Should all babies with oesophageal atresia have routine screening for midgut malrotation anomalies? A systematic review in search of evidence.

Background/Purpose Oesophageal Atresia (OA) is associated with co-existent anomalies. There is a controversy of literature pertaining to the risk (s) of intestinal malrotation. In order to guide management we critically evaluate the incidence of IM anomalies in OA newborns. Design MEDLINE and EMBASE databases were searched using keywords "(O)Esophageal Atresia and Malrotation/Associated Abnormalities/Associated Anomalies". Full texts of articles were screened if manuscripts exclusively reported patients with OA malrotation and/or associated anomalies. Larger case series (> 10patients) were included if abstract (s) showed that associated anomalies were systematically assessed. Full eligibility criteria required at least one case of malrotation in an OA index case. Data were collected on article type, number of patients and method (s) of diagnosis. Results 632 abstracts were screened of which 158 papers were analysed based on inclusion criteria-30 manuscripts documented the incidence (%) of malrotation. Incidence rate (s) were 0.5-13%. Malrotation was observed to have a higher incidence (10-44%) in OA babies with other gastrointestinal anomalies (VACTERL). Conclusion Newborns with OA appear to be at a higher risk (%) of having intestinal malrotation anomalies than healthy babies. Prospective studies are required to accurately quantify and define the ' true incidence ' of this association. Given the potential lethal consequences of midgut volvulus screening may be justified in OA babies. Consensus guidelines (DELPHI) exploring surgeons attitudes with regards management of ' asymptomatic malrotation ' disorders in OA newborns may further guide best practice.

Endoscopic vs laparoscopic paediatric gastrostomies: Time to change our practice?

BACKGROUND: Gastrostomy insertion is a common procedure for paediatric surgeons, with the percutaneous endoscopic gastrostomy (PEG) technique long favoured for its simplicity and speed. However, there is growing evidence to suggest that primary laparoscopic balloon gastrostomy (LBG) insertions may have lower complication rates. This study aimed to determine the relative safety and healthcare resource burden of PEG and LBG. METHODS: A retrospective review of all primary gastrostomy insertions (2011-2019). Primary outcome measures included return to theatre for emergency laparotomy and healthcare burden (total gastrostomy-related admissions, length of stay and total theatre utilisation). RESULTS: 338 PEGs and 277 LBGs were inserted with a minimum follow-up period of six months. Following PEG insertion 12/338(3.6%) children required an emergency laparotomy for gastrostomy-related complications. This compared to 2/277(0.7%) following LBG insertion (ARR2.8% (95%CI0.6-5.0), p < 0.0267). When considering all gastrostomy related admissions, there was no significant difference in total theatre utilisation (PEG = 85 [IQR58-117] minutes, LBG = 86 [IQR75-105] minutes, p = 0.12). However, PEGs were found to have an overall longer length of stay 4 [IQR3-7] vs 3 [IQR2-4] days. CONCLUSIONS: LBGs carry a significantly lower rate of major complications and are not associated with an increased healthcare burden. LBG should be considered as the first line method of gastrostomy insertion in children.

Effectiveness and tolerability of mirabegron in children with overactive bladder: A retrospective pilot study.

AIMS: Mirabegron has promising results for OAB symptoms in adults, although the potential for cardiovascular side effects has caused concern. Efficacy and tolerability in children have not been extensively studied. Effectiveness, tolerability, and side effects of Mirabegron are reported in children with refractory OAB. METHODS: A retrospective review of children receiving Mirabegron between February 2014 and November 2018 was completed. Frequency, urgency, nocturnal (NE), and daytime incontinence (DI) were analyzed at baseline and 6 months. RESULTS: 70 children (50 females), median age 15 [range 8-16] years, commenced Mirabegron 25 mg (n = 29) or 50 mg (n = 41). 37 (53%) were still receiving treatment at 6 months: monotherapy n = 30, and combination therapy n = 7 (Solifenacin n = 4, Desmopressin n = 2, both n = 1). Where undertaken, blood pressure monitoring and ECGs were normal. For patients on monotherapy, 6 of 17 (35%) had improvement in NE, 11 of 19 (58%) in DI, 12 of 20 (60%) in frequency, and 8 of 21 (38%) in urgency symptoms. For patients receiving combination therapy, 2 of 6 (33%) had improvement in NE, 2 of 4 in DI (50%), 2 of 4 (50%) in frequency, and 4 of 6 (67%) had improvement in urgency. Reasons for treatment discontinuation (entire cohort) were: ineffectiveness (n = 28), worse symptoms (n = 4) and/or adverse reactions (n = 7), including dry mouth (n = 2), headaches (n = 4), dizziness (n = 1), nausea/vomiting (n = 3), increased seizures (n = 1), and rash (n = 1). CONCLUSION: Mirabegron improved symptoms in 70% of patients with refractory OAB. A prospective RCT should be the next step to establish the role of Mirabegron for the treatment of OAB in children. LEVEL OF EVIDENCE: Level II.

