The Clinical Value of Cranial CT Venography for Predicting Fusobacterium necrophorum as the Causative Agent in Children with Complicated Acute Mastoiditis.

BACKGROUND AND PURPOSE: Fusobacterium necrophorum (F necrophorum) is an anaerobic bacteria that causes invasive head and neck infections in children. Several studies have demonstrated an increasing prevalence of F necrophorum as the causative agent in acute mastoiditis in children, with associated high rates of intracranial complications such as epidural abscess and sinus venous thrombosis, to name a few. F necrophorum requires a treatment protocol that differs from the empiric treatment that is tailored to more common pathogens (eg, group A streptococci, Streptococcus pneumonia), and hence expediting the diagnosis is important. For evaluating complicated acute mastoiditis in children, cranial CT venography remains the imaging study of choice in most medical centers due to its availability in emergency situations. Based on our clinical experience, our hypothesis is that children with F necrophorum-associated complicated acute mastoiditis can be differentiated from those with other etiologies using CT venography. MATERIALS AND METHODS: CT venography studies of 76 children hospitalized and treated for complicated acute mastoiditis were retrospectively reviewed. Retrieved imaging data included intracranial complications (epidural abscess, sinus venous thrombosis), cranial bone-related complications, and extracranial complications (subperiosteal abscess, temporomandibular joint abscess, and soft-tissue inflammation). The cohort was divided into children with F necrophorum-related disease (study group) and those with non-F necrophorum-related disease (control group). RESULTS: Thirty-seven children (49%) comprised the study group, and 39 children in whom the causative agents were other bacteria comprised the control group. There were significantly higher rates of complications in the study group: sinus venous thrombosis (P < .001), perisigmoid epidural abscess (P = .036), and extramastoid osteomyelitis (P < .001). Thrombosis in venous sites beyond the sigmoid sinus and jugular foramen (a pattern consistent with an otogenic variant of Lemierre syndrome) and emphysematous osteomyelitis were found only among children in the F necrophorum-related study group (32% and 22% accordingly). CONCLUSIONS: In children with complicated acute mastoiditis, CT venography findings of emphysematous osteomyelitis and/or thrombosis in venous sites beyond the sigmoid sinus and jugular foramen (a pattern consistent with the otogenic variant of Lemierre syndrome) should lead the radiologist to suggest F necrophorum-related mastoiditis.

Validation of the 2023 international diagnostic criteria for MOGAD in a pediatric cohort.

OBJECTIVE: To validate the recently published diagnostic criteria for Myelin Oligodendrocyte Glycoprotein-antibody associated disease (MOGAD) in real-world cohort of children with acquired demyelinating syndromes. METHODS: Patients <18yrs presenting with demyelinating disease to Pediatric neuroimmunology clinics at two Israeli tertiary centers who had MOG antibodies (MOG-Abs) tested between 01/07/2017 and 15/08/2023 were included. Diagnostic criteria for MOGAD were applied and sensitivity and specificities were calculated. RESULTS: MOG-Abs were detected in 28/63 (44 %). Median age at onset for all patients was 11.4 yrs (range 1.1-17.6 yrs) and 41 (65 %) were female. Of the patients testing negative, ADEM was the most common diagnosis (n = 11) followed by MS (n = 8). No patients without MOG-Abs were diagnosed with MOGAD. All patients with a clinical diagnosis of MOGAD had positive MOG-Abs and fulfilled the 2023 international diagnostic criteria for MOGAD. Sensitivity, specificity, positive predictive value, and negative predictive value were 100 %. We found no difference between younger (<10yrs old) and older (>10 yrs old) children in the number of supportive criteria fulfilled at onset (median 2 vs. 2.5, p = 0.4) The number of supporting features was higher in patients with relapsing (n = 5) vs. monophasic (n = 23) disease course at onset (median 3 vs. 2, p = 0.03) and at final follow-up (median 5 vs. 2, p = 0.004). CONCLUSION: Recent MOGAD diagnostic criteria had excellent performance in this pediatric cohort but did not add to the diagnostic accuracy of the antibody test alone.

Pott's Puffy Tumor in Pediatric Patients: Case Series and Literature Review.

