Mortality rates, cause and risk factors in people with spina bifida, register-based study over five decades.

AIM: Care for people with spina bifida can be improved. This may be done by evaluating mortality rates and causes of death. METHODS: Between 1973 and 2021, 1735 people with spina bifida appeared in registers of the Swedish population. Survival rates and causes of death were calculated according to age and decade. RESULTS: Over almost 50 years, the prevalence of spina bifida decreased from 5.2 to 1.2 per 10 000 births. Mortality fell sharply during the first year of life, with survival rising from 75% to 94%. For children aged 2-18 years and adults, mortality rates were low and differences between decades were minimal. Causes of childhood deaths were congenital abnormalities, hydrocephalus and infections, the latter two also in adults. Adult causes also included self-inflicted injuries and substance abuse, with suicidal or unclear intent, both more common than in the general population. Bladder malignancies were also more frequent, although after reconstructive bladder surgery, mortality rates were similar. CONCLUSION: Survival in the first year of life increased in children with spina bifida, whereas there was no difference in survival rates between adults born between 1973 and 1999. For adults, proactive prevention methods regarding self-inflicted injury, substance abuse and bladder cancer are warranted.

Transabdominal ultrasound of rectal diameter in healthy infants: a prospective cohort study during the first year of life.

AIM: Transabdominal rectal ultrasound (TRU) is used to measure transverse rectal diameter (TRD) in order to diagnose functional constipation (FC) and megarectum, and to evaluate treatment. The proposed cut-off value is 3.0 cm. Currently, no standardised values exist for children below the age of 4. We used repeated TRUs to establish reference TRD values in healthy infants and to describe rectal diameter in infants with FC. METHODS: This prospective observational cohort study enrolled healthy term babies from a maternity department. TRD measurements were taken at 2 and 12 months of age, and questionnaires completed in interviews helped diagnose FC according to Rome III criteria. RESULTS: Two hundred TRUs were performed on 110 infants (62 males). In infants without FC anytime, the mean TRD at 2 months was 1.56 (SD 0.32) cm and at 12 months 1.78 (0.47) cm, while the 95th percentiles were 2.26 and 2.64 cm, respectively. In 77 infants with two TRUs, the mean increase was 0.21 cm (95% confidence interval: 0.099-0.318). Thirteen infants were diagnosed with FC during the study period. At 2 and 12 months of age, there was no difference in TRD between infants with and without FC. CONCLUSION: TRD increased from 2 to 12 months. We suggest 2.3 cm as an upper limit for normal TRD at 2 months and 2.6 cm at 12 months. Infants diagnosed with FC did not have a greater TRD than infants without, either before or after treatment. Further studies are needed to evaluate the usefulness of TRU in infants with FC or megarectum.

Bowel habits in healthy infants and the prevalence of functional constipation, infant colic and infant dyschezia.

AIM: During infancy bowel habits change. Most infants with gastrointestinal problems have a functional gastrointestinal disorder (FGID), a major reason for visiting paediatricians. This study aims to provide data on stool frequency and consistency during the first year, prevalence rates for functional constipation (FC) and to establish associations with relevant demographic data. The occurrence of infant colic (IC) and infant dyschezia (ID) was also reported. METHODS: This prospective observational birth-cohort study enrolled 122 healthy full-term infants. Questionnaires were completed at 2 weeks and 2, 6 and 12 months. RESULTS: Stool frequency decreased with age and consistency changed, with fewer runny stools. At 2 weeks, 24.3% had at least one of the studied FGID. FC was found in 2.6%-14.3% (up to 12 months), IC was found in 4.9%-3.4% (up to 2 months) and ID in 22.1%-3.9% (up to 6 months). Infants with an FGID had a lower weight and more healthcare visits than infants without. Breastfeeding and a high percentage of runny stools at 2 weeks of age decreased the odds of developing FC. CONCLUSION: Data on bowel habits and the prevalence of FC, IC and ID are presented. FGID during infancy is common and affects children's well-being, while their families need support and advice.

Whole exome sequencing identifies KIF26B, LIFR and LAMC1 mutations in familial vesicoureteral reflux.

