Extravesical vs. intravesical ureteric reimplantation for primary vesicoureteral reflux: A systematic review and meta-analysis.

Purpose: This study aims to compare the outcomes of extravesical (EVUR) and intravesical (IVUR) ureteric reimplantation for primary vesicoureteral reflux (VUR) via systematic review and meta-analysis. Methods: Literature review from Medline, Embase, and Cochrane since inception to March 2022 was performed. Meta-analysis was conducted on eligible randomized controlled trials (RCT) and observational cohort studies (OCS) comparing outcomes between EVUR and IVUR. Results: Twelve studies were included, comprising 577 patients (778 ureters) operated by EVUR and 395 patients (635 ureters) by IVUR. Pre-operative VUR grade, postoperative VUR persistence and hydronephrosis was not statistically significant. EVUR had shorter operative time [mean differences (MD) -22.91 min; 95% confidence interval (CI), -44.53 to -1.30, P = 0.04] and hospital stay (MD -2.09 days; 95% CI, -2.82 to -1.36, P < 0.00001) compared to IVUR. Bilateral EVUR had higher risk of postoperative acute urinary retention (ARU) (8.1%) compared to bilateral IVUR (1.7%) (OR = 4.40; 95% CI, 1.33-14.58, P = 0.02). No patient undergoing unilateral EVUR or IVUR experienced ARU. Conclusion: Both EVUR and IVUR are equally effective in correcting primary VUR. Operative time and hospital stay are shorter after EVUR compared to IVUR. However, bilateral EVUR is associated with higher risk of postoperative ARU.

Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis): Early Diagnosis and Management of Asymptomatic Herlyn-Werner-Wunderlich Syndrome.

INTRODUCTION: Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare variant of Mullerian ductal anomaly associated with ipsilateral renal agenesis. Most patients are diagnosed after menarche with complications of uterovaginal obstruction, genitourinary infection and pelvic adhesions. Patients often undergo additional operations for misdiagnosis or treatment of complications. Our institution manages several HWWS patients diagnosed before symptoms by screening for antenatally-diagnosed renal agenesis. This study aims to improve the presymptomatic management of HWWS patients. METHODS: We carried out retrospective case review of patients diagnosed with HWWS from 2010 to 2017 on patient demographics, symptoms, clinical course and operative management and summarize the sparse literature published to date. RESULTS: There were 8 patients with HWWS but only 2 symptomatic patients presented acutely with hematocolpos requiring urgent vaginal surgery. The other six patients had early diagnosis through postnatal ultrasound screening. No patient required further operation for diagnosis or complications related to obstructed hemivagina. CONCLUSION: Our case series and literature review show that the majority of prepubertal patients with HWWS do not require early gynecological surgery. We recommend that female babies with renal agenesis should be screened for HWWS syndrome with ultrasound. Early diagnosis and presymptomatic elective surgery may prevent urogynecological complications that cause fertility and renal impairment. STUDY DESIGN: Case series, level IV evidence.

Posterior retroperitoneoscopic adrenalectomy for pediatric adrenal tumors.

BACKGROUND/AIMS: Posterior retroperitoneoscoic adrenalectomy has been reported as an option for adrenal tumor resection but is not commonly performed in children owing to the extreme semikneeling position advocated to flatten the lumbar lordosis in order to achieve adequate retroperitoneal space. As children have smaller lordosis angles, flattening of the lordosis and creation of optimal retroperitoneal space may be achieved with less hip flexion. We used pediatric lumbar lordosis measurements to develop a modified prone jackknife position and report our experiences with this setup for posterior retroperitoneoscopic adrenalectomy for adrenal tumors. METHODS: Lordosis angles were measured on sagittal computed tomography (CT) and magnetic resonance imaging (MRI) studies of patients with adrenal tumors and compared to normal references. The data were used to develop our modified prone jackknife position. Selected patients with adrenal tumors underwent posterior retroperitoneoscopic adrenalectomy in this position. Patient demographics, diagnoses, operative times, complications, postop analgesia requirements, and length of hospitalization were analyzed. RESULTS: CT and MRI studies were analyzed for 20 patients with adrenal tumors diagnosed in our institution from 2012 to 2017; median lordosis angle was 27.84° (range: 15.50°-36.48°) - less than reference lordosis angles of respective age groups, and flexion angles of common operating tables. Five patients underwent retroperitoneoscopic adrenalectomy between June 2016 and June 2018. Histological diagnoses were neuroblastoma, adrenal hyperplasia, pheochromocytoma, and adrenal angiomatoid fibrous histiocytoma. Median age was 4 years [range: 1-11]. Median operating time was 137 min [range 111-181 min]. No conversions to open surgery were required. One patient had intraoperative bleeding from the adrenal vein. Only 1 patient required postoperative opioids for analgesia. Median length of hospitalization after surgery was 2 days (range: 2-3 days). CONCLUSIONS: Pediatric patients can achieve flattening of lumbar lordosis with less extreme positioning. Posterior retroperitoneoscopic adrenalectomy in a modified prone jackknife position is a feasible operation for pediatric patients with small adrenal masses. TYPE OF STUDY: Clinical research paper. LEVEL OF EVIDENCE: Level III.