BACKGROUND: Pott's puffy tumor (PPT) is a rare complication of frontal sinusitis. Although it may occur at any age, the incidence is higher during adolescence. METHODS: We report a series of 10 pediatric patients (9-17 years of age) who presented with PPT in 2 tertiary care pediatric hospitals in central Israel between January 2018 and August 2022 and review the published literature on pediatric PPT. RESULTS: The most common clinical presentations included headache (10 cases), frontal swelling (6 cases) and fever (5 cases). Symptom duration before admission was between 1 and 28 days (median 10 days). The diagnosis of PPT was made by imaging studies at a median of 1 day after admission. All 10 patients underwent computed tomography studies, and 6 patients also underwent magnetic resonance imaging. The overall rate of intracranial complications was 70%. All 10 children were treated with systemic antibiotics and surgical interventions. Streptococcus constellatus group was the most common causal bacteria. All 10 patients recovered uneventfully. CONCLUSIONS: Our findings show that a high index of suspicion for PPT should be applied to adolescents presenting with prolonged headache and frontal swelling. Contrast-enhanced computed tomography is an appropriate first tool for the evaluation; however, an magnetic resonance imaging should be performed to determine the necessity of intracranial interventional treatments if there is any suspicion of intracranial involvement. Complete recovery can be expected with appropriate antibiotic treatment and surgical intervention in most of the cases.

Prospective Validation of the Lémann Index in Children: A Report From the Multicentre Image Kids Study.

BACKGROUND: The Lémann Index [LI] and the recently updated LI are tools for measuring structural bowel damage in adults with Crohn's disease [CD] but have not been evaluated in children. We aimed to validate the updated LI in the prospective multicentre ImageKids study of paediatric CD. METHODS: We included children with CD undergoing magnetic resonance enterography [MRE], pelvic magnetic resonance imaging [MRI] and ileocolonoscopy. Half were followed for 18 months, when MRE was repeated. Serum was collected for fibrosis-related proteomic markers. The LI was calculated by central readers from the MRE, ileocolonoscopy, physical examination and surgical data. Reliability and construct validity were assessed at baseline, while responsiveness and test-retest reliability were explored longitudinally. RESULTS: In total, 240 children were included (mean age, 14.2 ± 2.5 years; median disease duration, 2.2 years [interquartile range, IQR 0.25-4.42]; median baseline LI, 4.23 [IQR 2.0-8.8]). The updated LI had excellent inter-observer reliability (interclass correlation coefficient [ICC] = 0.94, 95% confidence interval [CI] 0.92-0.95) but poor, although statistically significant, correlation with radiologist and gastroenterologist global assessments of damage and with serum proteomic levels of fibrotic markers [rho = 0.15-0.30, most p < 0.05]. The updated LI had low discriminative validity for detecting damage (area under the receiver operating characteristic curve [AUC-ROC] 0.69, 95% CI 0.62-0.75). In 116 repeated MREs, responsiveness was suboptimal for differentiating improved from unchanged disease [AUC-ROC 0.58, 95% CI 0.45-0.71]. Test-retest reliability was high among stable patients [ICC = 0.84, 95% CI 0.72-0.91]. CONCLUSION: Overall, the updated LI had insufficient psychometric performance for recommending its use in children. An age-specific index may be needed for children with shorter disease duration than typical adult cohorts.

Development of a multimodal machine-learning fusion model to non-invasively assess ileal Crohn's disease endoscopic activity.

