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.

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.

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.

Survival Trends and Syndromic Esophageal Atresia.

BACKGROUND AND OBJECTIVES: Presence of a syndrome (or association) is predictive of poor survival in esophageal atresia (EA). However, most reports rely on historical patient outcomes, limiting their usefulness when estimating risk for neonates born today. We hypothesized improved syndromic EA survival due to advances in neonatal care. METHODS: A retrospective single-center review of survival in 626 consecutive patients with EA from 1980 to 2017 was performed. Data were collected for recognized risk factors: preterm delivery; birth weight <1500 g; major cardiac disease; vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities (VACTERL); and non-VACTERL syndromes. Cox proportional hazards regression models were used to evaluate temporal trends in survival with respect to year of birth and syndromic EA. RESULTS: Overall, 87% of 626 patients with EA survived, ranging from 82% in the 1980s to 91% in the 2010s. After adjusting for confounders, syndromic EA survival did not improve during the study, with no association found between year of birth and survival (hazard ratio [HR] 0.98, 95% confidence interval [CI]: 0.95-1.01). Aside from lethal non-VACTERL syndromes, patients with nonlethal non-VACTERL syndromes (HR 6.85, 95% CI: 3.50-13.41) and VACTERL syndrome (HR 3.02, 95% CI: 1.66-5.49) had a higher risk of death than those with nonsyndromic EA. CONCLUSIONS: Survival of patients with syndromic EA has not improved, and patients with non-VACTERL syndromes have the highest risk of death. Importantly, this is independent of syndrome lethality, birth weight, and cardiac disease. This contemporary survival assessment will enable more accurate perinatal counseling of parents of patients with syndromic EA.

H-type congenital tracheoesophageal fistula: Insights from 70 years of The Royal Children's Hospital experience.

BACKGROUND: The long-term outcomes of H-type tracheoesophageal fistula (TOF), an uncommon variant of esophageal atresia/tracheoesophageal fistula (OA/TOF), are rarely described in the literature. We reviewed our institutional experience of 70 years. METHODS: The Nate Myers Oesophageal Atresia Database was queried for patients with an H-type TOF (1948-2017). Data included presentation, diagnostic workup, surgical management, and outcomes. RESULTS: Of 1088 patients with OA/TOF, 56 (5.1%) had an H-type TOF. The most common presenting symptoms were cyanotic episodes (68%), choking with feeds (52%), and aspiration pneumonitis (46%). The majority (82%) were symptomatic in the first week of life. Coexisting congenital anomalies were present in 46%: cardiac (13/56, 23%), genitourinary (10/56, 18%), and vertebral/skeletal (9/56, 16%). Patients were consistently diagnosed with prone contrast tube esophagogram (77% sensitivity on the first study and 96% after a second study). The fistula was most commonly approached through a right cervical collar incision. Right vocal cord palsy occurred in 22%, with one case of bilateral palsies. Other complications included leak (5.6%), recurrence (9.3%), stricture (1.9%), and diverticulum (1.9%). Although there was a trend towards a lower recurrence rate when interposition material was used, this was not statistically significant (3.3% vs 16.7%, p = 0.16). Survival in operative cases was 98.2%, and when all diagnosed cases were considered was 89.3%. CONCLUSIONS: We have reported the largest single-center series of H-type TOF. Diagnosis is challenging, and surgical morbidity remains high. Despite this, long-term outcomes are favorable. LEVEL OF EVIDENCE: IV.

Esophageal morbidity in patients following repair of esophageal atresia: A systematic review.

BACKGROUND: Esophageal atresia (EA) is a life-threatening congenital condition, affecting one in 2600 newborns. Morbidity remains high, with many patients experiencing complications, including anastomotic leak/stricture, and gastro-esophageal reflux disease (GERD). Increased understanding of esophageal motility patterns may help explain the etiology of these complications. AIMS: We aimed to review knowledge regarding esophageal motility and related complications in children with EA, evaluate patients' symptomatology and relate this to esophageal motility. METHODS: We performed a systematic review (PROSPERO: CRD42018092277), according to the PRISMA protocol. Two investigators independently conducted search strategies (OvidMEDLINE, PubMed, Cochrane Review, BMJ BestPractice), identifying complications in patients following EA repair. Rates of esophageal dysmotility, GERD, dysphagia, anastomotic leak, anastomotic stricture, recurrent fistula formation, and esophagitis were sought. RESULTS: A total of 65 publications met selection criteria (n = 4882). Rates of morbidity were high: esophageal dysmotility (78%), GERD (43%), dysphagia (44%), anastomotic leak (19%), anastomotic stricture (26%), recurrent fistula formation (7%), and esophagitis (47%). No correlation appeared to exist with severity of symptoms. CONCLUSIONS: This systematic review identified high rates of complications in children with EA, with esophageal dysmotility present in the majority of patients. Increasing survival, with resultant longer timeframes to develop morbidities, makes standardized follow-up regimens crucial. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level 3.

