Impact of Colorectal Nurse Specialist supervised parental administration of rectal washouts on Hirschsprung's disease outcomes: a retrospective review.

PURPOSE: To highlight the utility of Colorectal Nurse Specialist (CNS) supervised parental administration of rectal washouts in the management of Hirschsprung's disease (HD). METHODS: Retrospective case note review of HD patients treated at a tertiary children's hospital in United Kingdom from January 2011 to December 2022. Data collected included demographics, complications, enterocolitis, obstructive symptoms and stomas. Primary pull-through (PT) is done 8-12 weeks after birth. Parental expertise in performing rectal washouts at home is ensured by our CNS team before and after PT. RESULTS: PT was completed in 69 of 74 HD patients. Rectal washouts were attempted on 63 patients before PT. Failure of rectal washout efficacy necessitated a stoma in four patients (6.4%). Of the 65 patients who had PT and stoma closed, three (4.5%) required a further stoma over a mean follow-up period of 57 months (Range 7-144 months). Two of these had intractable diarrhoea due to Total Colonic Aganglionosis (TCA). One patient (1.5%) had unmanageable obstructive symptoms requiring re-diversion. Hirschsprung-associated enterocolitis (HAEC) requiring hospital admission occurred in 14 patients (21%). CONCLUSION: Our stoma rates are lower compared to recent UK data. This could potentially be due to emphasis on parental ability to perform effective rectal washouts at home under CNS supervision.

Non-thermal disruption of ß-adrenergic receptor-activated Ca2+ signalling and apoptosis in human ES-derived cardiomyocytes by microwave electric fields at 2.4 GHz.

The ubiquity of wireless electronic-device connectivity has seen microwaves emerge as one of the fastest growing forms of electromagnetic exposure. A growing evidence-base refutes the claim that wireless technologies pose no risk to human health at current safety levels designed to limit thermal (heating) effects. The potential impact of non-thermal effects of microwave exposure, especially in electrically-excitable tissues (e.g., heart), remains controversial. We exposed human embryonic stem-cell derived cardiomyocytes (CM), under baseline and beta-adrenergic receptor (ß-AR)-stimulated conditions, to microwaves at 2.4 GHz, a frequency used extensively in wireless communication (e.g., 4G, Bluetooth™ and WiFi). To control for any effect of sample heating, experiments were done in CM subjected to matched rates of direct heating or CM maintained at 37 °C. Detailed profiling of the temporal and amplitude features of Ca2+ signalling in CM under these experimental conditions was reconciled with the extent and spatial clustering of apoptosis. The data show that exposure of CM to 2.4 GHz EMF eliminated the normal Ca2+ signalling response to ß-AR stimulation and provoked spatially-clustered apoptosis. This is first evidence that non-thermal effects of 2.4 GHz microwaves might have profound effects on human CM function, responsiveness to activation, and survival.

Rare Associations with Posterior Urethral Valves.

Posterior urethral valves are a common cause of congenital bladder outlet obstruction. Known associations include cardiac malformations and gastrointestinal abnormalities. In this case series, we report on two cases of PUV associated with anorectal malformations along with a case of PUV in monochorionic diamniotic twins. We explore the difficulty in achieving a diagnosis and the final management. The association of posterior urethral valves in a patient with anorectal malformation should be suspected in case of associated oligohydramnios or oliguria postnatally. There should be a high index of suspicion in twin pregnancy even if only one of the twins is suspected of bladder outlet obstruction.

Vasculitis as part of the fetal response to acute chorioamnionitis likely plays a role in the development of necrotizing enterocolitis and spontaneous intestinal perforation in premature neonates.