BACKGROUND AND OBJECTIVE: Recurrent attentive non-invasive observation of intestinal inflammation is essential for the proper management of Crohn's disease (CD). The goal of this study was to develop and evaluate a multi-modal machine-learning (ML) model to assess ileal CD endoscopic activity by integrating information from Magnetic Resonance Enterography (MRE) and biochemical biomarkers. METHODS: We obtained MRE, biochemical and ileocolonoscopy data from the multi-center ImageKids study database. We developed an optimized multimodal fusion ML model to non-invasively assess terminal ileum (TI) endoscopic disease activity in CD from MRE data. We determined the most informative features for model development using a permutation feature importance technique. We assessed model performance in comparison to the clinically recommended linear-regression MRE model in an experimental setup that consisted of stratified 2-fold validation, repeated 50 times, with the ileocolonoscopy-based Simple Endoscopic Score for CD at the TI (TI SES-CD) as a reference. We used the predictions' mean-squared-error (MSE) and the receiver operation characteristics (ROC) area under curve (AUC) for active disease classification (TI SEC-CD≥3) as performance metrics. RESULTS: 121 subjects out of the 240 subjects in the ImageKids study cohort had all required information (Non-active CD: 62 [51%], active CD: 59 [49%]). Length of disease segment and normalized biochemical biomarkers were the most informative features. The optimized fusion model performed better than the clinically recommended model determined by both a better median test MSE distribution (7.73 vs. 8.8, Wilcoxon test, p<1e-5) and a better aggregated AUC over the folds (0.84 vs. 0.8, DeLong's test, p<1e-9). CONCLUSIONS: Optimized ML models for ileal CD endoscopic activity assessment have the potential to enable accurate and non-invasive attentive observation of intestinal inflammation in CD patients. The presented model is available at https://tcml-bme.github.io/ML_SESCD.html.

Development, Validation, and Evaluation of the Pediatric Inflammatory Crohn's Magnetic Resonance Enterography Index From the ImageKids Study.

BACKGROUND & AIMS: Cross-sectional imaging is important in the assessment of transmural inflammation in Crohn's disease (CD). Small bowel involvement is often more extensive in pediatric CD, requiring a panentering measuring tool. We undertook to develop a magnetic resonance enterography (MRE)-based index that would measure inflammation in all segments of the intestine, without rectal contrast. METHODS: Children with CD underwent ileocolonoscopy and MRE and half were prospectively followed for 18 months when MRE was repeated. Item generation and reduction were performed by a Delphi panel of pediatric radiologists, a systematic literature review, a cross-sectional study of 48 MREs, and a steering committee. Formatting and weighting were performed using multivariate modeling adjusted by a steering committee. MREs were read locally and centrally. Reliability, validity, and responsiveness were determined using several clinimetric and psychometric approaches. RESULTS: Thirty items were initially generated and reduced to 5 using regression analysis on 159 MREs: wall thickness, wall diffusion weighted imaging, ulcerations, mesenteric edema, and comb sign. In the validation cohort of 81 MREs, the weighted global PICMI correlated well with the radiologist global assessment (r = 0.85; P < .001) and with the simple endoscopic score in a subsample with ileocolonic disease (r = 0.63; P < .001). Interobserver and test-retest reliability were high (interclass correlation coefficients, 0.84; 95% confidence interval [CI], 0.79-0.87; and 0.81, 95% CI, 0.65-0.90, respectively; both P < .001). Excellent responsiveness was found at repeated visits (n = 116 MREs; area under the receiver operating characteristic curve 0.96; 95% CI, 0.93-0.99). Transmural healing was defined as PICMI ≤10 and response as a change of >20 points with excellent discriminative validity (area under the receiver operating characteristic curve = 0.96; 95% CI, 0.93-0.99). CONCLUSIONS: The PICMI is a valid, reliable, and responsive index for assessing transmural inflammation in pediatric CD. It scores the entire bowel length and does not require intravenous contrast or rectal enema and, therefore, is suitable for use in children. (ClinicalTrials.gov, Number: NCT01881490.).

Development and Validation of a Pediatric MRI-Based Perianal Crohn Disease (PEMPAC) Index-A Report from the ImageKids Study.

BACKGROUND: As part of the prospective multicenter ImageKids study, we aimed to develop and validate the pediatric MRI-based perianal Crohn disease (PEMPAC) index. METHODS: Children with Crohn disease with any clinical perianal findings underwent pelvic magnetic resonance imaging at 21 sites globally. The site radiologist and 2 central radiologists provided a radiologist global assessment (RGA) on a 100 mm visual analog scale and scored the items selected by a Delphi group of 35 international radiologists and a review of the literature. Two weighted multivariable statistical models were constructed against the RGA. RESULTS: Eighty children underwent 95 pelvic magnetic resonance imaging scans; 64 were used for derivation and 31 for validation. The following items were included: fistula number, location, length and T2 hyperintensity; abscesses; rectal wall involvement; and fistula branching. The last 2 items had negative beta scores and thus were excluded in a contending basic model. In the validation cohort, the full and the basic models had the same strong correlation with the RGA (r = 0.75; P < 0.01) and with the adult Van Assche index (VAI; r = 0.93 and 0.92; P < 0.001). The correlation of the VAI with the RGA was similar (r = 0.77; P < 0.01). The 2 models and the VAI had a similar ability to differentiate remission from active disease (area under the receiver operating characteristic curve, 0.91-0.94). The PEMPAC index had good responsiveness to change (area under the receiver operating characteristic curve, 0.89; 95% confidence interval, 0.69-1.00). CONCLUSIONS: Using a blended judgmental and mathematical approach, we developed and validated an index for quantifying the severity of perianal disease in children with CD. The adult VAI may also be used with confidence in children.

