's variable anatomical drainage patterns and morphometry. It also evaluates the embryological factors contributing to these variations and discusses their surgical implications and technical considerations. Anatomical dissections were conducted on 21 adult human cadavers within the Department of Anatomy. Concurrently, a retrospective analysis was conducted on 15 patients who underwent various retroperitoneal surgical interventions in the Urology Department. Demographic variables and intraoperative findings were recorded and analyzed. Dissection analysis predominantly identified preaortic LRVs in 18 cadavers. Notable anatomical variations included a circumaortic left renal vein (CLRV), a delayed preaortic confluence of extrahilar duo LRVs, and an extrahilar tetramerous confluence with a retroiliac topography. The majority of LRVs usually end in the inferior vena cava. However, an extrahilar tetramerous variant had an unusual drainage pathway. Out of 15 cases, three (20%) had a retroaortic left renal vein(RLRV). One patient with a nonfunctioning kidney had type 1 RLRV, and another patient with pelvic ureteric junction obstruction had type 4 retroiliac left renal vein (RILRV). In both of these patients, symptoms were relieved after surgery. In a young patient with left varicocele and microscopic hematuria who had type 2 RLRV, symptoms resolved spontaneously after a few months. A thorough understanding of the variable anatomical drainage patterns of the LRV is crucial for surgeons. Accurate preoperative identification can provide valuable insights, potentially leading to improved surgical outcomes in renal procedures.

Dorsal Lumbotomy Pyeloplasty Via Transverse Incision, A Ten Years’ Experience

The Pelviureteric- junction obstruction (PUJO) is the commonest cause of hydronephrosis in children. Dismembered pyeloplasty is the gold standard procedure for PUJO. Dorsal transverse lumbotomy approach is our preferred approach to get access to Pelviureteric junction. This is a retrospective study of last 10 years of one Hundred and twenty three cases. All the patients were operated via dorsal lumbotomy approach. Pre-operative Investigations hemogram, Ultra sonography and a preoperative diuretic renal scintigraphy with diethyl tri-amino penta acetic acid (DTPA) / Ethylene dicysteine (EC.) and Dimercaptosuccinic acid (DMSA) was performed in every patient to assess the differential function and changes in renogram curve. Indication for pyeloplasty was PUJO with obstructed renogram with non-achievement or delayed excretion of Tracer (T ½). Patient were operated under General anaesthesia in prone position and standard Anderson-Hynes Dismembered pyeloplasty via transverse lumbotomy incision. A Total of 123 patients were operated with the median age of patients was 30 months; the average antero- posterior diameter of the renal pelvis was 24 mm. The Average time for surgery was 99 minutes, The Average days of admission were 4. Six patients had postoperative leak which were managed conservatively. Nine patients required redo surgery.

Differential renal length index: useful measure in management of isolated unilateral hydronephrosis?

To explore the usefulness of the 'differential renal length index' (iDRL) before and after pyeloplasty, as the anteroposterior diameter is commonly used to quantify hydronephrosis but inaccuracies arise due to interobserver variability, hydration status and pure intra-renal dilatation. Prospectively collected data, from two centres, of all children undergoing pyeloplasty for isolated unilateral pelvi-ureteric junction obstruction (PUJO) (2015-2021) were analysed. Subgroup analysis was undertaken: Group A - differential renal function (DRF) ≥40%, Group B - subnormal DRF (20-39%), and Group C - symptomatic. Children with structural anomalies of upper and lower urinary tract, bilateral involvement, and subnormal DRF (<20%) were excluded. All the children had a pre- and postoperative ultrasound scan and Tc99m mercapto-acetyltriglycine (MAG3) renograms. The iDRL was calculated as follows: iDRL = ([a - b]/b) × 100, where 'a' is the length of hydronephrotic kidney (cm) and 'b' is the length of contralateral normal kidney (cm). The mean difference and standard error of mean (SEM) between the pre- and postoperative iDRL was evaluated using the paired Student's t-test, with P < 0.05 considered statistically significant. A total of 119 children with 1-year follow-up were included. For the entire cohort, the mean (SEM) preoperative iDRL was 27.7 (1.4) and postoperatively was 12.5 (1.1), with a mean (range) DRF improvement of 54% (44-66%) (P < 0.001). In Group A (n = 97), the mean (SEM) preoperative iDRL was 26.6 (1.5) and postoperatively was 13.1 (1.2), with a mean (range) DRF improvement of 50% (38-63%) (P < 0.001). In Group B (n = 22), the mean (SEM) preoperative iDRL was 32.6 (3.5) and postoperatively was 10.0 (2.8), with a mean (range) DRF improvement of 69% (49-89%) (P < 0.001). In Group C (n = 28), the mean (SEM) preoperative iDRL was 19.9 (2.3) and postoperatively was 7.7 (1.9), with a mean (range) DRF improvement of 61% (38-85%) (P < 0.001). Our study identifies the iDRL as a useful measure of improvement following successful pyeloplasty. In the subgroup with DRF of >39% minimum improvement was >37%. Similar minimum DRF improvement was also noted (>37%) in hypo-functioning kidneys and symptomatic PUJO.

Unraveling Patterns of Congenital Structural Malformations in Infants: A Hospital-Based Descriptive Study.

