Outcomes 1 year after non-operative management of uncomplicated appendicitis in children: Children with AppendicitiS during the CoronAvirus panDEmic (CASCADE) study.

BACKGROUND: A major shift in treatment of appendicitis occurred early in the SARS-CoV-2 pandemic with non-operative management used commonly outside research protocols and in units with limited previous experience. This study aims to compare real-world outcomes of surgery versus non-operative management of uncomplicated appendicitis in children with 1-year follow-up. METHOD: A prospective multicentre observational study of children treated for uncomplicated appendicitis at 74 hospitals in the UK and Ireland from 1 April to 31 July 2020 was performed. Propensity-score matched analysis was conducted using age, sex, C-reactive protein at diagnosis and duration of symptoms as covariates. Primary outcomes were success of non-operative management defined as achieving 1-year follow-up without undergoing appendicectomy due to recurrent appendicitis or ongoing symptoms, and occurrence of any predefined complication (intra-abdominal collection, wound infection, bowel obstruction or reintervention). RESULTS: Of 1464 children with presumed uncomplicated appendicitis, 1027 (70.2 per cent) underwent surgery and 437 (29.9 per cent) underwent non-operative management. Ninety-four children (21.5 per cent) treated by initial non-operative management required appendicectomy during the index hospital admission while recurrent appendicitis after discharge occurred in 25 (10.4 per cent) children within 1 year. The overall success rate of non-operative management at 1 year was 63.1 per cent (95 per cent c.i. 58.0 to 68.3 per cent). For propensity-score matched analyses, 688 children undergoing surgery and 307 undergoing non-operative management were included. Any predefined complication occurred in 50 (7.3 per cent) children undergoing surgery and in four (1.3 per cent) children undergoing non-operative management (OR 5.9 (95 per cent c.i. 2.1 to 16.6)) in the propensity-score matched cohort. There was no mortality or stoma formation. CONCLUSION: Non-operative management is a safe and valid alternative to appendicectomy in children with uncomplicated appendicitis.

10 Year Follow-Up of Randomized Trial of Laparoscopic Nissen Versus Thal Fundoplication in Children.

Introduction: The aim of this study was to compare the long-term outcomes of laparoscopic complete (Nissen) fundoplication (LNF) with laparoscopic partial (Thal) fundoplication (LTF) in children. This is the only prospective, randomized study to follow patients up for more than 10 years. Interim results published in 2011 at median 2.5 year follow-up showed that LNF had a significantly lower failure rate compared with LTF. Materials and Methods: A randomized, controlled trial of LNF versus LTF in children (<16 years) was performed. The primary outcome measure was "absolute" failure of the fundoplication-recurrence of symptoms that merited either reoperation or insertion of transgastric jejunostomy (GJ). Secondary outcomes were "relative" failure (need for postop antireflux medication), complications (e.g., dysphagia), and death. Results: One hundred seventy-five patients were recruited; 89 underwent LNF, and 86 underwent LTF. Eight patients had no follow-up recorded. At long-term follow-up, 59 patients had died (35%); LNF 37/85 (43.5%) and LTF 22/82 (26.8%), P = .02. Median length of follow-up in survivors was 132 months. There was no statistically significant difference in "absolute" failure rate between LNF 8/85(9.4%) and LTF 15/82 (18%), P = .14. There was no difference in "relative" failure between LNF 7/85 (8.2%) and LTF 12/82 (14%), P = .23. Long-term dysphagia affected 5 out of 108 (4.6%) patients; 3/48 (6.2%) of LNF and 2/60 (3.3%) of LTF (P = .65). Conclusions: There was no statistically significant difference in 'absolute' failure between LNF and LTF at long-term follow-up. Neurologically impaired children have a high mortality rate following fundoplication due to comorbidities. This trial commenced in 1998 and was approved by the Oxfordshire Research Ethics Committee (No. 04.OXA.18-1998).

Impact of the COVID-19 pandemic on management and outcomes of children with appendicitis: The Children with AppendicitiS during the CoronAvirus panDEmic (CASCADE) study.

