Endoscopic repair of tracheoesophageal fistulas: A contemporary multi-institutional case series and literature review.

OBJECTIVES: Recurrent and primary tracheoesophageal fistulas (TEFs) are a challenging surgical pathology to treat, as standard open surgical approaches are associated with high morbidity and mortality. As such, endoscopic modalities have gained interest as an alluring alternative, yet variable success rates have been reported in the literature. The aim of this study was to provide a contemporary update of the literature and describe our institutional experience with the bronchoscopic obliteration of recurrent and primary TEFs. METHODS: Retrospective chart review of all pediatric patients having undergone endoscopic TEF repair at two pediatric academic centers in Montreal, Canada and Lille, France between January 1, 2008 to December 31, 2020. RESULTS: 28 patients with TEFs (20 recurrent, 8 primary) underwent a total of 48 endoscopic procedures. TEF repair was performed under endoscopic guidance using various combinations of techniques, including fistula de-epithelialization (endoscopic brush, thulium laser, trichloroacetic acid-soaked pledgets or electrocautery), tissue adhesives, submucosal augmentation, esophageal clip and stenting. Successful closure was achieved in 16 patients (57 %), while 12 (43 %) required eventual open or thoracoscopic repair. The mean number of endoscopic procedures was 1.7. There were no major treatment-related complications such as pneumothorax, mediastinitis or death (mean follow-up 50.8 months). CONCLUSIONS: Endoscopic repair of recurrent or primary TEFs is a valuable component of our therapeutic armamentarium and may contribute to decreased surgical morbidity in this complex patient population. Families should be counselled that endoscopic results may be more modest than with open or thoracoscopic approaches, and multiple procedures may be required.

Disease severity impacts perceived quality of life in congenital diaphragmatic hernia: a prospective observational study.

BACKGROUND: While research indicates comparable quality of life (QOL) in congenital diaphragmatic hernia (CDH) and healthy populations, the effect of CDH severity on patients' health perceptions remains unexplored. We aimed to assess QOL perception in CDH, hypothesising a decline correlated with increased disease severity. METHODS: In this prospective observational study, we analysed patients with CDH aged 5 years and above participating in a longitudinal outpatient programme. We excluded bilateral CDH, genetic/syndromic conditions, prematurity and late diagnosis. Participants self-administered the age-adapted Pediatric Quality of Life Inventory (PedsQL) survey, covering four domains (physical, emotional, social, school). After enrolment, data were collected blind to severity status (larger defects denoting significant/'severe' disease). Repeated measurements were managed using a random mixed-effects model. RESULTS: Of 34 participants (50% males) who completed the PedsQL, 10 provided measurements at two visits. Eight required a patch (type C), while 26 had primary repairs (type A=8; type B=18). Age at first evaluation was comparable across groups (no patch: median 11 (7-16), patch: 13 (8-15) years, p=0.78). Severe CDH correlated significantly with lower PedsQL scores (adjusted ß: -18%, 95% CI -28%; -7%, adjusted for age at visit and sex). Lower scores specifically occurred in walking, exercising, social and academic functioning. CONCLUSION: Severe CDH significantly lowers QOL. This finding is crucial for resource allocation in long-term CDH health surveillance and advocates for regular inclusion of patient experiences in quality improvement efforts.

Gastrostomy Tube Use in Pediatrics: A Systematic Review.