Sarcopenia is a Predictor for Adverse Clinical Outcome in Pediatric Inflammatory Bowel Disease.

OBJECTIVES: Inflammatory bowel disease (IBD) has a high impact on nutritional status. Sarcopenia is related to higher risk of surgery and rescue therapy in adults with IBD; however, comparable data in pediatric populations are scarce. We evaluated muscle mass as a predictor of disease outcome in pediatric IBD. METHODS: All pediatric IBD patients who underwent magnetic resonance enterography (MRE) during 2008 to 2019 were included. Muscle mass was assessed by measuring the area of the psoas muscle at the upper level of L3 on MRE. The psoas area divided by the body surface area (BSA) yielded the psoas index. Clinical and radiological data, including disease location, activity, course, and medications were documented. The control group included non-IBD children who underwent an MR imaging study. RESULTS: We enrolled 101 IBD patients, 69 (68.3%) with Crohn disease (CD) and 32 (31.7%) with ulcerative colitis (UC) (mean age 15.03 ±â€Š3.27 years). The psoas index was significantly lower in the IBD patients compared with the 87 controls (326 vs 528, respectively, P < 0.001). Patients with a psoas index in the lowest quartile had significantly higher risk for biologic therapy (multivariate analysis, hazard ratio [HR] = 12.1, P = 0.046) and disease exacerbation (HR = 9, P = 0.047) independently of body mass index, compared with patients with a psoas index in the uppermost quartile. CONCLUSIONS: Sarcopenia correlates with the radiological severity of pediatric IBD and serves as a predictor for adverse clinical disease outcome. Muscle mass measurement in MRE studies may serve as a possible marker for disease outcome in this population.

Clinical-stage Approaches for Imaging Chronic Inflammation and Fibrosis in Crohn's Disease.

The number of imaging-based indices developed for inflammatory bowel disease as research tools, objectively measuring ileocolonic and perianal activity and treatment response, has expanded in the past 2 decades. Created primarily to assess Crohn's disease (CD), there is increasing adoption of these indices into the clinical realm to guide patient care. This translation has been facilitated by validation in adult and pediatric populations, prompted by simplification of score calculations needed for practical application outside the research environment. The majority of these indices utilize magnetic resonance imaging (MRI), specifically MR enterography (MRE) and pelvic MRI, and more recently ultrasound. This review explores validated indices by modality, anatomic site and indication, including for documentation of the presence and extent of CD, disease progression, complications, and treatment response, highlighting those in clinical use or with the potential to be. As well, it details index imaging features used to quantify chronic inflammatory activity, severity, and to lesser extent fibrosis, in addition to their reference standards and any modifications. Validation in the pediatric population of indices primarily developed in adult cohorts such as the Magnetic Resonance Index of Activity (MaRIA), the Simplified Magnetic Resonance Index of Activity (MARIAs), and the MRE global score (MEGS), together with newly developed pediatric-specific indices, are discussed. Indices that may be predictive of disease course and investigational techniques with the potential to provide future imaging biomarkers, such as multiparametric MRI, are also briefly considered.

Incidental Findings on Brain Magnetic Resonance Imaging (MRI) in Pediatric Endocrine Patients.

