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BACKGROUND: Diagnosing paediatric Crohn's disease (CD) based on histology can present challenges. We evaluate the histological spectrum of treatment-naïve biopsies from children with CD and assess these findings' diagnostic and predictive value. METHODS: Three cohorts were identified: (1) 137 patients with CD, (2) 116 patients with ulcerative colitis (UC) and (3) 50 patients without inflammatory bowel disease. Biopsies from the gastrointestinal (GI) tract were re-examined for signs of active and chronic inflammation, including lymphocyte-pattern oesophagitis, focal enhancing gastritis and indicators of chronicity. Additionally, granulomas and microgranulomas (defined as clusters of 4-9 epithelioid histiocytes) were evaluated. RESULTS: Lymphocyte-pattern oesophagitis was observed in 15% of patients (n=20). Moderate-to-severe diffuse gastritis was noted in 50.4% of patients (n=68), while focal enhancing gastritis was identified in 11.1% (n=15). In terminal ileal biopsies, 46.1% exhibited activity and 5.3% showed features of chronicity. Active colitis was present in 73% of patients (n=100), with chronic colitis seen in 11.7% (n=16). Granulomas and microgranulomas were observed in 31.4% (43/137) and 48.9% (67/137) of patients, respectively. Notably, 30.7% (42/137) of patients with microgranulomas were without granulomas. Previously undetected microgranulomas were found in 20 of 27 cases. 2.5% of patients with UC and none of the control cohort showed microgranulomas. Lymphocyte-pattern oesophagitis was associated with an increased need for anti-tumor necrosis factor (TNF) therapy (p=0.007). CONCLUSIONS: GI microgranulomas, often overlooked, are specific to CD in the proper clinical context. Oesophageal lymphocytosis may predict a need for more aggressive treatment. The study brings to light under-recognised aspects of CD's histological diagnosis, including the oversight of microgranulomas, the high prevalence of diffuse gastritis and low prevalence of focal enhancing gastritis, the frequent absence of terminal ileitis and the infrequent occurrence of chronic colitis.
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BACKGROUND: The management of acute hemorrhage in patients with previously treated head and neck squamous cell carcinoma (HNSCC) is challenging due to the lack of substantial evidence to guide clinical decision making. METHODS: A systematic review and retrospective chart review were performed to identify patients with a history of HNSCC who underwent either primary or adjuvant radiation therapy (RT) and presented with hemorrhagic complications requiring embolization. Patient characteristics, history, presentation, and outcomes were reviewed. RESULTS: The systematic review included a total of 182 patients. Heterogeneity existed in outcomes reporting; 1-year overall survival approached 50%. From the retrospective chart review, 51 patients were included. Median survival time following hemorrhage was 2.2 months (range 1.2-11.4 months). Patients with malignancy at time of hemorrhage were identified as having worse survival. CONCLUSIONS: Acute hemorrhage in patients with a history of previously radiated HNSCC portends a high risk of mortality, with patients with active malignancy representing a worse prognostic group.
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Transient cortical blindness is a known complication of iodinated contrast administration and is believed to reflect osmotic injury or autoregulatory dysfunction of the posterior circulation. Here, we report 2 cases of postangiography transient cortical weakness, a rare clinical analog to transient cortical blindness that affects the anterior circulation. The symptoms, timeline, and imaging findings of transient cortical weakness are distinct from more common post-procedural complications such as acute ischemic stroke or transient ischemic attack.
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Defining monogenic drivers of autoinflammatory syndromes elucidates mechanisms of disease in patients with these inborn errors of immunity and can facilitate targeted therapeutic interventions. Here, we describe a cohort of patients with a Behçet's- and inflammatory bowel disease (IBD)-like disorder termed "deficiency in ELF4, X-linked" (DEX) affecting males with loss-of-function variants in the ELF4 transcription factor gene located on the X chromosome. An international cohort of fourteen DEX patients was assessed to identify unifying clinical manifestations and diagnostic criteria as well as collate findings informing therapeutic responses. DEX patients exhibit a heterogeneous clinical phenotype including weight loss, oral and gastrointestinal aphthous ulcers, fevers, skin inflammation, gastrointestinal symptoms, arthritis, arthralgia, and myalgia, with findings of increased inflammatory markers, anemia, neutrophilic leukocytosis, thrombocytosis, intermittently low natural killer and class-switched memory B cells, and increased inflammatory cytokines in the serum. Patients have been predominantly treated with anti-inflammatory agents, with the majority of DEX patients treated with biologics targeting TNFα.