Anorectal malformation & Hirschsprung's disease: A cross-sectional comparison of quality of life and bowel function to healthy controls.

BACKGROUND: Patients with anorectal malformation (ARM) and Hirschsprung's disease (HD) face long-term disturbance in bowel function even after definitive surgery. This study evaluates the quality of life (QOL) of patients with ARM and HD, and compares them to healthy controls using self-report questionnaires. METHODOLOGY: A prospective study was performed recruiting patients with ARM or HD from September 2013 to December 2014 who had primary surgery done in our institution at least 2 years prior to participation. Age-matched and gender-matched controls were enrolled from our patients with minor outpatient complaints. All participants completed the following PedsQL™ scales (maximum score 100): 4.0 Generic Core Scales, 3.0 General Well-Being (GWB) Scale and 2.0 Family Impact (FI) Module. All were also scored on bowel function (BFS), with a maximum score 20. Appropriate statistical analysis was performed, with significance level <0.05. RESULTS: There were 193 participants: 87 controls, 62 ARM, 44 HD. When comparing Core, GWB and FI scores, there were no significant differences between groups although controls had best scores indicating best QOL and general wellbeing, with least impact of the child's health on the family. BFS was significantly different with controls having best and ARM worst scores. There were no significant differences in scores between parent and child indicating intradyad consistency. There was significant positive correlation between BFS and Core (p<0.0001), and between BFS and GWB scores (p<0.005); and significant negative correlation between BFS and FI scores (p<0.0001). CONCLUSIONS: Bowel function impacts quality of life. Those with ARM and HD can achieve good quality of life comparable to controls, based on patient and caregiver self-reported outcomes. TYPE OF STUDY: Prospective comparative study LEVEL OF EVIDENCE: Level II.

Understanding Experiences of Youth Growing Up with Anorectal Malformation or Hirschsprung's Disease to Inform Transition Care: A Qualitative In-Depth Interview Study.

INTRODUCTION: The impact of anorectal malformation (ARM) or Hirschsprung's disease (HD) in children continues into adulthood despite early surgical correction. We aimed to explore the physical, social, and emotional impacts of these conditions on youth to inform best transition care strategies. MATERIALS AND METHODS: Eligible participants were those aged between 14 and 21 years who had undergone surgery for ARM/HD in our institution. We conducted one-on-one in-depth interviews to saturation using a question guide developed from literature review and clinician expertise. Deidentified transcripts were coded by four coders (two pediatric surgeons, one psychiatrist, and one qualitative expert) for major themes using a constant comparison approach. A theoretical model for understanding the transition experience was developed using grounded theory. RESULTS: Out of 120 patients identified as eligible, 11 youth (6 males) participated in the study. Interviews lasted from 50 to 60 minutes. Four major themes emerged: (1) social support (subthemes: family as core, friends as outer support), (2) cognitive and emotional change (subthemes: realization/recognition of illness, matching emotional response), (3) impact of physical symptoms (subthemes: adverse effects of abnormal bowel habits, gaining bowel continence control leading to overall feeling of control, need to keep disease private), and (4) healthcare providers (viewed as important information sources). Themes did not differ by gender. CONCLUSION: Our model suggests that participants' understanding of bowel disease evolved over time with a "lightbulb" moment in preteens or early teens accompanied by increasing disease ownership and self-management. Clinicians should independently engage with patients in late childhood to address evolving emotional and information needs and encourage increasing autonomy. Future studies should explore communication approaches to meet transition needs of patients.

Beliefs and values about intra-operative teaching and learning: a case study of surgical teachers and trainees.

