The principles of enhanced recovery after percutaneous endoscopic gastrostomy (ERaPEG): a UK tertiary center experience.

PURPOSE: Enhanced recovery after surgery (ERAS) pathways have been shown to improve surgical outcomes and patient satisfaction. The aim of the study was to assess whether the implementation of a perioperative enhanced recovery after percutaneous endoscopic gastrostomy (ERaPEG) pathway based on ERAS principles was safe, satisfactory to parents and improved outcomes. METHODS: Following a quality improvement project, a multimodal ERaPEG pathway was introduced as standard practice within the department and children undergoing elective same-day admission percutaneous endoscopic gastrostomy (PEG) at a single UK tertiary center were prospectively enrolled. Exclusion criteria were patients undergoing other concurrent procedures and those who underwent a laparoscopic assisted/open procedure. Data included patient demographics, underlying diagnosis, indication, length of stay (LOS) and 30-day readmission. Parental experience and satisfaction were determined using a questionnaire including 5-point Likert scales. A retrospective cohort was used for comparison. Data were analyzed using Chi-Square test and Mann-Whitney U tests. RESULTS: Ninety-five patients met the inclusion criteria: 50 pre and 45 post the implementation of ERaPEG. Median age was 3 and 2 years, respectively. Neurodisability was the underlying diagnosis in most patients (84%-pre-ERaPEG; 76%-post-ERaPEG). Most common PEG indication was medication/nutritional supplementation (52%-pre-ERaPEG; 51%-post-ERaPEG). The LOS significantly decreased from a median of 51.5 h (pre-ERaPEG) to 32 h (post-ERaPEG) (p < 0.001). Thirty-day readmission rates were similar (6% vs 11%). Most parents felt that the educational material was easy to access and understand. Post-operatively the majority of parents (≥ 80%) were confident in managing the gastrostomy device, setting up/giving the feeds and also felt that the LOS was appropriate. CONCLUSION: This study shows that the implementation of an ERaPEG pathway significantly reduced LOS following PEG. In addition, the pathway was satisfactory to parents and offered the benefit of improved resource utilization.

Oesophageal atresia is correctable and survivable in infants less than 1 kg.

INTRODUCTION: Management of oesophageal atresia (OA) and trachea-oesophageal fistula (TOF) in babies of low birth weight is challenging especially when associated with other anomalies. Birth weight of <1500 g has previously formed part of a classification system designed to predict outcome, alongside the cardiac status of the patient. Improvements in neonatal care have led to increasing numbers of premature low birth weight infants surviving. The aim of this study was to look at the experience of our institution in the extremely low birth weight (ELBW) patients. METHODS: A retrospective review of our institutions OA database was performed from 1993 to June 2015. Patients of birth weight less than 1000 g were included. A review of our OA/TOF clinical database and notes review established the following; gestation, birth weight, associated anomalies, operative procedures, morbidity and mortality. RESULTS: Of 349 patients with OA across the 22-year period, 9 ELBW patients were identified (<1000 g). Six males and three females. Gestational age ranged from 23 to 34 weeks and median birth weight was 815 g ranging from 630 to 950 g. Overall survival was 56 % (5/9). There were double the numbers of ELBW OA/TOF patients seen in the second half of the study period presumably the result of improving neonatal care. Seven patients had type C OA with TOF and underwent emergency TOF ligation, two had concomitant oesophageal repair. One of these patients died from NEC; the other survived. Of the five who had isolated TOF ligation three died-two from cardiac disease and one from prematurity. Both type A patients survived and after initial gastrostomy placement one had a primary delayed repair, the other a gastric transposition. All three babies under 800 g died-one from cardiac disease the others from conditions indicative of their prematurity-necrotising enterocolitis and intraventricular haemorrhage. CONCLUSIONS: 50 % survival is achievable in OA/TOF under 1 kg and the Spitz classification is still applicable in this group as a whole. However, none of the current classification systems are applicable in infants <800 g who in our study all had poor outcomes. We suggest these should be considered as separate group when predicting outcomes.

UK intussusception audit: a national survey of practice and audit of reduction rates.

AIM: To define current UK reduction practice and the reductions rates achieved. MATERIALS AND METHODS: Electronic surveys were sent to radiologists at 26 UK centres. This assessed methods of reduction, equipment, personnel, and protocol usage. Standardized audit proforma were also sent to evaluate all reductions performed in 2011. RESULTS: Twenty-two of 26 centres (85%) replied. All used air enema under fluoroscopic guidance. Equipment was not standardized but could be broadly categorized into hand-pumped air-supply systems (seven centres) and pressurized air systems (15 centres). Seventeen centres followed a protocol based on British Society of Paediatric Radiologists (BSPR) guidelines. In 21 of the 22 centres a consultant paediatric radiologist led reductions and only 12 centres reported a surgeon being present. Three hundred and ten cases were reported across 22 centres. Cases per centre ranged from 0-31 (median 14). Reduction rates varied from 38-90% (median 71%). The overall perforation rate was 2.5%. Caseload did not significantly correlate with reduction rate, and there was no significant difference between the two types of equipment used. Median reduction rates were 15% higher in centres with a surgeon present at reduction (p < 0.05). CONCLUSION: Intussusception care in the UK lacks standardization of equipment and personnel involved. National reduction rates are lower than in current international literature. Improved standardization may lead to an improvement in reduction rates and a surgeon should always be present at reduction.

