Long-Term outcomes of cecostomy tube insertion for patients with bowel dysfunction: A retrospective review.

BACKGROUND: Constipation is common in the pediatric population and in severe forms it can lead to debilitating fecal incontinence which has a significant impact on quality of life. Cecostomy tube insertion is a procedural option for cases refractory to medical management, however there is limited data investigating the long-term success and complication rate. METHODS: A retrospective review was performed evaluating patients at our centre undergoing cecostomy tube (CT) insertion between 2002 and 2018. The primary outcomes of the study were the rate of fecal continence at 1-year, and the incidence of unplanned exchanges prior to annual scheduled exchange. Secondary outcomes include the frequency of anaesthetic requirements and length of hospital stay. Descriptive statistics, t-test, and chi-square analysis was performed where appropriate using SPSS v25. RESULTS: Of 41 patients, the average age at the time of initial insertion was 9.9 years with the average length of stay in hospital being 3.47 days. The most common etiology of bowel dysfunction was spina bifida, which was present in 48.8% (n = 20) of patients. Fecal continence was achieved in 90% (n = 37) of patients at 1 year and the average rate of cecostomy tube exchange was 1.3/year with an average of 3.6 general anaesthetics being required by patients and the average age of no longer requiring one being 14.9 years. DISCUSSION: Analysis of patients undergoing cecostomy tube insertion at our centre has further supported the use of cecostomy tubes as a safe and effective option for management of fecal incontinence refractory to medical management. However, a number of limitations exist in this study including its retrospective design and failure to investigate changes in quality of life using validated questionnaires. Additionally, while our research provides greater insight to practitioners and patients what degree of care and types of complications or issues they may encounter with an indwelling tube over the long-term, our single-cohort design limits any conclusions that could be made regarding optimal management strategies for overflow fecal incontinence through direct comparison with other management strategies. CONCLUSIONS: CT insertion is a safe and effective method for managing fecal incontinence due to constipation in the pediatric population, however, unplanned exchange of tube due to malfunction, mechanical breakage, or dislodgment occurs frequently and may impact quality of life and independence. LEVEL OF EVIDENCE: IV.

Renal outcomes of children born with posterior urethral valves at a tertiary center: A 15-year retrospective review.

INTRODUCTION: Posterior urethral valves (PUVs) is a congenital condition in which an obstruction in the urethra prevents drainage of urine from the bladder in males, with up to 60% of children diagnosed developing chronic kidney disease (CKD). The primary aim of this study was to identify novel factors that may predict development of CKD and end-stage real disease (ESRD ) in children with PUVs to potentially address modifiable factors and delay progression. The secondary aim was to compare rates of catheterization and incontinence between our patients and other case series to provide information to parents about long-term bladder outcomes. METHODS: A single-center, retrospective cohort study was performed of all children referred to our multidisciplinary clinic for PUV diagnosis between 2005 and 2019. Univariable associations of different variables with the composite outcome CKD or ESRD were evaluated. RESULTS: Thirty of 46 patients (65%) developed CKD, with the majority (40%) being stage 2 CKD (n=12). Seven of 30 patients (23%) developed ESRD requiring renal replacement therapy. Fourteen of 26 (30%) required clean intermittent catheterization (CIC) initiation, with a median CIC initiation age of 4.3 years. Creatinine nadir post-valve ablation, oligohydramnios, and initiation of CIC are significant predictors of CKD development. CONCLUSIONS: This review reiterates that children born with PUVs have a high morbidity rate, with a high proportion developing CKD. Using a multidisciplinary approach to PUV patient care allows for better family education, early intervention of bladder dysfunction, and possibly better long-term preservation of renal function.

Contemporary management of pediatric high grade renal trauma: 10 year experience at a level 1 trauma centre.

