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Background: Continence problems occur often in children with anorectal malformations (ARM). The aim of this study was to evaluate parental experiences with toilet facilities at Dutch primary schools and their experience with how schools deal with ARM children. Methods: This survey was developed in collaboration with the national patient advocacy group (PAG). Recruitment for participation was conducted by the PAG (email listing and social media) and one expertise center for ARM. Participants were parents of school-attending ARM children aged 3 to 12 years. Results: Sixty-one participants (31.9%) responded to the survey. The median age of the children was 7.0 years (IQR 5.0–9.0). Schools were often located in a village (63.9%) and encompassed 100–500 children (77.0%). In total, 14 parents (23.0%) experienced difficulties in finding a primary school. Experiences with the school were described as solely positive (37.7%), solely negative (9.8%), positive and negative (34.4%), and neither positive nor negative (16.4%). Regarding school toilet facilities, 65.6% of the toilets were reported clean and 78.7% were easily accessible. Conclusions: About 25% of parents reported difficulties in enrolling their children into primary school, and 45% reported negative experiences. This highlights the need for improved guidance and the optimization of education in schools when dealing with ARM children.

Background: In children with anorectal malformations (ARM), additional anomalies can occur within the VACTERL-association. Routine screening is of great importance for early identification and potential treatment. However, uniformity in screening protocols is lacking and only small cohorts have been described in literature. The aim of this study was to assess and describe a unique large cohort of ARM patients who underwent VACTERL screening in the neonatal period. Methods: A retrospective mono-center cohort study was performed. Included were all neonates born between January 2000 and December 2020 who were diagnosed with ARM and screened for additional anomalies. Full screening consisted of x-ray and ultrasound of the spine, cardiac and renal ultrasound, and physical examination for limb deformities, esophageal atresia, and ARM. Criteria for VACTERL-classification were predefined according to the EUROCAT-definitions. Results: In total, 216 patients were included, of whom 167 (77.3%) underwent full VACTERL-screening (66% in 2000–2006 vs. 82% in 2007–2013 vs. 86% in 2014–2020). Median age at follow-up was 7.0 years (IQR 3.0–12.8). In 103/167 patients (61.7%), additional anomalies were identified. Some 35/216 patients (16.2%) fulfilled the criteria of a form of VACTERL-association. In 37/216 patients (17.1%), a genetic cause or syndrome was found. Conclusions: The majority of ARM patients underwent full screening to detect additional anomalies (77%), which improved over time to 86%. Yet, approximately a quarter of patients was not screened, with the potential of missing important additional anomalies that might have severe consequences in the future. Forms of VACTERL-association or genetic causes were found in 16% and 17% respectively. This study emphasizes the importance of routine screening. Level of evidence: III.

Background: Umbilical discharge is common in children and mostly attributed to infection or granuloma. However, an underlying congenital abnormality warranting surgery might also be present. Ultrasound is the imaging modality of choice to diagnose the presence of a congenital abnormality. The aim of this study is to investigate diagnostic accuracy of the ultrasound to detect pathology requiring surgical excision. Methods: All patients ≤ 18 years with umbilical discharge from January 2008 to September 2019 were retrospectively included. Diagnostic accuracy, i.e., sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (LR +) and negative likelihood ratio (LR −), were calculated. Results: Eighty-one patients were included and 56 were operated. The ultrasound was false positive in 10 patients and false negative in 13 patients. The sensitivity of ultrasound was 71.1% (95% CI 55.7–83.6), specificity 72.2% (54.8–85.8), PPV 76.2% (64.7–84.8), NPV 66.7% (54.8–76.8), LR + 2.6 (1.5–4.5) and LR − 0.40 (0.2–0.7). Conclusions: This study shows that the diagnostic accuracy of ultrasound for detecting underlying congenital abnormalities warranting surgery for umbilical discharge in the pediatric population is low, even with experienced pediatric radiologists. Therefore, the role of the ultrasound in the diagnostic workup and value in clinical decision making is limited. Type of study: Study of diagnostic test. Level of evidence: III

