E M Kiely’s research while affiliated with Great Ormond Street Hospital for Children NHS Foundation Trust and other places

Purpose The STAT trial is a multicenter randomized controlled trial in 12 centers worldwide aiming to determine the most effective operation for neonates with necrotizing enterocolitis (NEC) requiring intestinal resection: stoma formation (ST) or primary anastomosis (PA). Methods Infants having a primary laparotomy for NEC were randomized intraoperatively to PA or ST if the operating surgeon thought that both were viable treatment options for that patient. The primary outcome (duration of parenteral nutrition [PN]) was evaluated by Cox regression. Results Eighty patients were recruited from 2010 to 2019. Infants undergoing anastomosis finished PN significantly earlier than patients undergoing stoma (hazard ratio PA vs. ST 2.38, 95% CI 1.36–4.12 p = 0.004). There was no difference in mortality between the two groups (PA 4/35 vs. ST 8/38 p = 0.35) or in the rate of complications requiring further unplanned operations (p = n.s.). Multiple intestinal complications were more frequent in the stoma group compared to the anastomosis group (ST 12/26 vs. PA 5/31, p = 0.02, Fisher’s Exact test). Conclusion At laparotomy for NEC, when there is no disease distal to resected intestine, primary anastomosis should be performed as it enhances the recovery from NEC, reduces the risk of multiple intestinal complications and does not increase adverse outcomes.

Objective: In gastroschisis, there is evidence to suggest that gut dysfunction develops secondary to bowel inflammation; we aimed to evaluate the effect of maternal antenatal corticosteroids administered for obstetric reasons on time to full enteral feeds in a multicenter cohort study of gastroschisis infants. Methods: A three center, retrospective cohort study (1992-2013) with linked fetal/neonatal gastroschisis data was conducted. The primary outcome measure was time to full enteral feeds (a surrogate measure for bowel function) and secondary outcome measure was length of hospital stay. Analysis included Mann-Whitney and Cox regression. Results: Of 500 patients included in the study, 69 (GA at birth 34 [25-38] weeks) received antenatal corticosteroids and 431 (GA at birth 37 [31-41] weeks) did not. Antenatal corticosteroids had no effect on the rate of reaching full feeds (Hazard ratio HR 1.0 [95% CI: 0.8-1.4]). However, complex gastroschisis (HR 0.3 [95% CI: 0.2-0.4]) was associated with an increased time to reach full feeds and later GA at birth (HR 1.1 per week increase in GA [95% CI: 1.1-1.2]) was associated with a decreased time to reach full feeds. Conclusion: Maternal antenatal corticosteroids use, under current antenatal steroid protocols, in gastroschisis is not associated with an improvement in neonatal outcomes such as time to full enteral feeds or length of hospital stay. This article is protected by copyright. All rights reserved.

Background: Long term outcomes of gastric transposition (GT) for complex esophageal atresia (EA) are poorly reported. We aimed to perform comprehensive long term follow up of adults who had been treated with GT for EA as children. Methods: Consecutive patients who underwent GT for EA in childhood aged >18 years old were identified alongside age matched patients who had primary repair (PR). Type of EA, comorbidities and details of surgery were recorded. Telephone interviews included medical history, current symptoms - including gastrointestinal symptom rating scale (GSRS), morbidity and health related quality of life (HRQoL) using gastrointestinal quality of life index (GIQLI). Results: 32 participants were interviewed in each group (mean age 29 years). BMI (19.9 ± 3.5) was significantly lower (p = 0.0006) in GT group. 6/32 (19%) still required supplementary feeding. Adult morbidity included anastomotic stricture (34%), chronic respiratory disease (28%), dumping symptoms (25%), anemia (47%) and depression (19%). 3 patients required major revision surgery. Participants in both groups report regular upper gastrointestinal symptoms (GSRS: GT = 2.1, PR = 2.0) and were more symptomatic than the normal population (1.4) but not statistically different from each other. HRQoL (GIQLI = 113) was lower than after PR (122) but not significantly different (p = 0.29) and the normal population (125). 23% of GT participants had higher than normal HRQoL. Conclusions: GT for EA is associated with significant morbidity and symptoms, including issues previously unreported in adulthood such as mental health problems. This mandates long term follow up and quality transition of these patients into adult care. Type of study: Retrospective study. Level of evidence: Level III.