BACKGROUND: Current guidelines advocating the conservative management of renal injuries in children are primarily extrapolated from adult series due to a dearth of evidence in the pediatric population. OBJECTIVES: The aim of this study was to review our experience in the management of pediatric high-grade renal trauma and to clarify the role of conservative management in this cohort of patients. STUDY DESIGN: The Alberta Trauma Registry (ATR) is a comprehensive web-based registry which functions to prospectively collect data on all trauma patients in the province who sustain a severe injury (i.e. Injury Severity Score (ISS) ≥12). The ATR was used to identify all pediatric patients who attended hospitals within the Edmonton region with high grade renal injuries (grade III-V) between January 2006 and December 2018. Hospital records and imaging were reviewed to identify patient demographics, mechanism of injury, AAST grade, haemodynamic stability, associated injuries, management, length of hospital stay (LOS), complications, and follow-up outcomes. RESULTS: A total of 53 children (38 boys, 15 girls) were identified with a mean age of 13 years (1-16). The mechanism of injury was blunt trauma in 92.5% (49/53) of cases (Supplementary Table). AAST grade distribution was 37.8% Grade III (20/53), 49% Grade IV (26/53) and 13.2% Grade V (7/53). All Grade III injuries were successfully managed conservatively. Overall 11 patients with Grade IV/V injuries required urological intervention (ureteral stenting (5 patients), angioembolization (4 patients), bladder washout with clot evacuation (1 patient), emergency nephrectomy (3 patients)). The overall renal salvage rate was 92.4% (49/53). DISCUSSION: Our series confirms the safety of expectant management in high grade pediatric renal trauma. All grade III injuries in our study were managed conservatively without the need for intervention. This suggests that these injuries may be managed safely outside of designated trauma centres. One third of children with grade IV/V injuries required intervention. Therefore we recommend that patients with these injuries are transferred to specialized units with the capacity to provide such procedures if required. CONCLUSION: This study supports the conservative management of pediatric renal trauma in the setting of high-grade injury. Expectant management was associated with acceptable rates of intervention and excellent renal salvage rates.

An Antenatally Detected Pure Malignant Rhabdoid Tumor of the Bladder.

Pediatric malignant rhabdoid tumors are a rare entity with poor prognosis. The extrarenal variants of this disease are sparsely presented within the literature. Our case represents, to our knowledge, the first antenatally detected bladder-variant. At 19 months of age, this patient has benefited from early detection and intervention and remains disease free following partial cystectomy and chemotherapy.

Initially Asymmetrical Function on MAG3 Renography Increases Incidence of Adverse Outcomes.

PURPOSE: To discriminate pathological from physiological hydronephrosis (99m)Tc-mercaptoacetyltriglycine (MAG3) diuretic renography is a fundamental imaging modality. Initial asymmetrical differential function or decreasing function on serial MAG3 scans is a classic surgical indication. However, prognostic evidence supporting this is limited. Therefore, we examined the association between initial function and outcomes. We hypothesized that patients with initially asymmetrical function would have higher odds of functional decline, and experience symptoms and surgical intervention compared to patients with initially symmetrical scans. MATERIALS AND METHODS: We retrospectively reviewed the charts of 1,124 pediatric patients with hydronephrosis seen at Stollery Children's Hospital from 2000 to 2014. A total of 387 cases of unilateral ureteropelvic junction obstruction were grouped by initial postnatal differential function with asymmetrical defined as a greater than 10% difference in relative function. Postnatal surveillance, followup MAG3 results, pyeloplasty rates and post-pyeloplasty outcomes were compared. RESULTS: Of 387 patients 143 (37%) had initially asymmetrical function. Of those undergoing MAG3 scan surveillance 17% experienced a 10% or greater decline in ipsilateral differential function vs a 6% rate of function loss in the initially symmetrical group (OR 3.2, 95% CI 1.6-6.4, p = 0.0008). The overall pyeloplasty rate in patients with asymmetrical and symmetrical function was 27% and 16%, respectively (OR 1.9, 95% CI 1.1-3.2, p = 0.013). Patients with initially asymmetrical function had an increased rate of pyeloplasty secondary to symptoms compared to those with initially symmetrical function (16% vs 8%, OR 2.1, 95% CI 1.1-4.0, p = 0.019). Mean time to functional decline and pyeloplasty was 19 and 17 months, respectively. CONCLUSIONS: Patients with initially asymmetrical differential function had increased ORs for subsequent renal decline, symptom onset and pyeloplasty.

Randomized Crossover Trial of Single Use Hydrophilic Coated vs Multiple Use Polyvinylchloride Catheters for Intermittent Catheterization to Determine Incidence of Urinary Infection.