Vesicoureteral reflux (VUR) is a common urological problem in children and its hereditary nature is well recognised. However, despite decades of research, the aetiological factors are poorly understood and the genetic background has been elucidated in only a minority of cases. To explore the molecular aetiology of primary hereditary VUR, we performed whole-exome sequencing in 13 large families with at least three affected cases. A large proportion of our study cohort had congenital renal hypodysplasia in addition to VUR. This high-throughput screening revealed 23 deleterious heterozygous variants in 19 candidate genes associated with VUR or nephrogenesis. Sanger sequencing and segregation analysis in the entire families confirmed the following findings in three genes in three families: frameshift LAMC1 variant and missense variants of KIF26B and LIFR genes. Rare variants were also found in SALL1, ROBO2 and UPK3A. These gene variants were present in individual cases but did not segregate with disease in families. In all, we demonstrate a likely causal gene variant in 23% of the families. Whole-exome sequencing technology in combination with a segregation study of the whole family is a useful tool when it comes to understanding pathogenesis and improving molecular diagnostics of this highly heterogeneous malformation.

Bowel and bladder function in infant toilet training (BABITT) - protocol for a randomized, two-armed intervention study.

BACKGROUND: In the last decades, the average age for toilet training has increased in the western world. It is suggested that the postponed initiation of toilet training is a contributing factor to problems related to bowel and bladder control. Functional gastrointestinal and urinary tract disorders are prevalent in childhood, causing suffering in affected children and for their families, and consuming healthcare resources. To evaluate whether assisted infant toilet training can prevent functional gastrointestinal and urinary tract disorders in young children, we are conducting a randomized intervention study with a 4-year follow-up. METHODS: This randomized two-armed intervention study will include 268 Swedish infants recruited at six child healthcare centers in Region Dalarna located in the central part of Sweden. The intervention entails parents being instructed and practicing assisted infant toilet training with their child. Children are randomized to start assisted infant toilet training at 0-2 months or at 9-11 months of age. The primary objective is to determine the efficacy of assisted infant toilet training initiated at 0-2 months on the prevalence of functional gastrointestinal disorders (defined as infant colic, infant dyschezia and/or functional constipation) up to the age of 9 months. Secondary objectives are to evaluate whether assisted toilet training initiated during the first year of life reduce the prevalence of functional gastrointestinal disorders (defined as functional constipation, gastrointestinal symptoms and/or stool toileting refusal) and urinary tract disorders (defined as bladder dysfunction and/or urinary tract infections) up to the age of 4 years. Furthermore, infant-to-mother attachment, parental stress, the toilet training process and overall parental experiences will be evaluated/explored. DISCUSSION: This protocol article presents the rationale and design of a randomized two-armed intervention study that will determine the efficacy of assisted infant toilet training on functional gastrointestinal disorders up to the age of 9 months. Furthermore, the study will evaluate whether assisted infant toilet training during the first year of life can prevent functional gastrointestinal and urinary tract disorders in children up to 4 years of age. If effective, assisted infant toilet training could be recommended in child healthcare settings and new evidence-based guidelines on infant toilet training could be implemented. TRIAL REGISTRATION: The study protocol was retrospectively registered at ClinicalTrials. gov  ( NCT04082689 ), initial release June 12th, 2019).

A genome-wide scan to locate regions associated with familial vesicoureteral reflux.

Vesicoureteral reflux (VUR) is a congenital malformation carrying a high risk of recurrent urinary tract infections (UTI) and, at worst, chronic renal failure. Familial clustering implies a genetic etiology, but studies during the past few decades have demonstrated a causal gene variant in <10% of patients with VUR. The aim of the present study was to search for fully or partially shared ancestral haplotypes in 14 families from south-western Sweden with at least three affected members. High-density single nucleotide polymorphism microarray was used for genotyping prior to analysis with a compatibility matching method developed in-house, and the analysis of copy number variations (CNV). No single unique haplotype was revealed to be shared by the families, thereby excluding a common ancestry and founder mutations as a probable cause of VUR. After evaluation of haplotypes shared by subsets of families, a haplotype shared by nine families was found to be of particular interest. This haplotype, located at chromosomal region 4q21.21, harbours two tentative candidate genes (bone morphogenetic protein 3 and fibroblast growth factor 5), both expressed in metanephros and with known functions during nephrogenesis. As to CNV, only one family had a specific CNV shared by all affected members. This was a focal deletion at 5q31.1 including follistatin-like 4, a gene without a previous known connection to VUR. These data demonstrated the genetic heterogeneity of VUR and indicated that an interaction of environmental and genetic factors, including non-coding and epigenetic regulators, all contribute to the complexity of VUR.