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Artrite , Síndrome de Behçet , Produtos Biológicos , Doenças Inflamatórias Intestinais , Masculino , Humanos , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/genética , Doenças Inflamatórias Intestinais/diagnóstico , Doenças Inflamatórias Intestinais/genética , Artralgia , Proteínas de Ligação a DNA , Fatores de Transcrição/genéticaRESUMO
OBJECTIVE: Despite the adoption of same-day outpatient surgical procedures in some specialties, it remains common practice to admit patients for monitoring after elective endovascular treatment of brain aneurysms to monitor for complications. The necessity of such monitoring has not been fully characterized. Here, the authors reviewed the utilization of imaging during posttreatment hospitalization, a surrogate measure for workup of suspected complications requiring hospital resources, to infer the value of inpatient monitoring after endovascular aneurysm treatment. METHODS: Clinical and angiographic data from eligible patients were retrospectively assessed for demographic characteristics, imaging indications, timing of imaging, and imaging findings. Patients were included if they underwent elective endovascular brain aneurysm treatment, and patients were excluded if significant intraprocedural complications occurred. The recorded imaging modalities included CT, MRI, catheter-based imaging, and ultrasound; plain radiographs were excluded. Multivariable logistic regression analysis was performed to identify predictors of the need for posttreatment imaging. RESULTS: In total, 1229 elective endovascular procedures for brain aneurysm treatment were included. Patients underwent imaging before discharge in 13.4% (165/1229) of cases, with significant findings in 5.0% (61/1229) of cases. The median (interquartile range) time to first posttreatment imaging was 13.2 (4.2-22.8) hours. The need for imaging during posttreatment hospitalization was positively associated with larger aneurysm size (p < 0.05) and negatively associated with underlying cardiovascular disease (p < 0.05). CONCLUSIONS: More than 1 in 8 patients who underwent elective endovascular brain aneurysm treatment required imaging during posttreatment hospitalization, most within the first 24 hours, and 1 in 20 had significant findings. These results suggest the importance of short-term hospitalization after elective endovascular aneurysm treatment.
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Aneurisma da Aorta Abdominal , Implante de Prótese Vascular , Procedimentos Endovasculares , Aneurisma Intracraniano , Humanos , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/cirurgia , Aneurisma da Aorta Abdominal/etiologia , Aneurisma da Aorta Abdominal/cirurgia , Estudos Retrospectivos , Procedimentos Endovasculares/métodos , Resultado do Tratamento , Implante de Prótese Vascular/métodos , Hospitalização , Hospitais , Procedimentos Cirúrgicos Eletivos , Fatores de RiscoRESUMO
BACKGROUND: Intracranial aneurysms of the middle cerebral artery can be treated using several open surgical and endovascular approaches. Given the growing evidence of clinical equipoise between these various treatment strategies, there is a need to assess the costs associated with each. METHODS: Cost of aneurysm treatment was divided into two categories for comparison. "Initial cost" comprised the total in-hospital expenses for initial aneurysm treatment and "total cost" comprised initial aneurysm treatment and all expenses relating to readmission due to treatment-related complications, prescribed catheter angiograms for monitoring of treatment stability, and any retreatments needed for a given aneurysm. The open surgical group was subdivided into a pterional approach group and a lateral supraorbital (LSO) approach group for. RESULTS: Median initial cost was $37,152 (IQR $31,318-$44,947) for aneurysms treated with the pterional approach, $29,452 (IQR $27,779-$32,826) for aneurysms treated with the LSO approach, and $19,587 (IQR $14,125-$30,521) for aneurysms treated with endovascular approaches. The median total cost was $39,737 (IQR $33,891-$62,259) for aneurysms treated with the pterional approach, $31,785 (IQR $29,513-$41,099) for aneurysms treated with the LSO approach, and $24,578 (IQR $18,977-$34,547) for aneurysms treated with endovascular approaches. Analysis of variance test demonstrated variance across groups for both initial and total cost (p = 0.004, p = 0.008, respectively). In our subsequent analysis, initial cost and total cost were higher in the pterional group than the endovascular group (p = 0.003 and p = 0.006, respectively). CONCLUSIONS: Endovascular treatment of elective aneurysms has a significantly lower cost than open surgical treatment with the pterional approach, but not with the LSO approach. For aneurysms not amenable to endovascular treatment, a minimally invasive LSO approach carries a lower cost burden than a pterional approach.
