ACR Appropriateness Criteria® Antenatal Hydronephrosis-Infant.

Antenatal hydronephrosis is the most frequent urinary tract anomaly detected on prenatal ultrasonography. It occurs approximately twice as often in males as in females. Most antenatal hydronephrosis is transient with little long-term significance, and few children with antenatal hydronephrosis will have significant obstruction, develop symptoms or complications, and require surgery. Some children will be diagnosed with more serious conditions, such as posterior urethral valves. Early detection of obstructive uropathy is necessary to mitigate the potential morbidity from loss of renal function. Imaging is an integral part of screening, diagnosis, and monitoring of children with antenatal hydronephrosis. Optimal timing and appropriate use of imaging can reduce the incidence of late diagnoses and prevent renal scarring and other complications. In general, follow-up neonatal ultrasound is recommended for all cases of antenatal hydronephrosis, while further imaging, including voiding cystourethrography and nuclear scintigraphy, is recommended for moderate or severe cases, or when renal parenchymal or bladder wall abnormalities are suspected. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Serial MRI Findings After Endoscopic Removal of Button Battery From the Esophagus.

OBJECTIVE. The purpose of this study was to evaluate findings at serial MRI after endoscopic removal of a button battery from the esophagus in a series of pediatric patients. MATERIALS AND METHODS. Serial MRI examinations after removal of a button battery from the esophagus were reviewed retrospectively for the presence of mediastinal edema; imaging characteristics of the aorta and arteries; imaging characteristics of the trachea; and imaging characteristics of the esophageal wall at the level of injury. RESULTS. A total of 48 MRI examinations were performed on 19 patients, 89% (17/19) in the first 48 hours after battery removal. Serial MRI was performed for 84% (16/19) of patients. Initial MRI showed extensive mediastinal edema in all 17 patients who underwent MRI in the first 48 hours. Edema directly abutted major arteries in all 17 patients and abutted the airway in all 10 patients with proximal esophageal injury. Arterial vascular changes were seen in 30% (3/10) of patients with proximal esophageal injury and 57% (4/7) of patients with mid or distalesophageal injury. Airway changes were seen in 80% (8/10) of patients with proximal esophageal injury. Serial MRI showed improvement of airway changes in all patients and improvement in vessel wall changes in all but one (25%, 1/4) of the patients who had mid or distal esophageal injury. Four patients (21% [4/19]) had contained esophageal leak on esophagrams. No patients in our series developed a tracheoesophageal or vascular-enteric fistula. CONCLUSION. Our case series provides important information on natural history of MRI findings in children after endoscopic removal of a button battery from the esophagus. Further studies are needed to determine the imaging findings most sensitive and specific for severe complications, such as tracheoesophageal fistula and vascular-enteric fistula.

Incidence and importance of portal venous gas in children with hypertrophic pyloric stenosis.

BACKGROUND: Hypertrophic pyloric stenosis (HPS) is a common cause of gastric outlet obstruction in young infants. Infants with HPS present with projectile vomiting, sometimes have electrolyte abnormalities and typically undergo pyloromyotomy to alleviate the obstruction. Abdominal US is the gold standard imaging study for diagnosis. Case reports of incidental hepatic portal venous gas have been reported in infants with HPS; however, no large studies have been conducted to determine the incidence or possible clinical implications of this finding. OBJECTIVE: To assess the incidence of portal venous gas in infants with HPS and to determine whether the presence of this gas in infants with HPS indicates a more unstable patient, increased length of stay or worse outcome. MATERIALS AND METHODS: We conducted a retrospective review of sonographic reports containing "pyloric stenosis," excluding negative descriptor, at a tertiary-care children's hospital from November 2010 to September 2017. Data collected included pyloric thickness/length, liver evaluation, portal venous gas, any additional imaging, demographics, symptomatology days, electrolyte abnormality, and length of hospital stay. RESULTS: In a 7-year period, 545 US exams were positive for HPS. Of these, 334 exams included enough hepatic parenchyma to evaluate for portal venous gas. Infants in 6 of the 334 exams demonstrated portal venous gas (1.8%). Clinical presentation (length of symptoms and electrolyte abnormalities), demographics (male predominance and age at presentation) and imaging characteristics (pyloric thickness and length) were similar for the HPS groups with and without portal venous gas. There was no significant difference in outcome or length of hospital stay. CONCLUSION: Visualization of portal venous gas in infants with HPS is not rare and appears benign, without need for further imaging. Portal venous gas in infants with HPS does not portend a more severe patient presentation or outcome.

Risk factors and surveillance for reduced bone mineral density in pediatric cancer survivors.

