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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.

Objective: Discussion remains on how to advise women with a past medical history of surgically corrected anorectal malformations (ARMs) regarding vaginal delivery. The aim of this review is to evaluate and review the reported obstetrical complications and outcomes after vaginal delivery for these women.Data sources: A systematic search was performed from inception up to 8 December 2020 in PubMed, Embase.com and Clarivate Analytics/Web of Science Core Collection, with backward citation tracking. Study eligibility criteria / appraisal: All articles reported on the outcomes of interest in women with a past medical history of surgically corrected anorectal malformation and had a vaginal delivery were included with the exception of editorial comments or invitational commentaries. Screening and data extraction was done by two authors independently with a third and fourth reviewer in case of disagreement. Tool for Quality assessment depended on the type of article. As low quality evidence was expected no meta-analysis was performed. Results: Only four of the 2125 articles screened were eligible for inclusion with a total of 12 vaginal deliveries attempts in 7 women. In two patients complications were reported: failed vaginal delivery requiring urgent cesarean section (n=1), vaginal tears (n=1). Conclusion: high quality evidence regarding outcomes and complications after vaginal delivery in women with a history of surgically corrected anorectal malformation is lacking. Therefore, based upon this systematic review no formal recommendation can be formulated regarding its safety. Future studies are essential to address this problem. Prospero registration: CRD42020201390. Date: 28-07-2020s

Introduction: The process of improving one's skills over time is called a “learning curve”. This term has attracted great attention during the last decades, especially in relation to laparoscopic techniques. Aim: To assess the outcome of paediatric laparoscopic pyeloplasty (LP). Material and methods: Retrospective analysis of the consecutive LPs. The inclusion criteria: (1) children aged < 18 years, (2) transperitoneal approach, and (3) the same operating paediatric urologist (RC). Patients with a history of any procedure on the upper urinary tract were excluded. Any surgical reintervention during follow-up was defined as a failure. The outcomes of LPs performed before 2012 (G1) were compared to those conducted between 2012 and 2016 (G2). Fisher's exact test was used for statistical analysis. Results: Ninety patients met the inclusion criteria, and a total of 95 LPs were performed. The mean operation time was 155 min, and the mean hospitalisation period was 2.4 days. In G1, 19 patients underwent Anderson-Hynes LP, 16 had Fenger non-dismembered LP and two underwent vascular hitch. In G2, 54, 2 and 2 patients underwent these procedures, respectively. The overall success rate was 91.5%. There were six failures in G1 and three in G2 (p = 0.147). Of the Anderson-Hynes LPs, 1/19 in G1 and 3/58 in G2 required reintervention (p = 1). For Fenger LPs, this was 4/16 and 0/2, respectively (p = 1). Only one patient required reoperation after vascular hitch. Conclusions: The surgeons' learning curve reflects their experience with regard to the entire therapeutic process, but not exclusively their manual skills.

Background. Distal penile hypospadias account for about 70% of all cases of hypospadias. There is a variety of operative techniques that could be performed when foreskin reconstruction is an option. The urethral stent is left in the urethra to prevent complications. Objectives. To determine whether the duration of stenting influences the healing of foreskin after distal hypospadias repair. Material and methods. Data from 2 institutions was retrospectively analyzed. Inclusion criteria were as follows: 1) a modified meatal advancement glanuloplasty without tubularization of the urethral plate, 2) foreskin reconstruction and 3) follow-up - 12 months. All other types of reconstruction and re-do procedures were excluded. The period of urethral stenting was determined intraoperatively depending on the surgeon's preferences. Mean age at operation was 23.3 months. The cohort was divided into 3 groups. In Group I (G-I), no catheter was left or it was removed the next day after surgery. In Group II, the catheter was left for more than 5 days. In those 2 groups, the surgery was done by different surgeons. Group III consisted of 35 patients who had a stent for

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.