High-Resolution Anorectal Manometry-A Prospective Cohort Study in Healthy Infants.

OBJECTIVES: Functional gastrointestinal disorders (FGD) are common during early childhood. In severe defecation disorders, anorectal manometry (AM) is done to exclude aganglionosis. High-resolution anorectal manometry (HRAM) can probably improve diagnostics but normative data in infants are scarce. This study aims to provide HRAM data in healthy infants overall and in subgroups of infants with FGD, defined as functional constipation (FC), infant colic (IC), and infant dyschezia (ID) (ROME 3 criteria). METHODS: This prospective observational cohort study enrolled healthy term infants, having HRAM at 2 and 6 months, using a water-perfused pediatric anorectal silicone catheter. Data were provided for presence of anorectal waves and recto anal inhibitory reflex (RAIR), volume needed to elicit RAIR and anal resting pressure (ARP). Questionnaires at 2 and 6 months identified subjects with FC, IC, and ID. RESULTS: A total of 187 HRAM investigations were done in 107 infants, without any complications. Normal RAIR was found in all, at least on one occasion. Mean ARP increased between 2- and 6-month investigations in infants without FGD. No other differences in HRAM data could be seen between infants with or without an FGD. At 2 months FC, IC, and ID were seen (2%, 4%, and 17%), whereas at 6 months only FC and ID were seen (7% and 1%). CONCLUSION: We can provide data on HRAM in infants at 2 and 6 months of age. When performing repeated HRAM in healthy infants, RAIR was found in all subjects. HRAM is safe, easy tolerable, and can be recommended in infants with or without FGD.

Bladder/bowel dysfunction in pre-school children following febrile urinary tract infection in infancy.

BACKGROUND: An association between bladder-bowel dysfunction (BBD) and urinary tract infection (UTI) is well-known. However, a question less explored is whether children with UTI early in life also have increased prevalence of BBD after they are toilet-trained. In this study, consecutively selected children with pyelonephritis during their first year of life were assessed for BBD at pre-school age. METHODS: Ninety-two children (51 boys) hospitalized due to pyelonephritis during their first year of life were assessed for BBD at median age 5.4 years. A validated BBD questionnaire, along with urine flow and residual volume measurements, was used for diagnosing BBD. During follow-up, the group was well-characterized regarding renal status, vesicoureteral reflux (VUR), and recurrent UTI. RESULTS: BBD was diagnosed in 35/92 (38%), of which the majority was sub-diagnosed with dysfunctional voiding (DV). There was a strong association between BBD and recurrent UTI during follow-up (p < 0.0001), but only a slight association with VUR status at presentation. Nevertheless, in the group with both BBD and VUR, recurrent UTI was four times higher (12/13, 92%) than in children who had neither VUR nor BBD (23%), (p = 0.0008). BBD was also associated with kidney damage (p = 0.017). CONCLUSION: In children with pyelonephritis during the first year of life, 38% had BBD at pre-school age, regardless of whether they had VUR or not. The study shows an important association between BBD and recurrent UTI, so an assessment of BBD is therefore recommended for pre-school children with UTI, especially when they have history of pyelonephritis during infancy.

Urological results and patient satisfaction in adolescents after surgery for proximal hypospadias in childhood.