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Pediatric brain arteriovenous malformations are a major cause of morbidity and mortality, with the harmful effects of this disease compounded by the additional disability-years experienced by children with ruptured or other symptomatic arteriovenous malformations. In addition to the risks shared with their adult counterparts, pediatric patients frequently experience recurrence following radiographic cure, which presents an additional source of morbidity and mortality. Therefore, there is a need to synthesize potential mechanisms contributing to the elevated recurrence risk in the pediatric population and discuss how these translate to practical considerations for managing these patients.
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BACKGROUND: Dural arteriovenous fistulas (dAVFs) draining into the vein of Galen (VoG) are complex lesions that often necessitate treatment to minimize the risk of rupture and relieve symptoms. These lesions can be treated with open surgical resection, radiosurgery, or endovascular embolization. Unfortunately, endovascular treatment of dAVFs involving the VoG has not been robustly assessed across large patient cohorts. To meet this need, we performed a retrospective review of dAVFs involving the VoG at our center, and included these in a meta-analysis to identify the safety and efficacy of endovascular embolization, as well as describing current treatment trends for this disease. METHODS: Consecutive patients with dAVFs involving the VoG treated at a single center were identified from a prospective database and retrospectively reviewed. A literature search was conducted with defined search criteria, and eligible studies were included alongside our cohort in a meta-analysis. Rates of complete dAVF treatment and clinical complications were pooled across studies with a random effects model and reported with a 95% CI. RESULTS: Five dAVFs involving the VoG were treated endovascularly at our center during the study period. In this series, 80% of treatments led to complete occlusion of the fistula while no patients had clinical complications. Onyx was used for all treatments. In our meta-analysis, the overall rate of complete occlusion was 72.0% (95% CI 59.8% to 84.1%) and the overall rate of clinical complications was 10.0% (95% CI 4.7% to 15.3%). CONCLUSIONS: Endovascular approaches for dAVFs involving the VoG are technically feasible, but carry a risk of clinical complications. Future work should identify optimal endovascular embolic agents.
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BACKGROUND AND PURPOSE: Stent-assisted coiling of wide neck bifurcation aneurysms in the anterior communicating segment and basilar tip region can be performed with varying stent configurations, including single stenting or Y-stenting. Y-stenting requires two stents and thus incurs greater cost and procedural complexity than single-stent constructs. The influence of first stent type on the need for Y-stenting remains unknown. MATERIALS AND METHODS: Clinical and angiographic data were retrospectively obtained for patients that underwent stent-assisted coiling for basilar tip or anterior communicating aneurysms at a high-volume center. Patients were included in this study if stent-assisted coiling was performed using Neuroform Atlas or LVIS Jr stents. A multivariate binary logistic regression was performed to measure the influence of first stent type on the need for Y-stenting. RESULTS: Stent-assisted coiling was used to treat 82 aneurysms in 81 patients during the study period, and Y-stenting was performed in 18.3% (15/82) of cases. In multivariate logistic regression analysis, use of LVIS Jr. as the first stent did not significantly influence the need for subsequent Y-stenting after controlling for aneurysm morphology (OR 0.65, 95% CI 0.18-2.43). CONCLUSION: Controlling for aneurysm morphology and location, the use of Y-stenting for stent-assisted coiling was not independently influenced by the choice of LVIS Jr or Neuroform Atlas as the first stent. A larger cohort may reveal differences between these two stents, particularly for aneurysms with large neck sizes.