Surgical management of perianal abscess in neonates and infants.

BACKGROUND: The optimal management of perianal abscess in neonates and infants remains unclear, including the need for laying open of the fistula and the role of microscopy and culture studies (MCS). We aimed to report the recurrence rate following incision and drainage alone (I&D) compared to incision and drainage with laying open of the fistula (I&DF) and to determine the value of MCS in perianal abscess management. METHODS: Following ethical approval (16326Q), a 10-year (2007-2017) review of children younger than 1 year presenting with a perianal abscess was performed. Presence of a fistula was sought in all patients. Data are presented as number of cases (%), median (range) and analysed using Fisher's exact test and Mann-Whitney U-test. P-values of <0.05 were considered significant. RESULTS: We identified 108 patients (107 (99.1%) males) with 111 abscesses (three bilateral); 26 in I&D group and 85 in I&DF group. Initial abscess occurred to the right of midline in 64 cases (58%) and to the left of midline in 47 cases (42%). Twenty-two (20%) recurred after 30 (6-372) days. Sixty-five (59%) had MCS performed. Recurrence was higher in I&D group (9/26) versus I&DF group (13/85) (P = 0.04 (relative risk 2.2, 95% confidence interval 1.0-4.5)). There was no difference in recurrence within each group between patients with or without MCS (I&D group, P = 0.1; I&DF group, P = 0.3). CONCLUSION: The recurrence of surgically managed perianal abscess is lower when a fistula is identified and laid open at the initial operation. There is little value of MCS in the management of paediatric perianal abscess.

The burden of esophageal dilatations following repair of esophageal atresia.

AIM: To describe the burden of esophageal dilatations in patients following esophageal atresia (EA) repair. METHOD: A retrospective review was performed at The Royal Children's Hospital, Melbourne, of all neonates undergoing operative repair for EA over a 17-year period (1999-2015). Stricture was defined by radiological and/or intra-operative findings of narrowing at the esophageal anastomosis. Data recorded included EA type, perinatal details, operative approach, esophageal anastomosis outcome, dilatation requirement, and survival. Key endpoints were anastomotic leakage and tension, esophageal dilatation technique, dilatation frequency, fundoplication, and complications. RESULTS: During the study period, 287 newborn EA patients were admitted, of which 258 underwent operative repair and survived to primary discharge. Excluding 11 patients with isolated tracheoesophageal fistula, 247 patients were included in the final analysis. Intra-operative anastomotic tension was documented in 41/247 (16.6%), anastomotic leak occurred in 48/247 (19.4%), and fundoplication was performed in 37/247 (15.0%). Dilatations were performed in 149/247 (60.3%). Techniques included bougie-alone (92/149, 61.7%), combination of bougie and balloon (51/149, 34.2%), and balloon-alone (6/149, 4.0%). These patients underwent 1128 dilatations; median number of dilatations per patient was 4 (interquartile range 2-8). Long-gap EA and anastomotic tension were risk factors (p < 0.01) for multiple dilatations. Complications occurred in 13/1128 (1.2%) dilatation episodes: 11/13 esophageal perforation, 2/13 clinically significant aspiration. Perforations were rare events in both balloon (6/287, 2.1%) and bougie dilatations (4/841, 0.5%); one patient had a perforation from guidewire insertion. CONCLUSIONS: Esophageal dilatation occurred in a majority of EA patients. Long-gap EA was associated with an increased burden of esophageal dilatation. Perforations were rare events in balloon and bougie dilatations. TYPE OF STUDY: Original article - retrospective review. LEVEL OF EVIDENCE: II.

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.

Quality of life assessment in esophageal atresia patients: a systematic review focusing on long-gap esophageal atresia.