BACKGROUND: Proximal hypospadias repair is associated with a considerable complication risk. Long-term follow-up is required to present realistic expectations in pre-operative counseling. OBJECTIVE: To investigate adolescents after childhood surgery for proximal hypospadias in a prospective cohort study describing the urological outcome, complication rates and patient satisfaction with penile appearance. STUDY DESIGN: 39 adolescents ≥14 years with penoscrotal to perineal hypospadias and primary urethroplasty (tubularized incised plate (TIP), preputial flap as Onlay or tubularized (Duckett)) from 1996 to 2005 at a single center were evaluated. The clinical assessment, at Md 16.5 years (14-25), included voiding history, genital examination including the Hypospadias Objective Scoring Evaluation (HOSE), uroflowmetry plus chart data from previous urinary flows and evaluation of patient satisfaction using the Penile Perception Score (PPS). RESULTS: Twenty-nine patients with penoscrotal and 10 with scrotal/perineal hypospadias underwent surgery with TIP (N = 14), Onlay (N = 14) and Duckett (N = 11). Uroflows improved significantly compared with prepubertal maximal flows. Impaired flow rate (<10 mL/s) was found in 14% (5/36). Fifty-one percent (20/39) required reoperations, 29% (4/14) of TIP, 50% (7/14) of Onlay and 82% (9/11) of Duckett (p = 0.0062). Median penile length in adolescence was 8.7 cm (4.0-11.0). Forty-four percent (12/27) of patients were dissatisfied with penile length. Patients were 'satisfied' or 'very satisfied' with meatal position and shape despite HOSE for meatal position being 11% (4/38) distal, 76% (29/38) proximal glanular and 13% (5/38) coronal. TIP patients had more curvature at puberty than Duckett (p = 0.0062). Patients that had a decurvature procedure had shorter penile length (p = 0.019). DISCUSSION: A high complication rate is previously described, predominantly within the first years. Our study shows 50% of reoperations were performed after >3 years, illustrating the need for long-term follow-up. Patient satisfaction with a deviant meatal position is rarely reported [1,2]. Our results support a conservative approach to an asymptomatic retracted meatus. Limitations of this descriptive study are the non-comparable groups and the retrospective data for correlation, impeding evaluation of prognostic outcome-factors. The shorter penile length found in patients after plication, and increased curvature after TIP, is therefore merely descriptive. However, the findings are in line with earlier publications suggesting limited use of TIP, and plication (recommending ventral lengthening instead) to avoid penile shortening and curvature in these cases [3-5]. CONCLUSIONS: The urological long-term outcome after proximal hypospadias repair is good, although late reoperations are common. In adolescence, patients were dissatisfied with the short penile length but satisfied with meatal position, indicating that in proximal hypospadias, preserving penile length and correcting curvature are prioritized over a distal meatus.

Bladder/bowel dysfunction at school age is seen in children with high-grade vesicoureteral reflux and lower urinary tract dysfunction in infancy.

AIM: In approximately one third of cases, congenital high-grade vesicoureteral reflux (VUR) diagnosed during infancy is seen together with lower urinary tract dysfunction (LUTD), characterised by a high-capacity bladder and incomplete emptying. In an earlier study, 20 of these infants were treated with clean intermittent catheterisation during a 3-year period and with surgical treatment of the VUR before catheterisation was ended. In the present study, bladder function was evaluated in these children at school age. METHODS: Bladder function was evaluated in the 20 children at a mean age of 7.3 years using a validated voiding-bowel questionnaire with scores (cut-off score 7) and a urine flow/residual study. RESULTS: Four children (20%) had a normal voiding function at follow-up, whereas 11 (55%) had a clear bladder/bowel dysfunction (scores 7-19) and five (25%) had a mild dysfunction (score 6). Ten (63%) of the children with any dysfunction were recognised as dysfunctional voiding. Recurrent febrile urinary tract infections were correlated with the scores of faecal questions (P = .041), but for total scores P = .058. CONCLUSION: The follow-up of bladder function in children at 7.3 years, diagnosed with high-grade VUR and LUTD in infancy, revealed bladder/bowel dysfunction of varying severity in the majority of cases.

The Swedish infant high-grade reflux trial: UTI and renal damage.