Surgeons require advanced psychomotor skills, critical decision-making and teamwork skills. Much of surgical skills training involve progressive trainee participation in supervised operations where case variability, operating team interaction and environment affect learning, while surgical teachers face the key challenge of ensuring patient safety. Using a theoretical framework of situated learning including cognitive apprenticeship, we explored teachers' and trainees' beliefs and values about intra-operative training and reasons for any differences. A qualitative case study method was used where five teacher-trainee pairs participating in an observed teaching operation were separately interviewed about the same operation. Thematic analysis of transcribed interviews and observations was performed with iterative refinement and a reflexive approach was adopted throughout the study. We found that in all cases, teachers and trainees had shared recognition of learning about technical skills whereas they differed in three cases regarding non-technical skills such as surgical reasoning and team management. Factors contributing to teacher and trainee satisfaction with the process were successful trainee completion of operation without need for surgeon take-over, a positive learning environment and learning new things. Teaching-learning behaviours observed and discussed were modeling, coaching and scaffolding, while exploration, reflection and articulation were less common. Our study reveals differing teacher and trainee perspectives of some aspects of intra-operative training and surfaces new reasons other than amount of feedback and autonomy given. Factors contributing to different perspectives include teacher and trainee abilities, values and situational influences. Targeted teaching-learning strategies could enhance intra-operative learning.

Factors Predicting Testicular Atrophy after Testicular Salvage following Torsion.

INTRODUCTION: Testicular atrophy (TA) is a significant complication in patients who undergo salvage procedures for testicular torsion. Studies on outcome focus on factors predicting testicular viability during scrotal exploration but few assess factors predicting TA in patients who undergo salvage procedures. We assess the incidence of TA after salvage and identify associated factors. MATERIALS AND METHODS: With ethical approval, we reviewed patients who underwent salvage for testicular torsion in our institution from 2001 to 2013. Data was collected on patient demographics, duration of pain, sonographic findings, postoperative complications, and follow-up assessment of TA (defined as difference in testicular volume > 50% compared with the contralateral testis, based on measurement by Prader orchidometer or by ultrasound). We excluded patients with torted undescended testis, those under 1 month, and those with follow-up < 6 months. Chi-square or Mann-Whitney U tests were used as appropriate with significance level < 0.05. RESULTS: Of 85 patients who had scrotal exploration for testicular torsion, 53 had testicular salvage. Overall, 16 patients defaulted or had < 6 months follow-up, leaving 37 patients who were studied, median age 12 years (range, 0.5-16.0 years) at presentation. Median follow-up was 12.5 months (range, 6-88 months). A total of 20 patients (54%) developed TA. Median duration to TA was 12.5 months (range, 2-88 months). All had clinical evidence of atrophy by 14 months, except two who initially defaulted follow-up, but were diagnosed with TA at 35 and 88 months postoperatively when presenting with unrelated complaints. Factors associated with TA were duration of pain > 1 day (p = 0.004) and heterogeneous echogenicity on ultrasound (p = 0.001). Sonographic evidence of reduced vascularity was not predictive. Of 11 that had pain > 1 day, 10 (91%) had TA. No testes survived when pain ≥ 3 days. CONCLUSION: Half of patients with testicular torsion undergoing salvage surgery will develop testicular atrophy, even when intraoperatively assessed as viable, and should be counseled accordingly. Duration of pain > 1 day and sonographic heterogeneous echogenicity are predictive. Salvage rates are dismal when duration of symptoms exceeds 1 day.

Undescended testis: Level of knowledge among potential referring health-care providers.

AIM: Studies report that most boys with undescended testis(UDT) are referred and operated beyond the recommended age of 1 year, possibly due to lack of awareness of treatment guidelines. We investigate the level of knowledge of UDT among potential referring health-care providers. METHOD: We devised a survey on the clinical features and appropriate management of UDT. Using convenience sampling, we approached health-care professionals with regular contact with paediatric patients and final year medical students. Respondents were allowed to remain anonymous. They were categorised according to specialty and level of experience/training. RESULTS: Of 1179 approached, 203 responded. Thirty-six (24%) of 149 qualified doctors had never seen a case of UDT. Median score was 6 (range 1-9). There was no significant difference in scores when comparing specialty. Mean scores decreased significantly in trend according to level of experience. When questioned regarding timings of referral and orchidopexy, 24% of qualified doctors would not refer until 9 months of age, and 66% thought orchidopexy should be done after 1 year old. Half would stop examining for UDT after 2 years old. CONCLUSIONS: Inexperience with UDT and outdated knowledge may contribute to delays in referral for UDT. Many would stop examining for UDT at 2 years old, placing undue reliance on accurate physical examination in early childhood and indicating lack of awareness of the ascending testis. Community health initiatives must emphasise recent changes in guidelines for management of UDT.

