The Society for Pediatric Radiology Magnetic Resonance Imaging and Emergency and Trauma Imaging Committees' consensus protocol recommendation for rapid MRI for evaluating suspected appendicitis in children.

The imaging evaluation of acute abdominal pain in children with suspected appendicitis has evolved to include rapid abdominopelvic MRI (rMRI) over recent years. Through a collaborative effort between the Magnetic Resonance Imaging (MRI) and Emergency and Trauma Imaging Committees of the Society for Pediatric Radiology (SPR), we conducted a survey on the utilization of rMRI to assess practice specifics and protocols. Subsequently, we present a proposed consensus rMRI protocol derived from the survey results, literature review, and discussion and consensus between committee members.

Rib spur causing a hemothorax, pneumothorax, and diaphragmatic injury in a pediatric patient.

Pleural effusion is a relatively common condition encountered in the pediatric emergency department. Evaluation of pleural effusion in the emergency department typically includes advanced imaging such as computer tomography or ultrasound, as well as diagnostic thoracocentesis. We report a case of a 10-year-old female with a rib spur at the anterolateral left sixth rib that caused a hemothorax, pneumothorax, and diaphragmatic injury. The patient underwent video-assisted thoracoscopic surgery and resection of the rib spur. The procedure was well-tolerated without any complications.

Bifocal pineal and suprasellar germinomas with posterior fossa metastases in an adolescent patient.

Central nervous system germ cell tumors are rare lesions that are more frequently seen in the pediatric age group. Intracranial germinomas are a type of these germ cell tumors and commonly arise in the pineal region, suprasellar region, or less frequently at both areas (bifocal). Common features of this tumor depend on the location of the lesion(s) and include Parinaud's syndrome, obstructive hydrocephalus, diabetes insipidus, panhypopituitarism, strabismus, and visual acuity defects. We report a case of bifocal pineal and suprasellar germinoma with posterior fossa metastases in a 15-year-old male patient. The involvement of the third ventricular floor and nonthickened inferior pituitary stalk of the suprasellar lesion suggest that it is a metastasis of a primary pineal lesion rather than a dual-primary. This distinction, with the presence of posterior fossa metastases, favors the use of more aggressive treatment with combination radiation therapy and chemotherapy for a better outcome.

Child Neurology: Hypotonia and Delayed Teeth Eruption in a 2-Year-Old Girl.

POLR3-related disorders are rare hypomyelinating leukodystrophies associated with hypodontia. We present a female patient, who was referred to pediatric neurology at 2 years of age for tremor, low tone, and motor delays. In addition, she was noted to have a delay in her teeth eruption and myopia. Neurologic examination was significant for ataxic features and global developmental delay. Laboratory workup was unrevealing. MRI was significant for hypomyelination. Genetic testing confirmed a pathogenic variant of POLR3B POLR3-related leukodystrophies should be considered in patients who present with hypotonia, ataxia, and hypodontia. There are many different subtypes of POLR-related leukodystrophies each with distinguishing phenotypic and radiographic features. Although MRI can be helpful in initial evaluation, genetic testing is needed for confirmatory diagnosis and to guide prognosis.

Longitudinal Changes in the Sensorimotor Pathways of Very Preterm Infants During the First Year of Life With and Without Intervention: A Pilot Study.

Objective: Evaluate longitudinal changes in brain microstructure and volumes in very preterm infants during the first year of life with and without intervention.Design: Descriptive pilot study.Methods: Five preterm infants in a three-arm clinical trial, one SPEEDI Early, two SPEEDI Late, and two usual care. Brain structural and diffusion MRI's were acquired within 72 hours after neonatal intensive care unit discharge (n = 5), three months post-baseline (n = 5), and six months post-baseline (n = 3). Fractional anisotropy (FA), Mean diffusivity (MD), and volume metrics were computed for five brain regions.Results: More than 60% of eligible participants completed 100% of the scheduled MRIs. FA and volume increased from baseline to six months across all brain regions. Rate of white matter volume change from baseline to six months was highest in SPEEDI Early.Conclusions: Non-sedated longitudinal MRI is feasible in very preterm infants and appears to demonstrate longitudinal changes in brain structure and connectivity.

Aggressive vertebral hemangioma masquerading as neurological disease in a pediatric patient.

Aggressive hemangioma is a rare vertebral lesion in pediatric patients which can present with deteriorating neurological function. It can mimic malignancy on imaging, particularly as it regularly has an extrasosseous soft tissue component. We present a case of a 13-year-old male who presented with a three month history of lower extremity weakness that was found to have an infiltrative mass at T10 with associated cord compression from epidural extension of the lesion. In this report we review the characteristic imaging findings associated with aggressive hemangioma, including its appearance on read-out segmented diffusion-weighted images. It is imperative that radiologists who interpret studies of children be aware that this lesion exists and what it looks like, as it can be associated with massive hemorrhage if encountered unexpectedly during surgery.

