David M. Burge

Contemporary management and outcomes for infants born with oesophageal atresia

Reports on the management and outcome of rare conditions, such as oesophageal atresia, are frequently limited to case series reporting single‐centre experience over many years. The aim of this study was to identify all infants born with oesophageal atresia in the UK and Ireland to describe current clinical practice and outcomes.

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.

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.

Increasing incidence of detection of congenital lung lesions

Dear Sir, Re: The incidence of congenital cystic lung lesions Priest et al. discuss the dilemmas arising in the management of congenital lung cysts in children, and states that the incidence of such lesions is somewhere between 1 in 8,000 and 1 in 30,000 live births. There is increasing concern amongst many pediatric surgeons that resection of asymptomatic cystic lung lesions in children may not be merited. Since 1990 we have seen 90 patients with confirmed or suspected cystic lung lesions. Only six of these were diagnosed postnatally and the remainder on antenatal scan. Final diagnosis after CT scan was congenital cystic adenomatoid malformation (CCAM) 45 (6 postnatal presentation), sequestration 21 and other thoracic lesion 6. In four infants the lesion was found to have resolved on CT. Fourteen patients were not referred for cross-sectional imaging by their local unit and thus a final diagnosis has not been made. The frequency with which cystic lung lesions are being diagnosed antenatally in our area has increased over the last two decades. In the 1990s we were referred 2–3 such cases per year for prenatal counseling but we are now seeing 10–12. CCAM is the final diagnosis in those undergoing cross-sectional imaging postnatally in 6–8 cases per year now. Our experience suggests an incidence of cystic lung lesions is about 1 in 3,000 live births and of CCAM at least 1 in 6,000 births. This finding has considerable implications for the management of CCAM. If resection is advocated in these cases then there will be large numbers of children undergoing lung surgery. It is likely that the true incidence of the condition has not changed over the years, just that prenatal ultrasound imaging has become more sensitive. Assuming that our reported incidence has pertained for generations, it would be expected that many more cases of infection or malignancy in CCAMs would be apparent. We do not advocate CCAM resection in asymptomatic 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.

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

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.

A UK wide cohort study describing management and outcomes for infants with surgical Necrotising Enterocolitis.

The Royal College of Surgeons have proposed using outcomes from necrotising enterocolitis (NEC) surgery for revalidation of neonatal surgeons. The aim of this study was therefore to calculate the number of infants in the UK/Ireland with surgical NEC and describe outcomes that could be used for national benchmarking and counselling of parents. A prospective nationwide cohort study of every infant requiring surgical intervention for NEC in the UK was conducted between 01/03/13 and 28/02/14. Primary outcome was mortality at 28-days. Secondary outcomes included discharge, post-operative complication, and TPN requirement. 236 infants were included, 43(18%) of whom died, and eight(3%) of whom were discharged prior to 28-days post decision to intervene surgically. Sixty infants who underwent laparotomy (27%) experienced a complication, and 67(35%) of those who were alive at 28 days were parenteral nutrition free. Following multi-variable modelling, presence of a non-cardiac congenital anomaly (aOR 5.17, 95% CI 1.9–14.1), abdominal wall erythema or discolouration at presentation (aOR 2.51, 95% CI 1.23–5.1), diagnosis of single intestinal perforation at laparotomy (aOR 3.1 95% CI 1.05–9.3), and necessity to perform a clip and drop procedure (aOR 30, 95% CI 3.9–237) were associated with increased 28-day mortality. These results can be used for national benchmarking and counselling of parents.

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.

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.

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.