INTRODUCTION: High-grade vesicoureteral reflux (VUR) in children is associated with recurrent urinary tract infection (UTI) and renal damage. Breakthrough UTI despite continuous antibiotic prophylaxis (CAP) during the first years of life is a matter of concern and evokes early intervention. We investigated whether early endoscopic treatment (ET) of VUR grade 4-5 can reduce the risk of UTI recurrence and renal scarring. MATERIALS AND METHODS: This prospective, randomized, controlled, multicentre, 1-year follow-up trial comprised 77 infants, <8 months of age with VUR grade 4-5 (Table) randomized to CAP (n = 39) or ET (with prophylaxis until resolution) (n = 38). Voiding cystourethrogram, ultrasound, renal scintigraphy, and free voiding observation were performed at study entry and after 1 year. Parenchymal defects were seen in 67 (87%) children at entry, 39 (34 boys, 5 girls) of them characterized as generalized. At follow-up, renal deterioration (new scars or progress in old damaged area) and symptomatic UTIs were reported. RESULTS: There were 27 recurrent febrile UTIs in 6 (16%) children in the ET group and in 10 (26%) in the CAP group (p = 0.43), in eight (36%) girls and eight (15%) boys (p = 0.039). Successful VUR outcome (VUR 0-2) was seen in 22 (59%) in the ET and eight (21%) in the CAP group (p = 0.0014). Multiple recurrences were only seen in patients with persistent dilating reflux at follow-up (p = 0.019). Deterioration on scintigraphy was seen in eight children (9 kidneys) with no difference between treatment groups (p = 0.48) or sex (p = 0.17). Renal deterioration was associated with high bladder capacity (BC) and large residual volume (PVR) at 1 year (p = 0.0092 and p = 0.041). Six of the eight children with renal deterioration had a recurrent UTI (p = 0.0032). Seven of nine renal units with deterioration were seen in children with persistent VUR 3-5 at follow-up. Univariable logistic regression identified female sex and high PVR as positive predictors for recurrent UTI (p = 0.039 and 0.034) and high PVR tended to predict renal deterioration (p = 0.053). DISCUSSION: No differences between the treatment groups regarding recurrent UTI and renal deterioration could be found. Increased PVR and female sex were positive predictors for UTI recurrences. VUR grade at follow-up was correlated to UTI recurrence and renal deterioration. CONCLUSION: This study did not show any difference between ET and CAP in reducing the risk of UTI recurrence or renal deterioration. The rate of VUR resolution was higher in the ET group and VUR grade at follow-up correlated with both UTI recurrence and renal deterioration.

The Swedish infant high-grade reflux trial: Study presentation and vesicoureteral reflux outcome.

INTRODUCTION: High-grade vesicoureteral reflux (VUR) in infants is associated with congenital renal abnormalities, recurrent UTI, and bladder dysfunction. Endoscopic treatment (ET) is a well-established method in children with low to moderate reflux grades, but there is a lack of randomised controlled trials regarding the use of ET versus continuous antibiotic prophylaxis in infants with high-grade VUR. OBJECTIVE: This study aimed to determine whether high-grade VUR in infants can be treated with endoscopic injection and whether ET is superior to antibiotic prophylaxis in the treatment of VUR. MATERIALS AND METHODS: This prospective, randomised, controlled, multicentre, 1-year follow-up trial comprised 77 infants (55 boys, 22 girls) <8 months of age with VUR grade 4-5 (n = 30/n = 47). Of the infants, 52 (68%) had bilateral VUR. Thirty-nine were randomised to antibiotic prophylaxis and 38 to ET (with prophylaxis until resolution). Voiding cystourethrogram, ultrasound, renal scintigraphy, and free voiding observation were performed at study entry and after 1 year to evaluate VUR grade, and renal and bladder function. RESULTS: VUR grade ≤2 was seen in 22 (59%) infants in the endoscopy group and eight (21%) in the prophylaxis group at follow-up (p = 0.0014). The success rate in the endoscopy group was 100% in unilateral grade 4, falling to 31% in bilateral grade 5 (p = 0.0094). Correspondingly, the results in the prophylaxis group were 40% in grade 4 down to 0% in bilateral grade 5 (p = 0.037) (Table). Logistic regression analyses identified ET, VUR grade 4, unilaterality, and low residual urine at baseline as positive predictors of VUR down-grading to ≤2 (area under ROC curve 0.88). In four patients with reflux resolution after one injection, dilating reflux recurred at the 1-year follow-up. One patient had a UTI possibly related to ET. In our material four patients required re-implantation, of whom one was obstructive after injection. DISCUSSION: The opportunity to offer even small infants with high-grade VUR an alternative, minimally invasive treatment option is a great advance in paediatric urology. In this high-risk group, bilateral VUR grade 5 stands out with its poor bladder function and low chance of resolution. The recurrence rate of dilating VUR after successful ET is consistent with previous studies. The limitations are the relatively small number of patients and the short follow-up. CONCLUSION: High-grade VUR in infants can be treated with injection therapy and the resolution rate is higher than that of prophylaxis treatment. The complication rate is low and VUR grade 4, unilaterality, and low residual urine are favourable for the resolution and down-grading of VUR.