Pre- and postnatal MR imaging of an asymptomatic giant hypothalamic hamartoma.

Hypothalamic hamartomas are rare tumors that are most often diagnosed in early childhood. These lesions are classified as giant hypothalamic hamartomas when they exceed 4 cm in any 1 dimension. The most common presenting symptoms associated with these lesions are precocious puberty, gelastic seizures, and (less commonly) syndromic conditions such as Pallister-Hall syndrome. We present a unique case of an asymptomatic giant hypothalamic hamartoma diagnosed prenatally by fetal magnetic resonance imaging and followed throughout infancy. This case demonstrates the utility of multimetric analysis using difference sequences, including diffuse-weighted imaging, to assess specific properties of intracranial lesions detected in utero and to aid in accurate diagnosis prior to birth.

In Vitro Measurement of Regional Nasal Drug Delivery with Flonase,® Flonase® Sensimist,™ and MAD Nasal™ in Anatomically Correct Nasal Airway Replicas of Pediatric and Adult Human Subjects.

Background: The majority of current nasal delivery devices, commercialized for children, are developed for adults. Differences in the dose reaching the target are expected due to significant differences between the pediatric and adult nasal airway geometries and their inhalation patterns. This study aims to compare the efficacy of most common nasal drug delivery devices in terms of regional delivery of suspension and solution formulations in pediatric and adult subjects. Methods: Anatomically correct nasal models of 2-, 5-, and 50-year old subjects were developed to evaluate regional nasal delivery of suspensions of fluticasone propionate and fluticasone furoate delivered with Flonase® and Flonase® Sensimist™, respectively, and the delivery of an aqueous solution of a model drug, administered with MAD Nasal™. Relevant inhalation patterns were considered for each nasal airway geometry. Controlled administration methods were used, and all contributing parameters, including particle size, velocity, and plume geometry, are reported. Results: Regional deposition patterns resulting from Flonase® Sensimist™ and Flonase® were not significantly different in each replica (p > 0.05), despite their different plume geometry and droplet size distributions. However, there was a significant difference in deposition of nasal sprays between the pediatric (2- and 5-year old) and adult models (p < 0.05), while no statistical differences were found between the two pediatric models (p > 0.05). The MAD atomizer resulted in different deposition patterns in all three subjects (p < 0.05). Conclusion: Nasal sprays are not adequate delivery devices for pediatric population, due to the narrower nasal passage and greater anterior deposition (∼60%). MAD atomizer resulted in significantly less anterior deposition (∼10%-15%) compared to the nasal pumps, but there was ∼30% run off to the throat. An in vitro platform incorporating anatomically correct nasal geometries and inhalation patterns can guide the development of age-appropriate nasal drug delivery devices.

A Chromosomal Deletion and New Frameshift Mutation Cause ARSACS in an African-American.

Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS) is a rare, progressive, neurodegenerative disease characterized by ataxia, spasticity and polyneuropathy. First described in the French-Canadian population of Quebec in 1978, ARSACS has since been identified in multiple patients worldwide. In this clinical case report, we describe the evaluation of an 11-years-old African-American male who presented to neuromuscular clinic for assessment of a gait abnormality. He had a history of gross motor delay since early childhood, frequent falls and a below average IQ. Chromosomal microarray revealed a 1.422 megabase loss in the 13q12.12 region, which includes the SACS gene. Next Generation Sequencing then showed a novel, predicted to be pathogenic missense mutation (c.11824dup) of this gene. His clinical presentation and neurological imaging further confirmed the diagnosis of ARSACS. To our knowledge, this is the first reported case of this disease in the African-American population of the United States. This case report further highlights the growing trend of identifying genetic diseases previously restricted to single, ethnically isolated regions in many different ethnic groups worldwide.

Pediatric cholangiopathies: diseases of the gallbladder and biliary tract.

Pediatric gallbladder and bile duct disease encompasses a broad spectrum of processes, from congenital to developmental to neoplastic. We describe normal pediatric biliary anatomy and summarize the most common pathologic entities, with a focus on non-invasive imaging techniques and findings. Ultrasound is the first-line imaging modality in children with suspected biliary pathology based on its widespread availability, cost effectiveness, and lack of ionizing radiation. MRI and MRCP are often used for further evaluation in cases of diagnostic uncertainty and for surgical planning.

Review of diffusion tensor imaging and its application in children.

Diffusion MRI is an imaging technique that uses the random motion of water to probe tissue microstructure. Diffusion tensor imaging (DTI) can quantitatively depict the organization and connectivity of white matter. Given the non-invasiveness of the technique, DTI has become a widely used tool for researchers and clinicians to examine the white matter of children. This review covers the basics of diffusion-weighted imaging and diffusion tensor imaging and discusses examples of their clinical application in children.

Pediatric cavernous sinus thrombosis: A case series and review of the literature.