BACKGROUND: Necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) are causes of bowel perforation in premature neonates. Studies have demonstrated that both are associated with acute chorioamnionitis (ACA) of the placenta. AIM: The aim of our study was to identify any histopathological links between placental histopathological abnormalities and the later development of NEC and/or SIP in premature patients presenting at our institution. PATIENTS AND METHODS: Cases with a diagnosis of NEC/SIP were identified. Entry criteria were the diagnosis of NEC/SIP was confirmed clinically and/or histologically, had been made within the first 7 days of life, neonates were premature, and the placenta had been submitted for histological examination. In those cases with ACA, CD34 immunohistochemistry and Martius scarlet blue staining was performed. Medical records were reviewed for demographics, clinical variables, and clinical outcomes. Statistical analysis was performed using Fisher exact test. RESULTS: In total, 21 cases met defined inclusion criteria (12 NEC, 8 SIP, and 1 clinically indeterminate). Mean gestational age was 27 weeks. Median age of presentation was 5 days. Placental histology showed ACA in 16 of 21 cases (76.2%). Of those with ACA, 13 of 16 (81.3%) had umbilical phlebitis, 12 of 16 (75.0%) had umbilical arteritis, 6 of 16 (37.5%) funisitis, and 12 of 16 (75.0%) had chorionic vasculitis. No differences (p > 0.05) were seen between ACA and diagnosis or clinical outcome (Fisher exact test). Of the 16 cases, 14 with ACA that later developed either NEC or SIP showed vasculitis in the umbilical cord and/or chorionic plate and/or stem villi vasculature. The association between ACA and vasculitis was highly significant (p < 0.01). Of those with ACA on placental histology, 12 of 16 (75.0%) cases were found to have intermediate-advanced stage fetal inflammatory response (FIR), whereas 13 of 16 (81.3%) had grade 2 (severe) FIR. Of the 16 cases, 8 (50.0%) had evidence of fibrin deposition/early thrombus formation within placental and/or umbilical vasculature. These were associated with vascular endothelial injury in vessels with prominent vasculitis. CONCLUSION: NEC or SIP shows a significant association with ACA with presence of vasculitis as part of the FIR (p < 0.01). In a proportion of cases, the development of fibrin deposition in response to vasculitic endothelial damage of the placental vasculature may form part of the mechanism linking ACA and early postnatal development of NEC and/or SIP.

Gastroschisis: one year outcomes from national cohort study.

OBJECTIVE: To describe one year outcomes for a national cohort of infants with gastroschisis. DESIGN: Population based cohort study of all liveborn infants with gastroschisis born in the United Kingdom and Ireland from October 2006 to March 2008. SETTING: All 28 paediatric surgical centres in the UK and Ireland. PARTICIPANTS: 301 infants (77%) from an original cohort of 393. MAIN OUTCOME MEASURES: Duration of parenteral nutrition and stay in hospital; time to establish full enteral feeding; rates of intestinal failure, liver disease associated with intestinal failure, unplanned reoperation; case fatality. RESULTS: Compared with infants with simple gastroschisis (intact, uncompromised, continuous bowel), those with complex gastroschisis (bowel perforation, necrosis, or atresia) took longer to reach full enteral feeding (median difference 21 days, 95% confidence interval 9 to 39 days); required a longer duration of parenteral nutrition (median difference 25 days, 9 to 46 days) and a longer stay in hospital (median difference 57 days, 29 to 95 days); were more likely to develop intestinal failure (81% (25 infants) v 41% (102); relative risk 1.96, 1.56 to 2.46) and liver disease associated with intestinal failure (23% (7) v 4% (11); 5.13, 2.15 to 12.3); and were more likely to require unplanned reoperation (42% (13) v 10% (24); 4.39, 2.50 to 7.70). Compared with infants managed with primary fascial closure, those managed with preformed silos took longer to reach full enteral feeding (median difference 5 days, 1 to 9) and had an increased risk of intestinal failure (52% (50) v 32% (38); 1.61, 1.17 to 2.24). Event rates for the other outcomes were low, and there were no other significant differences between these management groups. Twelve infants died (4%). CONCLUSIONS: This nationally representative study provides a benchmark against which individual centres can measure outcome and performance. Stratifying neonates with gastroschisis into simple and complex groups reliably predicts outcome at one year. There is sufficient clinical equipoise concerning the initial management strategy to embark on a multicentre randomised controlled trial comparing primary fascial closure with preformed silos in infants suitable at presentation for either treatment to determine the optimal initial management strategy and define algorithms of care.

Hypertrophic pyloric stenosis: predicting the resolution of biochemical abnormalities.