To evaluate fertility potential after orchidopexy for bilateral undescended testis and compare two surgical fixation techniques for effect on fertility. Men older than 22 years who had either tunica albuginea orchidopexy (TAO) or “no-touch” technique (NTO) in childhood for bilateral undescended testis (BUDT) were selected. Participants filled out a questionnaire followed by physical examination, had testicular ultrasound, blood sample and semen analysis. Statistical testing was performed using general linear modelling. Sixty-seven out of 166 individuals responded. Forty-nine completed the questionnaire, and nine (18.3%) reported having fathered children. Thirty-six showed up for further examination, 26 had TAO and 10 NTO. Impaired hormonal spermatogenesis regulation (34.6% vs. 20%), higher subfertility rate (46% vs. 20%) and lower means of motile spermatozoa (58.1 × 106 spz vs. 177.9 × 106 spz) were observed in the TAO versus the NTO group; none of these were statistically significant. Four (15.4%) of the TAO and two (20%) of the NTO group have azoospermia. Although the operation technique did not have a significant impact on fertility, unfavourable outcomes were more common after surgery involving the tunica albuginea of the testis. Larger sample sizes are needed to ascertain whether the trends favouring the NTO technique are of any significance.

Lower urinary tract symptoms (LUTS) in children is a frequent occurring problem, together with astma the most frequent disease at pediatric age. For many children LUTS is a chronic condition with a major impact on the quality of life. Adequate treatment is needed and useful to enhance quality of life, prevent LUTS at later age and protect the upper urinary tracts in case of co-existing vesico-ureteric reflux. The advice to wait and see is outdated This manuscript tries to give an overview of the current management of LUTS in children.

We explored possible associations between long-term antimuscarinic use and behavioral problems in children with spinal dysraphism and neurogenic bladder. Children with open and closed spinal dysraphism were recruited from 2 pediatric hospitals, 1 in Amsterdam and 1 in Utrecht, The Netherlands. At the Amsterdam facility antimuscarinics were prescribed in selected patients with detrusor overactivity. At the Utrecht facility antimuscarinics were prescribed from birth onward in patients with spinal dysraphism beginning in the early 1990s. Parents of study participants were asked to fill out a Child Behavior Checklist. Demographics, data on level and type(s) of lesion, and presence of hydrocephalus with a drain (and, if applicable, number of drain revisions) were retrieved for each patient. Cases and controls (8 boys and 8 girls per group) were matched on a 1-to-1 basis. Data on 32 children were analyzed. Median age was 10.6 years in cases and 10.5 years in controls (p=0.877). In each group 9 of 16 patients had hydrocephalus with a drain. No significant difference in Child Behavior Checklist scores for total problems was found between cases and controls (median 52.0 vs 59.5, p=0.39). No differences were found between the groups on any subdomain of the Child Behavior Checklist. No significant differences in behavior were found between children with spinal dysraphism with and without long-term use of antimuscarinics

To assess the results of endoscopic bladder neck procedure on the anterior bladder wall in children. Surgery is done in the lithotomy position using three 5-mm ports. The bladder is insufflated with CO2. A U-shaped incision is made around the bladder neck. A mucosal strip is tabularized around a 12 French catheter and covered with the second layer of mucosa. Twenty procedures were performed on 18 patients (mean age, 9.8 years), and the follow-up period was >1 year (mean, 34 months). Mean operation time was 149 minutes. Twelve patients had neurogenic lower urinary tract dysfunction. Fifteen patients failed earlier bladder neck surgery, predominantly fascia sling suspension. Preoperatively, all patients had low detrusor leak point pressure. Clean intermittent catheterization (CIC) was resumed through the urethra in 11 patients and through a stoma in 6 patients. Two patients needed conversion because of CO2 leakage. Six patients were dry, and 4 improved in the short term (3-6 months). After 1 year of follow-up, 2 patients were dry, and 6 improved. In the long term, 1 (9%) out of 11 patients who were catheterized through the urethra was dry, and 3 of the 11 patients (27%) improved. Of the 6 patients with a CIC stoma, 1 (17%) was dry, and 3 (50%) improved. Endoscopic bladder neck surgery is, for most patients, a minor operation, but the long-term results are disappointing. The construction of a continent channel for CIC can improve the outcome when anterior bladder neck plasty is performed

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.