OBJECTIVE: To explore incidental findings on brain magnetic resonance imaging (MRI) studies of pediatric patients referred due to endocrine disorders. METHODS: A retrospective, observational study conducted in a tertiary referral center. The neuroimaging database of 17,445 brain MRI studies of 11,011 pediatric patients were searched for cases with endocrine referrals and without medical history of malignancy, genetic syndromes, and/or neurologic comorbidities. This database was linked to the pediatric neurosurgical database. Clinical data were retrieved from medical files. RESULTS: In total, 524 patients (50.2% males, mean age 8.5±3.5 years) were referred to brain MRI due to growth disturbances (n = 313), pubertal disorders (n = 183), prolactin hypersecretion (n = 18), central diabetes insipidus (n = 8), and obesity (n = 1). Incidental findings were found in 128 (24.4%) cases. Chiari type 1 malformation was more prevalent in patients with growth disturbances (P<.001). Small pituitary cysts were observed in 20 (3.8%) patients, and pineal cysts in 25 (4.8%) patients, mostly girls (68%, P<.001). White matter lesions were diagnosed in 30 (5.7%) patients, none with clinical evidence of neurologic disease. Brain asymmetry without clinical significance and developmental venous anomalies were observed in 14 (2.7%) and 8 (1.5%) patients, respectively. Twelve patients were diagnosed with intracranial tumors, and 5 required surgical intervention for a histopathologic diagnosis of juvenile pilocytic astrocytoma (n = 3), choroid plexus papilloma (n = 1), or inconclusive (n = 1). The rest were managed conservatively. CONCLUSION: Incidental findings on brain MRIs of pediatric patients referred by endocrinologists are common and raise dilemmas. The spectrum ranges from structural disruptions to tumors. Decision-making is individualized and patient-centered.

Mature teratoma splitting the brainstem in a newborn: case report.

Mature teratomas (MTs) of the posterior fossa are extremely rare. The authors present a case of a prenatal diagnosis of an MT splitting the brainstem. Representative images as well as the clinical and surgical course are presented. Literature regarding "split brainstem" and MT of the posterior fossa is discussed.

Use of EOS Low-Dose Biplanar X-Ray for Shunt Series in Children with Hydrocephalus: A Preliminary Study.

INTRODUCTION: Shunt series (SS) are a common diagnostic tool used to verify shunt integrity. SS include X-ray films of the skull, chest, and abdomen and often are performed either when a shunted patient presents with suspected shunt malfunction or as a screening test to identify shunt disconnections or dislodgment. EOS low-dose biplanar X-rays are associated with significantly reduced radiation doses compared with ordinary X-rays and are used for various indications. This is the first publication on the use of EOS as a SS technique. METHODS: Over a period of 6 months, EOS were performed at our center for various orthopedic indications, mostly for scoliosis evaluation. Nine children (<20 years of age) had a ventriculoperitoneal shunt and served as the study group. We retrospectively reviewed shunt visibility and integrity in the EOS scans as well as regular SS or plain spinal X-rays. RESULTS: Three patients had bilateral shunts, and 8 had previous X-rays for comparison. In all patients, the shunt integrity was easily demonstrated on the EOS images. Two patients had an identified shunt disconnection confirmed on the EOS images. No shunt-related information was missed on the EOS compared with the other X-ray images. CONCLUSIONS: These preliminary results suggest that EOS may be used as an alternative technology to demonstrate shunt integrity instead of regular X-ray SS. Favorable shunt visibility without the need for multiple radiation exposures and image processing (such as stitching) results in a significantly shorter examination time and significant less radiation.

Perianal Pediatric Crohn Disease Is Associated With a Distinct Phenotype and Greater Inflammatory Burden.

OBJECTIVES: Data on the outcomes of children with perianal Crohn disease (pCD) are limited, although its presence is often used for justifying early use of biologics. We aimed to assess whether pCD in children is associated with more severe outcomes as found in adults. METHODS: Data were extracted from the ImageKids database, a prospective, multicenter, longitudinal cohort study. The study enrolled 246 children at disease onset or thereafter. All patients underwent comprehensive clinical, endoscopic, and radiologic evaluation at enrollment; 98 children had repeat evaluation at 18 months. RESULTS: Of the 234 included patients (mean age 14.2 ±â€Š2.4 years; 131 [56%] boys), 57 (24%) had perianal findings, whereas only 21 (9%) had fistulizing perianal disease. Children with pCD had reduced weight and height z scores compared with non-pCD patients (-0.9 vs -0.35, P = 0.03 and -0.68 vs -0.23, respectively; P = 0.04), higher weighted pediatric CD activity index (32 [interquartile range 16-50] vs 20 [8-37]; P = 0.004), lower serum albumin (3.6 ±â€Š0.7 vs 4.5 ±â€Š0.8, P = 0.016), and higher magnetic resonance enterography global inflammatory score (P = 0.04). Children with pCD had more rectal (57% vs 38%, P = 0.04), and jejunal involvement (31% vs 11% P = 0.003) and a higher prevalence of granulomas (64% vs 23%, P = 0.0001). Magnetic resonance enterography-based damage scores did not differ between groups. Patients with skin tags/fissures only, had similar clinical, endoscopic, and radiologic characteristics as patients with no perianal findings. CONCLUSIONS: Pediatric patients with pCD with fistulizing disease have distinct phenotypic features and a predisposition to a greater inflammatory burden.