PURPOSE: Hypertrophic pyloric stenosis (HPS) is a common condition of infancy, often presenting with marked biochemical derangement, requiring correction. Previous studies have looked at the relationship between serum electrolytes and acid-base balance in HPS but not at the relationship between the degree of biochemical derangement and time taken to resolve the biochemical abnormality. METHODS: Retrospective analysis was performed on all 151 infants undergoing pyloromyotomy over a 3 year period. Of these, 105 met the inclusion criteria of: compliance with the unit HPS fluid protocol, and the documentation of at least three serial biochemical investigations. The rate of correction for each biochemical marker (sodium, potassium, chloride, urea, pCO2, hydrogen ion concentration, bicarbonate and the base excess) was plotted against the degree of disturbance and then against time. RESULTS: A significant relationship (P < 0.01) was found between the rate of correction of an abnormal chloride, urea or base excess and the degree of initial derangement. This enables the prediction of the time taken for the required correction of biochemical abnormalities prior to theatre. CONCLUSION: This method of analysis may be of value in comparing the effectiveness of different fluid regimes in use for the correction of biochemical abnormalities in infants with IHPS.

Gastroschisis: a national cohort study to describe contemporary surgical strategies and outcomes.

BACKGROUND: Information on adoption of newer surgical strategies for gastroschisis and their outcomes is largely limited to hospital-based studies. The aim of this study was to use a new UK national surveillance system to identify cases and thus to describe the contemporary surgical management and outcomes of gastroschisis. METHODS: We conducted a national cohort study using the British Association of Paediatric Surgeons Congenital Anomalies Surveillance System to identify cases between October 2006 and March 2008. RESULTS: All 28 surgical units in the United Kingdom and Ireland participated (100%). Data were received for 95% of notified cases of gastroschisis (n = 393). Three hundred thirty-six infants (85.5%) had simple gastroschisis; 45 infants (11.5%) had complex gastroschisis. For 12 infants (3.0%), the type of gastroschisis could not be categorized. Operative primary closure (n = 170, or 51%) and staged closure after a preformed silo (n = 120, or 36%) were the most commonly used intended techniques for simple gastroschisis. Outcomes for infants with complex gastroschisis were significantly poorer than for simple cases, although all deaths occurred in the simple group. CONCLUSIONS: This study provides a comprehensive picture of current UK practice in the surgical management of gastroschisis. Further follow-up data will help to elucidate additional prognostic factors and guide future research.

Contemporary postnatal surgical management strategies for congenital abdominal wall defects.

Early definitive closure of abdominal wall defects is possible in most cases. Staged reduction does offer distinct advantages, and mortality and morbidity may be better. Risk stratification may produce outcome and tailor management of difficult cases in the form of a clinical pathway. Stem cell technology may, in the future, offer the ideal allogenic prosthesis in complex cases.

Experience of bedside preformed silo staged reduction and closure for gastroschisis.

AIM: The purpose of this study was to assess the effectiveness of routine staged reduction and closure at the bedside, using preformed silos with no general anesthesia (PSnoGA), compared to emergency operative fascial closure (OFC) under general anesthesia for gastroschisis (GS). METHODS: A retrospective matched case-control analysis of neonates with GS was performed between 1990 and 2004 inclusively. Assessment included demographics, method of closure, days on ventilator, days to first enteral feed, days to full oral feeds, days on parenteral nutrition, length of hospital stay, and complications. RESULTS: Sixty-five patients with GS were treated in our institution between 1990 and 2004. Thirty-five underwent OFC, 4 had Bianchi ward reduction, and 26 received PSnoGA. Seventeen patients with bowel perforation, atresia, ward reduction, medical complications necessitating ventilation, or any other condition requiring urgent surgical intervention were excluded from the analysis. Patients were well matched for gestation and birth weight. Forty-eight patients (OFC = 27 and PSnoGA = 21) were compared by using Mann-Whitney U test. Median days on ventilator (4 vs 0; P < or = .0001) was significantly reduced, but there was no difference for days to full oral feeds (26 vs 31; P = .26), days on parenteral nutrition (25 vs 30; P = .28), and length of stay (32 vs 36; P = .32), respectively. Complications were similar for both groups. CONCLUSIONS: PSnoGA has outcomes statistically similar to OFC, although days on ventilator are significantly reduced. Slow reduction of the bowel avoids abdominal compartment syndrome and closure may be achieved without fascial sutures. PSnoGA is performed at the bedside and aims to avoid general anesthesia, a period of ventilation, and out-of-hours operating, thereby reducing costs. A prospective, multicenter, randomized control trial is needed to evaluate the effectiveness of PSnoGA.

Traction-compression-closure for exomphalos major.