Combined creatinine velocity and nadir creatinine: A reliable predictor of renal outcome in neonatally diagnosed posterior urethral valves.

INTRODUCTION: Nadir creatinine (lowest creatinine during the first year following diagnosis) is a recognised indicator of future chronic renal impairment (CRI) in posterior urethral valve (PUV) patients. We recently described "creatinine velocity" (Cvel), the rate of change of creatinine following initial bladder drainage, as a new early predictor of CRI in neonatally diagnosed PUV. Rising Cvel (>3 µmol/L/day) is associated with increased risk of CRI. OBJECTIVE: We studied these two prognostic indicators in combination, as a test for future CRI in neonatally diagnosed PUV patients. STUDY DESIGN: Medical records for patients treated by endoscopic valve ablation at our institution between 1993 and 2004 were reviewed. Simple linear regression was used to calculate Cvel. Creatinine velocity and nadir creatinine were considered predictive of future CRI if they were greater than 3 µmol/L/day or greater than 75 µmol/L (0.85 mg/dL), respectively. Chronic renal insufficiency was defined as CKD2 or higher. Outcomes in test groups were analysed by Fisher exact test. Statistical significance was defined as p < 0.05. RESULTS: Sixty-two patients were treated within the first 30 days of life and had sufficient data to calculate both Cvel and nadir creatinine. Mean follow-up was 9.4 years. Patients were grouped as having both risk factors (Group A), one risk factor (Group B), or neither risk factor (Group C). All four (100%) patients from Group A developed CRI, compared with 11 of 17 (64.7%) patients from Group B and three of 41 (7.3%) patients from Group C (p ≤ 0.0005). As a diagnostic test for future CRI, "presence of at least one risk factor" had a specificity of 86.4%, sensitivity of 83.3%, positive predictive value of 71.4%, and negative predictive value of 92.7%. Additional prognostic information was obtained by assigning a score from 1 to 3 for each prognostic indicator (Table). The sum of these scores gave a PUV Risk Score. No patient with a PUV Risk Score of 2 developed CRI, while all patients with a Score of 6 developed CRI. Incidence of CRI in patients with PUV Risk Scores of 3, 4, and 5 was 8.3%, 50%, and 63.6%, respectively (p ≤ 0.0005). CONCLUSION: Considered together, these prognostic indicators provide a powerful test for future CRI. Presence of at least one of these risk factors should be considered "at risk for CRI". Patients with neither risk factor are unlikely to develop CRI. Calculation of the PUV Risk Score provides an even more accurate prognosis.

Medicated Manuka honey in conservative management of exomphalos major.

PURPOSE: The aim of this study was to assess the effectiveness of Manuka honey ointment and dressings in the conservative management of exomphalos major (EM). METHODS: A retrospective review of five patients with EM who underwent non-operative management with Manuka honey ointments and dressings was carried out to assess the time to complete epithelialisation, time to full feeds, hospital stay, adverse effects, complications and outcome. RESULTS: The skin epithelialisation over the EM sac was achieved in a median of 63 days (48-119). The median time to full enteral feed was 13 days (3-29). The median hospital stay was 66 days (21-121). No adverse effects were noted related to Manuka honey. Three patients had pulmonary hypoplasia requiring prolonged hospitalization; one of those died with respiratory complications at home after achieving complete epithelialisation. The follow-up was a median 16 months (6-22). Two patients did not require repair of the ventral hernia. One patient had ventral hernia repair at 16 months with excellent cosmesis. The remaining patient is awaiting repair. CONCLUSION: This is the first description of the use of medicated Manuka honey ointment and impregnated dressings in the conservative management of EM. This treatment is safe, efficacious and promotes wound healing with favorable outcome.

Posterior urethral valves: creatinine velocity, a new early predictor of renal insufficiency.