CONTEXT: Despite frequency of gastrostomy placement procedures in children, there remains considerable variability in preoperative work-up and procedural technique of gastrostomy placement and a paucity of literature regarding patient-centric outcomes. OBJECTIVES: This review summarizes existing literature and provides consensus-driven guidelines for patients throughout the enteral access decision-making process. DATA SOURCES: PubMed, Google Scholar, Medline, and Scopus. STUDY SELECTION: Included studies were identified through a combination of the search terms "gastrostomy," "g-tube," and "tube feeding" in children. DATA EXTRACTION: Relevant data, level of evidence, and risk of bias were extracted from included articles to guide formulation of consensus summaries of the evidence. Meta-analysis was conducted when data afforded a quantitative analysis. EVIDENCE REVIEW: Four themes were explored: preoperative nasogastric feeding tube trials, decision-making surrounding enteral access, the role of preoperative imaging, and gastrostomy insertion techniques. Guidelines were generated after evidence review with multidisciplinary stakeholder involvement adhering to GRADE methodology. RESULTS: Nearly 900 publications were reviewed, with 58 influencing final recommendations. In total, 17 recommendations are provided, including: (1) tTrial of home nasogastric feeding is safe and should be strongly considered before gastrostomy placement, especially for patients who are likely to learn to eat by mouth; (2) rRoutine contrast studies are not indicated before gastrostomy placement; and (3) lLaparoscopic placement is associated with the best safety profile. LIMITATIONS: Recommendations were generated almost exclusively from observational studies and expert opinion, with few studies describing direct comparisons between GT placement and prolonged nasogastric feeding tube trial. CONCLUSIONS: Additional patient- and family-centric evidence is needed to understand critical aspects of decision-making surrounding surgically placed enteral access devices for children.

The impact of a lipid injectable emulsion (SMOF) on conjugated bilirubin levels in children receiving prolonged parenteral nutrition: A large single center experience.

BACKGROUND: The SMOFlipid is a composite emulsion that has showed benefits, but limited data is available on children receiving prolonged parenteral nutrition (PN). This study aimed to compare conjugated bilirubin (CB) levels at the end of ILE administration in this population. METHODS: Medical charts of all infants treated with Intralipid (Jan 2012-Sep 2013) or SMOFlipid (Oct 2013-Dec 2016) were reviewed. Only infants that received PN for ≥28 consecutive days were included. Laboratory data were extracted from the closest day of initiation and discontinuation of the ILE (±7 days). For the primary objective, an analysis of covariance was employed, adjusting for initial CB values and total days of ILE administration. CB values were log-transformed to normalize distribution. Statistical tests were two-sided and performed at the significance level <0.05. RESULTS: A total of 150 infants were included: 72 used Intralipid for 82 times and 88 received SMOFlipid in 92 occasions. The incidence of cholestasis was 20% (Intralipid) and 4.5% (SMOFlipid). Infants treated with SMOFlipid had significantly lower CB levels at the end of ILE administration with geometric mean ratio between groups of 1.7 (95% CI:1.0, 2.8; p < 0.05). CONCLUSION: In a large and heterogenous group of infants receiving PN for ≥28 consecutive days the final levels of CB were significantly lower with SMOFlipid when compared to Intralipid suggesting a protective role of this type of ILE in this high-risk population. CLINICAL RELEVANCY STATEMENT: SMOFlipid is an emulsion that has showed benefits, but limited data is available on children receiving prolonged parenteral nutrition (PN). This study compared conjugated bilirubin (CB) levels at the end of ILE administration in infants that received PN for ≥28 consecutive days with either SMOFlipid or Intralipid. In a large number of patients with several gastrointestinal diseases lower CB levels were observed with the use of SMOFlipid with geometric mean ratio between groups of 1.7 (95% CI:1.0, 2.8; p < 0.05). Our results demonstrate a protective role of this type of ILE in this high-risk population.

Risk stratification helps identify congenital diaphragmatic hernia (CDH) infants in need of formal neurodevelopmental assessment: Observations from a structured, interdisciplinary long-term follow-up clinic.