PURPOSE: Urinary tract infection is a key issue for long-term intermittent catheterization users. Various catheter designs and methods have evolved to decrease the risk but the evidence remains unclear regarding whether product type improves outcomes. We determined whether single use hydrophilic coated catheters reduced urinary tract infections compared to multiple use polyvinylchloride catheters for children with neurogenic bladder due to spina bifida. MATERIALS AND METHODS: This was a randomized crossover 4-center trial with 2 treatment periods of 24 weeks each, consisting of single use hydrophilic coated catheter and multiple use polyvinylchloride catheter (washed with soap and water, and air dried after each use). Each week participants recorded symptoms and urine results (Multistix® 8SG reagent strip). Primary outcome was person-weeks of urinary tract infection, defined as positive leukocytes plus fever, flank pain, increased incontinence, malaise, or cloudy or odorous urine requiring antibiotic treatment. Individuals were included if they were a child or young adult with spina bifida and used intermittent catheterization as the primary method of bladder emptying. RESULTS: Calculated sample size was 97. More than 120 patients were screened, of whom 66 were randomized and 45 completed both trial arms. Mean age was 10.6 years. Of the patients 21 were male and 24 were female. Mean ± SD person-weeks of urinary tract infection was 3.42 ± 4.67 in the single use hydrophilic coated catheter group and 2.20 ± 3.23 in the multiple use polyvinylchloride catheter group (p <0.001). There were no statistical differences in weeks of febrile urinary tract infection or antibiotic use. CONCLUSIONS: Results are consistent with the Cochrane Review in that single use hydrophilic coated catheters may not decrease the incidence of symptomatic urinary tract infection in community dwelling chronic intermittent catheterization users when compared to clean multiple use polyvinylchloride catheters.

Nocturnal enuresis: non-pharmacological treatments.

INTRODUCTION: Nocturnal enuresis affects 15% to 20% of 5-year-old children, 5% of 10-year-old children, and 1% to 2% of people aged 15 years and older. Without treatment, 15% of affected children will become dry each year. Nocturnal enuresis is not diagnosed in children younger than 5 years, and treatment may be inappropriate for children younger than 7 years. METHODS AND OUTCOMES: We conducted a systematic review and aimed to answer the following clinical question: What are the effects of non-pharmacological interventions for relief of symptoms of nocturnal enuresis? We searched: Medline, Embase, The Cochrane Library, and other important databases up to October 2013 (Clinical Evidence reviews are updated periodically, please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA), the European Medicines Agency (EMA), and the UK Medicines and Healthcare products Regulatory Agency (MHRA). RESULTS: We found five studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. CONCLUSIONS: In this systematic review, we present information relating to the effectiveness and safety of the following interventions: acupuncture, dry bed training, enuresis alarm, and hypnotherapy.

Global developmental delay, progressive relapsing-remitting parkinsonism, and spinal syrinx in a child with SOX6 mutation.

SOX6, a member of the SOX gene family, plays a key role in the development of several mammalian tissues and organs, including the central nervous system. Specifically, this gene modulates the differentiation and proliferation of interneurons in the medial ganglionic eminence, as well as oligodendrocytes in the spinal cord. We describe the case of a 4-year-old girl with global developmental delay and a spinal cord syrinx who presented with recurrent episodes of parkinsonian symptoms subsequent to febrile illnesses. The symptoms included gait instability, tremor, and dysarthria, with a progressive relapsing-remitting course over the span of 2 years. The patient was later found to have a large deletion-type mutation in the SOX6 gene. This case is the first report in humans implying a role for SOX6 in basal ganglia function, as well as spinal cord development.

Symptomatic versus asymptomatic pyeloplasties: A single institution review.

INTRODUCTION: Historically, pyeloplasties have been performed after symptoms and radiographic confirmation of an ureteropelvic junction obstruction (UPJO). However, with prenatal ultrasonography, the approach to patients has fundamentally changed. Increasingly, patients are diagnosed and treated before the advent of morbidity, based on imaging findings alone. However, optimum screening strategies and thresholds for intervention vary significantly, are controversial, and are not founded on outcome-based evidence. We examined all pyeloplasties performed at our institution and reviewed their indication for surgery. We hypothesized that, despite ubiquitous screening for UPJO, most pyeloplasties had been performed secondary to symptoms and did not benefit from antenatal screening. METHODS: A retrospective chart review was performed of all pyeloplasties performed at the Stollery Children's Hospital, Edmonton, Alberta, over the past 8 years. Patients were categorized according to indication for surgery: symptomatic or asymptomatic. RESULTS: Most (60%) of our pyeloplasties were performed for symptomatic indications. Furthermore, 12% of these patients had antenatally detected hydronephrosis that was thought to have resolved spontaneously during follow-up. Of our symptomatic patients, 37% were undergoing surveillance with the expectation for spontaneous resolution. Of the 29 patients who underwent pyeloplasty, 8 suffered a preoperative loss of function on renal scans; however, only 50% returned to within 90% of their original function. CONCLUSION: Despite active surveillance of antenatally detected hydronephrosis, most pyeloplasties at our institution were performed for de-novo symptoms. We believe that this simple observation reinforces that our current surveillance strategies are unable to predict and eliminate all morbidity from UPJO.

