Exploring the infectious contribution to intussusception causality using the effects of COVID-19 lockdowns in Australia: An ecological study.

INTRODUCTION: Intussusception is the primary cause of acute bowel obstruction in infants. The majority of cases under 2 years of age are classed as idiopathic with viral infection implicated as one of the causes. COVID-19 public health measures led to significant decreases in communicable disease prevalence. During these times, reductions in intussusception frequency were reported - reductions greater than would be expected with our previous understanding of its infectious aetiology. METHODS: We conducted a retrospective, multi-state, ecological study over a twelve-year period. Monthly case numbers of ICD-10-AM K56.1 'Intussusception' coded admissions were acquired from state-wide admissions datasets from New South Wales (NSW), Victoria and Queensland, representing 77.62% of the eligible Australian population. These counts within differing jurisdictional lockdowns were compared to non-lockdown periods in order to investigate a correlation between intussusception frequency and lockdown periods. RESULTS: We found a negative association between intussusception frequency and lockdown periods in both eligible states. The largest reductions were seen in the under 2 year age groups with Victoria experiencing a 62.7% reduction (Rate ratio (RR) = 0.37, p < 0.0001) and NSW a 40.1% reduction (RR = 0.599, p = 0.006) during lockdown times. Controls for variations in lockdown restrictions between both regional and metropolitan areas also showed expected decreases. CONCLUSION: Our ecological study demonstrates significant decreases in the frequency of paediatric intussusception admissions during the COVID-19 lockdown periods. The unexpected magnitude of the reductions suggests that the true proportion of infectious disease-caused idiopathic intussusception is greatly underestimated.

Esophago-gastric junction findings on high resolution impedance manometry in children with esophageal atresia.

OBJECTIVES: Using high resolution impedance manometry (HRIM), this study characterized the esophago-gastric junction (EGJ) dynamics in children with esophageal atresia (EA). METHOD: Esophageal HRIM was performed in patients with EA aged less than 18 years. Objective motility patterns were analyzed, and EGJ data reported. Controls were pediatric patients without EA undergoing investigations for consideration of fundoplication surgery. RESULTS: Seventy-five patients (M:F = 43:32, median age 1 year 3 months [3 months-17 years 4 months]) completed 133 HRIM studies. The majority (64/75, 85.3%) had EA with distal tracheo-esophageal fistula. Compared with controls, liquid swallows were poorer in patients with EA, as evident by significant differences in distension pressure emptying and bolus flow time (BFT). The integrated relaxation pressure for thin liquid swallows was significantly different between EA types, as well as when comparing patients with EA with and without previous esophageal dilatations. The BFT for solid swallows was significantly different when compared with EA types. CONCLUSIONS: We have utilized HRIM in patients with EA to demonstrate abnormalities in their long-term EGJ function. These abnormalities correlate with poorer esophageal compliance and reduced esophageal peristalsis across the EGJ. Understanding the EGJ function in patients with EA will allow us to tailor long-term management to specific patients.

The early years: hirschsprung disease and health-related quality of life.

PURPOSE: Chronic diseases are notorious in the way that they interfere with many aspects of a child's development, and this holds true for children with Hirschsprung disease (HD). The present research aims to (1) determine whether the health-related quality of life (HRQoL) of HD children differs from healthy paediatric populations; and (2) explore the relationship between HD children's HRQoL and psychosocial outcomes of parents. METHODS: Using a cross-sectional survey study design, children's HRQoL was assessed using the Pediatric Quality of Life Inventory (PedsQL), while parental psychosocial outcomes were measured using the Patient-Reported Outcomes Measurement Information System (PROMIS) anxiety and depression short-forms, Family Management Measure (FaMM), and Parent Experience of Child Illness. Surveys were administered over telephone to parents of 48 Australian children treated for HD (87.5% male, median age 4.5 years) during the period May to November 2021. RESULTS: While postoperative HRQoL of HD children was comparable to that of healthy age-matched controls, psychosocial quality of life of HD children was significantly poorer (mean difference = 3.40, CI [0.05, 6.76]). All parental outcome measures were significantly correlated with the PedsQL (r = - 0.77-0.67, p < 0.05) in expected directions, with FaMM subscales (except parent mutuality) demonstrating the most variation (R2 = 0.41-0.59). Of note, 31.3% of parents reported moderate to severe symptoms of anxiety on the PROMIS. CONCLUSION: Despite overall positive results for children, parents reported elevated symptoms of anxiety. This study highlights the importance of long-term follow-up care for HD patients and their families.