Aim of the study: Divided colostomy (DC) has been recommended in anorectal malformations (ARMs) with previously reported advantages of decreasing overflow into the distal limb and urinary tract infections (UTIs). Skin bridge loop colostomy (LC) is a technically easier alternative without an increase in these complications. We report our institutional experience of LC in ARM. Methods: Retrospective study (Institution-approved Clinical Audit) reviewing the clinical records of all patients with ARM undergoing stoma formation in a single UK tertiary pediatric surgical center (2000-2015). Data collected included type of ARM, associated anomalies, type and level of colostomy, time to stoma closure, complications and UTIs. Results: One hundred and eighty-two (95 female) patients underwent colostomy formation for ARM. The vast majority (171/ 94%) underwent LC; 9 (5%) had a divided colostomy (DC) and 2 (1%) had no available data. The spectrum of defects in girls included rectovestibular (62/65%), rectovaginal (4/4%) and cloaca (29/31%). In boys, 71 (82%) had a fistula to the urinary tract and 16 (18%) presented with a perineal fistula. Urological abnormalities coexisted in 87 (47.8%) patients. Thirty five (21%) patients developed UTIs. Among the 19 girls who developed UTI, 8 had rectovestibular fistula and 11 had cloaca. Of the 16 boys who developed UTI, 14 had a fistula to the urinary tract and 11 had an independent urological abnormality. The mean time from stoma formation to stoma closure was 10 (3-52) months. Complications were reported in 22 (12%) LCs. Fifteen patients (9%) developed a stoma prolapse following LC with 10 (6%) requiring surgical revision. Conclusions: This is the largest reported series of outcomes following LC for ARM. LC is easier to perform and to close, requiring minimal surgical access, with comparable complications and outcomes to those published for DC. Type of study: Retrospective comparative study. Level of evidence: III.

Conjoined twins can be subdivided into (a) symmetrical conjoined twins, and (b) heteropagus or parasitic twins. This chapter presents a brief summary of the history of conjoined twins followed by a classification of the various types according to the site of attachment, the investigations required, and the operative procedure. The experience of the team at Great Ormond Street Hospital is presented.

Conclusion: Less than half of children with achalasia are symptom free after initial treatment with either BD or HM. HM, however, when performed as first procedure, provided longer symptom-free period and reduced need for subsequent intervention. What is Known: • Balloon dilatation (BD) and Heller's myotomy (HM) are safe and effective treatment options for achalasia. • Controversy, however, exists regarding the most effective initial therapeutic approach. What is New: • HM with or without fundoplication may represent the initial therapeutic approach of choice. • Initial BD may negatively affect the outcome of a subsequent HM.

Aim The objective of the study is to describe management of exomphalos major and investigate the effect of congenital cardiac anomalies. Methods A single-center retrospective review (with audit approval) was performed of neonates with exomphalos major (fascial defect ≥ 5cm ± liver herniation) between 2004 and 2014. Demographic and operative data were collected and outcomes compared between infants who had primary or staged closure. Data, median (range), were analyzed appropriately. Results A total of 22 patients were included, 20 with liver herniation and 1 with pentalogy of Cantrell. Gestational age was 38 (30–40) weeks, birth weight 2.7 (1.4–4.6) kg, and 13 (60%) were male. Two were managed conservatively due to severe comorbidities, 5 underwent primary closure, and 15 had application of Prolene (Ethicon Inc) mesh silo and serial reduction. Five died, including two managed conservatively, none primarily of the exomphalos. Survivors were followed up for 38 months (2–71). Cardiac anomalies were present in 20 (91%) patients: 8 had minor and 12 major anomalies. Twelve (55%) patients had other anomalies. Primary closure was associated with shorter length of stay (13 vs. 85 days, p = 0.02), but infants had similar lengths of intensive care stay, duration of parenteral feeds, and time to full feeds. Infants with cardiac anomalies had shorter times to full closure (28 vs. 62 days, p = 0.03), but other outcomes were similar. Conclusion Infants whose defect can be closed primarily have a shorter length of stay, but other outcomes are similar. Infants with more significant abdominovisceral disproportion are managed with staged closure; the presence of major cardiac anomalies does not affect surgical outcome.