Outcomes following fecal continence procedures in patients with neurogenic bowel dysfunction.

PURPOSE: Malone antegrade continence enema and cecostomy button improve quality of life in patients with neurogenic bowel. However, they have not been compared regarding fecal continence outcomes. We compared these 2 procedures with respect to continence, complications and conversions. MATERIALS AND METHODS: We retrospectively reviewed the charts of patients who underwent Malone antegrade continence enema or cecostomy at the University of Alberta between January 2006 and January 2011. A total of 26 patients underwent Malone antegrade continence enema, of whom 20 underwent concomitant Monti procedure and bladder augmentation, 5 a laparoscopically assisted procedure and 1 concomitant ileovesicostomy. A total of 23 patients underwent cecostomy, of whom 1 underwent ileovesicostomy, 1 bladder augmentation, 1 a Monti procedure with bladder augmentation and 1 laparoscopic cecostomy. Continence was defined as ability to wear underwear with no accidents at most recent annual followup, which was a minimum of 1 year postoperatively. RESULTS: Fecal continence rates were 84.6% for Malone antegrade continence enema and 91.3% for cecostomy. There were no statistically significant differences in continence based on procedure (p = 0.48), age (p = 0.97) or gender (p = 0.54). Of patients who underwent cecostomy 8.7% switched to the Malone antegrade continence enema, while 11.5% with Malone antegrade continence enema switched to cecostomy. Mean length of hospital stay for patients undergoing cecostomy vs laparoscopically assisted Malone antegrade continence enema was 4.0 vs 5.2 days (p = 0.15). Complications included stomal pain (23.1% of patients) and difficulty with catheterizing (19.2%) following Malone antegrade continence enema, and difficulty flushing (26.1%) following cecostomy. CONCLUSIONS: There were no significant differences between Malone antegrade continence enema and cecostomy button with respect to fecal continence or complication rates. Each approach poses unique challenges, suggesting that patients and families need to understand the differences to make an individualized choice.

The association between urinary continence and quality of life in paediatric patients with spina bifida and tethered cord.

OBJECTIVE: To determine the association between urinary continence and quality of life (QoL) in a paediatric spina bifida population. METHODS: After appropriate ethics approval, a prospective study was initiated using multiple validated QoL instruments that were distributed to patients as they presented for their annual appointment at the Northern Alberta Spina Bifida Clinic (Edmonton, Alberta). General demographic information was collected and validated questionnaires were used. The survey package included two instruments to assess overall QoL: Global Pediatric QoL (PedsQL 4.0) and Health Specific QoL-Spina Bifida (HRQoL-SB). Two instruments were also included to quantify urinary symptoms and assess urinary specific QoL: the Urinary Incontinence Severity Index - Pediatric (ISI-P) and Urinary Specific QoL (PinQ). RESULTS: A total of 71 patients were enrolled in the study. The general QoL (PedsQL 4.0) and health-specific QoL (HRQoL-SB) scores for the population indicated an overall QoL of 66% (n=69) and 83% (n=67), respectively. Approximately 46% (33 of 71) reported >1 episode of urinary incontinence per week. Urinary continence was associated with a significantly higher urinary-specific QoL (PinQ; P<0.001), general QoL (PedsQL 4.0; P<0.05) and health-specific QoL (HRQoL-SB; P<0.05). Furthermore, urinary incontinence and its effect on QoL was not influenced by the presence of a shunt, level of the lesion or manner of dysraphism. CONCLUSION: These data suggest that QoL in patients with spina bifida is related to urinary continence. This effect appears to be independent of the type and level of the spinal dysraphism and the presence or absence of a shunt.