AIM: To report the impact of the SARS-CoV-2 pandemic on management and outcomes of paediatric appendicitis in the UK and Ireland. METHODS: Prospective, multicentre observational cohort study at general surgical and specialist paediatric surgical centres in the United Kingdom and Ireland from 1st April to 31st July 2020. Primary outcome was treatment strategy used for acute appendicitis. RESULTS: This study includes 2002 children treated for acute appendicitis of a median age of 10 (range 1-15) years and 605 children from a similar data set pre pandemic from 2017. In the pandemic cohort 560/2002(28%) were initially treated non operatively of whom 125/560(22%) proceeded to appendicectomy within initial hospital admission. Non operative treatment wasn't used in the pre pandemic cohort. Diagnostic imaging use was greater during the pandemic compared to pre pandemic (54vs31%; p < 0.00001) but overall use of laparoscopy was similar during both time periods (62.4vs66.6%). Hospital readmission rate was lower (8.7vs13.9%; p = 0.0002) during the pandemic than pre pandemic and Re-intervention rate was similar (2.9vs2.6%;p = 0.42). In cases treated operatively negative appendicectomy rate was lower during the pandemic than pre pandemic (4.4vs15.4%; p =0.0001), and during the pandemic was amongst the lowest ever reported in the UK. CONCLUSION: COVID-19 has had a significant impact on the management of children with appendicitis in the UK and Ireland. The rate of imaging and the use of non operative management increased, whilst the negative appendicectomy rate reduced. Overall, patient outcomes have not been adversely impacted by change in management during the pandemic. CONCLUSION: Level I. TYPE OF STUDY: Prognosis study.

The Relationship Between Present-Centered Awareness and Attention, Burnout, and Compassion Fatigue in Oncology Health Professionals.

OBJECTIVES: Oncology health professionals experience high levels of burnout and compassion fatigue, affecting their health and the care they provide. This study aimed to establish whether present-centered awareness and attention (a component of mindfulness) is uniquely associated with burnout and compassion fatigue in oncology professionals. METHODS: An international sample of oncology professionals (n = 118) completed an online questionnaire with validated measures of present-centered awareness and attention, empathy, compassion fatigue, and burnout. Hierarchical multiple regressions were used to model relations among the independent variable (present-centered awareness and attention) and the criterion variables of burnout (disengagement, exhaustion) and compassion fatigue (compassion satisfaction, compassion burnout, secondary traumatic stress) after statistically controlling for empathy, age, gender, years of experience, and patient contact hours. RESULTS: Mean hours of patient contact per week was 23.52 (SD = 13.62), with 26 (22.03%) reporting 40 h or more. Higher hours of patient contact per week were positively associated with secondary traumatic stress. Present-centered awareness and attention was associated with lower disengagement, lower emotional exhaustion, higher compassion satisfaction, lower secondary traumatic stress, and lower compassion burnout. In each model, present-centered awareness accounted for unique variance after controlling for age, gender, years of experience, patient contact hours per week, and empathy scores. The amount of unique variance accounted for by present-centered awareness ranged from 4 to 10%. CONCLUSIONS: Oncology professionals reporting higher levels of present-centered awareness and attention reported higher compassion satisfaction and lower secondary traumatic stress, compassion burnout, exhaustion, and disengagement. Promoting present-centered awareness may be a mechanism that contributes to less burnout in oncology professionals.

Insufflation in minimally invasive surgery: Is there any advantage in staying low?