Levobupivacaine plasma concentrations following repeat caudal anesthetics.

AIM: A single caudal anesthetic at the start of lower abdominal surgery is unlikely to provide prolonged analgesia. A second caudal at the end of the procedure extends the analgesia duration but total plasma concentrations may be associated with toxicity. Our aim was to measure total plasma levobupivacaine concentrations after repeat caudal anesthesia in infants and to generate a pharmacokinetic model for prediction of plasma concentrations after repeat caudal anesthesia in neonates, infants and children. METHODS: Infants undergoing definitive repair of anorectal malformations or Hirschsprung's disease received a second caudal anesthesia at the end of the procedure. Total levobupivacaine concentrations were assayed 3-4 times in the first 6 h after the initial caudal. These data were pooled with data from four studies describing plasma concentrations after levobupivacaine caudal or spinal anesthesia. Population pharmacokinetic parameters were estimated using nonlinear mixed-effects models. Covariates included postmenstrual age and body weight. Parameter estimates were used to simulate concentrations after a repeat levobupivacaine 2.5 mg kg-1 caudal at 3 or 4 h following an initial levobupivacaine 2.5 mg kg-1 caudal. RESULTS: Twenty-one infants (postnatal age 11-32 weeks, gestation 37-39 weeks, weight 5.2-8.6 kg) were included. The measured peak plasma concentration after repeat caudal levobupivacaine 2.5 mg kg-1 4 h after initial caudal was 1.38 mg L-1 (95% prediction interval 0.60-2.6 mg L-1 ) and 3 h after initial caudal was 1.46 mg L-1 (0.60-2.80) mg L-1 . Simulation of total plasma concentrations in neonates (7 kg, 57 weeks postmenstrual age) given caudal levobupivacaine 4 h after the initial caudal were 1.76 mg L-1 (0.68-3.50) mg L-1 if 2.5 mg kg-1 levobupivacaine was used and 0.88 mg L-1 (0.34-1.73) mg L-1 if 1.25 mg kg-1 of 0.125% levobupivacaine was used. In simulated older children (20 kg, 6 years), the mean maximum concentration was 1.43 mg L-1 (0.60-2.70) mg L-1 if 2.5 mg kg-1 levobupivacaine was repeated at 3 h. CONCLUSION: Repeat caudal levobupivacaine 2.5 mg kg-1 at 3 h after an initial 2.5 mg kg-1 dose does not exceed the concentration associated with systemic local anesthetic toxicity. In 2.5% of simulated neonates (weight 3.8 kg, PMA 40 weeks), repeat caudal anesthesia demonstrates broaching of the lower concentration limit associated with toxicity at both 3 and 4 h after initial caudal.

Preexisting neural factors that contribute to dysmotility in esophageal atresia: a systematic review.

Esophageal dysmotility in esophageal atresia (EA) relates to abnormal development of esophageal innervation and musculature and to the esophageal repair. Few studies have investigated the preexisting dysmotility in EA, present prior to surgery. This systematic review aims to summarize the literature on neuronal studies in EA, to understand the causative factors for esophageal dysmotility. We performed a systematic review (PubMed, EMBASE, EBM, CINAHL databases; January 1947-February 2021) in accordance with PRISMA (PROSPERO number CRD42020171014). Fourteen studies were identified (eleven human, 187 EA patients; three animal, 64 EA rat specimens). Neural factors affecting esophageal dysmotility in human and animal studies included proteins, enzymes, growth factors, and genes, which play a role in the nervous system or neuroendocrine system, some of which have functions as neuromodulators or neurotransmitters. This systematic review has identified neural factors that affect esophageal dysmotility and contributes toward our understanding of the underlying dysmotility in patients with EA. The studies identified are important and essential for successful translation of basic science knowledge to impact clinical practice and understanding. Level of evidence: III.

Quality of Life Outcomes in Primary Caregivers of Children with Esophageal Atresia.

