MR Imaging of the Fetal Genitourinary Tract.

Fetal MR imaging overcomes many of the technical barriers of ultrasound and is an important diagnostic tool for fetal genitourinary (GU) anomalies. It is suited for evaluation of GU anomalies because of the fluid-sensitive sequences and superior soft tissue contrast. Often GU malformations are part of a multisystem genetic or congenital condition, and imaging the entire fetus with MR adds additional clarity about the extent of disease. It adds confidence to diagnoses of renal agenesis, urinary tract dilation, cystic disease, and tumors. It is particularly useful to delineate anatomy in complex GU malformations. This additional information guides counseling.

Implementing a Quality Intervention to Improve Confidence in Outpatient Venous Thromboembolism Management.

INTRODUCTION: Guidelines recommend that patients with acute venous thromboembolism (VTE) represented by low-risk deep vein thrombosis (DVT) and pulmonary embolism (PE) receive initial treatment at home versus at the hospital, but a large percentage of these patients are not managed at home. This study assessed the effectiveness of a quality intervention on provider knowledge and confidence in evaluating outpatient treatment for patients with VTE in the emergency department (ED). METHODS: A pilot program to overcome obstacles to outpatient VTE treatment in appropriate patients was initiated at Baylor Scott & White Health Temple ED. Subsequently, a formalized quality intervention with a targeted educational program was developed and delivered to ED providers. Provider surveys were administered pre- and post-quality intervention in order to assess clinical knowledge, confidence levels, and perceived barriers. Patient discharge information was extracted from electronic health records. RESULTS: Twenty-five ED providers completed the pre- and post-surveys; 690 and 356 patients with VTE were included in the pre- and post-pilot and pre- and post-quality intervention periods, respectively. Many ED providers reported that a major barrier to discharging patients to outpatient care was the lack of available and adequate patient follow-up appointments. Notably, after the quality intervention, an increase in provider clinical knowledge and confidence scores was observed. Discharge rates for patients with VTE increased from 25.6% to 27.5% after the pilot intervention and increased from 28.5% to 29.9% after the quality intervention, but these differences were not statistically significant. Despite instantaneous uptick in discharge rates after the interventions, there was not a long-lasting effect. CONCLUSION: Although the quality intervention led to improvements in provider clinical knowledge and confidence and identified barriers to discharging patients with VTE, discharge rates remained stable, underscoring the need for additional endeavors.

When patients develop blood clots in their veins or have blood clots travel to their lungs, they may seek treatment at the hospital emergency department. As a best practice, most people can treat blood clots with medicines at home; however, many patients are treated at the hospital. This study looked at how an education program for doctors in the hospital could help more patients be treated at home. The education program improved doctors' knowledge and confidence when evaluating patients with blood clots who could be treated at home. However, this study found that the number of patients treated at home was the same before and after the doctors participated in the education program. Two major problems that prevented patients from being treated at home were not having follow-up appointments readily available and patients taking their medicine as needed. More and different types of programs may help doctors understand the best ways to treat patients with blood clots in the emergency department.

Pseudoclitoromegaly from acute T-cell lymphoblastic leukemia.

A 7-year-old girl presented with painful genital enlargement, which was first believed to be clitoromegaly of hormonal origin. However, on the physical exam the clitoris was not visible and the prepuce and labia minora were enlarged and tender. Magnetic resonance imaging demonstrated an infiltrative abnormal signal with restricted diffusion involving the enlarged clitoris and adjacent soft tissues of the prepuce and labia minora, confirming a nonhormonal infiltrative malignancy. The same abnormal signal was present in enlarged inguinal lymph nodes, the kidneys, and an anterior mediastinal mass. The pathologic diagnosis was T-cell acute lymphoblastic leukemia.

Acute acalculous cholecystitis in an infant after gastroschisis closure.

