Simplified Magnetic Resonance Index of Activity Is Useful for Terminal Ileal but not Colonic Disease in Pediatric Crohn Disease.

BACKGROUND: Ileocolonoscopy (IC) detects mucosal inflammation and magnetic resonance enterography (MRE) detects transmural inflammation in Crohn disease (CD). We aimed to evaluate the relationship between the simplified magnetic resonance index of activity (MARIAs) and measures of inflammation by IC in children with newly diagnosed CD. METHODS: Retrospective review of 140 patients 6-18 years of age with CD who had baseline IC and MRE within 5 weeks of diagnosis. MARIAs was calculated for each intestinal segment (terminal ileum [TI], ascending colon, transverse colon, descending colon, sigmoid colon, rectum), defined as (1 × thickness > 3 mm) + (1 × edema) + (1 × fat stranding) + (2 × ulcers). Sensitivity and specificity were derived using receiver operating characteristic (ROC) curves to compare MARIAs to IC findings. RESULTS: Using IC as the reference standard, the cutoff MARIAs ≥1 identified TI segments with active inflammation with 84% sensitivity, 73% specificity, 85% positive predictive value (PPV), 70% negative predictive value (NPV), and area under the curve (AUC) 0.782 (95% confidence interval [CI] 0.689-0.876). The cutoff MARIAs ≥2 identified TI segments with severe lesions with 87% sensitivity, 76% specificity, 87% PPV, 76% NPV, and AUC 0.814 (95% CI 0.712-0.916). There was poor sensitivity for all colonic segments. CONCLUSION: The MARIAs is feasible and accurate in reflecting disease activity in the TI, but not in the colon, in children with newly diagnosed CD. Although the MARIAs may be useful for monitoring TI disease activity over time, full assessment continues to require both IC and MRE.

Postnatal diagnosis of single-suture craniosynostosis with cranial ultrasound: a systematic review.

INTRODUCTION: The optimal protocol for diagnostic workup of craniosynostosis and the role of specific imaging modalities remain controversial. Skull X-rays and 3-dimensional head CTs are options when physical exam is equivocal but involve ionizing radiation. Ultrasound has emerged as an alternative modality for visualization of cranial sutures, but its use is not widespread. METHODS: The authors performed a systematic review of the literature on the use of ultrasound for the diagnosis of craniosynostosis. RESULTS: A total of 12 studies involving 1062 patients were included. Overall, 300 patients (28.2%) were diagnosed with craniosynostosis. A total of 369 (34.7%) patients had their diagnosis (craniosynostosis vs. patent sutures) confirmed with another imaging modality in addition to ultrasound. Among studies, the specificity of ultrasound ranged from 86 to 100%, and the sensitivity from 71 to 100%. CONCLUSIONS: Ultrasonography of cranial sutures is a feasible and accurate tool for the diagnosis of single-suture craniosynostosis when physical exam findings are insufficient. Although technical aspects of ultrasonography and its interpretation have an associated learning curve, ultrasound can achieve high sensitivity and specificity among patients with suspected craniosynostosis.

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.

Improvement of calcinosis using pamidronate in a patient with juvenile dermatomyositis.

A 7-year-old girl with juvenile dermatomyositis developed severe calcinosis, despite an extensive medication regimen. Three administrations of intravenous pamidronate produced significant improvement in calcinosis, pain and function, leading to remission less than 1 year after induction of therapy.

Lung Ultrasound Score for Prediction of Surfactant Administration in Preterm Infants with Respiratory Failure.

OBJECTIVE: We investigated the predictive value of a lung ultrasound score (LUS) for surfactant administration in a United States Level 4 Neonatal Intensive Care Unit. STUDY DESIGN: Thirty infants born at <37 weeks gestational age with respiratory distress syndrome associated respiratory failure requiring continuous positive airway pressure were included. A LUS was obtained within six hours of life. Surfactant administration in the first five days of life was recorded. Receiver operating characteristic (ROC) analysis for LUS and surfactant administration was performed. RESULTS: Median completed gestational age was 33 weeks (31-34 weeks interquartile range) and median birth weight was 2.0 kg (1.5-2.3 kg). LUS for predicting an initial surfactant dose had an area under the ROC curve of 0.97. A score > 9 provided 100% sensitivity and 91% specificity for predicting administration of an initial surfactant dose. CONCLUSION: A LUS > 9 provided excellent sensitivity and specificity for predicting which infants will receive surfactant for associated respiratory failure.

Validating the Modified McGill Thyroid Nodule Score for Assessment of Preoperative Risk of Pediatric Thyroid Malignancy.

OBJECTIVE: The McGill Thyroid Nodule Score (MTNS) is a preoperative tool used to predict the risk for well-differentiated thyroid cancer in adults. It was developed by a multidisciplinary team using established evidence-based risk factors for thyroid cancer. The modified McGill Thyroid Nodule Score (mMTNS) was developed to predict malignancy risk in children. A pilot study suggested the mMTNS was able to assess malignancy risk in children with indeterminate cytology on fine needle aspiration (FNA). This study seeks to validate these findings. METHODS: Retrospective chart review identified subjects who underwent FNA biopsy and subsequent resection. Each patient was assigned a score to compare to final pathology. Statistical analysis was performed with SPSS. All tests were 2-tailed and statistical significance defined p < 0.05. Logistic regression used to determine predictive values of scores. RESULTS: 46 patients ≤21 years of age underwent resection of a thyroid nodule. Female predominance of 85% (n = 39). 78% (n = 36) of patients had palpable nodule. 65% (n = 30) found to have benign pathology and 35% (n = 16) found to have malignancy. Malignant nodules associated with greater mean mMTNS compared to benign [13.63 vs 7.23]. An mMTNS greater >12 had sensitivity of 86.7%, specificity of 90.3%, positive predictive value of 81.3%, and negative predictive value of 93.3%. CONCLUSION: Our data suggests the mMTNS continues to be a useful adjunct in predicting malignancy risk of pediatric thyroid nodules. An mMTNS >12 has a high risk for malignancy, which can aid in counseling and clinical decision making, particularly when there is indeterminate cytology on FNA. LEVEL OF EVIDENCE: IV.