BACKGROUND/PURPOSE: Neurodevelopmental delay (NDD) affects congenital diaphragmatic hernia (CDH) infants. Initial assessment by experienced developmental pediatricians, supported by Bayley-3 tests, is a viable pathway for NDD identification and surveillance. We risk stratified CDH infants to observe differences in incidence and type of NDD based on disease severity. METHODS: Patients from a CDH long-term follow-up database started in 2012 were reviewed (REB#2019-4583). Risk stratification into low, moderate, and high-risk cohorts was performed using the CDH Study Group Mortality Prediction Score. Patients requiring ECLS, supplemental oxygen at 30 days and patch repair were also considered high-risk (i.e. usual clinical criteria). Post-discharge NDD assessments by developmental pediatricians and occupational therapists (Bayley-3) were analyzed for all patients >18months. NDD incidence and type per risk group was determined using descriptive statistics. RESULTS: Of 102 CDH patients included for study, 26% (27/102) had NDD. Risk stratification identified 2(2%), 7(7%), and 18(18%) patients with NDD in the low, moderate and high-risk groups, respectively. Language delay (2 low; 6 moderate; 10 high) was the most prevalent. Three patients had both expressive and receptive language delay. Motor deficits were observed almost exclusively in the high-risk group. CONCLUSION: Based on our experience, NDD affects one-quarter of CDH infants. Risk stratification helped identify infants at increased risk of NDD. While language delays predominated across all risk groups, multiple deficits occurred in higher risk cohorts. These patients should receive structured NDD assessment as part of an optimal interdisciplinary CDH care pathway.

Xanthan- and Rice Cereal-Based Thickeners in Infants: A Multidisciplinary Single-Center Experience.

Some infants aspirate thin liquids and must be fed thickened liquids in order to protect the lungs. However, thickeners have not been fully studied for safety. Xanthan-based thickeners have been implicated in the development of necrotizing enterocolitis and rice cereal-based thickeners have been associated with constipation and excessive weight gain. The aim of this study was to compare rates of adverse events between both thickeners. Methods: Single-center retrospective chart review conducted at a tertiary pediatric care center between January 2013 and July 2017. All infants deemed unsafe for oral feeding and treated with xanthan- or rice cereal-based milk thickeners were included. Data were extracted from the medical records and patients categorized according to the type of thickener. Primary outcome was the occurrence of diarrhea, constipation, overweight, and obesity at 3-6 and 6-12 months after thickener initiation. Appropriate statistical tests were used. In addition, an e-mail was sent to 14 level III Canadian Pediatric hospitals inquiring about their practice. Results: We identified 53 patients to be included in the study; 20 used xanthan-based- and 33 used rice cereal-based milk thickeners. Rates of diarrhea, constipation, overweight, and obesity at 3-6 and 6-12 months after initiation were not different between thickeners. Important variability concerning thickening practices was reported by the 8 centers that responded. Conclusions: In infants treated with milk thickeners, xanthan-based or rice cereal-based thickeners may have similar safety profiles that require further investigation including a larger number of patients.

Multiple Faces of Eyelid Involvement in Sarcoidosis.

PURPOSE: To report three cases of eyelid involvement by sarcoidosis. In one of them, it mimicked a malignant lesion. METHODS: Retrospective case reports. RESULTS: A 73-year-old man presented with destruction of the left lower eyelid for 2 years. He had granulomatous uveitis in the left eye. Chest CT scan showed parenchymal abnormalities that could correspond to sarcoidosis. Skin biopsy revealed noncaseating granuloma. Oral and topical corticosteroids resulted in improvement of the condition. A 72-year-old female patient presented with cutaneous infiltration of the left upper eyelid for 1 month. Biopsy was consistent with sarcoidosis. Endobronchial biopsies showed interstitial fibrosis. Oral prednisone improved the condition. A 65-year-old female patient presented with edema of the right upper eyelid for 2 months. Full-thickness biopsy showed granuloma without necrosis. There was an improvement with oral steroid. CONCLUSION: Although eyelid involvement in sarcoidosis is uncommon, different forms of presentation, including destructive lesions, can be observed.

Orbitopalpebral and ocular sarcoidosis: what does the ophthalmologist need to know.