Gastroschisis is a common type of congenital anterior abdominal wall defect with intraabdominal organs exposed outside the abdominal cavity. With modern neonatology and surgical practices, the overall prognosis for infants with gastroschisis is excellent. However, a subset of infants with gastroschisis will develop complications, requiring repeat surgical interventions. We present a case of a female infant with complicated gastroschisis who developed acute perforated acalculous cholecystitis, which was accurately diagnosed with abdominal ultrasound and successfully treated with medical management and a percutaneous cholecystostomy tube.

Neonatal neurosonography practices: a survey of active Society for Pediatric Radiology members.

BACKGROUND: While neonatal brain US is emerging as an imaging modality with greater portability, widespread availability and relative lower cost compared to MRI, it is unknown whether US is being maximized in infants to increase sensitivity in detecting intracranial pathology related to common indications such as hemorrhage, ischemia and ventriculomegaly. OBJECTIVE: To survey active members of the Society for Pediatric Radiology (SPR) regarding their utilization of various cranial US techniques and reporting practices in neonates. MATERIALS AND METHODS: We distributed an online 10-question survey to SPR members to assess practice patterns of neonatal cranial US including protocol details, use of additional sonographic views, perceived utility of spectral Doppler evaluation, and germinal matrix hemorrhage and ventricular size reporting preferences. RESULTS: Of the 107 institutions represented, 90% of respondents were split evenly between free-standing children's hospitals and pediatric departments attached to a general hospital. We found that most used template reporting (72/107, 67%). The anterior fontanelle approach was standard practice (107/107, 100%). We found that posterior fontanelle views (72% sometimes, rarely or never) and high-frequency linear probes to evaluate far-field structures (52% sometimes, rarely or never) were seldom used. Results revealed a range of ways to report germinal matrix hemorrhage and measure ventricular indices to assess ventricular dilatation. There was substantial intra-institutional protocol and reporting variability as well. CONCLUSION: Our results demonstrate high variability in neurosonography practice and reporting among active SPR members, aside from the anterior fontanelle views, template reporting and linear high-resolution near-field evaluation. Standardization of reporting germinal matrix hemorrhage and ventricular size would help ensure a more consistent application of neonatal US in research and clinical practice.

The Perfect Storm: A Case of Rapid-Onset Obesity With Hypoventilation, Hypothalamic, Autonomic Dysregulation, Neuroendocrine Tumor (ROHHADNET) With Heart Failure, Narcolepsy, and a Rare Location of a Pelvic Neuroendocrine Tumor.

Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome is a rare disease of concurrent respiratory dysfunction and autonomic dysregulation with endocrine abnormalities. ROHHADNET includes ROHHAD plus coexisting neuroendocrine tumors (NETs). We describe an eight-year-old boy, who originally presented at four years of age with rapid weight gain and hyperhidrosis and who developed mild obstructive sleep apnea (OSA). His clinical course was eventually complicated by hypoxic respiratory failure requiring admission to the pediatric intensive care unit (PICU). Echocardiogram at that time demonstrated dilated cardiomyopathy left ventricular ejection fraction (LVEF) of 28% at time of admission. His respiratory failure persisted despite average volume-assured pressure support (AVAPS) around the clock leading to tracheostomy placement for cardiopulmonary support. He also demonstrated autonomic instability with multiple pituitary hormone deficiencies. Computed tomography (CT) imaging of the abdomen and pelvis demonstrated a presacral soft tissue mass consistent with a tumor of neural crest origin. Daytime somnolence and confusion progressed and a low cerebrospinal fluid hypocretin level revealed a diagnosis of narcolepsy type 1.

Utilization of neonatal sedation and anesthesia: an SPR survey.

