Kaposiform Lymphangiomatosis as a Cause of Vaginal Bleeding & Discharge: A Case Report.

BACKGROUND: Prepubertal vaginal bleeding is a common presentation for pediatric adolescent gynecologists with a broad differential diagnosis that historically may not have included complex lymphatic anomalies. However, given recent consensus criteria and imaging capabilities, this may be a condition that pediatric adolescent gynecologists see more frequently in the future. CASE: We present a case of a 5-year-old pre-pubertal girl whose only presenting symptoms of a rare complex lymphatic anomaly was copious vaginal bleeding. After three vaginoscopies, two hysteroscopies, two pelvic MRIs, and a percutaneous ultrasound guided core needle biopsy, this patient was eventually diagnosed with Kaposiform lymphangiomatosis at age 9 years-old, and she is now being treated medically with sirolimus, a mammalian target of rapamycin (mTOR) inhibitor, with improvement in her symptoms. SUMMARY AND CONCLUSION: Complex lymphatic anomalies should be considered after initial and secondary workups for pre-pubertal vaginal bleeding or copious vaginal discharge are negative. Furthermore, this case illustrates the value of pelvic MRI in the setting of unknown cause of vaginal bleeding when typical workup is negative.

Analysis of treatment sequence and outcomes in patients with relapsed malignant peripheral nerve sheath tumors.

Background: Malignant peripheral nerve sheath tumors (MPNST) are aggressive soft tissue sarcomas originating from cellular components within the nerve sheath. The incidence of MPNST is highest in people with neurofibromatosis type 1 (NF1), and MPNST is the leading cause of death for these individuals. Complete surgical resection is the only curative therapeutic option, but is often unfeasible due to tumor location, size, or presence of metastases. Evidence-based choices of chemotherapy for recurrent/refractory MPNST remain elusive. To address this gap, we conducted a retrospective analysis of our institutional experience in treating patients with relapsed MPNST in order to describe patient outcomes related to salvage regimens. Methods: We conducted a retrospective electronic health record analysis of patients with MPNST who were treated at Johns Hopkins Hospital from January 2010 to June 2021. We calculated time to progression (TTP) based on salvage chemotherapy regimens. Results: Sixty-five patients were included in the analysis. Upfront therapy included single or combined modalities of surgery, chemotherapy, or radiotherapy. Forty-eight patients received at least 1 line of chemotherapy, which included 23 different regimens (excluding active clinical studies). Most patients (n = 42, 87.5%) received a combination of doxorubicin, ifosfamide, or etoposide as first-line chemotherapy. Salvage chemotherapy regimens and their TTP varied greatly, with irinotecan/temozolomide-based regimens having the longest average TTP (255.5 days, among 4 patients). Conclusions: Patients with advanced or metastatic MPNST often succumb to their disease despite multiple lines of therapy. These data may be used as comparative information in decision-making for future patients and clinical trials.

FDG PET of Isolated Right Ventricular Sarcoidosis.

ABSTRACT: A 62-year-old man with a history of allergic rhinitis and lightheadedness was admitted after an episode of sustained ventricular tachycardia detected by Holter monitor. A chest CT revealed peribronchial nodules, and mediastinal and hilar lymphadenopathy. The findings raised concern for sarcoidosis. MRI and FDG PET/CT were performed and revealed cardiac sarcoidosis isolated to the right ventricle only. Sarcoidosis of the right ventricle, sparing the left ventricle, is an extremely uncommon entity.

Estimation of Chest Wall Attenuation of 123I Emissions in Substernal Goiter: A Phantom Study.

In patients with substernal goiter, the generally accepted theory is that thyroid uptake measurements with iodine isotopes are underestimated because of attenuation by the chest wall. The extent of this underestimation is not well known. In this study, we calculated the attenuation of 123I emissions using a cadaver chest wall with a thyroid probe to better understand the potential severity of this underestimation. Methods: A 11.1-MBq capsule of 123I was measured using a thyroid probe directly in a standard neck phantom and behind a cadaver chest wall that included the soft tissues and bony structures (sternum). Results: The calculated attenuation of the iodine capsule was 18% for the neck phantom and 35% for the cadaver chest wall. Conclusion: Thyroid uptake in cases of substernal goiter may be underestimated by standard techniques using a neck phantom. The composition of the chest wall can vary greatly, and the substernal extent of the goiter would be difficult to calculate with high accuracy on a routine basis. Comparison between the cadaveric specimen and the phantom does give us a rough estimation of the differences in attenuation. Our findings suggest that attenuation by the chest wall can be substantial. Knowledge of the extent of the substernal component of the thyroid gland may be useful if the uptake measurement is used to calculate doses for treating hyperthyroidism in patients with substernal goiter.

The impact of particulate electron paramagnetic resonance oxygen sensors on fluorodeoxyglucose imaging characteristics detected via positron emission tomography.