Sarcoidosis is a chronic multisystemic disease of unknown aetiology, characterised by non-caseating granulomas. Ocular involvement rate ranges from 30% to 60% among individuals with sarcoidosis, and can vary widely, making the diagnosis a challenge to the ophthalmologist. Cutaneous manifestations occur in about 22% of sarcoidosis cases, but eyelid involvement is rare. Eyelid swelling and nodules are the most frequent forms of eyelid involvement, but other findings have been reported. The joint analysis of clinical history, ancillary exams and compatible biopsy is needed for the diagnosis, as well as the exclusion of other possible conditions. This review aims to describe the different forms of presentations, the clinical reasoning and treatment options for ocular, eyelid and orbital sarcoidosis.

Mammalian Target of Rapamycin Inhibitors Vs Calcineurin Inhibitors in Chronic Graft Rejection After Lung Transplantation: A Systematic Review and Meta-Analysis.

BACKGROUND: The number of lung transplantations has been rising constantly. However, use of this therapeutic resource is limited by several issues that are difficult to resolve, such as chronic graft rejection and complications secondary to immunosuppression. METHODS: This systematic review compared mammalian target of rapamycin (mTOR) inhibitor immunosuppression associated with low-dose calcineurin inhibitors with isolated calcineurin inhibitor immunosuppression on the new-onset chronic rejection development and mortality 12 months after lung transplantation. Three controlled randomized clinical trials (SHITRIT, NOCTET, and 4EVERLUNG) were selected from electronic databases. RESULTS: Meta-analysis of the data at 12 months postintervention showed that only 4EVERLUNG assessed chronic graft rejection, with a higher incidence in the control group; however, the difference was not statistically significant (P = .197). Significant data were related to an increase in the number of adverse events (P = .0064) and improved renal function (P < .0001) in the mTOR inhibitor-based scheme. The other outcomes indicated a trend toward greater risk of death and acute graft rejection with the use of mTORs. CONCLUSIONS: The researchers suggest considering the use of mTOR inhibitors, whose greatest benefit is felt by patients with renal dysfunction, in association with the use of calcineurin inhibitors, because of the imminent risk of death among patients with renal failure.

How did Covid-19 impact US household foods? an analysis six months in.

Using a nationwide survey of primary grocery shoppers conducted in August 2020, we examine household food spending when the economy had partially reopened and consumers had different spending opportunities in comparison to when the Covid-19 lockdown began. We estimate the impact of Covid-19 on household spending using interval and Order Probit regressions. Income levels, age, access to grocery stores and farmers markets, household demographic information, along with other independent variables are controlled in the model. Findings show that middle-class households (with income below $50,000, or with income between $50,000 and $99,999) are less likely to increase their grocery expenditures during the pandemic. Households with children or elderlies that usually require higher food quality and nutrition intakes had a higher probability of increasing their spending during Covid-19 than before. Furthermore, consumers' spending behaviors were also significantly affected by their safe handing levels and the Covid-19 severity and food accessibility in their residences.

Clinical Aspects of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis With Severe Ocular Complications in Brazil.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute and potentially fatal inflammatory vesiculobullous reactions that affect the skin and mucous membranes, and which are most often triggered by particular medications and infections. In Brazil, the drugs most frequently associated with TEN and SJS include cold medicine such as dipyrone and NSAIDs, followed by carbamazepine, phenobarbital, penicillin, and allopurinol. Genetic variations have been found to increase the risk of SJS/TEN in response to triggering factors such as medications. The most closely associated genes found in Brazilian cold-medicine-related SJS/TEN patients with severe ocular complications are HLA-A*66:01 in those of mixed African and European ancestry and HLA-B*44:03 and HLA-C*12:03 in those of solely European ancestry. Our classification system for grading ocular surface complication severity in SJS/TEN patients revealed the most severe complications to be limbal stem cell deficiency and dry eye. Changes to the conjunctival flora have also been observed in SJS/TEN patients. Our group identified bacterial colonization in 95% of the eyes (55.5% of which were gram-positive cocci, 25.5% of which were gram-negative bacilli, and 19% of which were gram-positive bacilli). Several new treatment options in the acute and chronic ocular management of the SJS/TEN patients have been described. This article highlights some Brazilian institutions' contributions to ocular surface care in both the acute phase (including the use of amniotic membrane transplantation) and the chronic phase (such as eyelid margin and fornix reconstruction, minor salivary gland transplantation, amniotic membrane and limbal transplantation, scleral contact lenses, anti-angiogenic eyedrops for corneal neovascularization, ex-vivo cultivated limbal epithelium transplantation, conjunctival-limbal autografting, oral mucosa transplantation, and keratoprosthesis).