Health-related quality of life experiences among children and adolescents born with esophageal atresia: Development of a condition-specific questionnaire for pediatric patients.

BACKGROUND/PURPOSE: The aims were to present the framework for the development a condition-specific health-related quality-of-life (HRQOL) questionnaire for children with esophageal atresia (EA) and to describe HRQOL experiences reported by children and by their parents. METHODS: Utilizing the well-established DISABKIDS methodology, standardized focus group discussions were held and transcribed. HRQOL experiences were identified, content analyzed and evaluated using descriptive statistics. RESULTS: 30 families (18 children 8-17years, 32 parents of children 2-17years) participated in ten focus group discussions. 1371 HRQOL experiences were identified referencing social, emotional and physical aspects of eating and drinking (n=368), relationships with other people (n=283), general life issues; physical activity, sleep and general health (n=202), communicative/interactive processes of one's health condition (n=161), body issues (n=109), bothersome symptoms (n=81), impact of health care use/medical treatment (n=78), confidence in oneself and in the future (n=65) and difficulties because of concomitant anomalies (n=24). A basis of two age-related HRQOL questionnaires for children with EA (2-7years, 8-17years) was subsequently constructed. CONCLUSIONS: EA interacts with various aspects of the child's life. In addition to HRQOL issues of eating and drinking, social dimensions like relationships and interactions with other people seem to be prominent condition-specific HRQOL parameters. The settings for the development of the first condition-specific HRQOL questionnaires for patients with EA are established.

Treatment and Follow-Up of Patients with Long-Gap Esophageal Atresia: 15 Years' of Experience from the Western Region of Sweden.

OBJECTIVE: This retrospective study aims to report treatment results in patients with long-gap esophageal atresia (LGEA), gross A + B type, and discuss the value of different clinical findings and physiological tests in the follow-up. METHODS: This retrospective observational study comprises all patients with LGEA admitted to our department between 1995 and 2010. RESULTS: A total of 16 patients were included. Their mean gestational age was 35(+2) weeks and their mean birth weight was 1,945 g (-2.5 standard deviation scores). No catch-up growth in height could be seen and they remained smaller than the average population during the study period. Gastrostomy was performed as the first surgical procedure. Overall, 11 of the 16 patients had a delayed primary anastomosis. Elongation of the distal esophageal segment was required in 3 of the 16 patients and a colonic interposition in 2 of the 16 patients. The median age at definitive surgery was 150 days. All the patients had gastroesophageal reflux after their definitive surgery. Three of the 16 patients required surgery due to aspiration and all 3 had a pathological lung clearance index (LCI) at multiple-breath washout (MBW). At the age of 1 or 7 years, the LCI was pathological in 4 of the 14 patients, and spirometry showed an obstruction in 9 of the 14 patients. CONCLUSION: LGEA is a severe congenital malformation, with severe morbidity. No mortality was seen. MBW could be a useful tool for the early detection of progressive pulmonary damage.

Added Value of pH Multichannel Intraluminal Impedance in Adults Operated for Esophageal Atresia.