BACKGROUND: Children born with esophageal atresia (EA) have inherent abnormalities in esophageal motility which may impact upon patient and family Quality of Life (QoL). Currently, paucity of data exists for long-term outcomes of long-gap EA. We aimed to: (1) summarize QoL tools reported in the literature, focusing upon studies involving long-gap EA patients, and (2) compare QoL for long-gap versus non-long-gap EA patients. METHOD: We performed a systematic review of Cochrane Register of Controlled Trials, PubMed, EMBASE, and Ovid databases (January 1980-May 2018) in accordance with the PRISMA protocol. RESULT: Six studies were identified (536 patients total), and 419/536 (78%) patients completed QoL assessment. Response rates ranged from 29% to 100%. Median study size was 86 (range 8-159). Esophageal atresia type was described in 477 patients, and 74/477 (16%) were long-gap. Common assessment tools were Gastrointestinal Quality of Life Index and 36-Item Short-Form Health Survey. Compared with healthy individuals, long-gap EA patients suffered more gastrointestinal symptoms. There were no significant differences in QoL outcomes between long-gap and non-long-gap EA patients. CONCLUSION: Current literature suggests no significant difference in QoL outcomes between long-gap and non-long-gap EA patients. However, due to questionnaire variability and range of response rates, the data should be interpreted with care. LEVEL OF EVIDENCE: Level II.

Medical and surgical management of pediatric perianal crohn's disease: A systematic review.

BACKGROUND: The timely management of pediatric Crohn's disease (CD), and specifically perianal CD, is important owing to the possible adverse effects on growth, development, and quality of life. Perianal involvement is increasingly common, with up to 62% of pediatric CD patients affected. Presently, literature addressing the management of perianal CD has focused primarily on adults, with findings that cannot always be extrapolated to the pediatric population. We aimed to review the rates of healing, recurrence, and need for surgical intervention in perianal CD to provide evidence-based recommendations for the ideal management in children. METHOD: We conducted a systematic review of CENTRAL, PubMed, Medline, and EMBASE databases (January 1997-December 2017) in accordance with PRISMA. Two independent reviewers performed data extraction. RESULT: Ten studies met the inclusion criteria with a combined total of 538 patients. Median study population size was 17 (range 7-276), with a median age at intervention of 13.9 years (range 1-18). Seton placement allowed complete healing in 28.6% of children. Similar results (28.5%) were seen in children undergoing fecal diversion. One study demonstrated complete resolution of fistulizing disease in 70% of children treated with infliximab (IFX). One quarter of patients treated with IFX required further surgical intervention for disease control. Recurrence occurred most frequently in children undergoing Seton placement alone (5/14, 35.7%), compared with IFX (46/197, 23.4%) and combination therapy (12/276, 4.3%). CONCLUSION: In the pediatric population, a combination of medical and surgical treatment is required to control perianal CD, with fewer side effects. LEVEL OF EVIDENCE: Level II.

Clinical state of the paediatric acute scrotum in south-eastern Victoria.

BACKGROUND: Acute scrotal pain is a common paediatric surgical presentation. Delays in treatment can result in testicular loss from torsion. It is unclear where delays occur. We aimed to investigate presentations with an acute scrotum to identify any potential areas of delay. METHODS: We conducted a prospective study (April 2017-November 2018) of paediatric patients (<18 years) presenting with acute scrotal pain. Data collected included: patient demographics, history/examination findings, mode of presentation, clinical timeline details and outcomes. RESULTS: A total of 107 acute scrotum presentations were identified: 58 (54.2%) testicular appendage torsion, 23 (21.5%) testicular torsion, 6 (5.6%) epididymo-orchidits and 20 (18.7%) other diagnoses. Median age at presentation was 11 years (4 months-16 years). Fifty-seven (53.3%) underwent emergency surgery, of whom 23 (40.4%) had testicular torsion, with 2 requiring orchidectomy. Median time from onset of symptoms to seeking medical opinion was 5.5 (0-135) h. Once assessed by a medical professional, the route to paediatric surgical review via general practitioner (GP) and local emergency department (ED) to paediatric ED was 4.84 (1.67-24.5) h; via GP to paediatric ED was 2.58 (0.75-25.5) h; via local ED to paediatric ED was 2.25 (1-7.75) h; and directly to paediatric ED was 0.45 (0-1.42) h. CONCLUSION: Delays in assessment and treatment of acute scrotal pain occur from the time parents are aware of symptoms to seeking medical opinion. Education to increase awareness may reduce time delays. GPs should refer patients directly to a paediatric ED. Local EDs should manage paediatric cases as per the local surgeons' skill base.