The effect of chemotherapy on optic pathway gliomas and their sub-components: A volumetric MR analysis study.

BACKGROUND: Optic pathway gliomas (OPG) represent 5% of pediatric brain tumors and compose a major therapeutic dilemma to the treating physicians. While chemotherapy is widely used for these tumors, our ability to predict radiological response is still lacking. In this study, we use volumetric imaging to examine in detail the long-term effect of chemotherapy on the tumor as well as its various sub-components. PROCEDURE: The tumors of 15 patients with OPG, treated with chemotherapy, were longitudinally measured using our novel, previously described volumetric method. Patients were treated with up to five lines of chemotherapy. Sufficient follow-up imaging data, and patient's numbers, allowed for analysis of two treatment lines. Volumetric measurements of the tumors were segmented into solid-non-enhancing, solid-enhancing, and cystic components. Outcome analysis was done per specific treatment line and for the overall follow-up period. RESULTS: An average reduction of 9.7% (±23%) in the gross-total-solid volume (GTSV) was noted following treatment with vincristine and carboplatin. The cystic component grew under therapy by an average of 12.6% (±39%). When measured over the course of the whole study period, the cystic component grew by an average of 35% (±100%) and the GTSV increased by 12% (±35%). CONCLUSION: Initial treatment with vincristine and carboplatin seems to have a minimal initial effect, mostly on the solid components. The cystic component in itself seems to be unaffected by chemotherapy, and contributes to the subsequent growth of the total volume. During the overall treatment period, both solid and cystic components grew regardless of combined treatment methods.

PNist: interactive volumetric measurements of plexiform neurofibromas in MRI scans.

PURPOSE: Volumetric measurements of plexiform neurofibromas (PNs) are time consuming and error prone, as they require the delineation of the PN boundaries, which is mostly impractical in the daily clinical setup. Accurate volumetric measurements are seldom performed for these tumors mainly due to their great dispersion, size and multiple locations. This paper presents a semiautomatic method for segmentation of PN from STIR MRI scans. METHODS: Plexiform neurofibroma interactive segmentation tool (PNist) is a new tool to segment PNs in STIR MRI scans. The method is based on histogram tumor models computed from a training set. RESULTS: Experimental results from 28 datasets show an average absolute volume difference of 6.8 % with an average user time of approximately 7 min versus more than 13 min with manual delineation. In complex cases, the PNist user time is less than half in compared to state-of-the-art tools. CONCLUSIONS: PNist is a new method for the semiautomatic segmentation of PN lesions. Its simplicity and reliability make it unique among other state-of-the-art methods. It has the potential to become a clinical tool that allows the reliable evaluation of PN burden and progression.

Interactive segmentation of plexiform neurofibroma tissue: method and preliminary performance evaluation.

Plexiform neurofibromas (PNs) are a major manifestation of neurofibromatosis-1 (NF1), a common genetic disease involving the nervous system. Treatment decisions are mostly based on a gross assessment of changes in tumor using MRI. Accurate volumetric measurements are rarely performed in this kind of tumors mainly due to its great dispersion, size, and multiple locations. This paper presents a semi-automatic method for segmentation of PN from STIR MRI scans. The method starts with a user-based delineation of the tumor area in a single slice and automatically segments the PN lesions in the entire image based on the tumor connectivity. Experimental results on seven datasets, with lesion volumes in the range of 75-690 ml, yielded a mean absolute volume error of 10 % (after manual adjustment) as compared to manual segmentation by an expert radiologist. The mean computation and interaction time was 13 versus 63 min for manual annotation.