BACKGROUND: Gastroesophageal reflux (GER) and dysphagia are common following repaired esophageal atresia (EA). The risk of esophagitis and Barrett esophagus is increased compared with the general population. As yet, the causes are not fully explained. PURPOSE: The aim of this study was to investigate how GER, measured by pH multichannel intraluminal impedance (pH-MII), is correlated to the esophageal symptoms and histological findings. METHODS: Twenty-nine adult subjects operated for EA in Gothenburg from 1968 to 1983 were evaluated with pH-MII, manometry, and gastroscopy. RESULTS: pH-MII was performed in 15, manometry in 19, and gastroscopy in 24 subjects. Eleven subjects displayed pathological reflux parameters of any kind, mainly nonacid reflux (10/15). Dysphagia correlated to the number of weakly acidic reflux episodes. Lower esophageal sphincter (LES) incompetence, which correlated to a pathological number of acid reflux episodes (p = 0.012), was noted in 21/24 subjects, but the majority had a normal resting pressure. Esophagitis was present in 14/24, two of whom had Barrett esophagus. Histological changes correlated to the reflux index and the number of weakly acidic reflux episodes (p = 0.028 and 0.040) and tended to correlate to dysphagia (p = 0.052). CONCLUSION: pH-MII adds further information when it comes to explaining what causes symptoms and esophageal histological changes in adults operated for EA.

Normalized Urinary Flow at Puberty after Tubularized Incised Plate Urethroplasty for Hypospadias in Childhood.

PURPOSE: An obstructive urinary flow pattern is frequently seen after tubularized incised plate urethroplasty for hypospadias. However, the significance of this finding has not been determined and long-term results are few. We describe postoperative long-term uroflowmetry results after puberty in males who underwent tubularized incised plate urethroplasty in childhood. MATERIALS AND METHODS: A total of 126 boys underwent tubularized incised plate urethroplasty for distal penile to mid shaft hypospadias at Queen Silvia Children's Hospital in Gothenburg between 1999 and 2003. Of the patients 48 were toilet trained at surgery. We report on 40 patients who had data available at 2 and 12 months postoperatively, 7 years postoperatively and at puberty (median age 15.0 years, range 13.7 to 17.1). Of the patients 31 had distal and 9 had mid penile hypospadias. Clinical examination, urinary medical history, uroflowmetry and ultrasound measuring residual urine were performed. Maximum urinary flow was correlated to age and voided volume, using Miskolc nomograms for comparison of percentiles. RESULTS: At 1 year postoperatively 15 boys (37.5%) had normal urinary flow (above 25th percentile), compared to 16 (40%) at 7 years and 38 (95%) at puberty (p <0.0001). Improvement was significant in patients with distal (p <0.0001) and mid penile hypospadias (p = 0.008), as well as in patients who did (p = 0.0078) and did not undergo intervention (p <0.0001). During followup 5 patients underwent meatotomy due to obstructive symptoms and 4 underwent dilation. Three of these 9 patients had lichen sclerosus. CONCLUSIONS: There is great potential for normalization of urinary flow at puberty for boys with hypospadias treated with tubularized incised plate urethroplasty. Unless symptoms occur, a conservative approach seems preferable.

International Children's Continence Society standardization report on urodynamic studies of the lower urinary tract in children.

AIMS: The objective of this document created by the ICCS standardization subcommittee is to provide a uniform guideline on measurement, quality control and documentation of urodynamic studies in children. METHODS: This guideline was created using expert opinion and critical review of the published literature on urodynamic studies in children. Currently no standardized guideline or level 1 data exists on the proper technique for this subject matter. RESULTS: The document provides a throughout explanation on how to approach a child who presents with lower urinary tract dysfunction, whether it be of neurogenic, anatomic or functional origin. Formation of an urodynamic question after a comprehensive history and physical examination is paramount in selecting the urodynamic study(ies) that will be most appropriate for each child. Appropriate application of each test with careful consideration of the needs of the child and family will provide the most accurate and reproducible results. Recommendations on how to execute each of the components of an urodynamic study as well as interpretation are included in the document. CONCLUSIONS: Urodynamic studies have become a major tool in evaluating lower urinary tract dysfunction in children. There are many subtleties in performing these studies in children in juxtaposition to adults; therefore, adaptations specific to children must be made to achieve accurate and reproducible results. Uniformity in how the studies are conducted from center to center will allow for healthier transparency and enhanced comparison of results in both clinical and research situations.