Vulvar swelling and the timely diagnosis of Crohn's disease: A case report.

Children with vulvar swelling often present to multiple physicians and face a prolonged period of ambiguity and extraneous treatments. Here, we report a case of an 8-year-old girl who presented to the dermatology clinic with a 2-month history of vulvar swelling and was ultimately diagnosed with Crohn's disease. Although extra-intestinal manifestations are common in inflammatory bowel diseases, they rarely present before an existing diagnosis. This report highlights this potential early manifestation and demonstrates how timely diagnosis leads to improved outcomes for patients, their families, and healthcare systems.

Prolonged Use of Ethanol Lock Prophylaxis With Polyurethane Catheters in Children With Intestinal Failure: A Single-Center Experience.

BACKGROUND: Children with intestinal failure (IF) receiving home parenteral nutrition (HPN) require long-term central venous catheters (CVCs). Ethanol lock prophylaxis (ELP) can reduce central line-associated bloodstream infections, but there are some concerns of increased breakage rates when used with polyurethane catheters. We reviewed our experience using ethanol locks in both polyurethane and silicone CVCs. METHODS: A 10-year retrospective study of children with IF receiving HPN that used ELP was conducted. Complications per 1000 catheter days were extracted and a multivariable, mixed-effects Poisson model was used to compare catheter breakage rates and other complications between polyurethane and silicone CVCs. RESULTS: A total of 10 patients were included, comprising 85 CVCs and 13,227 catheter days. The most common cause of IF was necrotizing enterocolitis. Breakages were the most common complication: polyurethane 1.46/1000 vs silicone 3.76/1000 catheter days. Silicone catheters had a significantly higher breakage rate (adjusted rate ratio [RR], 2.86; 95% confidence interval [CI], 2.84-2.88; P < .001) whereas polyurethane catheters had higher rates of occlusion (adjusted RR, 0.14; 95% CI, 0.07-0.28; P < .001) and displacements. However, there were no differences in the overall catheter replacement rates and any other catheter-related outcomes. CONCLUSIONS: In children with IF receiving long-term HPN, the use of ELP in polyurethane catheters was associated with a lower risk of breakages when compared with silicone CVCs. These results should be regarded as preliminary data, and further studies with a higher number of participants are necessary to provide a better level of evidence.

Ustekinumab for the treatment of refractory pediatric Crohn's disease: a single-center experience.