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Embolização Terapêutica , Procedimentos Endovasculares , Aneurisma Intracraniano , Humanos , Estudos Retrospectivos , Resultado do Tratamento , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/cirurgia , Angiografia Cerebral , StentsAssuntos
Fístula Arteriovenosa , Malformações Vasculares do Sistema Nervoso Central , Embolização Terapêutica , Humanos , Prevalência , Malformações Vasculares do Sistema Nervoso Central/epidemiologia , Malformações Vasculares do Sistema Nervoso Central/terapia , Fístula Arteriovenosa/terapia , Angiografia Cerebral , Dura-MáterRESUMO
Tools and techniques utilized in endovascular brain aneurysm treatment have undergone rapid evolution in recent decades. These technique and device-level innovations have allowed for treatment of highly complex intracranial aneurysms and improved patient outcomes. We review the major innovations within neurointervention that have led to the current state of brain aneurysm treatment.
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Studies involving human intestinal tissue are essential for advancing the field of celiac disease (CeD), as diagnosis requires duodenal biopsies. Performing studies in children helps to better understand CeD in this important subpopulation. This study aims to determine the risk in obtaining duodenal research biopsies during pediatric endoscopy. In this retrospective chart review from 2016 to 2022 of 1180 research subjects and controls, there were 18 procedure-related adverse events within 48 hours. Most adverse events were for symptoms of pain and fever. There was no increased risk of adverse events if additional duodenal research biopsies were taken during pediatric endoscopy.
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Doença Celíaca , Duodeno , Humanos , Criança , Estudos Retrospectivos , Biópsia/efeitos adversos , Duodeno/patologia , Endoscopia Gastrointestinal/efeitos adversos , Mucosa Intestinal/patologia , Doença Celíaca/diagnóstico , Doença Celíaca/patologiaRESUMO
BACKGROUND AND PURPOSE: Flow diversion of intracranial aneurysms with the Pipeline Embolization Device (PED) is frequently performed, but the outcomes of retreatment for aneurysms that failed to occlude after prior treatment with PED have not been well studied. Here, we report the safety and efficacy of PED retreatment after initial failure to occlude. MATERIALS AND METHODS: Clinical and angiographic data from eligible patients were retrospectively assessed for demographics, aneurysm occlusion status, and clinical outcomes. Patients were included in this study if they underwent PED retreatment to treat an aneurysm that had previously been treated with PED. RESULTS: Retreatment of previously flow-diverted aneurysms with PED was performed in 42 cases. At final angiographic follow-up, angiographic improvement was observed after 45% (19/42) of retreatments and complete aneurysm occlusion was observed following 26% (11/42). Significant clinical complications occurred in 10% (4/42) of PED retreatments. CONCLUSIONS: Retreatment of intracranial aneurysms with PED following initial failure to achieve aneurysm occlusion has a low rate of subsequent complete aneurysm occlusion.
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Embolização Terapêutica , Aneurisma Intracraniano , Humanos , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/terapia , Aneurisma Intracraniano/etiologia , Resultado do Tratamento , Estudos Retrospectivos , Embolização Terapêutica/métodos , Angiografia Cerebral , Retratamento , SeguimentosRESUMO
BACKGROUND: The assessment of pediatric residents applying to subspecialty fellowship programs relies on faculty letters of recommendation (LOR). However, it is unclear if pediatric faculty are confident that their LOR are effective. OBJECTIVE: This study aims to assess the confidence of pediatric faculty in writing an effective LOR for pediatric residents applying to subspecialty fellowship programs. METHODS: Survey development was conducted using evidence-based best practices. Surveys were distributed via email in 2021 to all full-time pediatric faculty members who taught pediatric residents in a large academic medical center. Categorical values were compared by chi-square test. RESULTS: Eighty-five out of 150 (57%) faculty members completed the survey. Forty-one percent of participants were very confident that their LOR provided adequate content to assess residents during the application process. Confidence was associated with higher academic rank (p=0.02), frequent contact with residents (p=0.01), and writing >2 LOR in the last five years (p=0.0002). Confident LOR writers were more likely to describe their own background, details about the resident's scholarly activity, and the resident's ability to work as part of a team. Thirty-five percent of respondents reported never considering gender bias when writing LOR, whereas 28% reported always considering gender bias. Eighty-seven percent of respondents reported an interest in receiving LOR writing guidelines. CONCLUSION: Half of the faculty respondents were not very confident in their ability to write an effective LOR for pediatric residents applying for a fellowship. Faculty development and standardized instructions on writing effective LOR may be helpful both at the institutional and national levels, including the importance of considering gender bias when writing LOR.
