Association Between Administration of Antacid Medication and Anastomotic Stricture Formation After Repair of Esophageal Atresia.

BACKGROUND: Anastomotic stricture is a significant cause of morbidity after repair of esophageal atresia (EA). Exposure to gastric acid has been postulated to contribute to stricture development and severity leading to prophylactic antacid use by some surgeons. We investigated the association between administration of antacid medication and the development of anastomotic strictures. METHODS: Retrospective case-note review of consecutive infants undergoing repair of EA with distal tracheoesophageal fistula (type C) between January 1994 and December 2014. Only infants who underwent primary esophageal anastomosis at initial surgical procedure were included. Stricture-related outcomes were compared initially for infants who received prophylactic antacid medication (PAAM) versus no prophylaxis, and the role of PAAM in stricture prevention was explored in a multivariate model. Outcomes were also compared for infants grouped by antacid use at any stage. RESULTS: One hundred fourteen infants were included. Sixteen infants received PAAM at surgeon preference. Of the remaining 98 infants, 44 subsequently received antacid as treatment for gastroesophageal reflux (GER) and 54 never received antacid medication. There was no statistically significant association between incidence of stricture in the first year (10 of 16 versus 41 of 98; P = 0.18) nor time to first stricture (median, 57 d [41-268] versus 102 d [43-320]; P = 0.89) and administration of PAAM. Similarly, there were no statistically significant associations between incidence of stricture, age at first stricture and number of dilatations, and administration of antacid medication either as prophylaxis nor when given as treatment for symptoms or signs of GER. CONCLUSIONS: These data do not support the hypothesis that PAAM reduces the incidence or severity of anastomotic stricture after repair of EA. Treatment with antacids may be best reserved for those with symptoms or signs of GER. Further prospective investigation of the role of antacid prophylaxis on stricture formation after EA repair is warranted.

Growth pattern of infants with gastroschisis in the neonatal period.

BACKGROUND/AIM: Early postnatal growth patterns may have significant long term health effects. Although preterm infants on parenteral nutrition (PN) exhibit poor growth, growth pattern of term or near-term infants requiring PN is not well reported. We aimed to investigate this in infants born with gastroschisis. METHODS: Retrospective review of all infants with gastroschisis requiring PN treated at a single centre over a 4 year period. Growth and clinical data were retrieved, and weight SDS scores for corrected gestational age calculated. Weight SDS (mean ± SD) were compared at clinically relevant timepoints and multi-level regression used to model growth trends over time. MAIN RESULTS: During the study period 61 infants with gastroschisis were treated; all were included. Infants were small for gestational age at birth for weight (SDS score -0.87 ± 0.85). Weight SDS decreased significantly during the first 10 days of age (mean decrease 0.81 ± 0.56; p < 0.0001) and between birth and discharge (mean decrease 0.81 ± 0.56; p < 0.0001). Despite tolerating full enteral feeds, weight SDS velocity was negative around the time of transition from parenteral to enteral feed. There was evidence of 'catch up' growth between 3 and 6 months of age. CONCLUSION: Despite nutritional support with PN, infants with gastroschisis demonstrate significant growth failure during the newborn period. Further efforts are required to understand the underlying mechanisms, improve nutritional support and to evaluate the long term consequences of postnatal growth failure in this population.

Esophageal replacement by gastric transposition: A single surgeon's experience from a tertiary pediatric surgical center.

BACKGROUND: Many pediatric surgeons have limited experience of esophageal replacement. This study reports outcomes of esophageal replacement by gastric transposition performed by a single UK-based pediatric surgeon. METHODS: Consecutive patients were identified who underwent esophageal replacement by gastric transposition over a 28 year period. Clinical and demographic data were collected. Weight-for-age Z-scores were calculated for esophageal atresia patients. RESULTS: Nineteen patients were identified. Indication in the majority was long-gap esophageal atresia (n = 17; 10 with tracheoesophageal fistula). At surgery, median age was 8.5 months (range 2-55); median weight was 7.4 kg (range 4.0-17.4 kg). A right-sided thoracotomy or transhiatal approach was used. Median postoperative length of stay was 17.5 days (range 7-130); median intensive care stay was three days (range 1-63). There were no deaths. Anastomotic leak rate at 30 days was 10.5% (n = 2). One patient required early stricture dilatation. Median weight-for-age Z-score increased from -2.17 at one year of age to -1.86, -1.70 and -1.93 at 5, 10 and 15 years. CONCLUSIONS: Esophageal replacement by gastric transposition offers a potentially life-changing treatment; however, it is associated with significant morbidity. The majority of patients eventually achieve full oral feeding and maintenance of weight gain trajectory. A right-sided approach to the esophagus is feasible. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: IV.