BACKGROUND: Pediatric cancer survivors are at increased risk of developing low bone mineral density (BMD) due to cancer treatment. This study assessed the yield of screening for low BMD in pediatric-aged cancer survivors as per the Children's Oncology Group Long-Term Follow-Up (COG-LTFU) Guidelines, which recommend screening survivors who received steroids, methotrexate, or hematopoietic cell transplant (HCT). METHODS: This is a retrospective cohort study of 475 pediatric blood cancer and noncentral nervous system solid tumor survivors screened for low BMD with dual-energy X-ray absorptiometry (DXA) as per the COG-LTFU Guidelines from 2003 to 2010. Risk factors for low BMD (DXA Z-score ≤-2) were evaluated by univariate and multivariate analysis. RESULTS: The mean DXA Z-score was -0.1 for both whole body and lumbar spine measurements. Among at-risk survivors, 8.2% (39/475) had low BMD. Multivariate analysis of survivors with low BMD showed significant association with male gender (odds ratio [OR] 3.4, 95% confidence interval [CI], 1.3-9.0), exposure to total body irradiation (TBI), cranial, or craniospinal radiation (OR 5.2, 95% CI, 1.8-14.9), and gonadal dysfunction (OR 4.3, 95% CI, 1.4-13.0). Methotrexate exposure was not significantly associated with low BMD. Survivors receiving HCT had a reduced risk of low BMD (OR 0.2, 95% CI, 0.1-0.9). CONCLUSION: The highest risk factors for low BMD were male gender, exposure to TBI, cranial, or craniospinal radiation, and gonadal dysfunction. Survivors receiving methotrexate or HCT therapy have the lowest risk for low BMD among those screened. Future studies should investigate risk of low BMD for survivors receiving HCT without radiation exposure.

ACR Appropriateness Criteria Fever Without Source or Unknown Origin-Child.

The cause of fever in a child can often be determined from history, physical examination, and laboratory tests; infections account for the majority of cases. Yet in 20%, no apparent cause can be found, designated as fever without source (FWS). The yield of chest radiography in children with FWS is low, and it is usually not appropriate. However, in children with respiratory signs, high fever (>39°C), or marked leukocytosis (≥20,000/mm(3)), chest radiography is usually appropriate, as it has a higher yield in detecting clinically occult pneumonia. In newborns with FWS, there is higher risk for serious bacterial infection, and the routine use of chest radiography is controversial. In children with neutropenia, fever is a major concern. In some clinical circumstances, such as after hematopoietic stem cell transplantation, chest CT scan may be appropriate even if the results of chest radiography are negative or nonspecific, as it has higher sensitivity and can demonstrate specific findings (such as lung nodule and "halo sign") that can guide management. In a child with prolonged fever of unknown origin despite extensive medical workup (fever of unknown origin), diagnosis is usually dependent on clinical and laboratory studies, and imaging tests have low yield. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Care Partnerships: Toward Technology to Support Teens' Participation in Their Health Care.

Adolescents with complex chronic illnesses, such as cancer and blood disorders, must partner with family and clinical caregivers to navigate risky procedures with life-altering implications, burdensome symptoms and lifelong treatments. Yet, there has been little investigation into how technology can support these partnerships. We conducted 38 in-depth interviews (15 with teenage adolescents with chronic forms of cancer and blood disorders, 15 with their parents, and eight with clinical caregivers) along with nine non-participant observations of clinical consultations to better understand common challenges and needs that could be supported through design. Participants faced challenges primarily concerning: 1) teens' limited participation in their care, 2) communicating emotionally-sensitive information, and 3) managing physical and emotional responses. We draw on these findings to propose design goals for sociotechnical systems to support teens in partnering in their care, highlighting the need for design to support gradually evolving partnerships in care.

Adolescent and Caregiver use of a Tethered Personal Health Record System.

Supporting adolescent patient engagement in care is an important yet underexplored topic in consumer health informatics. Personal Health Records (PHRs) show potential, but designing PHR systems to accommodate both emerging adults and their parents is challenging. We conducted a mixed-methods study with teenage adolescent patients (ages 13-17) with cancer and blood disorders, and their parents, to investigate their experiences with My-Chart, a tethered PHR system. Through analyses of usage logs and independently-conducted surveys and interviews, we found that patients and parents both valued MyChart, but had different views about the role of the PHR for care communication and management, and different attitudes about its impact on the patient's ability to manage care. Specific motivations for using MyChart included patient-parent coordination of care activities, communication around hospital encounters, and support for transitioning to adult care. Finally, some parents had concerns about certain diagnostic test results being made available to their children.