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Purpose: The aim of this study was to explore potential correlation of the MR imaging features and clinical characteristics with formation of perianal abscess in children with Crohn's perianal fistulas (CPF). Methods: From 2010 to 2020, pediatric patients with CPF diagnosis on their first pelvic MRI were identified retrospectively. All patients were divided into two groups based on the presence or absence of perianal abscess. Baseline clinical and MRI characteristics were recorded for each patient. All the statistical calculations were performed using R (version 3.6.3). Results: A total of 60 patients [F:M 17:43, median age 14 years (IQR 10-15), ranging 3-18 years] were included in this study. Forty-four abscesses were identified in 36/60 children (mean volume 3 ± 8.6â ml, median 0.3â ml). In 24/60 patients with perianal disease, no abscess was detected on the MRI. Ten patients (28%) showed perianal abscess on pelvic MRI at the initial diagnosis. The rate of active disease on colonoscopy (visible ulcerations/aphthous ulcers) was similar in both groups (95% vs. 94%). With regards to disease location, the majority of patients (40/60, 66.6%) in both groups had ileocolonic CD. All patients without abscess had a single perianal fistula (n = 24; 3 simple and 21 complex fistulae), however, patients with perianal abscess tended to have >1 fistulous tracts (n = 50 fistulas; all complex, 27 single, 10 double and 1 triple). Intersphincteric fistula was the most common fistula type in both groups (79% and 66%, p = 0.1). The total length of fistula (3.8 ± 1.7 vs. 2.8 ± 0.8â cm, p = 0.006) and presence of multiple external openings (n = 25 vs. 7, p = 0.019) were significantly higher in patients with abscesses, and fistula length >3.3â cm showed 80% specificity and 83% PPV for the presence of perianal abscess. Fistulas were symptomatic (pain, bleeding or drainage) at similar rates in both groups (68% and 70%, p = 0.1). Conclusion: Pediatric patients with CPF who develop perianal abscess have a distinct imaging phenotype defined by longer fistula length (>3.3â cm), multiple skin openings and multiple fistulous tracts (≥2) on MRI. Patients who have these features but does not have an abscess on imaging may merit more aggressive treatment (and close monitoring) to prevent the development of an abscess.
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OBJECTIVE: Flow diversion of intracranial aneurysms with the pipeline embolization device (PED) may produce angiographically apparent stenosis within the PED, which can lead to secondary ischemic complications. In-stent stenosis can be treated medically with dual antiplatelet therapy (DAPT), but the safety and efficacy of this approach are unknown. In this work, we review the safety and efficacy of DAPT to prevent progression of in-stent stenosis or development of cerebral ischemia. METHODS: Clinical and angiographic data from eligible patients were assessed from a prospectively maintained neurointerventional database. Details surrounding in-stent stenosis and DAPT were extracted. Patients were included in this study if in-stent stenosis was detected at any angiographic follow-up and managed with DAPT. The primary efficacy endpoint was lack of angiographic progression of in-stent stenosis or new ipsilateral infarct following initiation of medical therapy. RESULTS: In total, 23 PED constructs developed in-stent stenosis and were managed with DAPT. Follow-up angiography was available for 19 constructs. Eighty-nine percent (17/19) of PED constructs achieved the primary endpoint of lack of stenosis progression and lack of new ipsilateral ischemic events. Of the 2 PED constructs that failed to achieve the primary endpoint of this study, one demonstrated worsening of in-stent stenosis from 55% to 76% over 16 months, while the other developed ipsilateral ischemic stroke 4 months after detection of in-stent stenosis. In addition, one patient experienced intracranial hemorrhage 9 months after the initiation of DAPT. CONCLUSIONS: Progression of in-stent stenosis and new ipsilateral ischemic events are limited in the presence of DAPT. However, hemorrhagic events related to DAPT may occasionally occur.