The impact of adult major trauma centre status on paediatric trauma activity.

OBJECTIVES: The London Trauma Network was launched in April 2010 in order to centralise trauma care in the capital city. The consultation and resourcing of the four new major trauma centres (MTC) was adult focused. The objective of this study was to assess the impact that adult MTC status has on paediatric trauma workload. METHODS: A retrospective review of paediatric major trauma calls was performed between 1 April 2009 to 31 January 2010, before MTC status, and the same time period in 2010/11 when St George's Healthcare Trust was a designated adult MTC. The following variables were assessed; number of trauma calls, admissions to hospital, radiological services usage, inpatient stay, mechanism of injury and injury severity score (ISS)--calculated from abbreviated injury score. RESULTS: There was a 200% increase in trauma calls between the two time periods and a 191% increase in admission to hospital. The usage of radiology increased 221% for CT and 161% for plain radiology. Mean inpatient stay decreased by 0.2 days. Mechanisms of injury were similar. Despite becoming a MTC the relative volume of major injuries (ISS>15) decreased between the two time periods by 1% with a relative increase in minor trauma (ISS<5) from 63% to 72%. These results may suggest at present paediatric trauma patients are being over triaged. CONCLUSIONS: Major adult trauma centre status has a significant effect on paediatric trauma workload and hence resources. When reconfiguration of trauma services are being considered it is essential to take into account the impact on paediatric services alongside those of the adult population.

Air enema reduction of intussusception: a registrar-led, protocol-driven service is safe and effective.

PURPOSE: In the UK, air enema reduction of intussusception is predominantly performed by consultant radiologists. At our institution, it is usually performed by a team involving radiology and surgical registrars. The purpose of this study was to assess who performs air enemas in the UK and the efficacy and safety of our registrar-led service. METHODS: A telephone survey of paediatric surgical centres in the UK was performed asking who performs air enema in these institutions. Following this, a retrospective review of all air enemas at our institution between January 2005 and 2011 was performed. Cases were identified from radiology databases and reviewed for grade of radiologist, perforation and outcome. RESULTS: At all 25 centres, consultant radiologists performed air enemas. At our institution 145 enemas were performed in 6 years. 141 were analysed (54 girls, 87 boys). Median age was 9 months (range 3-107 months). 82 % were performed by the registrar-only team. Over 6 years the registrar-led reduction rate was 77.5 % and in the last 3 years 84 %. The perforation rate was 0.9 %. CONCLUSION: A paediatric surgical registrar-led service for air enema reduction can be safe and effective ensuring a team approach is adopted, equipment is efficient and a strict protocol is applied.

Extubation strategies after esophageal atresia repair.

BACKGROUND/PURPOSE: Early extubation following repair of esophageal atresia (EA) is desirable unless the anastomosis is under tension, in which case paralysis and post-operative ventilation may reduce the risk of anastomotic leakage. However, complications from emergency reintubations do occur with either strategy. We aim to examine the risk/benefit balance of early and delayed extubation following EA repair. METHODS: A seven-year retrospective review of all babies that underwent EA repair was performed. Babies extubated within 24 h of surgery were classified as early extubation (EE). Babies intubated beyond the first 24 h were classified as delayed extubation (DE). The EE group was subdivided into babies extubated in operating room (EIOR), and babies who returned to the neonatal intensive care unit (NICU) intubated but extubated within 24 h (EW24). RESULTS: Forty-six babies were analyzed, and overall 15 (32.6%) required 24 reintubation episodes. Eight (28.6%) babies in the EE group required reintubation. The EIOR group (n = 12) had significantly increased risk of requiring reintubation (OR:7, 95%CI:1.08 to 45.16:p = 0.04) compared to the EW24 group (n = 16). Seven (38.9%) babies in the DE group required reintubation. The complication rate from reintubation after EA repair was 17%. CONCLUSIONS: Extubation on the NICU within 24 h of surgery carried the lowest risk of reintubation. For babies with a tight anastomosis, elective postoperative ventilation appeared to confer a protective benefit without incurring a high risk of complications from reintubation.

Lessons learned from pre-clinical testing of xenogeneic decellularized esophagi in a rabbit model.

Decellularization of esophagi from several species for tissue engineering is well described, but successful implantation in animal models of esophageal replacement has been challenging. The purpose of this study was to assess feasibility and applicability of esophageal replacement using decellularized porcine esophageal scaffolds in a new pre-clinical model. Following surgical replacement in rabbits with a vascularizing muscle flap, we observed successful anastomoses of decellularized scaffolds, cues of early neovascularization, and prevention of luminal collapse by the use of biodegradable stents. However, despite the success of the surgical procedure, the long-term survival was limited by the fragility of the animal model. Our results indicate that transplantation of a decellularized porcine scaffold is possible and vascular flaps may be useful to provide a vascular supply, but long-term outcomes require further pre-clinical testing in a different large animal model.