AIM: Antenatal screening has led to early detection of posterior urethral valves (PUV). However, despite early intervention, a proportion of children will develop chronic renal insufficiency. We studied the trend of serum creatinine following urinary tract decompression during the neonatal period in infants as a possible predictor of chronic renal insufficiency. METHODS: Patients treated by endoscopic resection of posterior urethral valves between 1993 and 2004 were identified. From these, infants treated within the first 30 days of life were identified. Serum creatinine values taken within the first 5 days following initial drainage were recorded. A creatinine velocity for each patient was calculated by linear regression analysis. Creatinine was considered rising if velocity was >3 µmol/L/day, or falling if velocity was <-3 µmol/L/day. Chronic renal insufficiency was defined as CKD2 or higher. RESULTS: Sixty-four neonates had decompression of the urinary tract. Of these, 16 had rising creatinine despite drainage, 10 had a plateau in creatinine level, and 36 had falling creatinine following drainage. Insufficient data were available in two to calculate creatinine velocity. Progression to renal insufficiency was significantly higher in patients with an initial rise in creatinine (62.5%) than in those with plateau creatinine (40%) or falling creatinine (8.6%) (P ≤ 0.0005 by Fisher exact test). Mean follow-up was 9.2 years. CONCLUSIONS: Rising creatinine, even transiently, following urinary tract drainage in neonates with posterior urethral valves is significant and is a new and important indicator of long-term prognosis.

Varicocele surgery: 10 years of experience in two pediatric surgical centers.

AIM: The study was designed to compare recurrence rates and complications after laparoscopic versus open varicocele surgery in children. SUBJECTS AND METHODS: A retrospective case-note review of all varicocele surgery over a 10-year period (April 1999-March 2009) in two pediatric surgical centers was performed. Multivariate analysis using logistic regression was performed using SPSS Statistics version 18 (SPSS Inc., Chicago, IL). RESULTS: Thirty-seven patients had varicocele surgery during the study period. The median age at surgery was 14 years (range, 11-16 years). Most children had left-sided Grade 2 varicocele. Twenty-five (68%) primary procedures were laparoscopic (17 artery-sparing), and 12 (32%) procedures were open (9 artery-sparing). Six (16%) children had recurrence, and 6 (16%) had postoperative hydrocele. Recurrence rates after laparoscopic (16%) and open (17%) surgery were similar. Increasing age significantly decreased recurrence (odds ratio, 0.373; 95% confidence interval 0.161-0.862; P = .021). Although laparoscopy was associated with higher rates of postoperative hydrocele (odds ratio, 2.817; 95% confidence interval, 0.035-3.595; P = .380) and artery-sparing ligation was associated with higher rates of recurrence (odds ratio, 2.667; 95% confidence interval, 0.022-4.235; P = .787), these associations were not statistically significant. CONCLUSIONS: The best results of varicocele surgery in terms of recurrence and postoperative hydrocele were achieved by open mass ligation; however, larger prospective studies are warranted.

Gallbladder contractility in liver cirrhosis: comparative study in patients with and without gallbladder stones.

An increased prevalence of gallstones was demonstrated in patients with liver cirhosis, higher in the advanced stages of the disease. Some studies have found impaired emptying of the gallbladder in cirrhotic patients. Our aim here was to investigate gallbladder emptying in cirrhotic patients with and without gallstones to find out whether emptying is further impaired in the presence of gallstones. The study group comprised 24 patients with liver cirrhosis and gallstones, 8 in each Child class. The controls were represented by 18 cirrhotic patients without gallstones, 6 in each Child class. Fasting gallbladder volume was calculated by ultrasound using the ellipsoid formula. Gallbladder emptying was evaluated for 90 min after ingestion of a solid-liquid meal (14 g fat, 425 kcal), by assessing minimal residual volume, gallbladder ejection fraction, and area under emptying curve at 15-min intervals. Statistical analysis was performed using the two-tailed Students' t test and Pearson's correlation coefficient. In controls, gallbladder fasting and residual volumes increased with the severity of cirrhosis, but gallbladder emptying did not change significantly. In cirrhotics with gallstones, gallbladder emptying decreased in Child C compared with Child A class patients and, also, compared to Child C controls. The number or size of gallstones, as well as the thickness of the gallbladder wall, did not correlate with gallbladder emptying parameters. Gallbladder contractility is impaired in patients with liver cirrhosis and gallstones. Hypomotility is proportional to the severity of liver disease. Gallbladder hypomotility might contribute to the increased gallstone formation in patients with advanced cirrhosis.