BACKGROUND: There is little data regarding the use of sedation and anesthesia for neonatal imaging, with practice patterns varying widely across institutions. OBJECTIVE: To understand the current utilization of sedation and anesthesia for neonatal imaging, and review the current literature and recommendations. MATERIALS AND METHODS: One thousand, two hundred twenty-six questionnaire invitations were emailed to North American physician members of the Society for Pediatric Radiology using the Survey Monkey platform. Descriptive statistical analysis of the responses was performed. RESULTS: The final results represented 59 institutions from 26 U.S. states, the District of Columbia and three Canadian provinces. Discrepant responses from institutions with multiple respondents (13 out of 59 institutions) were prevalent in multiple categories. Of the 80 total respondents, slightly more than half (56%) were associated with children's hospitals and 44% with the pediatric division of an adult radiology department. Most radiologists (70%) were cognizant of the neonatal sedation policies in their departments. A majority (89%) acknowledged awareness of neurotoxicity concerns in the literature and agreed with the validity of these concerns. In neonates undergoing magnetic resonance imaging (MRI), 46% of respondents reported attempting feed and bundle in all patients and an additional 46% attempt on a case-by-case basis, with most (35%) using a single swaddling attempt before sedation. Sedation was most often used for neonatal interventional procedures (93%) followed by MR (85%), nuclear medicine (48%) and computed tomography (31%). More than half of respondents (63%) reported an average success rate of greater than 50% when using neonatal sedation for MR. CONCLUSION: Current practice patterns, policies and understanding of the use of sedation and anesthesia for neonatal imaging vary widely across institutions in North America, and even among radiologists from the same institution. Our survey highlights the need for improved awareness, education, and standardization at both the institutional level and the societal level. Awareness of the potential for anesthetic neurotoxicity and success of non-pharmacologic approaches to neonatal imaging is crucial, along with education of health care personnel, systematic approaches to quality control and improvement, and integration of evidence-based protocols into clinical practice.

Gastroschisis in one twin neonate with extracorporeal liver.

We report a rare case of gastroschisis with extracorporeal liver suspected on late first trimester ultrasound and confirmed with second trimester ultrasound and magnetic resonance imaging in one fetus in a twin pregnancy. Liver herniation is common in omphalocele, a membrane-covered abdominal wall defect associated with other congenital anomalies. However, it is highly uncommon in gastroschisis, an uncovered abdominal wall defect aside of the cord insertion. Presence of liver herniation complicates prenatal differentiation between omphalocele and gastroschisis. The twins were born at 31 weeks' gestation due to preterm labor. The baby was treated with a silo device, followed by biologic mesh and a wound vac with instillation of fluid to prevent desiccation. Ultimately, the baby died of sepsis, with multiorgan failure and polymicrobial infection.

Mixed gonadal dysgenesis with an ovotestis on imaging mimicking ovotesticular disorder of sexual differentiation.

Mixed gonadal dysgenesis (MGD) is a rare disorder of sexual development. Also known as 45XO/46XY mosaicism, MGD is characterized by highly variable sexual phenotypes and an increased risk of gonadal malignancy. Patients with MGD often have a unilateral descended gonad and contralaterally either a streak gonad or no gonad. We present the case of a patient with a dysgenetic, nonpalpable gonad with imaging features of an ovotestis. These imaging features are generally more indicative of ovotesticular disorder of sexual development (previously true hermaphrodite), which is a condition with low risk of gonadal malignancy. Further evaluation with histology and genetic analysis confirmed the diagnosis of MGD. It is important to diagnose MGD to allow for early operative intervention and screening for malignancy.

Magnetic resonance imaging diagnosis of a skeletal dysplasia mimicking erosive arthropathy.

This case report of a 14-year-old boy with arthralgia and clinically suspected inflammatory arthropathy highlights how magnetic resonance imaging (MRI) ultimately diagnosed skeletal dysplasia. A genetic evaluation revealed a transient receptor potential vanilloid 4 (TRPV4) pathogenic variant. This is a rare description of the MRI appearance of this type of dysplasia in long bone epiphyses corresponding with the histological findings of disrupted endochondral ossification. This report offers imaging support to the description of endochondral bone growth disruption in TRPV4-related skeletal dysplasias.

Imaging of solid congenital abdominal masses: a review of the literature and practical approach to image interpretation.