OBJECTIVE To investigate whether children with lower urinary tract (LUT) anomalies are at greater risk for postoperative complications after laparoscopic pyeloplasty stented with a double-J catheter (JJC). MATERIALS AND METHODS Prospectively collected data of laparoscopic pyeloplasty (LP) performed between 2006 and 2015 were analyzed. Inclusion criteria are (1) toilet-trained child and (2) unilateral dismembered pyeloplasty stented with a JJC done by the same surgeon. Our pyeloplasty protocol includes cystoscopy and retrograde pyelography. JJC is left in for 3weeks. Asymptomatic patients with infravesical LUT anomalies (a-LUTA) and those with history of LUT symptoms (LUTS) were identified. Any short-term complication was classified according to Clavien-Dindo. Fisher's exact test was used for statistical analysis. RESULTS Fifty-four children (mean 9.8 years) were included. Ten of 54 patients had LUTS. In 4 of those 10, anatomical infravesical anomaly was found during cystoscopy. Accidental urethral anomaly was found in 11 patients (a-LUTA). The control group (CG) consisted of 33 patients. Postoperative hospital stay ranged from 1 to 8 days (mean 2 days). Overall complication rate was 8 of 54 (14%). Grade 1 complications occurred in 3 patients in the CG. Five patients had grade 3 complications (2 needed replacement of bladder catheter, and 3 had diversion of the upper tract). Those problems occurred in 1 of 10 patients with LUTS and 3 of 11 patients with a-LUTA compared to 1 of 33 in the CG. This difference was statistically significant (P

To assess the outcomes of nondismembered pyeloplasty for ureteropelvic junction obstruction in a pediatric population of children and adolescents. Between 2005 and 2009, a total of 129 pyeloplasties were performed at our institution. In all, 34 (24%) renal units underwent primary nondismembered Fenger-type plasty, 22 open (OPEN) and 12 laparoscopic (LAP). Gender distribution, left to right ratio, follow-up period, grade of kidney dilatation and split renal function were similar in both groups. The decision to perform a nondismembered procedure was made by the surgeon intraoperatively. The mean age at surgery was 4.5 years for the OPEN group and 14.2 years for the LAP group. Mean follow-up was 30 months (range 12-70 months). The overall success rate was 91% (95.5% the OPEN group and 83.5% in the LAP group). Secondary surgery was required for 3 renal units. Other complications included pyelonephritis in 3 patients (2 in OPEN and 1 in LAP), ileus in 1 patient (OPEN), and prolonged postoperative pain in 1 patient (LAP). The mean operation time was significantly shorter for open surgery (95 minutes for OPEN vs 179 minutes for LAP; P < .05). There was no significant difference in the length of the postoperative hospital stay (2.5 days for OPEN vs 3 days for LAP). Nondismembered pyeloplasty is an effective procedure for curing ureteropelvic junction obstruction in the pediatric population, and can be considered as an option in well selected cases

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.

Minimal invasive surgery (MIS) has developed tremendously during the last 20 years. Nowadays, a wide spectrum of ablative and reconstructive procedures in urology can be done in adults as well as in children. MIS has been proven to be a safe and effective technique regardless of the age of a patient, provided it is done by a well prepared team. MIS has its disadvantages and limitations. In this article the differences between adults and children in different aspects of MIS are described. The current status of MIS in paediatric urology is provided. The authors show their own experience in laparoscopic surgery for the treatment of congenital and acquired anomalies of the urogenital tract. The future of MIS in paediatric urology is briefly discussed. Proper training is crucial to reduce the complication rate and to improve the results. The numbers of laparoscopic procedures in childhood are relatively low. Therefore, there is a need for concentration of the MIS care to ensure surgeons’ exposition to higher volumes, which can improve the quality of care.

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.

INTRODUCTIONMidgut volvulus due to intestinal malrotation is a rare cause of intestinal obstruction when occurring in adult life. This paper documents the difficulties in reaching an early diagnosis.PRESENTATION OF CASEWe describe the case of an 85-year-old man with non-specific abdominal complaints for 20 years, who presented with sudden onset central abdominal pain. An acute median laparotomy under general anaesthesia was performed during which a 360° clockwise rotation of the small bowel around the mesenteric pedicle of the superior mesenteric artery and vein was found.DISCUSSIONMalrotation is considered any deviation of the normal rotation of the midgut in embryological development, causing intermittent episodes of gastrointestinal obstruction or acute events of midgut volvulus. Although mainly a paediatric diagnosis, some cases do present in adult life. Radiologic investigations include: upper gastrointestinal contrast studies, Doppler sonography and a contrast enhanced CT of the abdomen. If a true malrotation is diagnosed or found by coincidence, a Ladd's procedure is advised, even if the patient is asymptomatic. There is no proven surgical strategy for preventing the recurrence of small bowel volvulus in case of malfixation of the midgut.CONCLUSIONNon-specific recurrent abdominal complaints in adults of any age should raise suspicion of the possibility of a midgut malrotation or malfixation with or without intermittent volvulus. This case highlights the importance of diagnosis in an early stage.

