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.

Nutritional role of amniotic fluid: clues from infants with congenital obstruction of the digestive tract.

AIMS: To investigate the role played by amniotic fluid in late fetal nutrition by analysis of infants born with digestive tract atresia. METHODS: Birth weight (BW), gestational age and gender of infants born with oesophageal (OA), duodenal (DA), jejunal (JA) and ileal atresia (IA) were recorded and BW Z-scores compared. Infants with incomplete obstruction (stenosis), chromosomal or syndromic conditions and multiple congenital malformations were excluded. Term infants admitted with suspected postnatal intestinal obstruction in whom no congenital malformation was found were used as a control group. RESULTS: A total of 584 infants were identified comprising 148 OA, 60 DA, 26 JA and 57 IA with 293 in the control group. Infants with OA and DA had statistically significantly lower BW Z-score than controls. However, BW Z-score for infants with more distal atresia (JA and IA) was similar to controls. When compared with infants with OA, BW Z-score for infants with more distal atresia was higher than that for OA. BW Z-score in infants with OA was significantly lower in those born at term compared with those born preterm (mean±SD -0.92±1.0 vs -0.48±0.87; p=0.01) with a significant negative correlation between BW Z-score and increasing gestational age (R2=0.12; p<0.0001). This effect of gestational age was not seen in other atresias. CONCLUSION: These observations support the concept that reduced enteral absorption of amniotic fluid due to high digestive tract obstruction in utero reduces fetal growth. The effect is greater when the obstruction is more proximal and with advancing gestation.

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.

Paleolimnology and resurrection ecology: The future of reconstructing the past.

Paleolimnologists have utilized lake sediment records to understand historical lake and landscape development, timing and magnitude of environmental change at lake, watershed, regional and global scales, and as historical datasets to target watershed and lake management. Resurrection ecologists have long recognized lake sediments as sources of viable propagules ("seed or egg banks") with which to explore questions of community ecology, ecological response, and evolutionary ecology. Most researchers consider Daphnia as the primary model organism in these efforts, but many other aquatic biota, from viruses to macrophytes, similarly produce viable propagules that are incorporated in the sediment record but have been underutilized in resurrection ecology. The common goals shared by these two disciplines have led to mutualistic and synergistic collaborations-a development that must be encouraged to expand. We give an overview of the achievements of paleolimnology and the reconstruction of environmental history of lakes, review the untapped diversity of aquatic organisms that produce dormant propagules, compare Daphnia as a model of resurrection ecology with other organisms amenable to resurrection studies, especially diatoms, and consider new research directions that represent the nexus of these two fields.

Emergency laparotomy in infants born at <26 weeks gestation: a neonatal network-based cohort study of frequency, surgical pathology and outcomes.

OBJECTIVE: Identify the proportion of infants born at <26 completed weeks' gestation who require emergency laparotomy, and review the surgical pathology, incidence of subsequent surgical procedures and outcome. DESIGN: Retrospective cohort review. SETTING: Tertiary neonatal surgical unit. PATIENTS: All infants born at <26 weeks' gestation in a neonatal network over an 8-year period. RESULTS: Of 381 infants, laparotomy was indicated in 61 (16%) and performed in 57. Surgical pathology encountered included spontaneous intestinal perforation (SIP) (28), necrotising enterocolitis (NEC) (14), volvulus without malrotation (1), strangulated inguinal hernia (1), milk curd obstruction (4), NEC stricture (1) and meconium obstruction of prematurity (2). No intestinal pathology was found in six. Four infants with indications for laparotomy and severe comorbidity had intensive care withdrawn without surgery. The most frequent procedure performed was resection with primary anastomosis. Nine infants (16%) required more than one laparotomy. Of the 16 infants who had stoma formation, eight had closure before discharge. Fifteen infants required surgical patent ductus arteriosus ligation following laparotomy, and 17 had laser therapy for retinopathy of prematurity. Overall 42 infants with indication for laparotomy (69%) survived to discharge. CONCLUSIONS: Nearly one in six infants born at <26 weeks required emergency laparotomy. The most frequent pathology encountered was SIP (49%), followed by NEC (25%). Over one-quarter required subsequent gastrointestinal surgery, with many also requiring cardiothoracic and ophthalmic procedures. These data are important for those caring for extremely preterm infants, the provision of information to parents and organisation of neonatal services.

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.

The burden of excluding malrotation in term neonates with bile stained vomiting.

PURPOSE: To determine the number of term infants with bilious vomiting (BV) referred to a neonatal surgical centre for exclusion of malrotation by upper gastrointestinal contrast (UGI) examination. METHODS: Retrospective review of term (>37/40) neonates <28 days of age undergoing UGI for exclusion of malrotation between Jan 2010 and Dec 2014 in a neonatal network with 30,000 term deliveries annually. Only infants with BV in the absence of alternative clinical/radiological diagnosis were included. RESULTS: One hundred and sixty-six infants met the inclusion criteria. Fourteen (9 %) infants had malrotation diagnosed by UGI and confirmed at laparotomy. Only 1 of 110 infants referred at 0-2 days of age had positive UGI compared to 13 of 56 infants referred after this age (p < 0.01). An increase in referrals followed the death of an infant from midgut volvulus and as a result one in 500 term infants are currently being referred. CONCLUSION: Increasing awareness of the potential consequences of bilious vomiting appears to have resulted in increased referrals with no increase in detection of malrotation. Prospective studies are required to determine whether investigation of all infants with unexplained bilious vomiting is required and if it is possible to select cases for surgical referral.