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Embolização Terapêutica , Aneurisma Intracraniano , Humanos , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/terapia , Aneurisma Intracraniano/complicações , Inibidores da Agregação Plaquetária/uso terapêutico , Constrição Patológica/complicações , Stents , Angiografia Cerebral , Resultado do Tratamento , Estudos Retrospectivos , SeguimentosRESUMO
BACKGROUND: Rates of aneurysm occlusion following treatment with flow-diverting stents have been quantified at predefined time points in clinical trials, but data characterizing the continuous temporal progression of aneurysm occlusion are lacking. This study used real-world variability in timing of angiographic follow-up to characterize the time line of aneurysm occlusion following treatment with the Pipeline embolization device (PED). METHODS: All aneurysms treated with a PED at our institution between 2011 and 2020 were screened. Nonsaccular or ruptured aneurysms were excluded. Aneurysm occlusion status and time since treatment were recorded for each follow-up angiogram. Aneurysm occlusion was characterized using Kaplan-Meier and Cox proportional hazards analysis after censoring at last follow-up or subsequent treatment. RESULTS: There were 290 aneurysms in 222 patients analyzed. The median time of observed aneurysm occlusion was 7.5 months, and overall rate of aneurysm occlusion was 77.9%. Larger aneurysms demonstrated a longer median time to occlusion and lower rate of aneurysm occlusion (P = 0.029). There were no observed differences in the time line of occlusion for aneurysms treated with a single PED or multiple PEDs (P = 0.889) or without or with adjunctive coiling (P = 0.771). CONCLUSIONS: Aneurysms treated with a PED had a median time to observed occlusion of 7.5 months. Occlusion of larger aneurysms occurred more slowly than occlusion of smaller aneurysms following flow diversion. The number of PEDs deployed or the use of adjunctive coiling did not affect the time line or likelihood of aneurysm occlusion. These findings may guide optimal timing of follow-up after treatment with a PED.
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Embolização Terapêutica , Aneurisma Intracraniano , Prótese Vascular , Seguimentos , Humanos , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/terapia , Estudos Retrospectivos , Stents , Resultado do TratamentoRESUMO
INTRODUCTION: Granulomas are a pathologic hallmark of Crohn disease (CD) although they are found in only a subset of patients. Well-formed granulomas are associated with an aggressive phenotype although it is unknown if microgranulomas confer a similar phenotype. This study sought to define the incidence of microgranulomas in pediatric CD and compare the clinical course with cases with granulomas and those without granulomatous inflammation. METHODS: We performed a single-center, retrospective study of pediatric CD patients who had at least 3âyears of follow-up. initial diagnostic biopsies were systematically re-examined by a gastrointestinal pathologist. A priori definitions of granuloma (10+ histiocytes) and microgranuloma (4-9 histiocytes) were used. Disease outcomes of hospitalization, development of complicated disease behavior, perianal disease, and the use of anti-tumor necrosis factor (anti-TNF) therapy were assessed by Kaplan-Meier survival plots. RESULTS: This study included 138 subjects with an average follow-up of 4.6âyears. Granulomas were seen in 38 of 138 subjects (27.5%) and an additional 38 subjects (27.5%) had at least 1 microgranuloma (in the absence of granulomas). Escalation to anti-TNF therapy was higher in CD with granulomas (Pâ =â0.001) and microgranulomas (Pâ =â0.0001) compared with those without granulomatous inflammation. CONCLUSIONS: A significant subset of pediatric CD patients have microgranulomas (in the absence of well-defined granulomas). Children with CD who have microgranulomas are escalated to anti-TNF therapy more frequently than those without granulomatous inflammation (and at a similar rate to those with granulomas). Pathologists should have a low threshold to report microgranulomas as they may help to predict disease behavior.