AIM: Minimally invasive repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) and congenital diaphragmatic hernia (CDH) is feasible and confers benefits compared to thoracotomy or laparotomy. However, carbon dioxide (CO2) insufflation can lead to hypercapnia and acidosis. We sought to determine the effect of lower insufflation pressures on patients' surrogate markers for CO2 absorption - arterial partial pressure of CO2 (PaCO2), end tidal CO2 (EtCO2) and pH. METHODS: Single center retrospective review, including neonates without major cardiac anomaly. Selected patients formed 2 groups: Historical pressure (HP) group and low pressure (LP) group. We reported on the patients' preoperative characteristics that potentially confound the degree of CO2 absorption or elimination. Outcome measures were perioperative PaCO2, EtCO2, arterial pH and anesthetic time. RESULTS: 30 patients underwent minimally invasive surgery for CDH and 24 patients for EA/TEF with similar distribution within the HP and LP group. For CDH patients as well as for EA/TEF patients, there were no significant differences in their preoperative characteristics or surgery duration comparing HP and LP groups. With a decrease in insufflation pressure in CDH patients, there were a significant decrease (p = 0.002) in peak PaCO2 and an improvement in nadir pH (p = 0.01). For the EA/TEF patients, the decrease in insufflation pressure was associated with a significant decrease (p = 0.03) in peak EtCO2. Considering all 54 patients, we found EtCO2 to be highly significantly inversely correlated with pH and positively correlated with intraoperative PaCO2 (p < 0.001). Baseline Hb was inversely correlated with mean EtCO2 (p < 0.001). CONCLUSION: With lower insufflation pressures, CDH patients had significantly improved hypercapnia and acidosis, while EA/TEF patients had significantly reduced EtCO2. EtCO2 was correlated with acidosis and hypercapnia. TYPE OF STUDY: Retrospective case control study. LEVEL OF EVIDENCE: Level III.

Patch repair of congenital diaphragmatic hernia is not at risk of poor outcomes.

PURPOSE: Recurrence of congenital diaphragmatic hernia (CDH) was retrospectively evaluated after correction with or without a patch in an institution where tension-free repair is advocated. METHODS: Demographics and outcomes of patients with a postero-lateral CDH repaired (2000-2016) were analyzed (univariate tests and binary logistic regression adjusting for time since start of study, gender, defect side, liver herniation, patch, surgical approach, absence of postero-lateral rim and length of follow-up). RESULTS: Of 203 patients, 107 received a patch (P), and 96 were not patched (NP). Groups were not different for gestational age birthweight, gender, defect side and minimally invasive approach rate. Preoperative ECMO incidence (P:29.9% vs. NP:2.1%, p < 0.01), liver herniation (P:57.0% vs. NP:22.9%, p < 0.01) and absence of a postero-lateral rim (P:61.7% vs. NP:8.3%, p < 0.01) were higher in the P group. The mortality rate was 10.8% (P:15.0% vs. NP:6.2%, p = 0.07). Recurrence was not different (P:9.3% vs. NP:4.2%, p = 0.15). Multivariate analysis showed that recurrence was higher after thoracoscopy compared to open (OR = 12.2 [2.2-68], p < 0.01); neither the use of patch (OR = 2.3, [0.5-10.4], p = 0.28) nor any other factors were associated with recurrence. CONCLUSION: In this single centre series where tension-free repair was advocated, patch repair of CDH was not associated with higher recurrence, though access route was. TYPE OF STUDY: Cohort Study. LEVEL OF EVIDENCE: Level III.

Splenic Rupture Secondary to Vascular Ehlers-Danlos Syndrome Managed by Coil Embolization of the Splenic Artery.

Aim Atraumatic splenic rupture is uncommon and life-threatening. It may be related to underlying pathology and be the initial manifestation of the condition. Vascular Ehlers-Danlos syndrome (V-EDS) is a rare autosomal dominant collagen vascular disorder, associated with vessel fragility and rupture. We describe a child presenting with splenic rupture managed by embolization of the splenic artery. She was subsequently diagnosed with V-EDS. Case Description A 11-year-old girl with thalassemia trait presented with sudden onset of abdominal pain and hypovolemic shock. There was no history of trauma. Following resuscitation, abdominal computed tomography showed hemoperitoneum and active splenic arterial extravasation. Angiography demonstrated four bleeding points, from irregular vessels supplying the upper two-thirds of the spleen. These were not amenable to supraselective embolization. Therefore, coil embolization of the main splenic artery was performed, with no splenic supply seen on the postembolization angiogram. Her postoperative recovery was complicated by pancreatitis secondary to partial ischemia of the pancreatic tail. Subsequent extensive investigations excluded hematological, myeloproliferative, and infective causes for her splenic rupture. A safeguarding investigation was completed, with no pertinent factors identified. Findings of thin skin, abnormal bruising, and hypermobile joints raised a clinical suspicion of a connective tissue disorder. Genetic testing revealed a de novo mutation of the COL3A1 gene. Conclusions There are only four reports of V-EDS causing splenic rupture in the literature to date. These patients were all adults and only one had not previously been diagnosed with V-EDS. All underwent splenectomy. While V-EDS presenting with abdominal visceral rupture in children has been reported, this is the first report of a child with V-EDS presenting with splenic rupture. It is the only case of splenic rupture secondary to V-EDS that has been managed minimally invasively by embolization.