Pseudomembranous trigonitis in a male with Klinefelter syndrome: a case report and evidence of a hormonal etiology.

Klinefelter syndrome is a clinical syndrome with a distinct 47, XXY karyotype. Patients are characterized by a tall eunuchoid stature, small testes, hypergonotrophic hypogonadism, gynecomastia, learning difficulties and infertility. These patients have also been found to have raised estrogen levels. We report a 16 year old boy with Klinefelter syndrome presenting to our institution with gross hematuria. Cystoscopy and biopsy revealed the diagnosis of pseudomembranous trigonitis. Immunohistochemical stains showed an increase in estrogen and progesterone receptors in the trigone area but not in the rest of the bladder. In view of the patient's mildly raised estrogen levels and the histological findings, we postulate that estrogen is the driver of the development of pseudomembranous trigonitis. This is the first reported case of pseudomembranous trigonitis seen in association with Klinefelter syndrome, and also the first case of pseudomembranous trigonitis occurring within the male adolescent age group.

Undescended testis: 513 patients' characteristics, age at orchidopexy and patterns of referral.

OBJECTIVE: Undescended testis (UDT) affects 1-6% of males. Current recommendations are to correct maldescent by 1 year of age. We identify the population characteristics of children referred and managed for UDT, age at referral and orchidopexy, and patterns of referral. DESIGN, SETTING AND PATIENTS: Retrospective 5-year review of all patients operated for UDT from 2007 to 2011 in our institution. Patient demographics, neonatal diagnosis of UDT, age at referral, referral source and age at first orchidopexy were recorded. Data are reported as median (range). RESULTS: There were 513 boys with 576 undescended gonads; 450 (88%) had unilateral UDT. Congenital (present at birth) UDT was diagnosed in 287 (56%) children. Seventy-nine (15%) were premature births, 41 (8%) had associated major genitourinary abnormalities. Median age at referral was 1.1 (0-16.2) years; median age at first orchidopexy was 1.6 (0-17.2) years. When corrected for age, those with a history of prematurity and associated major genitourinary malformations were referred and operated on earlier. There was no difference in age at referral and orchidopexy when comparing unilateral versus bilateral maldescent, and palpability of UDT. Of those with congenital UDT, 70% were operated at beyond 1 year of age. Those referred from public tertiary hospitals were younger than those referred from community clinics (p<0.0001) and private healthcare institutions (p=0.003). CONCLUSIONS: Despite early diagnosis in many patients with UDT, most are referred and operated after 1 year of age, even in congenital UDT. Premature babies, those with major genitourinary anomalies, and those seen in public tertiary hospitals are referred earlier. Community health initiatives must emphasise prompt referral to allay the impact of delayed surgery.

Transanal endorectal pull-through versus transabdominal approach for Hirschsprung's disease: a systematic review and meta-analysis.

AIM: Transanal endorectal pull-through (TERPT) has become popular for single-stage treatment of Hirschsprung's disease. The benefits of TERPT over the conventional transabdominal approach (TAB) are still unclear. We performed a comprehensive meta-analysis comparing the clinical outcomes of TERPT and TAB. METHODS: Original articles published from 1998 to 2012 were searched from Medline, Embase, and Cochrane databases. Randomized controlled trials (RCT) and observational clinical studies (OCS) comparing TERPT and TAB were included. Outcomes evaluated included operative time, hospital stay and incidence of postoperative incontinence/soiling, constipation and enterocolitis. Pooled odds ratios (OR) were calculated for dichotomous variables; pooled mean differences (MD) were measured for continuous variables. RESULTS: Of 93 studies, 1 RCT and 11 OCS were included, comprising 444 cases of TERPT and 348 cases of TAB (215 Soave, 94 Duhamel, 24 Swenson, 15 Rehbein procedures). TERPT had shorter operative time (MD=-57.85 min; 95% confidence interval [CI], -83.11 to -32.60; P<0.00001) and hospital stay (MD=-7.06 days; 95% CI, -10.95 to -3.16; P=0.0004). TERPT had less postoperative incontinence/soiling (OR=0.58; 95% CI 0.37-0.90; P=0.01) and constipation (OR=0.49; 95% CI 0.30-0.81; P=0.005). There was no difference in incidence of postoperative enterocolitis. CONCLUSION: TERPT is superior to TAB in operative time, hospital stay, postoperative incontinence and constipation. However, more randomized controlled trials are necessary to verify the benefit of TERPT for Hirschsprung's disease.

Anomalies associated with anorectal malformations according to the Krickenbeck anatomic classification.