Summary Introduction and objective Syringocele is a rare cystic dilatation of the duct of Cowper's gland, afflicting mostly the pediatric population. Syringoceles have a wide range of symptoms and may cause urethral obstruction. The authors analyzed to clarify the clinical manifestation, diagnostic approach, management, and incidence in the pediatric population. Materials and methods All patients (122 cases) diagnosed with a syringocele at the department of Pediatric Urology in a tertiary referral university children's hospital between August 1991 and October 2016 were analyzed retrospectively by assessing medical charts. Results The clinical manifestation, diagnostic findings, and follow-up are summarized in the table. Half of the patients (50.0%) also had typical posterior urethral valves (PUVs) and/or a single valve in the 12 o'clock position (flap-valve). The symptoms of open and closed syringoceles showed no significant difference. Treatment consisted of incision of the syringocele with a diathermia hook. The incidence of urinary tract infection (UTI) before and after surgery in the group that had a syringocele only was significant different. The overall incidence of syringoceles seen at urethrocystoscopy in this series was 3.0%. Discussion This series suggests that the presenting age is strongly related to the consequences of syringoceles, as the youngest half of the patients had significantly more UTIs at presentation than older patients, who presented with significantly more obstructive voiding symptoms, postvoiding residuals, and incontinence. In addition, the younger group had a significantly higher incidence of vesicoureteral reflux and dilatation of the upper urinary tract. The found association between syringoceles and PUV may be due to overgrowth of epithelium, as possible origin in both anomalies. Conclusion With an incidence of 3.0%, syringoceles, in this tertiary referral series, should be considered in the differential diagnosis of obstructive urethral lesions. The presentation ranges between signs of severe obstructions in the prenatal and postnatal period to mild urinary incontinence problems at later age. Urethrocystoscopy proved to be useful in confirming the diagnosis and allows for immediate transurethral incision. Summary Table . Clinical manifestation Most frequent presenting symptoms Urinary tract infection (UTI) (50.0%), Obstructive voiding symptoms (45.9%) Postvoiding residual urine (13.9%) Median age at diagnosis 3.0 (IQR: 0.0–9.0) years Diagnostics (UCS) − 83 closed syringoceles and 31 open syringoceles. − 8 patients had two coexisting syringoceles. − 22 patients had a syringocele without additional urethral abnormalities. Follow-up Median follow up 24.0 (IQR: 7.00–52.50) months 42 (34.4%) patients were symptom-free directly after surgery. 23 (18.9%) patients needed a second incision of the rim of the opened syringocele.

Objective To gain insight into the efficacy and safety of urethral de-obstruction in boys with overactive bladder (OAB) complaints refractory to conservative treatment. Materials and methods All boys, older than 5 years, referred in 2009 for OAB complaints were included, n = 180. Nine had abdominal or penile pain as predominant complaint. 82% were tertiary referrals after unsuccessful conservative treatment with antimuscarinic medication and/or urotherapy for OAB. In 121, urethral obstruction was urodynamically proven or seriously suspected, and they underwent urethrocystoscopy with relief of obstruction, when present. Average duration of unsuccessful conservative pre-treatment in this group of patients was 1.2 years. Postoperative results, in terms of relief of complaints, were analyzed. Safety was assessed by analyzing those patients who had a secondary transurethral procedure in the same year, or in the 3 years after primary treatment. Results Of 106 boys with OAB, urge incontinence or therapy-resistant bedwetting, after de-obstruction 33 became free of complaints and 39 showed significant improvement, totaling 72 (68%); dry after additional urotherapy 11 (10%); no change 21 (20%). Nine boys had de-obstruction because of penile or abdominal pain, with 5 completely cured after the procedure. Follow-up treatment was cognitive training in 39, temporary anticholinergic treatment in 26 and CIC in 2 cases. Recurrence of obstruction was seen in 10% during the 3-year follow-up period. Conclusion After failure of conservative therapy, one should actively look for any urethral obstruction as underlying cause of OAB. For such patients, urethral de-obstruction is highly effective, with only a few minor late complications resulting in recurrence of obstruction.