The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey.

Consensus on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) is lacking, and comparison between studies remains difficult due to a large variety in outcome measures. We aimed to define a core outcome set (COS) for pediatric patients with an asymptomatic CPAM. An online, three-round Delphi survey was conducted in two stakeholder groups of specialized caregivers (surgeons and non-surgeons) in various European centers. Proposed outcome parameters were scored according to level of importance, and the final COS was established through consensus. A total of 55 participants (33 surgeons, 22 non-surgeons) from 28 centers in 13 European countries completed the three rounds and rated 43 outcome parameters. The final COS comprises seven outcome parameters: respiratory insufficiency, surgical complications, mass effect/mediastinal shift (at three time-points) and multifocal disease (at two time-points). The seven outcome parameters included in the final COS reflect the diversity in priorities among this large group of European participants. However, we recommend the incorporation of these outcome parameters in the design of future studies, as they describe measurable and validated outcomes as well as the accepted age at measurement.

Renal abscess in Papillion-Lefèvre syndrome.

A 5-year-old female with Papillon-Lefèvre syndrome presented with renal mass. A radiological diagnosis of malignancy was made; however, partial nephrectomy revealed granulomatous disease indicative of chronic infection. Although liver abscess is an emerging complication in patient with Papillon-Lefèvre syndrome, this case represents the first renal abscess described in such patients.

Outcomes in adulthood of gastric transposition for complex and long gap esophageal atresia.

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 ±â€¯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.

A patient led, international study of long term outcomes of esophageal atresia: EAT 1.

INTRODUCTION: Long term outcomes of esophageal atresia (OA) are poorly understood. The Federation of Esophageal Atresia and Tracheo-Esophageal Fistula support groups (EAT), a collaboration of patient support groups aimed to define patient reported long term outcomes and quality of life (QoL) in a large international cohort of OA patients. METHODS: Questionnaires were designed focusing on patient/parent reported outcomes including surgical history, current symptomatology and quality of life. Members of support groups within EAT were invited to complete questionnaires electronically via SurveyMonkey®. RESULTS: 1100 patients from 25 countries responded to the questionnaire and 928 were analyzed. 80% had type C anatomy, 19% type A and 1% type E. Patient ages were <5 years (42%), 5-10 years (26%), 11-17 years (16%) and 18 years and older (16%). 49% of all patients reported previous dilatations which was similar across age groups. Reflux symptoms affected 58% of patients and persisted into adulthood. Dysphagia also persisted in the adult population with 50% reporting sometimes or often getting food stuck. Reflux was significantly more frequent in 'long gap' versus 'standard gap' patients (p<0.005). Respiratory symptoms and chest infections decreased in frequency with age. In children median SDS for height was -0.41 (IQR -1.4 to 0.67) and that for weight was -0.63 (-1.6 to 0.67). BMI in adults was 21.5. Quality of life was described as significantly affected by OA in 18% of patients while 25% reported no effect on QoL. CONCLUSIONS: These results highlight the significant long term morbidity suffered by OA patients as children and into adulthood and suggest the need for quality transitional care. The patient designed and reported nature of the study gives a unique perspective to the results and emphasizes the benefits of collaboration.

Multi-stage bioengineering of a layered oesophagus with in vitro expanded muscle and epithelial adult progenitors.

A tissue engineered oesophagus could overcome limitations associated with oesophageal substitution. Combining decellularized scaffolds with patient-derived cells shows promise for regeneration of tissue defects. In this proof-of-principle study, a two-stage approach for generation of a bio-artificial oesophageal graft addresses some major challenges in organ engineering, namely: (i) development of multi-strata tubular structures, (ii) appropriate re-population/maturation of constructs before transplantation, (iii) cryopreservation of bio-engineered organs and (iv) in vivo pre-vascularization. The graft comprises decellularized rat oesophagus homogeneously re-populated with mesoangioblasts and fibroblasts for the muscle layer. The oesophageal muscle reaches organised maturation after dynamic culture in a bioreactor and functional integration with neural crest stem cells. Grafts are pre-vascularised in vivo in the omentum prior to mucosa reconstitution with expanded epithelial progenitors. Overall, our optimised two-stage approach produces a fully re-populated, structurally organized and pre-vascularized oesophageal substitute, which could become an alternative to current oesophageal substitutes.

Cervical approach to the thoracic inlet in paediatric patients with broncho-pulmonary foregut malformations.

Lesions of the thoracic inlet present a significant challenge to the surgeon due to the difficulty of access and proximity to important neurovascular structures within the region. We describe two cases of benign disease of the thoracic inlet in children, one bronchogenic cyst and an esophageal duplication, and report the cervical approach used to manage them. Both lesions extended from the neck through the thoracic inlet, but demonstrate how benign lesions in this area can be delivered up into a cervical incision, negating the need for the more invasive modified thoracotomies. A cervical approach can be safely and successfully used to approach benign pathology, such as bronchogenic cysts and oesophageal duplications of the thoracic inlet. Careful multidisciplinary planning is required for such procedures.