OBJECTIVE: To investigate the quality of life (QoL) impact on primary caregivers of children with esophageal atresia. STUDY DESIGN: We used a prospective cohort study design, inviting primary caregivers of children with esophageal atresia to complete the following questionnaires: Parent Experience of Child Illness (PECI), Patient-Reported Outcomes Measurement Information System (PROMIS) Anxiety, PROMIS Depression, 12-Item Short Form Survey (SF-12), and Pediatric Quality of Life Inventory (PedsQL). The PECI, PROMIS Anxiety and Depression, and SF-12 assessed caregiver QoL, and the PedsQL assessed patient QoL. Patients with Gross type E esophageal atresia served as controls. RESULTS: The primary caregivers of 100 patients (64 males, 36 females; median age, 4.6 years; range, 3.5 months to 19.0 years) completed questionnaires. The majority (76 of 100) of patients had Gross type C esophageal atresia. A VACTERL (vertebral anomalies, anorectal malformation, cardiac anomalies, tracheoesophageal fistula, renal anomalies, limb anomalies) association was found in 30, ≥1 esophageal dilatation was performed in 57, and fundoplication was performed in 11/100. When stratified by esophageal atresia types, significant differences were found in 2 PECI subscales (unresolved sorrow/anger, P = .02; uncertainty, P = .02), in PROMIS Anxiety (P = .02), and in SF-12 mental health (P = .02) and mental component summary scores (P = .02). No significant differences were found for VACTERL association, nor esophageal dilatation. Requirement for fundoplication resulted in lower SF-12 general health score, and lower PedsQL social and physical functioning scores. CONCLUSIONS: We have demonstrated that caring for a child with esophageal atresia and a previous requirement for fundoplication impacts caregiver QoL.

Reducing Underdiagnosis of Hirschsprung-Associated Enterocolitis: A Novel Scoring System.

BACKGROUND: Hirschsprung-Associated Enterocolitis (HAEC) is a life-threatening and difficult to diagnose complication of Hirschsprung Disease (HSCR). The goal of this study was to evaluate existing HAEC scoring systems and develop a new scoring system. METHODS: Retrospective, multi-institutional data collection was performed. For each patient, all encounters were analyzed. Data included demographics, symptomatology, laboratory and radiographic findings, and treatments received. A "true" diagnosis of HAEC was defined as receipt of treatment with rectal irrigations, antibiotics, and bowel rest. The Pastor and Frykman scoring systems were evaluated for sensitivity/specificity and univariate and multivariate logistic regression performed to create a new scoring system. RESULTS: Four centers worldwide provided data on 200 patients with 1450 encounters and 369 HAEC episodes. Fifty-seven percent of patients experienced one or more episodes of HAEC. Long-segment colonic disease was associated with a higher risk of HAEC on univariate analysis (OR 1.92, 95% CI 1.43-2.57). Six variables were significantly associated with HAEC on multivariate analysis. Using published diagnostic cutoffs, sensitivity/specificity for existing systems were found to be 38.2%/96% for Pastor's and 56.4%/86.9% for Frykman's score. A new scoring system with a sensitivity/specificity of 67.8%/87.9% was created by stepwise multivariate analysis. The new score outperformed the existing scores by decreasing underdiagnosis in this patient cohort. CONCLUSIONS: Existing scoring systems perform poorly in identifying episodes of HAEC, resulting in significant underdiagnosis. The proposed scoring system may be better at identifying those underdiagnosed in the clinical setting. Head-to-head comparison of HAEC scoring systems using prospective data collection may be beneficial to achieve standardization in the field.

Audit of enuresis referrals on the waiting list for a tertiary hospital outpatient clinic.

AIM: Enuresis, defined as intermittent incontinence occurring exclusively during sleep, affects 4-19% of children, but can be effectively treated using education and alarm-bell therapies. However, delays in treatment are likely to impact upon the quality of life of the child, parents and carers. Poor quality and incomplete referrals are thought to be a major driver of inefficiencies. The aim of this study was to explore characteristics of enuresis referrals on the waiting list for a general medicine clinic at a tertiary paediatric hospital. METHODS: An audit was conducted to examine all enuresis referrals on the general medicine outpatient clinic waiting list in February 2019 at The Royal Children's Hospital, Melbourne. Enuresis referrals with an organic cause and those for children less than 5 years of age were excluded. RESULTS: Of the 2613 referrals on the general medicine waiting list, 486 of 2613 (19%) were related to enuresis. The median age of patients on the waiting list was 8 years and 65% (315/486) were male. Sufficient detail was provided to determine temporal and disease stratification in 45% (218/486) of referrals; primary versus secondary enuresis, and monosymptomatic versus non-monosymptomatic enuresis. The mean number of days on the waiting list calculated at the time of data extraction (13 February 2019) was 226 (±179) days. CONCLUSIONS: The findings from this study suggest that there are long waiting times for enuresis services and referrals often do not contain complete information.