A systematic review of the current interventions available to support children living with parental cancer.

OBJECTIVE: Children living with parental cancer are vulnerable to distress and developmental disruption. This review aims to identify current interventions to support cancer patients' children and summarise how effective these are based on children's reports. METHODS: Between 25 May 2015 and 6 August 2018, a broad search strategy was used to identify relevant references. Seven databases were searched, and grey literature was also vetted. This review was informed by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and Cochrane guidelines. RESULTS: Eight studies evaluating six interventions were retained. Research designs and interventions were heterogenous, and study quality was low. A limited number of significant results were reported by studies. These evidenced improvement for PTSD symptoms, emotional regulation, and depression. However, overall current interventions do not appear effective among patients' children. CONCLUSION: Despite encouraging preliminary findings, interventions do not yet adequately support cancer patients' children. There is a need for more tailored and targeted interventions. A theoretical model conceptualising the impact of parental cancer may assist this. PRACTICE IMPLICATIONS: Findings will assist future intervention research by promoting standardised levels of care among cancer patients' children, that is empirically supported, effective, and meets principles of non-maleficence.

Gastrointestinal bleeding as initial presentation of extramedullary plasma cell neoplasms: A case report and review of the literature.

BACKGROUND: Plasma-cell neoplasms rarely involve the gastrointestinal tract and manifest as gastrointestinal bleeding. Plasmablastic myeloma is an aggressive plasma cell neoplasm associated with poor outcomes. A small number of cases with gastrointestinal involvement is reported in the literature and therefore high index of suspicion is essential for avoiding delays in diagnosis and treatment. CASE SUMMARY: Our aim is to present our experience of a 70-year-old patient with a secondary presentation of plasmablastic myeloma manifesting as unstable upper gastrointestinal bleeding and to review the literature with the view to consolidate and discuss information about diagnosis and management of this rare entity. In addition to our case, a literature search (PubMed database) of case reports of extramedullary plasma cell neoplasms manifesting as upper gastrointestinal bleeding was performed. Twenty-seven cases of extramedullary plasmacytoma (EMP) involving the stomach and small bowel presenting with upper gastrointestinal bleeding were retrieved. The majority of patients were males (67%). The average age on diagnosis was 62.7 years. The most common site of presentation was the stomach (41%), followed by the duodenum (15%). The most common presenting complaint was melena (44%). In the majority of cases, the EMPs were a secondary manifestation (63%) at the background of multiple myeloma (26%), plasmablastic myeloma (7%) or high-grade plasma cell myeloma (4%). Oesophagogastroscopy was the main diagnostic modality and chemotherapy the preferred treatment option for secondary EMPs. CONCLUSION: Despite their rare presentation, upper gastrointestinal EMPs should be considered in the differential diagnosis of patients with gastrointestinal bleeding especially in the presence of systemic haematological malignancy.

Impaired cellular and humoral immunity is a feature of Diamond-Blackfan anaemia; experience of 107 unselected cases in the United Kingdom.

Diamond-Blackfan anaemia (DBA) is a rare bone marrow failure syndrome characterised by anaemia, congenital anomalies and cancer predisposition. Although infections are the second leading cause of mortality in non-transplanted patients, immune function is largely unexplored. We identified quantitative deficits in serum immunoglobulins and/or circulating T, natural killer and B lymphocytes in 59 of 107 unselected patients (55·1%) attending our centre over a 7-year period. Immune abnormalities were independent of ribosomal protein genotype and arose in both steroid-treated and steroid-untreated patients. In summary, these data highlight the high prevalence and spectrum of infections and immune defects in DBA.

Post-Prandial Hyperinsulinaemic Hypoglycaemia after Oesophageal Surgery in Children.

