Kiery Braithwaite

Magnetic Resonance Enterography Healing and Magnetic Resonance Enterography Remission Predicts Improved Outcome in Pediatric Crohn Disease.

Background: Mucosal healing predicts clinical remission and improved outcomes in patients with Crohn disease (CD). Magnetic resonance enterography (MRE) is a noninvasive imaging modality that can assess small and large bowel wall inflammation. Evidence suggests that MRE may be an acceptable alternative to evaluate mucosal healing over endoscopy. Our objective is to determine whether MRE remission predicts clinical remission at follow-up in children with CD. Methods: We performed an institutional review board–approved retrospecitve chart review using our prospectively maintained MRE CD database. Inclusion criteria were all children who underwent an MRE more than 6 months after diagnosis with CD who had follow-up of at least 1 year from imaging. Results: A total of 101 children with CD underwent MRE, a median of 1.3 years from diagnosis with a median follow-up of 2.8 years after MRE. Active inflammation was detected in 65 MRE studies, whereas 36 MRE studies demonstrated MRE remission. A total of 88.9% of children demonstrating MRE remission were in clinical remission at follow-up, whereas only 44.6% of those demonstrating MRE active inflammation achieved clinical remission. Children demonstrating MRE-active inflammation were more likely to have a change in medication (44.6% vs 8.3%) and more likely to undergo surgery (18.5% vs 2.8%). Conclusions: MRE remission is associated with clinical remission at follow-up at least 1 year after MRE. MRE remission was associated with fewer medication changes and fewer surgeries suggesting that, similar to endoscopic remission, MRE remission demonstrates improved outcome. Additional research is needed to confirm that MRE can be used as a surrogate for mucosal healing.

Secondary signs may improve the diagnostic accuracy of equivocal ultrasounds for suspected appendicitis in children.

INTRODUCTION Ultrasound (US) is the preferred imaging modality for evaluating appendicitis. Our purpose was to determine if including secondary signs (SS) improve diagnostic accuracy in equivocal US studies. METHODS Retrospective review identified 825 children presenting with concern for appendicitis and with a right lower quadrant (RLQ) US. Regression models identified which SS were associated with appendicitis. Test characteristics were demonstrated. RESULTS 530 patients (64%) had equivocal US reports. Of 114 (22%) patients with equivocal US undergoing CT, those with SS were more likely to have appendicitis (48.6% vs 14.6%, p<0.001). Of 172 (32%) patients with equivocal US admitted for observation, those with SS were more likely to have appendicitis (61.0% vs 33.6%, p<0.001). SS associated with appendicitis included fluid collection (adjusted odds ratio (OR) 13.3, 95% confidence interval (CI) 2.1-82.8), hyperemia (OR=2.0, 95%CI 1.5-95.5), free fluid (OR=9.8, 95%CI 3.8-25.4), and appendicolith (OR=7.9, 95%CI 1.7-37.2). Wall thickness, bowel peristalsis, and echogenic fat were not associated with appendicitis. Equivocal US that included hyperemia, a fluid collection, or an appendicolith had 96% specificity and 88% accuracy. CONCLUSION Use of SS in RLQ US assists in the diagnostic accuracy of appendicitis. SS may guide clinicians and reduce unnecessary CT and admissions.

Imaging findings in megacystis-microcolon-intestinal hypoperistalsis syndrome

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare and frequently lethal form of severe functional intestinal obstruction more commonly found in girls. Imaging features characteristic of this disease include a large dilated bladder, microcolon and intestinal dysmotility. Additional imaging findings may include intestinal malrotation, hydronephrosis and vesicoureteral reflux. It is usually fatal in the first year of life. Because presenting clinical and imaging features can mimic other causes of neonatal bowel obstruction, we compiled examples of this disorder to help the pediatric radiologist recognize imaging findings associated with MMIHS and aid in the development of a long-term management plan and in counseling the family regarding implications of this disorder. We reviewed recent and historical literature relevant to MMIHS and present the imaging and clinical features of four patients with MMIHS treated at our institution as examples of this uncommon disorder.

Use of the star sign to diagnose internal fistulas in pediatric patients with penetrating Crohn disease by MR enterography

Development of internal fistula due to extramural spread of inflammatory bowel disease is a characteristic feature of penetrating disease in patients with Crohn disease. The “star sign” is a radiological finding of internal fistula that has previously been described in the gastroenterology literature in adult Crohn disease patients undergoing MR enteroclysis. The goal of this paper is to review the clinical and imaging features of penetrating disease in pediatric Crohn disease patients, highlighting the star sign as a useful diagnostic tool for diagnosing internal fistula in children by MR enterography. The recognition of penetrating complications by MR imaging can have important therapeutic and prognostic implications.

Improving ultrasound for appendicitis through standardized reporting of secondary signs

OBJECTIVE Our aim was to implement a standardized US report that included secondary signs of appendicitis (SS) to facilitate accurate diagnosis of appendicitis and decrease the use of computed tomography (CT) and admissions for observation. METHODS A multidisciplinary team implemented a quality improvement (QI) intervention in the form of a standardized US report and provided stakeholders with monthly feedback. Outcomes including report compliance, CT use, and observation admissions were compared pretemplate and posttemplate. RESULTS We identified 387 patients in the pretemplate period and 483 patients in the posttemplate period. In the posttemplate period, the reporting of SS increased from 5.4% to 79.5% (p<0.001). Despite lower rates of appendix visualization (43.9% to 32.7%, p<0.001) with US, overall CT use (8.5% vs 7.0%, p=0.41) and the negative appendectomy rate remained stable (1.0% vs 1.0%, p=1.0). CT utilization for patients with an equivocal ultrasound and SS present decreased (36.4% vs 8.9%, p=0.002) and admissions for observations decreased (21.5% vs 15.3%, p=0.02). Test characteristics of RLQ US for appendicitis also improved in the posttemplate period. CONCLUSION A focused QI initiative led to high compliance rates of utilizing the standardized US report and resulted in lower CT use and fewer admissions for observation. Study of a Diagnostic Test Level of Evidence: 1.