Management of thoracic empyema in children: a survey of the Australia and New Zealand Association of Paediatric Surgeons (ANZAPS).

PURPOSE: To define the spectrum of management for thoracic empyema in children in Australia and New Zealand. METHODS: Online survey of members of the Australian and New Zealand Association of Paediatric Surgeons (ANZAPS), limited to consultant/attending paediatric surgeons. RESULTS: A total of 54/80 (67.5%) members, from 16 paediatric surgical centres, responded. The majority (33/54, 61%) preferred chest drain with fibrinolytics, whilst 21/54 (39%) preferred video-assisted thoracoscopic surgery (VATS) with drain insertion. Urokinase was the most commonly used fibrinolytic (64%). There were no significant differences in management preferences between practising surgeons in Australia and New Zealand (p = 0.54), nor between consultants who had been practising a shorter (< 5 years) or longer (> 20 years) amount of time (p = 0.21). The practices described by the surveyed ANZAPS members were in line with the Thoracic Society of Australia and New Zealand recommendations for the management of paediatric empyema. CONCLUSION: Across Australia and New Zealand there exists significant variation surrounding the intra- and post-intervention management of thoracic empyema in children. The surveyed paediatric surgeons demonstrated a preference for fibrinolytics over the use of VATS. All management regimens were within published local guidelines.

Selective approach to preoperative echocardiography in esophageal atresia.

PURPOSE: Preoperative echocardiography is used routinely in neonates with esophageal atresia to identify patients in whom congenital cardiac disease will impact upon anesthetic and surgical decision-making. We aimed to determine the suitability of selective preoperative echocardiography. METHODS: We performed a single-center retrospective review of neonates with esophageal atresia over 6 years (2010-2015) at our tertiary pediatric institution. Data included preoperative clinical examination, chest x-ray, and echocardiography. Endpoints were cardiovascular, respiratory, radiological, and echocardiography findings. Selective strategies were assessed using sensitivity, specificity, positive predictive value, and negative predictive value. RESULTS: We identified 115 neonates with esophageal atresia. All underwent preoperative echocardiography. Cardiac defects were identified in 49/115 (43%) (major 9/115, moderate 4/115). Sensitivity, specificity, positive predictive value, and negative predictive value of abnormal clinical and radiologic assessment for major and moderate cardiac defects were 92%, 25%, 13%, 96%; for clinical examination alone were 92%, 25%, 14%, 96%; for absence of murmur, cyanosis, and abnormal respiratory examination were 92%, 28%, 13%, 97%. Selective strategies reduce echocardiograms performed by 22%. CONCLUSION: Selective strategies allow for identification of neonates with esophageal atresia who may have deferral of echocardiogram unill after surgery. Selection may improve timeliness of care and resource utilization, without compromising patient safety.

Radiation burden in patients with esophageal atresia: a systematic review.

Esophageal atresia (EA) is the most common congenital esophageal disorder. Radiological imaging facilitates diagnosis, surgical interventions, and follow-up. Despite this, standardized monitoring guidelines are lacking. We aimed to: (1) review the literature regarding radiation burden in children with EA; (2) establish the presence of guidelines for diagnosis and follow-up in children with EA. The systematic review was performed according to PRISMA protocol. Two investigators conducted independent searches (PubMed, Ovid, Cochrane Review) and data extraction. Analysis focused on pre- and post-operative imaging type and frequency to determine the radiation burden. Seven studies met the inclusion criteria (337 patients). All authors agreed upon the need to minimize radiation burden, recommending symptoms-guided management, use of dosimeters, and non-radiating imaging. One study identified a median 130-fold increase in cumulative lifetime cancer risk in children with EA compared with other babies in the special care unit. The most common investigations were X-ray and CT (pre-operatively), and X-ray and contrast swallow (post-operatively). Standardized guidelines focused upon the frequency and type of radiological imaging for children with EA are lacking. Children with EA are subjected to more radiation exposure than the general population. Implementation of non-radiating imaging (ultrasonography, manometry) is recommended.

Predictors of Mortality after Primary Discharge from Hospital in Patients with Esophageal Atresia.