Fetal abdominal tumors are rare, usually benign, and cause a great deal of anxiety for expectant parents and the physicians counseling them. In this paper the author reviews the most common fetal abdominal tumors in the liver (hemangioma, mesenchymal hamartoma, hepatoblastoma, metastases) and the kidney (congenital mesoblastic nephroma, Wilms tumor, malignant rhabdoid tumor, and clear cell sarcoma), and suprarenal mass lesions (adrenal neuroblastoma, adrenal hemorrhage, and subdiaphragmatic extralobar pulmonary sequestration). The author describes the imaging approach, imaging appearance and differentiating features of tumors, and differences between fetal and childhood appearances of tumors.

Evolution and regression of imaging findings in treated pulmonary Langerhans cell histiocytosis.

In rare instances, pediatric Langerhans cell histiocytosis (LCH) may manifest as lung disease. While the imaging features at presentation have been reported, we present sequential computed tomography (CT) scans of a 3-year-old boy with pulmonary LCH, revealing the evolution and regression of the disease. Sequential CT scans during treatment demonstrated variable evolution of pulmonary cysts, including changes in size, thinning of walls, and a pattern of collapse into irregular nodules and involution. Our case represents a rare opportunity to examine sequential CT findings of pediatric pulmonary LCH regression.

Turner syndrome and autosomal dominant polycystic kidney disease.

Turner syndrome is a chromosomal disorder that involves multiple organ systems and is typically associated with short stature. A multidisciplinary approach with regular screening and surveillance is key to managing this condition's multiple comorbidities. We present a case of a young girl with Turner syndrome and associated short stature on growth hormone treatment who presented with cystic renal disease found to be autosomal dominant kidney disease. We propose reevaluation of renal screening guidelines in this population due to the potential association of growth hormone and cyst proliferation.

Hyperuricosuria, hematuria, and novel bladder images with IgA nephropathy.

Episodic (recurrent) macroscopic hematuria in patients with IgA nephropathy is usually associated with a benign prognosis, although some patients experience a transient fall in glomerular filtration rate during the episodes. We present a 15-year-old girl with mild IgA nephropathy who had multiple episodes of macroscopic hematuria associated with severe but transient decreases in estimated glomerular filtration rate, low levels of serum uric acid, and marked increases in fractional excretion of uric acid. Ultrasound studies showed marked inflammatory changes in the bladder, especially involving the trigone. Cystoscopic findings were consistent with these changes. We hypothesize that the macroscopic hematuria may have resulted, at least in part, from hyperuricosuria causing acute irritation of the bladder mucosa in the trigone area.

Calcific tendonitis of the flexor pollicis longus tendon at the thumb interphalangeal joint in childhood.

In rare instances, calcific tendonitis may manifest in the pediatric population as inflammatory calcium hydroxyapatite deposition. To our knowledge, there have been no previous case reports involving the flexor pollicis longus tendon at the thumb interphalangeal joint. We present a 9-year-old boy with a painful mass at the right thumb interphalangeal joint. Initial radiographs revealed a 7-mm ovoid calcific mass along the volar soft tissues of the thumb interphalangeal joint. Subsequent ultrasound and magnetic resonance findings further confirmed calcification with surrounding edema. Because the pain was limiting the patient's school activities, his family elected for excisional biopsy of the calcific mass. Pathology ultimately revealed prominent dystrophic calcifications with surrounding granulomatous inflammation, consistent with calcific tendonitis.

Magnetic resonance imaging as a diagnostic modality in a child with acute lymphoblastic leukemia.

Acute lymphoblastic leukemia is the most common form of childhood cancer. It presents with nonspecific symptoms, such as bone pain, that can be easily misdiagnosed at initial presentation. We present a 2-year-old boy with bilateral foot pain that worsened over 6 months. X-rays of the feet showed no obvious abnormalities. Magnetic resonance imaging revealed abnormal bone marrow consistent with an infiltrative process. The patient was found to have precursor B-cell acute lymphoblastic leukemia. Cases such as these highlight the possible future use of magnetic resonance imaging in the early diagnostic workup for bone and muscle pain.

Pedunculated focal nodular hyperplasia in a healthy toddler.