Background Minimally invasive surgery (MIS) is being utilized more frequently as a surgical technique in general surgery and in paediatric urology. It is associated with a steep learning curve. Currently, the centre does not offer a MIS training programme. It is hypothesized that the number of MIS procedures performed in the low-volume specialty of paediatric urology will offer insufficient training potential for surgeons. Objective To assess the MIS training potential of a highly specialized, tertiary care, paediatric urology training centre that has been accredited by the Joint Committee of Paediatric Urology (JCPU). Study design The clinical activity of the department was retrospectively reviewed by extracting the annual number of admissions, outpatient consultations and operative procedures. The operations were divided into open procedures and MIS. Major ablative procedures (nephrectomy) and reconstructive procedures (pyeloplasty) were analysed with reference to the patients' ages. The centre policy is not to perform major MIS in children who are under 2 years old or who weigh less than 12 kg. Results Every year, this institution provides approximately 4300 out-patient consultations, 600 admissions, and 1300 procedures under general anaesthesia for children with urological problems. In 2012, 35 patients underwent major intricate MIS: 16 pyeloplasties, eight nephrectomies and 11 operations for incontinence (seven Burch, and four bladder neck procedures). In children ≥2 years of age, 16/21 of the pyeloplasties and 8/12 of the nephrectomies were performed laparoscopically. The remaining MIS procedures included 25 orchidopexies and one intravesical ureteral reimplantation. Discussion There is no consensus on how to assess laparoscopic training. It would be valuable to reach a consensus on a standardized laparoscopic training programme in paediatric urology. Often training potential is based on operation numbers only. In paediatric urology no minimum requirement has been specified. The number of procedures quoted for proficiency in MIS remains controversial. The MIS numbers for this centre correspond to, or exceed, numbers mentioned in other literature. To provide high-quality MIS training, exposure to laparoscopic procedures should be expanded. This may be achieved by centralizing patients into a common centre, collaborating with other specialities, modular training and training outside the operating theatre. Conclusion Even in a high-volume, paediatric urology educational centre, the number of major MIS procedures performed remains relatively low, leading to limited training potential.

Infections caused by Mycobacterium haemophilum in immunocompetent patients are unusual. M. haemophilum have been associated with cervicofacial lymphadenitis in children, but inguinal infections have not yet been described. We present a case of an inguinal lymphadenitis caused by M. haemophilum in an immunocompetent girl.

To present an overview of the clinical presentation and pathological anatomy, and the results of surgical correction of 7 cases of epispadias with intact prepuce; a rare condition that has only occasionally been reported in literature. A retrospective search was performed in the surgical and diagnoses database between 1991 and 2011. Seven cases of epispadias with intact prepuce were identified. Five presented as a webbed and buried penis, 1 as phimosis and 1 with suspicion for congenital anomaly of the genitalia. In 3 of 7 cases, epispadias was suspected or diagnosed at first presentation and could be surgically corrected in the first intervention. In the other 4 cases, epispadias was discovered during surgery, requiring an additional intervention to perform epispadias repair in 3 cases. One boy was diagnosed with glandular, 3 with coronal, 1 with shaft and 2 with penopubic epispadias. Epispadias repair was successful with regard to cosmesis and erectile function. Five patients developed normal continence after surgery, 1 after intensive urotherapy. An under average penile length was the main reported problem during follow-up. In the diagnostic process for a concealed penis, the possibility of epispadias should be considered. If epispadias is suspected or confirmed, epispadias repair can occur in the first intervention, reducing the number of additional interventions. Epispadias with intact prepuce appears to have a better prognosis concerning urinary continence compared to classical epispadias

Contains fulltext : 88206.pdf (Publisher’s version ) (Closed access) BACKGROUND/PURPOSE: Usually, anorectal malformations (ARM) are treated in 2 or 3 stages for fear of disturbed wound healing and subsequent damage to the anal sphincter complex. The aim of this study was to assess the feasibility, safety, advantages, and follow-up of an anterior or posterior sagittal anorectoplasty in low-type ARM (rectoperineal or rectovestibular), performed without colostomy. MATERIALS AND METHODS: Prospective collection of data regarding demographics, VACTERL (Vertebral-, Anal-, Cardiac-, Tracheo-Esophageal-, Renal-, Limb malformations) screening, perioperative measurements, surgeons, and complications. RESULTS: In 35 consecutive children (13 boys, 22 girls), repair of a low-type ARM was performed without colostomy. There were 13 boys and 10 girls with a rectoperineal and 12 girls with a rectovestibular fistula. The median age at operation was 4 months (range, 0-73 months); 34% being performed in the newborn period. Seventeen children had one or more other congenital anomaly. Preoperatively, all patients had rectal washouts with oral and rectal neomycin, and perioperative antibiotics, either 24 h (prophylaxis) or for 2 to 5 days. An anterior or posterior sagittal anorectoplasty was performed. Postoperatively, 9 children had no enteral feeding and total parenteral nutrition (TPN). All children had postoperative anal dilatations according to the Pena scheme. Two children (both with rectoperineal fistula) had a wound abscess; in the first child (with renal insufficiency), a colostomy was performed and in the other child a successful correction of the anoplasty was done. In 7 children (4 rectoperineal, 3 rectovestibular fistulae), the anus eventually healed after minor wound dehiscence. There was 1 anal stricture, after a median follow up of 14 months (range, 1-84 mo). After therapeutic antibiotics (2-5 days), 11% (2/18) had some degree of wound infection, versus 41% (7/17) after either no antibiotics or after prophylactic antibiotics (24 hours). Patients with TPN did not seem to profit with regard to wound healing and one patient experienced a central line related sepsis. At last follow-up, 12 children needed regular laxatives and/or enemas. Anal dilatations were well accepted above 6 months, and a trend was seen towards less need for laxatives when dilatations were continued longer. CONCLUSION: Repair of a low-type ARM without colostomy, with therapeutic antibiotics, and followed by a long period of postoperative anal dilatations has low morbidity and good outcome, which does not seem to be improved with TPN. 01 juli 2010