Introduction Congenital malformation studies serve several purposes, including establishing baseline rates, monitoring changes over time, exploring the origins of these defects, and helping in planning health services. Increasing public awareness about pediatric surgical interventions is another goal of these studies. However, the impact of congenital malformations is often underestimated in developing countries due to insufficient healthcare data and diagnostic facilities, particularly in rural areas. Families affected by the birth of a child with congenital malformations face significant stress and hardship. Methods The main aims of this study were to evaluate the clinical pattern of congenital structural malformations in our region (Uttarakhand, India), identify possibly associated factors of congenital malformations, and find out the immediate outcome of congenital malformations in enrolled participants. Results Among a total of 150 cases, 73 (48.7%) cases were inborn, whereas 77 (51.3%) cases were outborn. Investigation of congenital malformation revealed cleft lip or palate in 37 (24.7%) cases, congenital heart disease (CHD) in 33 (22%) cases, meningomyelocele (MMC) in 18 (12.0%) cases, anorectal malformation (ARM) in 11 (7.3%) cases, hypospadias in 10 (6.7%) cases, congenital talipes equinovarus (CTEV) in nine (6.0%) cases, tracheoesophageal fistula (TEF) in nine (6.0%) cases, polydactyly in seven (4.7%) cases, pelviureteric junction obstruction (PUJO) in four (2.7%) cases, duodenal atresia in three (2.0%) cases, midgut volvulus in three (2.0%) cases, umbilical sinus in two (1.3%) cases, sacrococcygeal teratoma (SCT) in one (0.7%) case, phimosis in one (0.7%) case, microtia in one (0.7%) case, and micrognathia in one (0.7%) case. Mortality was observed in 11 (7.3%) cases, whereas 105 (70%) cases were successfully discharged. Among 11 mortality cases, the cause of death was CHD in seven (63.2%) cases, TEF+CHD in two (18.1%) cases, MMC in one (9%) case, and duodenal atresia in one (9%) case. Conclusion Contrary to the common belief that advanced maternal age of greater than 35 years is a major cause, 86.6% of the congenital structural anomalies in our hospital-based study in Uttarakhand occurred in babies of mothers belonging to the age group of 18-30 years. Also, consanguineous marriage was observed in only 3.3% of cases, indicating that it may not be a major contributing factor causing congenital structural malformations in our region.External congenital anomalies are most commonly observed (60.7%), with cleft lip and cleft palate being the most common.The most frequently observed internal congenital anomaly is CHD (22%) followed by gastrointestinal (GI) (18.6%) and urinary anomalies (10.1%).Death and referral are commonly seen in CHD.

Histological and Immunohistological Findings in Vesicoureteric and Pelviureteric Junction Obstructions; a Spectrum of the Same Mishap – An Observational Study

Background: Vesicoureteric and pelviureteric junction obstructions (VUJOs and PUJOs) are the most common causes of congenital obstructions of the urinary tract, and yet the pathophysiology of these conditions is unclear. This study aimed to compare the histological findings of age-matched VUJO and PUJO cases for wall thickness, fibrosis, and the presence of interstitial cells of Cajal (ICC). Methodology: This was an observational, inter-institutional study done in the period between January 2020 and May 2021. Twelve age-matched cases of VUJO and PUJO were studied to compare histological findings when stained with hematoxylin and eosin, Masson trichrome, and immunohistochemical stains. Results: Results showed that the thickness of the muscle layer was increased in both VUJO and PUJO with no statistically significant difference. Perifascicular fibrosis was significantly more in VUJO as compared to PUJO. ICCs were less than grade 3 in most cases of VUJO and PUJO. Conclusions: The histological findings of VUJO and PUJO showed similarities, with more fibrosis in VUJO. The density of ICCs was less in both upper and lower urinary obstructions. As both conditions are the result of the same mishap – failure of maturation of fetal ureters, there may be a possibility that patients of PUJO can also have VUJO.

Contemporary status of diagnostic endoluminal ultrasound and optical coherence tomography in the ureter

AbstractObjectiveTo evaluate via a review of published literature, the efficacy of endoluminal ultrasound (ELUS) and optical coherence tomography (OCT) in the following ureteric diseases: urolithiasis, upper tract urothelial carcinoma, stricture disease and pelvic‐ureteric junction obstruction (PUJO).Patients and methodsUreteric high‐frequency ELUS provides 360° imaging, to a depth of 20 mm, and has been demonstrated to assess ureteric stricture length, degree of fibrosis and aetiology. OCT produces high‐quality images with a penetration depth of 2 mm. ELUS has proven to be useful at the time of endopyelotomy for PUJO as it can identify crossing vessels, some not detectable on CT angiography, allowing the urologist to avoid these when making their incision. Ureteric ELUS may be utilised for submucosal ureteric stones as they are highly visible. Endoluminal ultrasound may be deployed in the case of known sub‐mucosal urolithiasis when the ureter appears stone‐free. It may help identify sub‐mucosal stones or stones within diverticulum.ResultsEndoluminal ultrasound has been analysed for its use in determining muscle‐invasive urothelial carcinoma of the ureter. The PPV for ≥pT2 was only 16.7% in one study of six patients with MIBC and 76.2% in 21 patients with &lt;pT2 disease. Analysis of OCT for staging UTUC during ureteroscopy and biopsy demonstrated sensitivity for tumour invasion of 100% and specificity of 92%, 83% of lesion staging matched with histological analysis. Imaging analysis did not match histology in three patients with large exophytic tumours that exceeded the OCT depth penetration. Due to its superficial penetration, OCT cannot reliably stage large tumours.ConclusionsUreteric ELUS has been reported to be a useful tool in endopyelotomy, urolithiasis and stricture disease. The staging of ureteric urothelial carcinoma remains unsatisfactory with current imaging techniques and biopsy methods, and, based on the current literature, ELUS does not appear to have a strong enough PPV to determine muscle invasion. Ureteric OCT may be a useful tool in the future staging of upper tract urothelial carcinoma, particularly in differentiating the stage of small tumours. Further studies are needed in this area.