PURPOSE: We evaluated the incidence of congenital anomalies associated with anorectal malformations (ARMs) in relation to the anatomic type of ARM as defined by the Krickenbeck classification. METHODS: We reviewed 99 children with ARM in our institution from 2002 to 2011. Data were collected on patient demographics, type of ARM, and associated congenital anomalies, which were categorized according to organ systems. Statistical analysis was performed for comparison between groups using 'perineal fistula' as the base group. RESULTS: There were 62 (63%) male patients. The majority had perineal fistulas (35, 35%). Seventy-seven (78%) had at least one associated malformation. The most frequent malformations seen were genitourinary (28, 28%) and spinal anomalies (26, 26%). Those with rectovesical fistula had the highest proportion of genitourinary malformations (Odds Ratio [OR], 41.3; 95% confidence interval [CI], 4.7-363.4). Those with cloaca (OR, 49.5; 95% CI, 3.4-718.9) and those with rectovestibular fistula (OR, 12.4; 95% CI, 2.3-65.6) were most likely to have major spinal abnormalities, with tethered cord seen in all groups. The rectovestibular group was also most likely to have other associated malformations (OR, 8.6; 95% CI, 2.2-32.8). CONCLUSION: More than 75% of children with anorectal malformation have other associated malformations. Genitourinary anomalies are the most common. Major spinal anomalies are seen in all groups, affecting nearly half of those with rectovestibular fistula and those without fistula. The incidence of associated malformations in the rectovestibular group is higher than described in the literature. Thorough systematic evaluation of all infants with ARM should be done regardless of type of ARM.

Primary hepatic artery embolization in pediatric blunt hepatic trauma.

Non-operative management of isolated blunt hepatic trauma is recommended except when hemodynamic instability requires immediate laparotomy. Hepatic artery angioembolization is increasingly used for hepatic injuries with ongoing bleeding as demonstrated by contrast extravasation on the CT scan. It is used primarily or after laparotomy to control ongoing hemorrhage. Hepatic angioembolization as part of multimodality management of hepatic trauma is reported mainly in adults, with few pediatric case reports. We describe our institution experience with primary pediatric hepatic angioembolization and review the literature with regard to indications and complications. Two cases (3 and 8 years old), with high-grade blunt hepatic injuries with contrast extravasation on the CT scan were successfully managed by emergency primary hepatic angioembolization with minimal morbidity and avoided laparotomy. To date, the only reports of pediatric hepatic angioembolization for trauma are 5 cases for acute bleeding and 15 delayed cases for pseudoaneurysm. The role of hepatic angioembolization in the presence of an arterial blush on CT in adults is accepted, but contested in a pediatric series, despite higher transfusion rate and mortality rate. We propose that hepatic angioembolization should be considered adjunct treatment, in lieu of, or in addition to emergency laparotomy for hemostasis in pediatric blunt hepatic injury.

The learning curve in learning the curve: a review of Nuss procedure in teenagers.

BACKGROUND: The Nuss procedure is a new minimally invasive repair for pectus excavatum that was first published in 1998. Modifications in technique are constantly evolving to minimize complications, especially bar displacement, which are higher in adolescents and adults. The present study reviews our early experience with this procedure in a much older group of children than previously reported and suggests an alternative method of avoiding bar displacement. METHODS: Retrospective chart review was carried out on 78 consecutive patients who underwent the Nuss procedure between December 1999 and January 2004. All patients underwent a uniform technique using bilateral lateral stabilisers and thoracoscopy was not used. Operative details, subjective cosmetic results and complication rates were assessed. RESULTS: The mean age was 15.4 +/- 3.2 years. Single bars were used in 95%, double bars in 5%. The defect was asymmetrical in 26%. The defect was mild in 7%, moderate in 59% and severe in 34%. The median length of stay was 6.0 days (range 4-11). Total operating time was 58 min (range 35-95). Patient controlled analgesia (morphine) for pain relief was used for 105 h (range 61-169) or 4.4 days; the epidural infusion was stopped 1 day earlier. Cosmetic results were excellent in 80.3%. There was residual asymmetry in 75% of the asymmetrical defects although all were improved. Complications included eight (10%) reoperations for bar displacement. This was related to the learning curve as seven of these occurred in the first 2 years of the series. Removal of the bar has been accomplished in 31 (40%) patients. This was elective in all but four patients (three early removals for pain and one for infection). CONCLUSIONS: The Nuss procedure gives good results even in teenagers. Modification of technique and increased experience has reduced complications. The use of bilateral lateral stabilisers without additional wire fixation is an alternative method to avoid bar displacement.