INTRODUCTION: Post-prandial hyperinsulinaemic hypoglycaemia (PPHH) is a recognized complication of various gastric surgeries in children, but rarely reported after oesophageal atresia repair. We report 2 children diagnosed with PPHH after oesophageal surgery and the challenges of their management. Case 1: A 2-year-old boy diagnosed with oesophageal atresia at birth was surgically repaired requiring 6 oesophageal dilatations in the first year of life. At 11 months of age, he manifested hypoglycaemic seizures and investigations confirmed PPHH. Acarbose and diazoxide trials failed. He was managed with 17-h continuous gastrostomy feeds. Currently, he is 28 months old with euglycaemia on daytime bolus gastrostomy feeds and overnight 12-h continuous gastrostomy feeds. Case 2: A 6-month-old girl diagnosed with Wolf-Hirschhorn syndrome and tracheo-oesophageal fistula was surgically repaired, requiring monthly oesophageal dilatations. At 5 months of age, she was reported to have hypoglycaemia and PPHH was confirmed. She responded to diazoxide and continuous nasogastric tube feeds, but developed pulmonary hypertension pos-sibly diazoxide-induced. Subsequently, diazoxide was stopped and normoglycaemia was secured via 20-h continuous gastrostomy feeds. CONCLUSION: PPHH may be an underdiagnosed complication in children undergoing surgery for oesophageal atresia. These children must be monitored closely for symptoms of hypoglycaemia and if there are concerns must be screened for possible PPHH. Our cases demonstrate that continuous feeding regimens might be the only therapeutic option, until PPHH gradually lessens in intensity over time.

Morgagni hernia repair in children over two decades: Institutional experience, systematic review, and meta-analysis of 296 patients.

BACKGROUND/PURPOSE: Morgagni diaphragmatic hernia (MH) is rare. We report our experience based on routine patch use in MH repair to curb recurrence. A systematic review and meta-analysis were performed to study the recurrence and complications associated with minimally invasive surgery and the use of patch. METHODS: We retrospectively reviewed all cases of MH who underwent first-time repair in 2012-2017 in our institution to determine recurrence and complication rate. A MEDLINE search related to minimally invasive surgery (MIS) and patch repair of MH was conducted for systematic review. Eligible articles published from 1997-2017 with follow-up data available were included. Primary outcomes measured were recurrence and complication. Meta-analysis to compare open versus MIS and primary versus patch repair in the MIS group were performed in comparative cohorts. Continuous data were presented as median (range), and statistical significance was P<0.05. RESULTS: In our institution, 12 consecutive patients aged 17-month-old (22 days-7 years), underwent laparoscopic patch repair of MH, with one conversion to laparotomy. No recurrence or significant complication occurred over a follow-up period of 8 months (1-48 months). Thirty-six articles were included from literature review and were combined with the current series. All were retrospective case reports or series, of which 6 were comparative cohorts with both MIS and open repairs. A total of 296 patients from 37 series were ultimately used for analysis: 80 had open repair (4 patch) and 216 had MIS repair (32 patch), with a patch rate of 12%. There were 13 recurrences (4%): no difference between open and MIS repairs (4/80 vs 9/216, p=0.75); recurrence rate following primary repair was 13/260 (5%), but no recurrence occurred with 36 patch repairs. Meta-analysis showed no difference in recurrence between open and MIS repair (p=0.83), whereas patch repair was associated with 14% less recurrence compared with primary repair, although it did not reach statistical significance (p=0.12). There were 13 complications (5%): no difference between open and MIS repairs (5/80 vs 8/216, p=0.35). One small bowel obstruction occurred in a patient who had laparoscopic patch repair. CONCLUSION: In MH, recurrence and complication rates are comparable between MIS and open repairs. Use of patch appeared to confer additional benefit in reducing recurrence. TYPE OF STUDY: Systematic review LEVEL OF EVIDENCE: 3A.

Primary versus Staged Closure of Exomphalos Major: Cardiac Anomalies Do Not Affect Outcome.

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.

Current Research on the Epidemiology, Pathogenesis, and Management of Necrotizing Enterocolitis.