Focal nodular hyperplasia (FNH) is a benign hepatic tumor rarely seen in pediatric patients, with most cases reported in school-aged children with a history of malignancy, liver disease, chemotherapy, or hematopoietic stem cell therapy. Despite having advanced radiographic imaging, diagnosing FNH before surgical resection can be difficult. We report a rare case of pedunculated FNH presenting as a large abdominal mass palpated on physical exam in a healthy 3-year-old girl with no history of malignancy or underlying liver disease. Ultrasound, computed tomography, and magnetic resonance imaging (MRI) did not demonstrate the typical imaging characteristics of FNH, because the mass was pedunculated with a poorly visualized central scar. Because approximately 75% of all primary hepatic tumors in this age group are malignant, this report also discusses the importance of adding hepatobiliary phase imaging with MRI to avoid, if possible, the need for biopsy or surgical resection of a benign hepatic tumor.

Limited, fast magnetic resonance imaging as an alternative for preoperative evaluation of pectus excavatum: a feasibility study.

OBJECTIVE: The purpose of this study was to determine the reliability, feasibility, and image quality of a limited, fast magnetic resonance imaging (MRI) protocol for preoperative evaluation of pectus excavatum in a pediatric population referred for presurgical imaging. MATERIALS AND METHODS: A total of 47 patients, median age 14 years, referred for preoperative imaging of pectus excavatum, underwent axial balanced steady-state free precession MRI of the chest, with a limited patient charge. Two pediatric radiologists independently conducted a blinded retrospective study. The Haller and asymmetry indices were calculated at the level of greatest anterior-posterior chest narrowing. In addition, right heart compression and image quality were subjectively assessed, and scan duration was determined. RESULTS: Intraclass correlation coefficient reliability was between 0.85 and 0.98, indicating almost perfect agreement for quantitative measurements. Subjective evaluation of right heart compression and image quality showed moderate interreader agreement. Image quality was graded as good or excellent by both readers for all studies. No difference in the Haller index was observed between modalities in 3 patients on both computed tomographic scan and MRI (P = 0.2697). The median scan duration was 8 minutes. CONCLUSIONS: Limited MRI is a reliable and cost-effective alternative for preoperative assessment of pectus excavatum. It is fast, free of ionizing radiation, and there is excellent interreader reliability for measurements of chest wall deformity.

Breathe in... breathe out... stop breathing: does phase of respiration affect the Haller index in patients with pectus excavatum?

OBJECTIVE: The purpose of this article is to determine whether the phase of respiration at the time of imaging affects chest wall measurements and compression of internal structures in patients with pectus excavatum. MATERIALS AND METHODS: Forty-seven patients (median age, 14 years) imaged for preoperative pectus excavatum underwent limited axial balanced steady-state free precession MRI of the chest at inspiration, expiration, and stop quiet breathing. Two radiologists, who were blinded to prior measurements, independently calculated the Haller index, asymmetry index, and sternal tilt in each phase of respiration. Compression of internal structures was recorded. Statistical comparison was performed. RESULTS: The Haller index was significantly lower at inspiration, compared with stop quiet breathing and expiration, with medians (interquartile ranges) of 3.96 (3.27-4.61), 5.16 (4.02-6.48), and 5.09 (4.14-6.63), respectively (p < 0.0001 for both). No significant difference in Haller indexes was observed between expiration and stop quiet breathing (p = 0.1171). Of 11 patients with a Haller index less than 3.25 at inspiration, eight (72.7%) had an index greater than 3.25 on expiration and stop quiet breathing, which accounted for 17% (8/47) of all patients imaged. Compression of the liver or vascular structures was present in 24 (51%) patients. There was no significant difference in the asymmetry index, sternal tilt, or right heart compression between phases of respiration. CONCLUSION: Obtaining the Haller Index at inspiration may result in a value significantly lower than that at expiration, potentially affecting surgical and financial decision making. Compression of the liver and vascular structures was observed in 51% of patients, but additional research is needed to determine the clinical significance of this finding.