Current surgical practice in pediatric ulcerative colitis: A systematic review.

BACKGROUND: Surgical management of adult ulcerative colitis (UC) is well-studied, but not readily applicable to children. Restorative proctocolectomy with ileal pouch-anal anastomosis (RPC-IPAA), performed as one-, two-, or three-stage procedure, is preferred in pediatric patients with adequate anal sphincter function. PURPOSE: METHODS: Systematic review of Cochrane Register of Controlled Trials, PubMed, and EMBASE databases was conducted (January 1987-December 2016), in accordance with PRISMA. RESULTS: Twelve retrospective studies were identified (568 patients total); 31, 334, and 203 patients underwent one-, two-, and three-stage procedures, respectively. Median study size was 31 patients (range 10-202), median age was 13 years (range 2-21), median follow-up was 4 years (range 0.08-16). Postoperative complications included pouchitis, bowel obstruction, stricture, fistula, pouch failure, anastomotic leak, and wound infections. Calcineurin inhibitor usage improved pediatric ulcerative colitis activity index (PUCAI) score. Higher PUCAI scores correlated with likelihood of staged procedures. Number of stages did not restrict quality of life. CONCLUSIONS: Paucity of data exists, comparing preoperative factors leading to staged procedures in pediatric UC. This systematic review identifies an area for future studies. LEVEL OF EVIDENCE: II.

Inflammatory Myofibroblastic Tumor of the Urinary Bladder: A Case Report.

Inflammatory myofibroblastic tumor is a rare but benign clinical entity. Its ability to mimic malignancy poses a diagnostic challenge. Here, we report the first case in Australia, of inflammatory myofibroblastic tumor in the bladder in a 40-year-old male, removed via transurethral resection.

Synovial fluid findings in children with knee monoarthritis in lyme disease endemic areas.

BACKGROUND: Although Lyme and septic arthritis of the knee may have similar clinical presentations, septic arthritis requires prompt identification and treatment to avoid joint destruction. We sought to determine whether synovial fluid cell counts alone can discriminate between Lyme, septic, and other inflammatory arthritis. METHODS: We conducted a retrospective cohort study of children aged 1 to 18 years with knee monoarthritis who presented to 1 of 2 pediatric emergency departments located in Lyme endemic areas. We included children who had both a synovial fluid culture and an evaluation for Lyme disease. Septic arthritis was defined as a positive synovial fluid culture or synovial fluid pleocytosis (white blood cell [WBC] ≥40,000 cells/µL) with a positive blood culture. Lyme arthritis was defined as positive Lyme serology without a positive bacterial culture. All other children were considered to have other inflammatory arthritis. We compared the synovial fluid counts by arthritis type. RESULTS: We identified 384 children with knee monoarthritis, of whom 19 (5%) had septic arthritis, 257 (67%) had Lyme arthritis and 108 (28%) had other inflammatory arthritis. Children with other inflammatory arthritis had lower synovial WBC and absolute neutrophil count, as well as percent neutrophils, than those with either Lyme or septic arthritis. There were no significant differences in the synovial fluid WBC, absolute neutrophil count, and percent neutrophils for children with Lyme and septic arthritis. CONCLUSIONS: In Lyme endemic areas, synovial fluid results alone do not differentiate septic from Lyme arthritis. Therefore, other clinical or laboratory indicators are needed to direct the care of patients with knee monoarthritis.

Cost-effectiveness of thrombolysis within 4.5 hours of acute ischemic stroke: experience from Australian stroke center.

BACKGROUND AND PURPOSE: Previous economic studies outside Australia have demonstrated that patients treated with tissue-type plasminogen activator (tPA) within 4.5 hours of stroke onset have lower healthcare costs than those not. We aim to perform cost-effectiveness analysis of intravenous tPA in an Australian setting. METHODS: Data on clinical outcomes and costs were derived for 378 patients who received intravenous tPA within 4.5 hours of stroke onset at Royal Melbourne Hospital (Australia) between January 2003 and December 2011. To simulate clinical outcomes and costs for a hypothetical control group assumed not to have received tPA, we applied efficacy data from a meta-analysis of randomized trials to outcomes observed in the tPA group. During a 1-year time-horizon, net costs, years of life lived, and quality-adjusted life-years were compared and incremental cost-effectiveness ratios derived for tPA versus no tPA. RESULTS: In the study population, mean (SD) age was 68.2 (13.5) years and 206 (54.5%) were men. Median National Institutes of Health Stroke Scale score (interquartile range) at presentation was 12.5 (8-18). Compared with no tPA, we estimated that tPA would result in 0.02 life-years and 0.04 quality-adjusted life-years saved per person>1 year. The net cost of tPA was AUD $55.61 per patient. The incremental cost-effectiveness ratios were AUD $2377 per life-year saved and AUD $1478 per quality-adjusted life-years saved. Because the costs of tPA are incurred only once, the incremental cost-effectiveness ratios would decrease with increasing time-horizon. Uncertainty analyses indicated the results to be robust. CONCLUSIONS: Intravenous tPA within 4.5 hours represents a cost-effective intervention for acute ischemic stroke.