Gender-Based Differences in Pediatric Nuclear Medicine

Gender-based differences commonly encountered in pediatric nuclear medicine reflect both basic embryologic differences of the sexes, which are evident from infancy, and evolving physiological changes due to gender, which occur as the pediatric patient grows, undergoes puberty, and matures to adulthood. It is important for a nuclear medicine physician or radiologist to know both the gender and the age of a patient when interpreting her or his studies. It is also important that the reading physician be familiar with the normally evolving physiological changes that are specific for that patients stage of development. It is particularly important that the reading physician consider such changes when comparing serial studies of the patient that are acquired during the patients transitions through her or his different significant stages of development. Many pediatric nuclear medicine imaging protocols are modifications or adaptations of the protocols for adult imaging. Physicians reading pediatric studies must routinely incorporate knowledge on age and gender that is relevant to the patient for any given study. The age-defined gender-based subtleties of potential findings in pediatric nuclear medicine studies are often underrecognized. However, they are often of interest and at times important in the workup of both benign entities and pathologic processes of the pediatric patient.

The Skeletally Immature and Newly Mature Throwing Athlete

Injuries to the shoulder and elbow in the pediatric and adolescent throwing athlete are common. Both knowledge of throwing mechanics and understanding of normal bone development in the immature skeleton are key to the diagnosis, treatment, and potential prevention of these common injuries. Pathologic changes from chronic repetitive trauma to the developing shoulder and elbow manifest as distinctly different injuries that can be predicted by the skeletal maturation of the patient. Sites of vulnerability and resulting patterns of injury change as the child evolves from the skeletally immature little league player to the skeletally mature high school/college athlete.

Complete sternal cleft associated with right clavicular, manubrial, and thyroid hypoplasia, pectus deformity, and spinal anomalies.

Sternal cleft anomalies are rare. Associated anomalies include pentalogy of Cantrell and posterior fossa abnormalities, hemangiomas, arteriopathy, cardiac anomalies, eye abnormalities, and sternal defects syndrome. There is only a single report of complete sternal cleft, pectus excavatum, and right clavicular hypoplasia in an adult. Thyroid hemiagenesis is also very rare. To our knowledge, this is the first case of complete sternal cleft, pectus deformity, and right clavicular hypoplasia in a child and the first case with right thyroid hemiagenesis.

Malignant degeneration of pulmonary juvenile-onset recurrent respiratory papillomatosis

Juvenile-onset recurrent respiratory papillomatosis (JORRP) is a rare disease associated with the human papilloma virus (HPV) in which papillomas form along the aerodigestive tract in children. Pulmonary involvement is uncommon, but associated with worse clinical outcomes, including the rare complication of malignant transformation. We present a patient with JORRP in which lung disease underwent malignant transformation during adolescence. Our goal is to raise awareness of the potential for malignant transformation in children, as well as to familiarize pediatric radiologists with imaging features of malignant lung disease in JORRP. We advocate for the identification of the subgroup of JORPP patients with pulmonary disease who, due to increased risk for malignant transformation, may benefit from closer clinical and imaging surveillance by a multidisciplinary team.

707 MRI Identifies Unsuspected Liver Dysfunction in Patients After Fontan Procedure

Since the development of the original NAFLD activity score (NAS), it became evident that pediatric NAFLD has a distinct histopathological pattern including the presence of portal inflammation (PI). The aim of this study was to develop and validate a new grading score for Pediatric NAFLD that takes into account the presence of PI and the weight of each histological feature. Methods: Our training set consisted of 203 pediatric patients with biopsy-proven NAFLD. The diagnosis of nonalcoholic steatohepatitis (NASH) versus not NASH was based on Brunt criteria. The individual histological features of NAFLD were scored as follows: steatosis (0-3), lobular inflammation (0-3), ballooning (0-2), and portal inflammation (0-2; 0= no PI, 1= mild PI, 2= more than mild). Logistic regression analysis was performed to apply weight to each histological feature (to improve correlation with NASH). The new score was called the Pediatric NAFLD Histological Score or PNHS. A p value < 0.05 was considered statistically significant. The validation set consisted of 100 children with biopsy-proven NAFLD. Results: The mean age of the initial cohort was 12.4 ± 3.4 years, the median BMI percentile was 97% (94%-98%), and significant fibrosis (fibrosis stage ≥ 2) was present in 26 patients (12.8%). NASH was diagnosed in 135 patients with a mean NAS of 4.5 ± 1.4. The mean PNHS in the NASH group was 89 ± 20.5 compared to 21.9 ± 24.5 in not NASH group, p< 0.001. PNHS correlated with the presence of NASH according to pathologist diagnosis better than NAS, p= 0.011. The area under the ROC curve (AUC) for diagnosis of NASH was 0.96 for PNHS. Similar findings were noted in the validation set with AUC of 0.94. Conclusion: PNHS may be used for histological grading of pediatric NAFLD with better correlation with the presence of NASH than NAS. The utility of this score in other populations and in therapeutic trials should be further investigated.