Active observation versus interval appendicectomy after successful non-operative treatment of an appendix mass in children (CHINA study): an open-label, randomised controlled trial.

BACKGROUND: Despite a scarcity of supporting evidence, most surgeons recommend routine interval appendicectomy after successful non-operative treatment of an appendix mass in children. We aimed to compare routine interval appendicectomy with active observation. METHODS: We enrolled participants in the CHildren's INterval Appendicectomy (CHINA) study, a multicentre, open-label, randomised controlled study at 19 specialist paediatric surgery centres, 17 of which were in the UK, one in Sweden, and one in New Zealand. 106 children aged 3-15 years were assigned (1:1) by weighted minimisation to interval appendicectomy or active observation with minimisation for age, trial centre, sex, and presence of a faecolith on imaging. Eligible children had acute appendicitis with an appendix mass and were successfully treated without appendicectomy or other surgical intervention. Children were excluded from the study if they had coexisting gastrointestinal disease or had a substantial coexisting medical condition or immune defect. Because of the nature of the interventions, blinding was not possible. The primary outcome was the proportion of children developing histologically proven recurrent acute appendicitis or a clinical diagnosis of recurrent appendix mass within 1 year of enrolment after successful non-operative treatment of appendix mass (active observation group) and incidence of severe complications related to interval appendicectomy. Data were analysed on an intention-to-treat basis. This study is registered with ISRCTN, number 93815412. FINDINGS: Between Aug 8, 2011, and Dec 31, 2014, we randomly assigned 106 patients, 52 patients to interval appendicectomy and 54 to active observation. Two children in the interval appendicectomy group were withdrawn due to withdrawal of consent; two in the active observation group were withdrawn because they became ineligible after allocation. Six children under active observation had histologically proven recurrent acute appendicitis. Three children in the interval appendicectomy group had severe complications. Thus, the proportion of children with histologically proven recurrent acute appendicitis under active observation was 12% (95% CI 5-23) and the proportion of children with severe complications related to interval appendicectomy was 6% (95% CI 1-17). INTERPRETATION: More than three-quarters of children could avoid appendicectomy during early follow-up after successful non-operative treatment of an appendix mass. Although the risk of complications after interval appendicectomy is low, complications can be severe. Adoption of a wait-and-see approach, reserving appendicectomy for those who develop recurrence or recurrent symptoms, results in fewer days in hospital, fewer days away from normal daily activity, and is cheaper than routine interval appendicectomy. These high-quality data will allow clinicians, parents, and children to make an evidence-based decision regarding the justification for interval appendicectomy. FUNDING: BUPA Foundation.

Intrauterine death rate in gastroschisis following the introduction of an antenatal surveillance program: Retrospective observational study.

AIM: To investigate whether an antenatal surveillance protocol including ultrasound and cardiotocograph monitoring reduces intrauterine death (IUD) in cases of gastroschisis. Secondary outcomes included neonatal death rate, mode of delivery and rate of intervention before planned time of delivery. METHODS: This was a retrospective observational study of all women with antenatally diagnosed gastroschisis who were managed according to the surveillance program between 2002 and 2015 in a tertiary fetal medicine and pediatric surgical center covering the Wessex region of England. We reviewed and analyzed data from the Wessex Antenatally Detected Anomalies (WANDA) database as well as prospectively managed maternity, ultrasound and neonatal databases over the given time period. Case notes were reviewed when delivery was expedited. RESULTS: The IUD rate was 2.2%, a 58% reduction since the introduction of the surveillance protocol. Delivery was expedited in 35.4% of cases, and in 86% of these, delivery was by cesarean section. In women being induced as planned at 38 weeks, the vaginal delivery rate was 88%, and for those in spontaneous labor before 38 weeks it was 75%. CONCLUSIONS: An antenatal surveillance program appears to reduce the IUD in gastroschisis. In one-third of cases, delivery was indicated before the planned date of delivery. When expedited delivery was indicated, the chance of cesarean section was high.

Oesophageal atresia with no distal tracheoesophageal fistula: Management and outcomes from a population-based cohort.