To present the primary results of laparoscopic, Burch-type colposuspension (LBC) in children. LBC was performed in eight children (mean age, 14 years) with urinary incontinence after failed extensive urotherapy program. In all patients, bladder neck (BN) incompetence was proven by clinical observation, perineal ultrasound (US), and video-urodynamic study (V-UDS). The LBC was performed with patients in the lithotomy position by means of three 5-mm ports. The anterior wall of the vagina, lateral to the BN, was mobilized, and the vaginal wall was bilaterally lifted and sutured to Cooper's ligament, resulting in elevation and fixation of the BN. A catheter was left for 4 days. The mean operation time was 101 minutes (range, 56-150 minutes), and the follow-up time was >6 months. The postoperative period was uneventful for all patients. Shortly after the procedure, 5 patients (62.5%) were dry, and 1 patient improved (12.5%). After 6 months, 3 patients (37.5%) were dry, and 2 (25%) improved. In 3 wet patients, control US and V-UDS focused on the BN revealed hypermobility and persistent flat vesicourethral angle (VUA) in 1 patient, hypermobility with a good restoration of the VUA in 1 patient, and good fixation with good restoration of the VUA in 1 patient. LBC is a challenging procedure in children because of the small operation field. This procedure can be considered to cure refractory stress urinary incontinence in children with BN insufficiency when noninvasive methods have failed

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

In the search for new techniques to improve venous valve insufficiency, a peritoneal patch with a mesothelial layer on either side was employed in the venous circulation to construct a venous valve de novo. This material was used because of the resemblance in fibrinolytic activity of mesothelial cells to endothelium. The behavior of double-sided flaps of peritoneum was first studied on only one side in the caval vein wall of 10 rats. All veins remained open, and smooth incorporation of the patch into the vein wall was observed. It was therefore decided to use the same material for construction of a cusp blade in the venous circulation. This venous reconstruction was microsurgically performed in 21 rats and 4 piglets. In our hands, intramuscular anesthesia proved superior to intraperitoneal analgesia in rats. For the piglets mechanical ventilation and general anesthesia were used. In 9 of 21 rats a fair or good result was observed, although it did not seem possible to create a fully competent valve with only one cusp blade in the 1.5-mm-diam caval veins. The same fact evolved from the study using four 7-mm-diam piglet veins. All peritoneal flaps in the venous circulation demonstrated rebuilding of their structure, perhaps induced by the relatively exaggerated central venous flow. To our knowledge, this technique has never been used before. It seems to offer many advantages in the venous circulation, where hardly any thrombogenic surface is tolerated in contrast to the arterial circulation. Application in humans is yet premature, but future research may resolve several problems that occurred in this study.

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.

Of noninvasive venous function tests two are well known: photoplethysmography (PPG) and strain-gauge plethysmography (SGP). Their effectiveness in assessment of insufficiency in candidates for venous reconstructive surgery however, is debated. The two methods were studied in these patients, and compared to invasive measurements. Nineteen limbs of patients with deep venous disease, and seven limbs of healthly volunteers were evaluated. Measurements were taken in standing, as well as in the more patient-compliant sitting position. Venous reflux time (RT) proved to be significantly different in patients vs. controls. Differences between expelled volume of controls and patients were only significant for invasive measurements in any position ( p < 0.01), but not with one of the noninvasive methods. RT invasively and non-invasively measured were well correlated in both positions with PPG ( R = 0.83 – 0.86), and with SGP ( R = 0.85 — 0.92). The percentage residual pressure however, showed in both methods a poor correlation of < 0.50. The studied noninvasive techniques offer no alternative to invasive measurements in the evaluation of patients with deep venous disease, considered for venous reconstructive surgery.