Kaefer et al. [1] advocate, in case an indication exists for surgical intervention, to turn an obstructive megaureter into a refluxing one as a temporary solution to avoid reimplant surgery in infants. They do this based on a concern that reimplant surgery should be avoided under the age of 12 months. No scientific proof exists to support this concern that for some people in pediatric urology is a dogma that they strictly adhere to. In contrast, ample scientific proof exists that infant recalibration and reimplantation can be done safely with good results and without any risk for the urodynamic properties of the bladder [2e5]. In this current series, the children are brought into a compromised situation twice because of the need for extra definitive reconstructive surgery at a later age. Therefore, in our opinion, the message should be: if one is not confident to do a major reconstruction in a neonate, refer the patient to a center where, regardless of age, the patient can be treated adequately in one step, instead of bringing the patient at risk by creating a refluxing megaureter. An obstructive megaureter is not an indication for any temporary measures except for a nefrostomy tube in case of a pus-filled system in a septic child or a cutaneous ureterostomy when severe doubts exist on the viability of the kidney, to observe whether

Summary Introduction Candida bezoar (CB) is a rare finding in neonates and infants with candiduria, presenting as necrotic debris with proliferating mycelia in the collecting system of the kidney. If initial antifungal medical treatment does not result in clearance of candiduria and disappearance of CB on ultrasound in dilated kidneys, invasive interventions like insertion of nephrostomy tubes (NT) or surgical interventions to drain the kidney are sometimes advocated . . However, NT placement can be a technical challenge, especially in pre- and dysmature neonates, and NT displacement or obstruction by the CB can lead to suboptimal treatment. Identification of those children who will benefit from invasive renal drainage is important. Objective This study evaluates the management of patients with CB in three tertiary referral hospitals to determine criteria for intervention. Materials and methods A retrospective multicenter chart analysis was conducted of children with candiduria and ultrasonographic demonstration of CB (diagnosed between March 1995 and August 2012). The indication for invasive renal drainage (if performed) and subsequent clinical outcome, serum creatinine levels and ultrasound findings were assessed. Results A total of 12 children were included, two of which were premature neonates. Eight children had congenital urogenital anomalies. One older child with acute myeloid leukemia had CB during chemotherapy and one ex-premature developed CB following cerebral candidiasis. All children received systemic antifungal medication; in seven children invasive treatment was added. Indications for invasive treatment were clinical deterioration, progressive renal dilation, pyonephrosis, rising creatinine levels and persistence of CB. Two underwent a Y-cutaneous ureterostomy and nephrostomy tubes were inserted in five children. Percutaneous renal drainage by nephrostomy led to complications in 3 of 6 procedures. In all patients, irrespective of therapeutic modality, follow-up ultrasound demonstrated no de novo changes. No additional parenchymal defects or deterioration of split renal function were seen on DMSA or MAG-3 scan. Table . Children with invasive treatment Indication renal drainage Type of drainage & complications Renal follow up Premature 28 wks ♀ Clinical deterioration, pyonephrosis, creatinine ↑ Bilateral NT Displacement right NT Normal US Premature 25 wks ♀ Clinical deterioration, progressive dilation, normal creatinine Right sided NT Urinoma, & JJ placement Normal US Urosepticemia bilateral VUR ♂ Clinical deterioration, progressive dilation creatinine ↑ Left sided NT Obstruction & replacement NT Normal US Ex premature cerebral & renal candidiasis ♂ Clinical deterioration, pyonephrosis, creatinine ↑ Right sided NT – Right sided nephrolithiasis Bilateral VUR ♂ Clinical deterioration, progressive dilation, normal creatinine Left sided NT – Normal US Unilateral kidney agenesis & VUR ♀ MAG3 proven obstruction, creatinine ↑ Unilateral Y uretero-cutaneostomy Unchanged DMSA Obstructive mega ureter ♂ Persistent dilation, normal creatinine Unilateral Y uretero-cutaneostomy Unchanged MAG 3 L 63 %: R 37 % Children