OBJECTIF: Déterminer l'association entre la continence urinaire et la qualité de vie (QdV) au sein de la population d'âge pédiatrique ayant un spina-bifida. MÉTHODOLOGIE: Après l'approbation déontologique pertinente, les chercheurs ont amorcé une étude prospective au moyen de multiples instruments validés relatifs à la QdV, qui ont été distribués aux patients lors de leur rendez-vous annuel à la clinique de spina-bifida du nord de l'Alberta située à Edmonton, en Alberta. Ils ont colligé les renseignements démographiques généraux et utilisé des questionnaires validés. Le dossier de l'étude se composait de deux instruments pour valider la QdV globale : la QdV pédiatrique globale (PedsQL 4.0) et la QdV propre à la santé (HRQoL-SB). Deux instruments ont également été utilisés pour quantifier les symptômes urinaires et évaluer la QdV propre aux symptômes urinaires : l'indice de gravité de l'incontinence urinaire ­ pédiatrique (ISI-P) et la QdV propre aux symptômes urinaires (PinQ). RÉSULTATS: Au total, 71 patients ont participé à l'étude. Les indices de QdV générale (PedsQL 4.0) et de QdV propre à la santé (HRQoL-SB) pour cette population ont indiqué une QdV globale de 66 % (n=69) et de 83 % (n=67), respectivement. Environ 46 % (33 sur 71) ont déclaré plus d'un épisode d'incontinence urinaire par semaine. La continence urinaire s'associait à une QdV propre aux symptômes urinaires, à une QdV générale et à une QdV propre à la santé nettement plus élevées (PinQ : P<0,001, PedsQL 4.0 : P<0,05 et HRQoL-SB : P<0,05, respectivement). Par ailleurs, la présence d'un shunt, le niveau de la lésion et la gravité de la dysraphie n'avaient pas d'influence sur l'incontinence urinaire et son effet sur la QdV. CONCLUSION: Selon ces données, la QdV des patients ayant un spina-bifida est liée à la continence urinaire. Cet effet semble indépendant du type et du niveau de dysraphie spinale et de la présence ou de l'absence d'un shunt.

Quality of life in children with vesicoureteral reflux.

Vesicoureteral reflux (VUR) is commonly diagnosed in children presenting with urinary tract infections. Antibiotic prophylaxis and ureteric surgery are standard treatments for these children. Our aim was to investigate whether health-related quality of life (HRQOL) was altered in children treated for VUR. Children aged 1-5 years with grade III or higher VUR were identified through electronic records at the Stollery Children's Hospital. Parents of these children were mailed the TNO-AZL Netherlands Organisation for Applied Scientific Research Academic Medical Centre Quality of Life (TAPQOL) questionnaire. QOL scores for this group were compared with normative controls from the instrument's creators using the Mann-Whitney U test. Thirty-two of the 96 (33%) mailed surveys were returned. Eight children had surgery, and 19 were treated with antibiotic prophylaxis. When comparing the VUR group with the control group, we found that anxiety and social functioning scores were significantly better in patients with VUR (p < 0.01). The VUR group had worse scores in problem behavior, stomach complaints ,and communication (p < 0.01). This study reveals that children with VUR have a reasonable QOL when compared with controls. However, the diagnosis of VUR and its management does have an impact on gastrointestinal complaints, behavior, and communication, which may occur as a result of chronic medical intervention.

Gonadotropin-releasing hormone agonist in laparoscopic myomectomy: systematic review and meta-analysis of randomized controlled trials.

A systematic review and meta-analysis of randomized controlled trials was performed to estimate the effects on surgical outcomes of pretreatment with gonadotropin-releasing hormone (GnRH) agonist before laparoscopic myomectomy. The electronic bibliographic databases MEDLINE, EMBASE, Web of Knowledge, Scopus, and Cochrane Library, and reference lists from relevant articles were searched for English-language publications describing randomized controlled trials of GnRH agonist pretreatment vs placebo or no treatment before laparoscopic myomectomy. Three studies including 168 participants were identified. Pretreatment with GnRH agonist did not reduce operative time; however, intraoperative blood loss was statistically lowered (mean difference, 60 mL; 95% confidence interval [CI], 39-82). Statistical difference was also observed in postoperative hemoglobin concentration (mean difference, 1.15 g/dL; 95% CI, 0.46-1.83]) and red blood cell count (mean difference, 0.65 × 10(6) cells/mL; 95% CI, 0.16-1.14]) but not serum iron concentration. None of the patients in the studies experienced any major intraoperative or postoperative complications, and only 1 patient in each group required blood transfusion. This study does not show a difference in operative time with GnRH agonist pretreatment, which clarifies the previous conflicting reports on the effect of GnRH agonist therapy on the duration of laparoscopic myomectomy. Furthermore, despite previously reported decreased bleeding conferred by the laparoscopic approach compared with laparotomy, this meta-analysis demonstrates a further reduction in intraoperative blood loss with GnRH agonist pretreatment in patients undergoing laparoscopic myomectomy. Additional high-quality studies with appropriate power and follow-up are needed to corroborate these findings and to evaluate the treatment effects on short- and long-term outcomes such as perioperative complications and fertility.