Normal conus medullaris FDG uptake in children.

BACKGROUND: The biodistribution of 18F-FDG has been well described in both adults and children. Many findings are limited to children and warrant understanding prior to interpretation. OBJECTIVE: To determine the normal level of conus medullaris uptake, not previously reported in the literature to date, in a series of consecutive FDG PET/CT scans performed in children. MATERIALS AND METHODS: With IRB approval, we retrospectively reviewed 100 consecutive whole-body pediatric 18F-FDG PET/CT examinations obtained for various clinical indications. Scans that showed visible uptake of FDG at the conus were objectively evaluated, and standardized uptake value (SUV) was determined. Maximum SUV of the conus was compared to background, normal liver and lung, and ratios were recorded. Pathology in the conus was excluded. RESULTS: The scans of 100 patients ages 5 months to 24 years (mean 11.7 years) were reviewed. Three patients were excluded. Seventy percent showed uptake at the conus medullaris. SUVs ranged from 1.33 to 4.2 (mean 2.14). CONCLUSION: Low-level 18F-FDG uptake is common in the conus medullaris, represents normal distribution in children and should not be interpreted as metastatic disease to the CNS.

Fibrocartilaginous mesenchymoma, a unique osseous lesion: case report with review of the literature.

Fibrocartilaginous mesenchymoma is a rare osseous tumor that primarily arises in the long bones of children and adolescents. This lesion can grow quickly and reach a considerable size, despite its benign nature. It has proved challenging to diagnose and can be mistaken for a spectrum of benign and malignant bone tumors. The histological presentation of unique epiphyseal plate-like cartilage with destruction of the surrounding cortical bone and exhibition of dense fibrous stroma are important indicators for the diagnosis of fibrocartilaginous mesenchymoma. An 11-year-old boy presented with a left proximal humerus mass thought to be an aneurysmal bone cyst. The patient was lost to follow-up and came back 3 years later with massive growth of the lesion. Owing to the aggressive nature of the tumor, a left forequarter amputation was performed. Histological examination demonstrated numerous islands of cartilage with an exuberant spindle cell component characteristic of FCM. No distant metastases or local recurrences were identified at 2 years post-amputation. Because of the rapid growth of this lesion, it should be considered in the differential diagnosis of bone lesions in children and young adults.

Role of PET/CT in congenital histiocytosis.

Congenital Langerhans cell histiocytosis (LCH) is a rare, but often severe, form of LCH. Although a more benign single-system congenital LCH has been described, most cases present as multisystem disease with poorer prognosis and are often treated with systemic chemotherapy. Imaging plays a central role in diagnosis, initial staging and assessment of treatment response. PET/CT is increasingly utilized for pediatric LCH. We report a unique case with PET/CT used as an imaging tool in staging and evaluating treatment response in congenital multisystem LCH.

Blastic plasmacytoid dendritic cell neoplasm presenting as a subcutaneous mass in an 8-year-old boy.

Blastic plasmacytoid dendritic cell neoplasm (also referred to as CD4+/CD56+ hematodermic neoplasm) is a rare hematological malignancy typically seen in older adults. The disease presents with nonspecific cutaneous lesions and advances toward a fatal leukemic phase despite an often favorable initial response to chemotherapy. Fewer than 200 cases have been documented, and it is exceedingly rare in children. We report a case of histopathologically proven blastic plasmacytoid dendritic cell neoplasm arising in an otherwise healthy and asymptomatic 8-year-old boy who noticed a painless mass within the subcutaneous tissues below the left calf. In addition to contrast-enhanced MRI, the imaging work-up included PET/CT, which showed metastatic involvement within left inguinal and retroperitoneal lymph nodes. The case is notable both for the young age of the patient and for the absence of characteristic cutaneous manifestations of this disease.

Laryngeal and tracheal anomalies in an infant with oral-facial-digital syndrome type VI (Váradi-Papp): report of a transitional type.

The oral-facial-digital syndromes (OFDS) comprise a group of disorders involving malformations of the mouth, face, and digits. There are 13 subtypes of the OFDS, and much overlap exists among OFDS patients. Distinct syndromes such as Joubert and Pallister-Hall display many of the same features. This report describes an infant with abnormalities including a hypoplastic/absent cerebellar vermis and forked third metacarpals, consistent with a diagnosis of OFDS type VI (Váradi-Papp). The girl's abnormalities also included malformations of the larynx and trachea, findings never before described in type VI but described in other OFDS subtypes and similar syndromes. Our patient represents a transitional OFDS type, further supporting evidence of a common molecular pathway among these disorders. This report highlights the importance of the radiologist's role in diagnosis.