with antifungal treatment No renal drainage, CB resolution during antifungal treatment Renal follow up Unilateral kidney agenesis & sepsis ♂ CB during renal replacement therapy for multi organ failure Unchanged DMSA with scars Left sided VUR ♀ Febrile UTI with dilation and left sided CB, initially antibiotics switched to fluconazol Unchanged DMSA L 34 %: R 64 % Bladder exstrophy ♂ CB Postoperatively after primary closure with double J in situ Normal US AML and chemotherapy ♂ Neutropenic fever, UTI and CB initially antibiotics switched to fluconazol Normal US Prune belly syndrome ♂ Bilateral CB postoperatively after ureter reimplantation with stents in situ Unchanged DMSA L 60 %: R 40 % VUR = Vesicoureteral reflux; AML = Acute myeloblastic leukemia; US = Ultrasound; DMSA = Dimercaptosuccinic acid scan; MAG3 = Technetium-99m Mercaptoacetyltriglycine scan. Discussion In the literature renal drainage is suggested in case of complete obstruction. However dilatation is a frequent finding in children as part of the congenital renal anomaly and does not necessarily mean that there is obstruction of the urinary tract. Even in children without candida infections the diagnosis of obstruction is not straightforward, while the results of a MAG 3 scan can be obscured by compromised kidney function, parenchymal bacterial infiltration and neonatal immaturity of the kidney. If candiduria and CB persist despite intensive medical treatment, intensive consultation is required before renal drainage, because NT insertion might be a surgical challenge. Complications such as displacement of the NT, urinoma development, or NT obstruction can occur and was seen in three of six procedures. Premature neonates seem to be more prone to complications due to their small anatomical proportions, requiring medical equipment with small diameters prone to displacement and obstruction. Some studies describe successful pharmaceutical management in the majority of patients with CB. Other studies describe unilateral surgical intervention in children with bilateral CB where unilateral drainage did not influence overall renal outcome. This is in line with our results. A limitation of the present study is its retrospective design. In this population, the motivation for invasive renal drainage or conservative management was not well documented in all cases, and was mainly based on clinical and diagnostic parameters like creatinine levels and radiographic findings. Conclusion Renal drainage should be considered in selected cases after failure of systemic antifungal treatment. Inserting and maintaining a nephrostomy tube in young children is associated with a high rate of complications; conservative treatment is likely to be sufficient in the majority of patients with candiduria and CB.

Purpose We introduce a modification of ileocystoplasty combined with double Monti tube. This modification prevents the dog ear of the ileum during detuberalisation and also the Monti tube is supported by the surrounding mesenterium of the ileal augment. Material and Methods In 5 patients that needed ileocystoplasty the new technique has been used. Four patients had a small neuropathic bladder, one patient had a small contracted bladder without neurological disorder. The surgical technique is as follows: 30 or 40 cm ileum is isolated and plicated in a U shape and completely divided in the middle. A 16 mm wide strip of ileum is then cut from the top of the right ileal part, and a similar strip is cut from the bottom of the left ileal part. The strips are cut near the mesentery to form the Monti tubes. The small strips are tubelarised and joined in the middle to form a catheterisable tube. The ileal segments are opened antimesenterially. These ileal flaps are closed over the Monti tube in the middle. The lower part of the Monti tube is implanted with a submucosal tunnel in the bladder wall and the ileal patch is then anastomosed with the bladder. The Monti tube is anastomosed to the umbilicus in an ordinary way without any traction. Results All patients have umbilical stoma's without leakage or strictures. Intermittend cathetrisation is very easy. It seems that the Monti tubes are strait and well supported by the surrounding tissues so that kinking is not possible. The augmented bladders show excellent volume and compliance. Conclusions Although we treated only a small number of patients we are convinced that this modification provides advantages during the procedure and produces better results in the future.