PURPOSE: To describe the incidence and outcomes to one-year in infants born with oesophageal atresia (OA) with no distal tracheoesophageal fistula within a population cohort. METHODS: A subgroup analysis of a prospective multicentre population cohort study was undertaken describing the outcomes of infants with OA and no tracheoesophageal fistula, (type A) and those with only an upper pouch fistula, (type B). MAIN RESULTS: Twenty-one of 151 infants in the whole cohort were diagnosed with type A or B oesophageal atresia (14%). Fifteen were type A (71%) and six type B (29%). Infants with type B had a shorter gap length than those with type A: 2.5 vertebral bodies (2-3) vs. 5 (4-6) (p=0.008). All infants with type B OA underwent oesophageal anastomosis, 83% (n=5) as the primary procedure. All infants with type A, underwent staged management. Six (40%) had delayed primary anastomosis and eight required oesophageal replacement (53%). One infant died prior to reconstruction. The median time to delayed primary anastomosis in infants with type A or B OA was 82days (75-89days) (n=7). The median time to oesophageal replacement was 94days (89-147days) (n=8). Median length of stay for infants with type A or B OA from first operation to first discharge was 101days (31-123days). CONCLUSIONS: Infants with type B OA had a shorter gap length and all were managed with oesophageal anastomosis. OA with no distal tracheoesophageal fistula is uncommon at a population level and frequently has a complex course. LEVEL OF EVIDENCE: Rating: II.

Antenatally diagnosed right-sided stomach (dextrogastria): A rare rotational anomaly.

AIM: Antenatal detection of right-sided stomach (dextrogastria) is rare, and its significance in regards to intestinal rotation is unclear. We aimed to review all cases of antenatally-diagnosed dextrogastria in our regional fetal medicine unit over 10years. METHODS: A retrospective case-note review of patients identified from a prospectively-maintained database was performed. RESULTS: Twenty cases of antenatally-diagnosed dextrogastria were identified from 2004 to 2014. There were 8 terminations and 1 intra-uterine death. One patient has no post-natal information obtainable. Ten infants were live-born, and 2 died secondary to cardiac disease in the neonatal period. All had significant cardiac/vascular anomaly on postnatal assessment, including the 3 neonates in whom dextrogastria was the only antenatal finding. Two neonates developed bilious vomiting and underwent Ladd's procedure. Operative findings were dextrogastria/malrotation in both. A third child had gastro-oesophageal reflux, and contrast demonstrated stable duodenal/midgut position. This child has not developed symptoms attributable to malrotation and not undergone surgery. All 3 of these infants had asplenia or polysplenia and were managed with antibiotic prophylaxis/immunisation. Five children in the series were not investigated for malrotation and have not come to surgical attention (one is known to be asplenic). CONCLUSION: Antenatally-detected dextrogastria, even if apparently isolated, was always associated with postnatal significant cardiovascular anomaly, splenic abnormality or situs inversus. This may be important for antenatal counselling. We currently recommend postnatal echocardiography and splenic assessment, but reserve GI investigation/intervention for symptomatic malrotation owing to potential significant cardiac comorbidity.

Conservative management of antenatally diagnosed cystic lung malformations.

AIM: To review the outcome of all antenatally diagnosed conservatively managed congenital lung malformations (CLMs) managed at our centre. METHODS: All patients diagnosed antenatally with cystic lung malformations from 2001 to 2011, at a tertiary referral paediatric surgical centre practising a policy of conservative management of asymptomatic cases, were retrospectively reviewed. Data were collected from medical case notes and radiology reports. Ethical approval was obtained from our institutional research and development department. RESULTS: The complete records of 74 fetuses antenatally diagnosed with CLM were reviewed. There were 72 live births, at a median gestation of 39.6 weeks. Emergency lobectomy was performed in one symptomatic neonate. Elective lobectomies were performed at parental request in three asymptomatic infants, one of whom had a family history of synovial sarcoma. Two patients developed pneumonia in the affected lobe during early childhood and proceeded to lobectomy at the age of 3 years. One patient with a bronchopulmonary sequestration required embolisation for cyanotic episodes. The remaining 65 patients have been conservatively managed to date, and none have required hospital admission. Less than a quarter report mild respiratory symptoms such as cough or wheeze. Median follow-up is 5 years. CONCLUSIONS: This retrospective cohort study of 74 consecutive CLMs diagnosed antenatally over a 10-year period demonstrates that most of these lesions will remain asymptomatic throughout childhood. Although the natural history of CLMs in later years remains to be elucidated, we hope that this report on medium-term outcomes will be useful to clinicians who undertake antenatal counselling and may inform the discussion on how best to manage these children.

Operative intercostal chest drain is not required following extrapleural or transpleural esophageal atresia repair.