PURPOSE: We present our experience with traction-compression-closure (TCC) for exomphalos major (EM) to achieve a safe and embryologically correct midline supraumbilical aesthetic closure with preservation of the umbilicus. METHODS: Nineteen neonates with EM were paralyzed and ventilated. The abdominal domain was increased by upward cord traction to assist liver-bowel reduction by gravity and sac ligation, followed by circumferential elastic body binder compression. The supraumbilical abdominal wall anomaly cicatrized spontaneously or was closed surgically as a midline scar, with preservation of the umbilicus. RESULTS: Over 7 years (1998-2004), 19 patients with EM were treated by TCC, 18 of whom survived. The patients' median gestational age was 36 weeks (range, 24-40 weeks); their median birth weight was 2312 g (range, 890-3000 g). The median time to reduction was 4 days (range, 3-5 days), whereas that to full enteral feeds was 6 days (range, 4-6 days). Mechanical ventilation for 7 days (range, 6-8 days) was not associated with any morbidity, and the time to home discharge was 11 days (range, 8-12 days). Five patients did not require any surgery. There was no episode of sac rupture or infection. CONCLUSION: Abdominal expansion by vertical cord traction followed by compression reduction (TCC) under muscle relaxation and ventilation is time well spent toward a safe and aesthetic midline abdominal wall closure without tension for EM.

Interval laparoscopic appendectomy in children.

BACKGROUND: Conservative management of advanced complicated appendicitis in children is becoming more common. Formation of an appendiceal mass or abscess may mitigate against urgent appendectomy during the acute stage, and conservative treatment followed by interval appendectomy has been advocated. We present our experience of interval laparoscopic appendectomy in our institution. MATERIALS AND METHODS: All children who were offered interval laparoscopic appendectomy between January 2000 and December 2004 were included. Retrospective case note analysis was performed and data collected included demographics, duration of symptoms, method of diagnosis, days of antibiotics, length of interval, operative time, length of hospital stay (conservative treatment and interval laparoscopic appendectomy), analgesia requirements, complications, and histology. RESULTS: Thirty-six children, median age 8 years (range, 1-15 years) diagnosed with appendiceal mass or abscess were offered interval laparoscopic appendectomy by two surgeons in our institution: one patient declined interval laparoscopic appendectomy. Median antibiotic treatment was 10 days (range, 3-23 days). Median length of stay for conservative treatment was 6 days (range, 1-27 days). Five children required percutaneous drainage. For the 35 children who had interval laparoscopic appendectomy, the median interval was 93 days (range, 34-156 days) and median operative time was 55 minutes (range, 25-120 minutes). Median length of stay for interval laparoscopic appendectomy was 1 day (range, 1-3 days). There were no complications following interval laparoscopic appendectomy. CONCLUSION: Interval laparoscopic appendectomy can be safely performed in children, is associated with a short hospital stay and minimal morbidity, analgesia, and scarring following conservative management of appendiceal mass or abscess. Interval laparoscopic appendectomy eliminates the risk of recurrent appendicitis and serves to excise undiagnosed carcinoid tumors. In future it may be possible to perform interval laparoscopic appendectomy as a day-case procedure in selected patients.

Surgery for intestinal perforation in preterm neonates: anastomosis vs stoma.

BACKGROUND: Traditionally, a stoma is established after resection of perforated or necrotic intestine for isolated intestinal perforation (IIP) and necrotizing enterocolitis (NEC). We compared the outcome of resection and anastomosis (RA) with stoma formation (RS). METHODS: Sixty-eight neonates undergoing laparotomy for IIP (n = 20), NEC (n = 43), and indeterminate cause (n = 5) were reviewed retrospectively. Intestinal resection was followed by either anastomosis or stoma. The primary outcome measure was the frequency of anastomosis- and stoma-related complications. RESULTS: The median gestational age (GA) was 28.5 weeks and birth weight (BW) was 940 g. Thirty-seven neonates had RA (NEC 22, IIP 14, 1 unknown), 28 RS (NEC 21, IIP 6, 1 unknown), and 3 laparotomy only. Twenty-five neonates died postoperatively. The mean +/- SD GA of those who survived was 30 +/- 4.5 weeks and those who died was 27.2 +/- 3.5 weeks (P = .008). The mean BW for those that survived was 1440.5 +/- 865.1 g and those who died was 827.7 +/- 385.1 g (P = .002). There was no statistically significant difference between the RA and RS groups for GA (P = .93), BW (P = .4), general complications (P = .96), anastomosis and stoma complications (P = .48), and deaths (P = .42). CONCLUSIONS: RA, rather than stoma, is an acceptable option in the surgical management of preterm neonates with IIP or NEC.