OBJECTIVE: To describe esophageal atresia mortality rates and their associations in our cohort. STUDY DESIGN: Patients with esophageal atresia, managed at The Royal Children's Hospital, Melbourne (1980-2018), who subsequently died, were retrospectively identified from the prospective Nate Myers Oesophageal Atresia database. Data collected included patient and maternal demographics, vertebral anomalies, anorectal malformations, cardiovascular anomalies, tracheoesophageal fistula, renal anomalies, and limb defects (VACTERL) associations, mortality risk factors, and preoperative, operative, and postoperative findings. Mortality before discharge was defined as death during the initial admission. RESULTS: A total of 88 of the 650 patients (13.5%) died during the study period; mortality before discharge occurred in 66 of the 88 (75.0%); mortality after discharge occurred in 22 of the 88 (25.0%). Common causes of mortality before discharge were palliation for respiratory anomalies (15/66 [22.7%]), associated syndromes (11/66 [16.7%]), and neurologic anomalies (10/66 [15.2%]). The most common syndrome leading to palliation was trisomy 18 (7/66 [10.6%]). Causes of mortality after discharge had available documentation for 17 of 22 patients (77.3%). Common causes were respiratory compromise (6/17 [35.3%]), sudden unexplained deaths (6/17 [35.3%]), and Fanconi anemia (2/17 [11.8%]). Of the patients discharged from hospital, 22 of 584 (3.8%) subsequently died. There was no statistical difference in VACTERL association between mortality before discharge (31/61 [50.8%]) and mortality after discharge (11/20 [55.0%]), nor in incidence of twins between mortality before discharge (8/56 [14.3%]) and mortality after discharge (2/18 [11.1%]). CONCLUSIONS: We identified predictors of mortality in patients with esophageal atresia in a large prospective cohort. Parents of children with esophageal atresia must be counselled appropriately as to the likelihood of death after discharge from hospital.

Polyethylene Glycol Dosing for Constipation in Children Younger Than 24 Months: A Systematic Review.

OBJECTIVES: Evaluate safety and effectiveness of Polyethylene glycol (PEG) for chronic constipation in children aged younger than 24 months. Identify the optimum dose of PEG to manage chronic constipation in children aged younger than 24 months. METHODS: In this systematic review, Embase, Medline Ovid, Pubmed, and the Cochrane Library were searched between January 1, 2000 and February 1, 2019. Studies investigating functional constipation, in which patients younger than 24 months of age were treated with PEG, were considered as potentially eligible for review. Two authors screened the studies against inclusion/exclusion criteria. Study quality was assessed with the PEDro quality assessment, Cochrane risk of bias tool, and/or the Newcastle-Ottawa Scale. RESULTS: Five studies (2 randomized controlled trials, 3 retrospective chart reviews) satisfied selection criteria (n = 459). All studies employed different dosage categories: mean effective maintenance dose, mean initial dose, mean short-term and long-term dose, and mean daily dose. Dosage regimens were variable, with 0.45 to 1.1 g ·â€Škg ·â€Šday for PEG3350 and 0.48 to 0.65 g ·â€Škg ·â€Šday for PEG4000. Adverse effects were transient across all studies for all types of PEG; these included diarrhea and abdominal pain. CONCLUSIONS: This systematic review provided evidence for a lack of reported side effects from PEG for children aged younger than 24 months. Evidence to establish appropriate dosage regimens does not exist.An infographic accompanying this article can be found at http://links.lww.com/MPG/B839.

Retrospective audit of referral and triage pathways of paediatric patients with constipation and soiling.

AIM: This study aimed to explore referral and triage pathways for paediatric patients referred to an Australian hospital with bowel dysfunction (isolated or mixed bowel and bladder). METHODS: We conducted a retrospective audit of patients who attended their first clinic appointment during April to June 2014. Patients were included if they: (i) were a new patient referred for symptoms of constipation, soiling, daytime urinary incontinence or enuresis; and (ii) attended the encopresis, general medicine, continence, gastroenterology, paediatric surgery, urology, renal or Child and Adolescent Mental Health clinic. Patients with an organic cause (e.g. Hirschsprung disease) for their dysfunction were excluded. RESULTS: Of 1485 new patients seen at our targeted clinics, 281 (18.9%) had symptoms of bowel and/or bladder dysfunction. After excluding patients aged younger than 3 years (n = 43) and those with isolated bladder dysfunction (n = 130), 56 were referred for isolated bowel dysfunction and 52 for mixed bowel and bladder dysfunction. The median wait time from referral to first appointment was 3.8 months. Median wait times varied across symptom groups (isolated bowel, 4.6 months; mixed 3.4 months) and clinics (encopresis, 7.7 months; general medicine, 2.5 months). Over a 12-month period, patients attended an average of 3.5 appointments (isolated bowel, mean 3 appointments; mixed, mean 4 appointments). CONCLUSION: Paediatric patients with symptoms of bowel and bladder dysfunction wait several months to be seen in a public tertiary referral hospital. Alternative pathways for care, such as community-based primary care, need to be explored to improve timely management.