Follicular Dendritic Cell Sarcoma in a Patient With Adolescent-Onset Crohn's Disease Exposed to Multiple Immunomodulator and Biologic Therapies.

Children and adolescents with inflammatory bowel disease are often treated with immunomodulators (thiopurines, methotrexate) and biologics (anti-TNF, anti-integrin) for extended periods despite concerns about long-term safety. Here, we report a case of follicular dendritic cell sarcoma, a very rare malignancy, and the first reported presentation in a patient with inflammatory bowel disease exposed to infliximab, methotrexate, and vedolizumab. We review the key clinical features and diagnostic factors of this malignancy. The pathogenesis of follicular dendritic cell sarcoma is largely unknown, however, knock out of B-cell TNF in mice has been related to follicular dendritic cell dysregulation through its impact on NF-κB pathways and CXCL13 chemokines. It is unknown whether any relationship exists between this patient's diagnosis of Crohn's disease and therapeutic exposures to this rare malignancy. We document this case in the literature to raise awareness among other clinicians who may observe a similar case.

Concussion of the colon: paediatric patient with traumatic cecal haematoma.

A 14-year-old boy was injured from playing football, and subsequently developed worsening abdominal pain. CT of the abdomen and pelvis was concerning for a mass of ascending colon, with free fluid in the pelvis. The patient was emergently taken to the operating room where he was found to have a cecal haematoma. The patient underwent a right hemicolectomy and ileocolostomy and was discharged home just days later without any acute complications. As cecal haematomas are a rare entity, the best approach to management is not well defined. When the diagnosis is uncertain, or if the possibility of full-thickness injury exists, exploratory laparoscopy or laparotomy is the safest approach.

Standardized method for the measurement of Grabb's line and clival-canal angle.

OBJECTIVE Grabb's line-the perpendicular distance from the basion-C2 line (pB-C2)-is a widely used radiographic measurement with significant clinical implications in patients with a complex Chiari malformation. Rigorous demonstration of the reproducibility of this measurement has not previously been reported. The authors report a standardized measurement technique with excellent inter- and intrarater reliability on T1-weighted sagittal MRI. METHODS The authors developed a standardized measurement technique that included specifications of midline slice selection, landmark and reference line definitions, and measurement technique on T1-weighted sagittal images. Twenty MR images were reviewed by 2 pediatric neurosurgeons, 1 pediatric radiologist, and 1 undergraduate student. Measurements were performed using the technique specified on 2 separate occasions. Intrarater and interrater reliabilities were calculated using the intraclass correlation coefficient. RESULTS A combined interrater reliability of 0.879 was achieved for the pB-C2, and 0.916 for the clival-canal angle, another measure of interest in patients with complex Chiari malformations. Intrarater reliability for these measurements exceeded 0.858 for all 4 reviewers. CONCLUSIONS Grabb's line-the pB-C2-can be measured with excellent reliability using a standardized measurement protocol. Individual clinicians and collaborative databases should consider using validated measurement techniques to guide clinical decision making in patients with craniocervical junction pathology.

McGill Thyroid Nodule Score in Differentiating Benign and Malignant Pediatric Thyroid Nodules: A Pilot Study.

Objective The McGill Thyroid Nodule Score (MTNS) is a preoperative tool used to predict the risk for well-differentiated thyroid cancer given a specific nodule in adults. We evaluated the clinical utility of a modified pediatric MTNS with children and adolescents. Study Design Case series with chart review. Setting Tertiary care children's hospital. Subjects and Methods This is a retrospective chart review of 46 patients ≤18 years of age presenting with a solitary or dominant thyroid nodule treated with surgical resection between September 2008 and December 2015. The cumulative MTNS for each nodule was calculated and compared with the final pathology. Results Of 46 patients, 10 (21.7%) were diagnosed with well-differentiated thyroid cancer (80% papillary thyroid carcinoma, 10% follicular variant of papillary thyroid carcinoma, 10% follicular thyroid carcinoma). Malignant nodules were associated with a greater mean MTNS (benign, 5.72 ± 3.03; malignant, 16 ± 3.13; P < .05). The sensitivity, specificity, and positive predictive value of malignancy were 100%, 94.4%, and 83.3% for scores ≥10 and 80%, 100%, and 100% for scores ≥11, respectively. In nodules with indeterminate cytology (Bethesda III and IV), the pediatric MTNS showed good differentiation between benign and malignant disease, with mean scores of 7.95 and 12.5, respectively ( P = .006). Conclusion This pilot study suggests that a comprehensive scoring system may help assess the risk of malignancy in pediatric thyroid nodules and differentiate nodules with indeterminate cytology into higher- and lower-risk categories. Given these findings, larger, multi-institutional studies are warranted.