BACKGROUND: Approximately half of the United Kingdom patients undergoing esophageal atresia (OA) repair have an operative intercostal chest drain (ICD) placed (2008 British Association of Pediatric Surgeons Congenital Anomalies Surveillance Study data). We reviewed our experience of OA repairs to evaluate if an ICD placement is necessary. METHODS: Patients with OA/distal tracheoesophageal fistula (TOF), treated between January 1990 and January 2010, were identified by retrospective review of a prospectively maintained electronic database and patient case notes. MAIN RESULTS: A total of 112 consecutive patients were identified, of whom 107 were included (73 male). Five were excluded as no case notes were available. Median birth weight was 2,597 g (range 924 to 4,245 g) and median gestational age was 38 weeks (27 to 41 weeks). Median age at discharge was 22 days (3 to 440 days) and median follow-up was 3.5 years (0 to 18 years). Patients were analyzed in two groups-group 1 (n = 73) had an extrapleural (EP) repair, of which 23 had a pleural breach and group 2 (n = 34) had a purposeful transpleural (TP) approach (surgeon preference). Eleven patients (10%) had an operative ICD, of which six patients were in group 1 and five in group 2. These 11 patients had an uncomplicated postoperative course and all operative ICD were removed within 48 hours of surgery. Of the 96 patients that did not have an operative ICD, only 2 (2%) required postoperative intervention. One patient, in group 2, had a postoperative ICD inserted for a simple pneumothorax at 12 hours and removed at 48 hours. The other patient, in group 1, had a clinically detected anastomotic leak after 48 hours and required operative repair. CONCLUSION: An operative ICD is not required following OA/distal TOF repair, whether the approach is EP or TP. ICD that were electively placed (in 10%) served no clinical purpose.

The use of mouth brushings for screening girls who present with inguinal hernia for complete androgen insensitivity syndrome.

INTRODUCTION: Published guidance recommends that all girls with inguinal hernia should be screened for complete androgen insensitivity syndrome (CAIS). We report a novel, noninvasive screening technique. METHODS: Retrospective review of all girls undergoing inguinal herniotomy from April 2009 to October 2010. Those screened using the novel technique of extraction of Y chromosome specific DNA from a buccal mucosal sample obtained by mouth brushing are reported. RESULTS: A total of 29 girls were screened by mouth brushing at median age 2.9 years (range 29 days to 9.3 years). Of the 29 samples, 25 were adequate for DNA extraction; 4 were inadequate and screening was repeated (3 repeat mouth brushing, 1 perioperative blood test). Mouth brushing was well tolerated by children and acceptable to parents. A preoperative blood test was avoided in all girls who had a mouth brushing. None of the girls in this study had CAIS. Turn-around time for mouth brushing was mean 4.9 days compared with a minimum of 10 days for a karyotype. This technique is cheaper than a karyotype (£ 87 vs. £ 205). CONCLUSION: Extraction of Y chromosome specific DNA from a mouth brushing sample is effective for screening girls with inguinal hernia for CAIS. It is acceptable, cheaper, and quicker than alternatives.

Ten-year experience of home parenteral nutrition in a single centre.

AIM: Recent data are scarce on the provision of home parenteral nutrition (HPN) in children from the UK but would help to commission intestinal failure services. Our aim was to describe 10 years of HPN experience in our centre, which serves a population of 650,000 children. METHODS: Outcome and complication data were collected retrospectively from hospital records of children receiving HPN from April 2001. Data from other centres were used to compare complications and outcomes in the provision of HPN. RESULTS: Nineteen children (12 females) received 10,213 days (28 years) of HPN. In this group, incidence of blood culture positive sepsis was four episodes/1000 days PN. Two children had early intestinal failure-associated liver disease. Of the 19, seven still receive HPN at our centre, six survived PN, three were transferred to other services while still on HPN and three died. CONCLUSION: Outcome and complication data for HPN from a single UK regional paediatric centre are similar to larger centres. These data provide recent evidence of the disease burden of HPN, which are important for the commissioning of intestinal failure services.

Workload and costs associated with providing a neonatal surgery service.

AIM: To identify the workload related to provision of a neonatal surgical service in a UK neonatal network in order to inform local and national service commissioning. METHOD: Data relating to neonatal surgical admissions to a level 3 perinatal centre serving a network with 36,000 births per year collected prospectively over a 5-year period were analysed to identify annual activity. Daily dependency was assessed prospectively over a 6-month period and service costs calculated using existing local tariffs. Admissions from outside the network were excluded from analysis, and allowance was made for refused network admissions. RESULTS: On average 140 admissions required 2137 cot-days per year. At 80% occupancy, the service requires seven neonatal cots suggesting that there is a national requirement for one neonatal surgical cot per 5000 births. Intensive care, high care (HC) and special care accounted for 37%, 46% and 17% of cot-days, respectively. This equates to an annual service cost of £2m, about £250,000 per 5000 births. CONCLUSIONS: This assessment of the facilities and costs required to provide a neonatal surgical service in a level 3 perinatal centre in the UK may be used to inform network and national commissioning.