Ross procedure in children: 17-year experience at a single institution.

BACKGROUND: The Ross procedure in children carries substantial mortality and reoperation rate. Aortic root dilatation is of concern. To prevent dilatation of the neoaortic root, but permit normal growth, we began to apply an absorbable poly-(p-dioxanone)-filaments (PDS) band at the sino-tubular (ST)-junction. METHODS AND RESULTS: All children (n=100) who underwent Ross procedure during 1995-2012 were studied. Mean age at operation was 8.6±6.1 years (median 8.3 years, range 3 days to 18 years); 19 patients were younger than 1 year of age. The root replacement (n=91, Ross-Konno procedure in 29 patients), root inclusion (n=6), and subcoronary implantation (n=3) techniques were used. Operative mortality was 6% (6/100, 4 neonates, 2 infants). Age of <1-year at time of operation was a risk factor for early death (P<0.001). Mean follow-up time was 7.0±4.8 years (median 7.4 years, range 5 days to 16 years). Late mortality was 4.3% (4/94). Freedom from moderate or greater neoaortic valve insufficiency (AI) at 5 and 10 years was 89% and 83%, respectively. Freedom from neoaortic valve reoperation at 5 and 10 years was 96% and 86%, respectively. Aortic dilatation to Z-score >4 was greatest at the ST-junction (23%, 11/48) compared to the aortic annulus (17%, 11/66) and sinuses (14%, 7/50). Since 2001, a PDS band was placed around the ST-junction in 19 patients. Survivors with the PDS band had less AI (0 versus 20%, P=0.043) compared to survivors (n=35) without the PDS at 4.1±3 years. CONCLUSIONS: The Ross procedure in children can be performed with acceptable results. Children younger than 1 year of age have higher mortality, but not an increased autograft reoperation rate. Stabilization of the ST-junction may reduce AI.

Distinguishing Lyme from septic knee monoarthritis in Lyme disease-endemic areas.

OBJECTIVE: Because Lyme and septic arthritis may present similarly, we sought to identify children with knee monoarthritis at low risk for septic arthritis who may not require arthrocentesis. METHODS: We performed a retrospective study of children with knee monoarthritis presenting to 1 of 2 pediatric centers, both located in Lyme disease-endemic areas. Septic arthritis was defined by a positive result on synovial fluid culture or synovial fluid pleocytosis with a positive blood culture result. Lyme arthritis was defined as a positive Lyme serologic result or physician-documented erythema migrans rash. All other children were considered to have other inflammatory arthritis. A clinical prediction model was derived by using recursive partitioning to identify children at low risk for septic arthritis, and the model was then externally validated. RESULTS: We identified 673 patients with knee monoarthritis; 19 (3%) had septic arthritis, 341 (51%) had Lyme arthritis, and 313 (46%) had other inflammatory arthritis. The following predictors of knee septic arthritis were identified: peripheral blood absolute neutrophil count ≥10 × 10(3) cells per mm(3) and an erythrocyte sedimentation rate ≥40 mm/hour. In the validation population, no child with a absolute neutrophil count <10 × 10(3) cells per mm(3) and an erythrocyte sedimentation rate <40 mm/hour had septic arthritis (sensitivity: 6 of 6 [100%], 95% confidence interval [CI]: 54-100; specificity: 87 of 160 [54%], 95% CI: 46-62). Overall, none of the 19 children with septic arthritis were classified as low risk (10%, 95% CI: 0-17). CONCLUSIONS: Laboratory criteria can be used to identify children with knee monoarthritis at low risk for septic arthritis who may not require diagnostic arthrocentesis.