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.

Do infants with gastroschisis may have a high incidence of non-IgE-mediated cow's milk protein allergy?

BACKGROUND/PURPOSE: To determine the frequency and characteristics of suspected cow's milk protein allergy (CMPA) in infants with gastroschisis and response to change in milk. METHODS: A retrospective cohort study of 111 consecutive infants with gastroschisis. RESULTS: 64 episodes suggesting non-IgE-mediated CMPA occurred in 50 infants (45 %) at a median age of 44 days (9-186) and during the primary admission in 38 %. At the time of the episode the infant feed was breast milk (BM, n = 24), term formula (TF, n = 20) or extensively hydrolysed formula (EHF, n = 6). The feed was changed to EHF (34), amino acid formula (AAF) (14) or BM with maternal CMP-free diet (2). Partial or complete resolution of symptoms occurred in all. There was histological evidence of an allergic reaction to CMP in all four infants in whom tissue was available. Recurrent episodes occurred in 13/50 infants (26 %), 10 of whom were receiving EHF. There were no recurrent episodes in infants being fed with AAF. CONCLUSION: Features suggesting non-IgE-mediated CMPA appear common in infants with gastroschisis.

Outcomes at one-year post anastomosis from a national cohort of infants with oesophageal atresia.

BACKGROUND AND AIMS: We aimed to provide a contemporaneous assessment of outcomes at one-year post oesophageal atresia/tracheoesophageal fistula (OA-TOF) repair, focussing particularly on post-operative complications. It is generally accepted that oesophageal stricture is the most common complication and causes significant morbidity. We also aimed to assess the efficacy of prophylactic anti-reflux medication (PARM) in reducing stricture formation. METHOD: A prospective, multi-centre cohort study of all infants live-born with oesophageal atresia in the United Kingdom and Ireland in 2008/9 was performed, recording clinical management and outcomes at one year. The effect of PARM on stricture formation in infants with the type-c anomaly was assessed using logistic regression analysis. RESULTS: 151 infants were live-born with oesophageal atresia in the defined reporting period, 126 of whom had the type-c anomaly. One-year follow-up information was returned for 105 infants (70%); the mortality rate was 8.6% (95% CI 4.7-14.3%). Post-operative complications included anastomotic leak (5.4%), recurrent fistula (3.3%) and oesophageal stricture (39%). Seventy-six (60%) of those with type-c anomaly were alive at one-year with returned follow-up, 57(75%) of whom had received PARM. Of these, 24 (42%) developed a stricture, compared to 4 (21%) of those who had not received PARM (adjusted odds ratio 2.60, 95% CI 0.71-9.46, p = 0.147). CONCLUSIONS: This study provides a benchmark for current outcomes and complication rates following OA-TOF repair, with oesophageal stricture causing significant morbidity. The use of PARM appeared ineffective in preventing strictures. This study creates enough doubt about the efficacy of PARM in preventing stricture formation to warrant further investigation of its use with a randomised controlled trial.

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.

Total colonic aganglionosis: a 15-year single center experience.

PURPOSE: Total colonic aganglionosis (TCA) is a rare condition, which is challenging to manage. Outcome data are sparse. We aimed to review the demographics, treatment, and outcomes of TCA in our center. PATIENTS AND METHODS: A retrospective case note review of 15 years from a single center was undertaken. RESULTS: A total of nine patients (five male) were managed. Gestational age at birth was 39 weeks (range, 32.5-41 weeks). All patients were referred with distal intestinal obstruction at a median of day 2 (range, 1-6 days) of life. Two patients were managed with a long-term stoma. One died with persistent functional obstruction (despite a ganglionic stoma). Of the nine patients, seven patients underwent staged pull-through: three Soave, three Duhamel, and one Martin procedure with no short-term complications. All patients had at least one readmission with enterocolitis, diarrhea, or high stoma output. Further procedures were required in four of the seven patients. Only one child (older than 3 years) has achieved continence. Two children (both aged 8 years) requested reformation of a stoma to manage incontinence. CONCLUSION: In this series, we observed high morbidity and poor functional outcome, which should be anticipated in TCA. Patients with TCA have a high probability of requiring a long-term stoma and this should be considered as a management option.

C-reactive protein (CRP) levels in babies with gastroschisis managed with preformed silos.

BACKGROUND/PURPOSE: The purpose of this study was to assess the degree of elevation of serum C-reactive protein (CRP) levels in infants with gastroschisis managed by placement of a preformed silo and subsequent nonoperative closure. METHODS: CRP measurements were collected prospectively in infants with gastroschisis undergoing preformed silo placement until defect closure. Infants requiring operative closure or surgical silo placement were excluded as were any infants with confirmed sepsis during the closure period. RESULTS: Data were available for 43 infants. Median CRP at birth was 8 mg/L (range 6 to 55) rising to 42 mg/L (range 35 to 68) at the time of closure. CONCLUSIONS: Elevation of serum CRP levels is to be expected in infants with gastroschisis managed with a preformed silo in the absence of infection. This data may be used to prevent unnecessary use of antibiotics in this group of patients.

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.