OBJECTIVE: To describe clinical characteristics, imaging findings, morbidity, and mortality in a single-center cohort of 12 pediatric cavernous sinus thrombosis cases and to review all cases available in recent English literature. METHODS: Clinical data and radiographic studies on 12 cases from our institution were analyzed retrospectively. A literature search and review was conducted, with additional cases pooled with the new cohort for an aggregate analysis. RESULTS: Twelve cases of cavernous sinus thrombosis in children from the Children's Hospital of Philadelphia between January 1, 2000, and December 31, 2013, were reviewed. All patients survived to discharge; 3 of 12 (25%) experienced neurologic morbidity. Contrast-enhanced MRI and contrast-enhanced head CT were 100% sensitive for detecting cavernous sinus thrombosis, while noncontrast time-of-flight magnetic resonance venography (TOF MRV) and noncontrast head CT were 0% sensitive. Literature review produced an additional 40 cases, and the aggregate mortality rate was 4 of 52 (8%) and morbidity rate was 10 of 40 (25%). Outcomes did not vary by treatment or with unilateral vs bilateral cavernous sinus involvement. There was a trend toward worse outcomes with fungal infections. CONCLUSION: Our case series demonstrates low morbidity and mortality with early, aggressive surgical, antimicrobial, and anticoagulation therapies. Although anticoagulation and surgery were not associated with significantly different outcomes, more study is needed.

Fourth-year medical student opinions and basic knowledge regarding the field of radiology.

RATIONALE AND OBJECTIVES: This study evaluates the opinions and knowledge of fourth-year US medical students regarding radiology and analyzes the influence of a required or nonrequired radiology rotation as a reflection of the effectiveness of radiology medical student education. METHODS AND MATERIALS: Our institutional review board granted exempt status. An invitation e-mail was sent to 137 US medical schools. Upon receiving approval a second email was sent containing our voluntary anonymous online survey hyperlink to forward to their fourth-year class. Survey topics included demographics, radiology educational experiences, attitudes toward the field, and basic radiology knowledge. Responses were collected between August 4 and September 26, 2011. RESULTS: A total of 444 fourth-year medical students from 37 medical schools participated: 89% planned to enter a nonradiology specialty, 10.8% were required to take a dedicated radiology rotation, 34.9% completed one, 77% planned to complete one by graduation, 88.4% thought radiology often changes patient care or is at least as important as physical exam, 91.4% underestimated the cancer risk of an abdomen and pelvis computed tomography by at least one order of magnitude, and 72.9% by at least two orders. Seventy-seven percent had never heard of the American College of Radiology (ACR) Appropriateness Criteria. Respondents underestimated the potential risks of magnetic resonance imaging (MRI); with 58.3% aware intravenous gadolinium can cause nephrogenic systemic fibrosis and 79.4% aware of potential injury from metallic projectiles. 40.4% indicated that non-radiologist clinicians in specific medical specialties interpret their respective imaging studies at least as accurately as corresponding subspecialty radiologists. Other results include student opinions regarding teleradiology, radiologist lifestyle, and compensation. CONCLUSIONS: Fourth-year medical students recognize the importance of radiology but are poorly informed regarding radiation safety, MRI safety, and ACR Appropriateness Criteria, despite 34.9% having a dedicated rotation. This highlights the need for adoption of the Alliance of Medical Student Educators in Radiology curriculum.

Reducing abdominal CT radiation dose with the adaptive statistical iterative reconstruction technique in children: a feasibility study.

BACKGROUND: The use of the adaptive statistical iterative reconstruction (ASIR) algorithm has been shown to reduce radiation doses in adults undergoing abdominal CT studies while preserving image quality. To our knowledge, no studies have been done to validate the use of ASIR in children. OBJECTIVE: To retrospectively evaluate differences in radiation dose and image quality in pediatric CT abdominal studies utilizing 40% ASIR compared with filtered-back projection (FBP). MATERIALS AND METHODS: Eleven patients (mean age 8.5 years, range 2-17 years) had separate 40% ASIR and FBP enhanced abdominal CT studies on different days between July 2009 and October 2010. The ASIR studies utilized a 38% mA reduction in addition to our pediatric protocol mAs. Study volume CT dose indexes (CTDI(vol)) and dose-length products (DLP) were recorded. A consistent representative image was obtained from each study. The images were independently evaluated by two radiologists in a blinded manner for diagnostic utility, image sharpness and image noise. RESULTS: The average CTDI(vol) and DLP for the 40% ASIR studies were 4.25 mGy and 185.04 mGy-cm, compared with 6.75 mGy and 275.79 mGy-cm for the FBP studies, representing 37% and 33% reductions in both, respectively. The radiologists' assessments of subjective image quality did not demonstrate any significant differences between the ASIR and FBP images. CONCLUSION: In our experience, the use of 40% ASIR with a 38% decrease in mA lowers the radiation dose for children undergoing enhanced abdominal examinations by an average of 33%, while maintaining diagnostically acceptable images.