BACKGROUND/AIMS: Despite the well-established efficacy of tumor necrosis factor (TNF) antagonists as treatment options for Crohn's disease, many pediatric patients need a change in therapy due to adverse events and loss of response, highlighting the necessity for medications with a different mechanism of action. Ustekinumab has been shown to be effective in inducing clinical remission in some adults with disease refractory to anti-TNF agents, however, minimal data exists in the pediatric population. METHODS: We conducted a retrospective chart review of 11 pediatric patients receiving ustekinumab, specifically extracting baseline data, information on prior treatment and response, indications for starting ustekinumab, clinical information, and laboratory parameters pre- and post-therapy. Clinical response was defined as a decrease in abbreviated Pediatric Crohn's Disease Activity Index score. RESULTS: Patients ranged from 12 to 17 years of age upon initiation of treatment with ustekinumab. Five of 11 patients demonstrated a clinical response. Among these patients, 2 remained in clinical remission, while the remaining 3 experienced a secondary loss of response. The other 6 patients were primary nonresponders who either remained unwell or demonstrated slight clinical worsening. All patients who clinically responded to ustekinumab and had an initially elevated CRP experienced complete normalization of their values. Mucosal healing was seen on endoscopy in 1 responder, with 2 other patients showing endoscopic improvement. CONCLUSIONS: These results demonstrate for the first time that ustekinumab has the potential to induce not only clinical and biochemical remission, but also endoscopic improvement, in the pediatric population. Future research is needed to determine factors that influence response to therapy.

Validação de instrumento avaliativo para capacitação de enfermeiros em ressuscitação cardiopulmonar / Validation of evaluating instrument for training nurses in cardiopulmonary resuscitation / Validación de instrumento evaluador para la formación de enfermeras en reanimación cardiopulmonar

Objetivo: validar critérios de um instrumento para avaliação da capacitação de enfermeiros em ressuscitação cardiopulmonar. Método: estudo metodológico para validação de critério de um instrumento previamente validado quanto ao conteúdo. Realizou-se a aplicação de um instrumento pré e pós-teste de uma capacitação com 20 enfermeiros de um hospital do interior do Estado de São Paulo. Resultados: realizou-se a análise das respostas do pré e pós-teste sobre o entendimento dos participantes antes e após a realização de teoria atrelada a simulação sobre ressuscitação cardiopulmonar. Foram avaliadas as variáveis objetividade, simplicidade, clareza e pertinência, destacando-se que os participantes concordaram que os critérios citados foram atendidos. Conclusão: o instrumento investigado possui relação com os padrões definidos, sendo comprovadamente validado. Ao utilizar-se deste instrumento durante a avaliação de capacitações em ressuscitação cardiopulmonar, as equipes de trabalho, alunos e pacientes serão beneficiados, garantindo a padronização e melhor efetividade no atendimento a PCR

Objective: to validate criteria of an instrument for assessing the qualification of nurses in cardiopulmonary resuscitation. Method: methodological study for criterion validation of a previously validated instrument for content. The application of a pre and post test instrument of a training with 20 nurses from a hospital in the interior of the state of São Paulo was performed. Results: pre and post test answers were analyzed on the participants' understanding before and after the theory of the cardiopulmonary resuscitation simulation. The variables objectivity, simplicity, clarity and relevance were evaluated, highlighting that the participants agreed that the criteria mentioned were met. Conclusion: the instrument investigated is related to the defined standards and has been validated. By using this instrument during the assessment of training in cardiopulmonary resuscitation, workteams, students and patients will benefit through standardization and effectiveness of care

Objetivo: validar los criterios de un instrumento para evaluar la calificación de las enfermeras en reanimación cardiopulmonar. Método: estudio metodológico para la validación de criterios de un instrumento previamente validado para el contenido. Se realizó la aplicación de un instrumento de prueba previa y posterior a una capacitación con 20 enfermeras de un hospital en el interior del estado de São Paulo. Resultados: las respuestas previas y posteriores a la prueba se analizaron según la comprensión de los participantes antes y después de la teoría de la simulación de reanimación cardiopulmonar. Se evaluaron las variables objetividad, simplicidad, claridad y relevancia, destacando que los participantes estuvieron de acuerdo en que se cumplieron los criterios mencionados. Conclusión: el instrumento investigado está relacionado con los estándares definidos y ha sido validado. Al utilizar este instrumento durante la evaluación de la capacitación en reanimación cardiopulmonar, los equipos de trabajo, los estudiantes y los pacientes se beneficiarán mediante la estandarización y la eficacia de la atención