Is interval appendicectomy justified after successful nonoperative treatment of an appendix mass in children? A systematic review.

BACKGROUND/PURPOSE: Interval appendicectomy (IA) is commonly performed after successful nonoperative treatment of appendix mass (AM); although, this approach has recently been challenged. We systematically reviewed the pediatric literature with regard to the justification for this practice. METHODS: Using a defined search strategy, studies were identified and data were extracted independently by 2 reviewers. Incidences of recurrent appendicitis, complications after IA, and carcinoid tumor were estimated accounting for interstudy heterogeneity. Cost and length of stay of IA were analyzed. RESULTS: Three studies (127 cases) reporting routine nonsurgical treatment were identified; all were retrospective. There was marked interstudy heterogeneity and variable follow-up. After successful nonoperative treatment of AM, the risk of recurrent appendicitis is 20.5% (95% confidence interval [CI], 14.3%-28.4%). The incidence of complications after IA (23 studies, n = 1247) is 3.4% (95% CI, 2.2-5.1), and the incidence of carcinoid tumor found at IA (15 studies, n = 955) is 0.9% (95% CI, 0.5-1.8). No reports compared costs. Mean length of stay for IA was 3 days (range, 1-30 days). CONCLUSIONS: Prospective studies comparing routine IA with nonoperative treatment without IA in children are lacking. Available data suggest that 80% of children with AM may not need IA. A prospective study to evaluate the natural history of this condition compared with the morbidity and costs of IA is warranted.

Late-onset volvulus without malrotation in preterm infants.

PURPOSE: The aim of the study was to report a new observation of late-onset volvulus without malrotation (VWM) in preterm infants. METHODS: The study used medical note review of infants with VWM identified at a single regional centre between 1996 and 2007. RESULTS: Ten patients were identified. Group 1 includes 4 patients (gestation, 28-38 weeks; median, 32) who presented within 4 days of age (range, 1-4; median, 3). Group 2 includes 6 patients (gestation, 25-33 weeks; median, 27), who presented later (range, 22-57 days; median, 45). Characteristics of group 2 patients included recurrent episodes of abdominal distension and bile vomiting (6/6), long-term continuous positive airway pressure requirement (5/6), and sudden, severe deterioration with acute abdominal signs (6/6). Small bowel volvulus was found at laparotomy requiring resection (30%-70% of total small bowel) and either primary anastomosis (4) or stoma formation (2). All babies survived. CONCLUSIONS: There appear to be 2 clinical groups with VWM-one presenting within the first few days of life and the other presenting after the first month of life associated with a specific clinical history. This latter group has not been described before.

Gastroschisis: the cost of an epidemic.

BACKGROUND: The rising incidence of gastroschisis has been highlighted by the Department of Health as a growing concern. As well as the health implications for the increasing number of affected infants, this increase in incidence will have an impact of the costs of health care. This study was undertaken to estimate the financial cost of treating this condition in one tertiary neonatal surgical center. METHODS: A retrospective analysis was performed of all patients admitted to a tertiary neonatal surgical center with gastroschisis from January 1996 to December 2005. The main outcome measures were incidence, length of hospital stay, and total cost for all patients each year. RESULTS: The incidence of gastroschisis has risen 3-fold in 10 years. The median cost per patient is relatively constant. A few patients with severe intestinal dysmotility require prolonged hospital stay. As the condition becomes more common, there are an increasing number of complex patients and thus an increase in annual costs, which is disproportionate to the increase in numbers of cases. We estimate that the annual cost to the National Health Service (NHS) of this condition in England and Wales has risen from pound3.6 million in 1996 to in excess of pound15 million in 2005. CONCLUSIONS: Urgent research is required into the etiology of gastroschisis and into the severe intestinal dysmotility that occurs in some complex patients.

Operating and anaesthetic time required for inguinal hernia repair in the neonate.

Inguinal hernia repair in neonates can be a challenging operation and anaesthetic. However, there is no information on how much anaesthetic and operating time is required for such surgery. Planning operating time and resources requires this knowledge. This study looked at 94 neonatal hernia repairs and compared anaesthetic and operating times for these patients with 297 day-case hernia repairs. The median anaesthetic and operating times for neonates with unilateral repair were 20 and 45 min compared with 13 and 30 min for day-cases. However, the ranges for anaesthetic time (10-185 min) and operating time (20-170 min) were considerably in excess of those for day-cases (0-54 and 15-80 min) respectively. Neonatal hernia operations accounted for a total of one theatre list per month in our centre.