Despite decades of research on necrotizing enterocolitis, we still do not fully understand the pathogenesis of the disease, or how to prevent or how to treat it. However, as a result of recent significant advances in the microbiology, molecular biology, and cell biology of the intestine of preterm infants and infants with necrotizing enterocolitis, there is some hope that research into this devastating disease will yield some important translation into effective prevention, more rapid diagnosis, and novel therapies.

Probiotics for the prevention of surgical necrotising enterocolitis: systematic review and meta-analysis.

AIM OF THE STUDY: Probiotic administration to preterm infants has the potential to prevent necrotising enterocolitis (NEC). Data from randomised controlled trials (RCT) are conflicting but meta-analyses seem to support this intervention. To date, these analyses have not focused on surgical NEC. We aimed to determine the effect of probiotic administration to preterm infants on prevention of surgical NEC. METHODS: A systematic review of RCTs of probiotic administration to preterm infants was performed. Studies were included if RCT outcomes included any of (1) Bell's stage 3 NEC; (2) surgery for NEC; and (3) deaths attributable to NEC. Article selection and data extraction were performed independently by two authors; conflicts were adjudicated by a third author. Data were meta-analysed using Review Manager V.5.3. A random effects model was decided on a priori because of the heterogeneity of study design; data are risk ratio (RR) with 95% CI. MAIN RESULTS: Thirty-five RCTs reported NEC as an outcome. Seventeen reported surgical NEC; all RCTs were included. A variety of probiotic products was administered across studies. Description of surgical NEC in most studies was poor. Only 6/16 specifically reported incidence of surgery for NEC, 12/17 Bell's stage 3 and 13/17 NEC-associated mortality. Although there was a trend towards probiotic administration reducing stage 3 NEC, this was not significant (RR 0.74 (0.52-1.05), p=0.09). There was no effect of probiotics on the RR of surgery for NEC (RR 0.84 (0.56-1.25), p=0.38). Probiotics did, however, reduce the risk of NEC-associated mortality (RR 0.56 (0.34-0.93), p=0.03). CONCLUSION: Despite 35 RCTs on probiotic prevention of NEC, evidence for prevention of surgical NEC is not strong, partly due to poor reporting. In studies included in this meta-analysis, probiotic administration was associated with a reduction in NEC-related mortality.

Current research in necrotizing enterocolitis.

Despite decades of research on necrotizing enterocolitis, we still do not fully understand the pathogenesis of the disease, how to prevent or how to treat the disease. However, as a result of recent significant advances in the microbiology, molecular biology, and cell biology of the intestine of premature infants and infants with necrotizing enterocolitis, there is some hope that research into this devastating disease will yield some important translation into improved outcomes.

Early reported rectal sensation predicts continence in anorectal anomalies.

BACKGROUND: Straining at stool is an automatic reflex in babies and implies the presence of rectal sensation. We hypothesised that early reported rectal sensation would predict future continence in children with anorectal anomalies. AIM OF THE STUDY: The aim of this study is to determine if early straining at stool was a useful predictor of future continence in infants born with high anorectal malformations. METHODS: A retrospective case note review of prospectively collected clinical information was performed with institutional review board approval. All patients with intermediate/high anorectal malformation operated on by a single surgeon from 1984 to 2010 were included. After stoma closure, parents were asked: The responses were noted within the first year of stoma closure and then all patients were followed up until they were at least 3 ½years old and continence could be assessed using the Krickenbeck outcome classification. Data were compared using Fisher's exact test and sensitivity, specificity and positive predictive value (PPV) were calculated. MAIN RESULTS: Forty-eight patients were included in the study. Sixteen (33%) were female (12 cloacal malformation, 3 rectovaginal fistula, 1 rectal atresia) and 32 (66%) were male (6 rectovesical fistulae, 22 rectourethral fistulae, 4 no fistula). Median follow-up was 9.7years (range 3.5-17.9). Twenty-one children were noted by their parents to exhibit early straining at stool after stoma closure. Twenty of them achieved long term continence. The sensitivity of early straining as a predictor for long term continence was 77%, specificity 95% and positive predictive value 95%. CONCLUSION: The presence of early rectal sensation reported by parents is a good predictor of long term continence. This allows more informed discussion with families in the early years of life.