Paediatric constipation for general paediatricians: Review using a case-based and evidence-based approach.

Constipation is a common problem in childhood. The most common type of constipation is functional, accounting for 90-95% of all cases. The aim of this review is to provide clinical scenarios with treatment using evidence-based information, and management strategies and a clinical algorithm to guide the management of constipation in children. Recent guidelines and online information sites are detailed. Clinical red flags and organic causes of constipation are included. Four clinical scenarios are presented: case (1) 4-month-old child with constipation since birth and likely Hirschsprung disease; case (2) 6-month-old infant with infant dyschezia; case (3) 4-year old with functional constipation; and; case (4) 9-year old with treatment resistant constipation. Children with functional constipation need a thorough history and physical exam to rule out the presence of any 'red flags' but do not require laboratory investigations. Management includes education and demystification, disimpaction followed by maintenance therapy with oral laxatives, dietary counselling and toilet training. Treatment options differ between infants and children. Disimpaction and maintenance regimens for common laxatives are presented. On treatment failure or on suspicion of organic disease the patient should be referred for further evaluation. The radionuclide intestinal transit study (scintigraphy) is a useful modality for evaluation and planning of management in treatment-resistant children. Treatment options for treatment-resistant patients are presented. High-level evidence (meta-analyses) for pharmalogical and non-pharmalogical treatment modalities are reviewed and an algorithm for assessment and treatment are presented.

Paediatric bladder dysfunction: A single centre experience of public hospital wait times.

AIM: Paediatric bladder dysfunction, including daytime urinary incontinence and enuresis, is a common and distressing condition. Unfortunately, children with these symptoms are often on waitlists for several months. This treatment delay may significantly impact upon the child and family unit. This study aimed to quantify waiting times for children who had attended hospital outpatient clinics for symptoms of wetting. METHODS: A retrospective review was undertaken for patients who had been referred to The Royal Children's Hospital, Melbourne outpatient clinics for symptoms of wetting (with/without bowel symptoms). Data regarding the referral and triage pathway, up to the time of the first clinic appointment, were collected. These data were compared to a previous audit conducted in the same setting. RESULTS: A total of 101 clinic attendances were included in this study. The overall waiting time, from receipt of referral to the patient's first clinic attendance, was a median of 181 days (n = 94 valid responses; range 7-695). Wait times for patients with isolated symptoms of wetting were similar to patients with mixed bowel and bladder dysfunction (187 and 171.5 days, respectively). Most patients were triaged to the continence clinic (n = 68), whilst smaller proportions of patients were seen in the encopresis (n = 14), urology (n = 13), general medicine (n = 2), gastroenterology (n = 1) and nurse-led enuresis clinic (n = 3). CONCLUSIONS: The waiting times for patients with wetting generally exceeded 5 months. Alternative pathways for triage need to be explored to manage demand and improve wait times.

Management of Hirschsprung disease in Australia and New Zealand: a survey of the Australian and New Zealand Association of Paediatric Surgeons (ANZAPS).

PURPOSE: To define the practice of management for Hirschsprung disease (HD) in Australia and New Zealand. METHODS: Online survey of Australian and New Zealand Association of Paediatric Surgeons (ANZAPS) members. RESULTS: 56/80 (70%) members from 17 centres responded. DIAGNOSIS: 100% perform suction rectal biopsies; 40% perform a contrast enema. HISTOPATHOLOGICAL STAINING: H&E (94%), ACHE (70%) and calretinin (75%). SURGERY: Primary pull-through (PT) is performed by 88% (100% by < 6/12 months). The Soave-Boley PT is the preferred approach (85%), with laparoscopic assistance (77%) and muscle cuff division (93%). Routine post-operative dilatations are performed by 63% of respondents. If symptoms persist following PT, majority adopt a conservative approach (enemas/laxatives 90%; Botox 74%). If a long-segment is identified at PT, 60% fashion a stoma and delay definitive surgery. If total colonic aganglionosis is identified at PT, 76% fashion a stoma and delay definitive surgery. A dedicated bowel management program is available in 45% of centres with transition to adult services in 29%. CONCLUSIONS: A laparoscopic-assisted Soave-Boley PT is the most common technique for recto-sigmoid HD. Differences are noted in both the management of long-segment/total aganglionosis HD and post-operative management/follow-up.

Impact of Esophageal Atresia on the Success of Fundoplication for Gastroesophageal Reflux.

OBJECTIVES: Fundoplication is commonly performed in patients with a history of esophageal atresia (EA), however, the success of this surgery is reduced, as reflected by an increased rate of redo fundoplication. We aimed to determine whether EA impacts the prevalence of fundoplication, its timing, and performance of a redo operation. STUDY DESIGN: A single-center, retrospective review of all patients undergoing fundoplication over a 20-year period (1994-2013) was performed. Redo fundoplication was used as a surrogate for surgical failure. RESULTS: A total of 767 patients (patients with EA 85, those who did not have EA 682) underwent fundoplication during the study period. Median age (months) at primary fundoplication was lower in patients with EA (7.2 vs those who did not have EA 23.3; P < .001). Redo fundoplication rates between groups were not significantly different (EA 11/85 vs 53/682; P = .14). Median time (months) between primary and redo fundoplication was greater in patients with EA (36.2 vs 11.7; P = .03). CONCLUSIONS: Contrary to popular belief, the incidence of redo fundoplication was not significantly increased in patients with a history of EA. However, patients with EA underwent fundoplication at younger ages, which may be related to early life-threatening events in these patients. These results inform perioperative counseling, and highlight the importance of sustained surgical follow-up in patients with EA.

Can MR enterography screen for perianal disease in pediatric inflammatory bowel disease?

BACKGROUND: Pediatric Crohn's disease is associated with perianal disease (PAD). Magnetic resonance enterography (MRE) assesses small bowel involvement in pediatric inflammatory bowel disease (PIBD). Pelvic MRI (P-MRI) is the gold standard for assessing PAD. PURPOSE: To determine if MRE can accurately detect PAD in PIBD, distinguishing perianal fistulae (PAF) from perianal abscesses (PAA), referenced against P-MRI. STUDY TYPE: Retrospective. POPULATION: Seventy-seven PIBD patients, 27 females (mean age 14.1 years), with P-MRI and MRE within 6 months. FIELD STRENGTH/SEQUENCE: 1.5T and 3T; P-MRI: sagittal fat suppressed (FS) T2 fast spin-echo (FSE), coronal short tau inversion recovery, axial T1 FSE, coronal and axial postcontrast FS T1 FSE; MRE: coronal balanced steady-state free-precession (SSFP), coronal cine SSFP, coronal and axial single-shot T2 FS, axial SSFP, coronal ultrafast 3D T1 -weighted gradient echo FS (3D T1 GE), axial diffusion-weighted imaging, coronal and axial postcontrast 3D T1 GE FS. ASSESSMENT: Two radiologists independently, then by consensus, assessed randomized MRI exams, recording PAF number, location, and length; and PAA number, location, length, and volume. Sensitivity analysis used clinical disease as the gold standard, calculated separately for P-MRI and MRE. STATISTICAL TESTS: Comparing MRE and P-MRI consensus data, sensitivity, specificity, positive, and negative predictive values (P/NPV) were calculated. Inter- and intrareader reliability were assessed using kappa statistics. RESULTS: P-MRI and MRE were paired, detecting PAD in 73 patients, PAF in 63, and PAA in 31 P-MRI. MRE sensitivities, specificities, PPV, and NPV were: PAD 82%, 100%, 100%, 23%; PAF 74%, 71%, 92%, 38%; PAA 51%, 85%, 69%, 72%; clinical 82%, 22%, 37%, 69%; clinical P-MRI 96%, 8%, 37%, 80%. MRE interreader agreement for PAD was moderate (kappa = 0.51 [0.29-0.73]), fair for PAF and PAA. DATA CONCLUSION: Using a standard technique, MRE can detect PAD with high specificity and moderate sensitivity in PIBD, missing some PAF and small PAA. LEVEL OF EVIDENCE: 3 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018;47:1638-1645.