During a first-in-humans clinical trial investigating electron paramagnetic resonance tumor oximetry, a patient injected with the particulate oxygen sensor Printex ink was found to have unexpected fluorodeoxyglucose (FDG) uptake in a dermal nodule via positron emission tomography (PET). This nodule co-localized with the Printex ink injection; biopsy of the area, due to concern for malignancy, revealed findings consistent with ink and an associated inflammatory reaction. Investigations were subsequently performed to assess the impact of oxygen sensors on FDG-PET/CT imaging. A retrospective analysis of three clinical tumor oximetry trials involving two oxygen sensors (charcoal particulates and LiNc-BuO microcrystals) in 22 patients was performed to evaluate FDG imaging characteristics. The impact of clinically used oxygen sensors (carbon black, charcoal particulates, LiNc-BuO microcrystals) on FDG-PET/CT imaging after implantation in rat muscle (n = 12) was investigated. The retrospective review revealed no other patients with FDG avidity associated with particulate sensors. The preclinical investigation found no injected oxygen sensor whose mean standard uptake values differed significantly from sham injections. The risk of a false-positive FDG-PET/CT scan due to oxygen sensors appears low. However, in the right clinical context the potential exists that an associated inflammatory reaction may confound interpretation.

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.

ACR Appropriateness Criteria® Pneumonia in the Immunocompetent Child.

Pneumonia is one of the most common acute infections and the single greatest infectious cause of death in children worldwide. In uncomplicated, community-acquired pneumonia in immunocompetent patients, the diagnosis is clinical and imaging has no role. The first role of imaging is to identify complications associated with pneumonia such as pleural effusion, pulmonary abscess, and bronchopleural fistula. Radiographs are recommended for screening for these complications and ultrasound and CT are recommended for confirmation. The second role of imaging is to identify underlying anatomic conditions that may predispose patients to recurrent pneumonia. CT with intravenously administered contrast is recommended for this evaluation. 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.

ACR Appropriateness Criteria® Suspected Small-Bowel Obstruction.

Small-bowel obstruction is a common cause of abdominal pain and accounts for a significant proportion of hospital admissions. Radiologic imaging plays the key role in the diagnosis and management of small-bowel obstruction as neither patient presentation, the clinical examination, nor laboratory testing are sufficiently sensitive or specific enough to diagnose or guide management. This document focuses on the imaging evaluation of the two most commonly encountered clinical scenarios related to small-bowel obstruction: the acute presentation and the more indolent, low-grade, or intermittent presentation. This document hopes to clarify the appropriate utilization of the many imaging procedures that are available and commonly employed in these clinical settings. 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.

Regulation of intrinsic polarity establishment by a differentiation-type MAPK pathway in S. cerevisiae.

All cells establish and maintain an axis of polarity that is critical for cell shape and progression through the cell cycle. A well-studied example of polarity establishment is bud emergence in the yeast Saccharomyces cerevisiae, which is controlled by the Rho GTPase Cdc42p. The prevailing view of bud emergence does not account for regulation by extrinsic cues. Here, we show that the filamentous growth mitogen activated protein kinase (fMAPK) pathway regulates bud emergence under nutrient-limiting conditions. The fMAPK pathway regulated the expression of polarity targets including the gene encoding a direct effector of Cdc42p, Gic2p. The fMAPK pathway also stimulated GTP-Cdc42p levels, which is a critical determinant of polarity establishment. The fMAPK pathway activity was spatially restricted to bud sites and active during the period of the cell cycle leading up to bud emergence. Time-lapse fluorescence microscopy showed that the fMAPK pathway stimulated the rate of bud emergence during filamentous growth. Unregulated activation of the fMAPK pathway induced multiple rounds of symmetry breaking inside the growing bud. Collectively, our findings identify a new regulatory aspect of bud emergence that sensitizes this essential cellular process to external cues.

ACR Appropriateness Criteria® Developmental Dysplasia of the Hip-Child.

Developmental dysplasia of the hip (DDH) is the most common hip pathology in infants. Although its exact pathophysiology remains incompletely understood, its long-term prognosis depends not only on the severity of the dysphasia, but also on the timely implementation of appropriate treatment. Unrecognized and untreated hip subluxations and dislocations inevitably lead to early joint degeneration while overtreatment can produce iatrogenic complications, including avascular necrosis of the femoral head. In the past two decades, imaging has become an integral part of the clinical screening, diagnosis, and monitoring of children with DDH. Optimal timing for imaging and appropriate use of imaging can reduce the incidence of late diagnoses and prevent iatrogenic complications. In general, ultrasound of the hips is recommended in infants under the age of 4 months while pelvic radiography is recommended in older infants due to the fact that the femoral head ossific nucleus typically is not formed until 4 to 6 months of age. 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.

Impact of Donor Milk on Short- and Long-Term Growth of Very Low Birth Weight Infants.

Mother's own milk (MOM) reduces the risk of morbidities in very low birth weight (VLBW) infants. When MOM is unavailable, donor breastmilk (DM) is used, with unclear impact on short- and long-term growth. This retrospective analysis compared anthropometric data at six time points from birth to 20⁻24 months corrected age in VLBW infants who received MOM supplements of preterm formula (n = 160) versus fortified DM (n = 161) during neonatal intensive care unit (NICU) hospitalization. The cohort was 46% female; mean birth weight and gestational age (GA) were 998 g and 27.3 weeks. Multilevel linear growth models assessed changes in growth z-scores short-term (to NICU discharge) and long-term (post-discharge), controlling for amount of DM or formula received in first 28 days of life, NICU length of stay (LOS), birth GA, and sex. Z-scores for weight and length decreased during hospitalization but increased for all parameters including head circumference post-discharge. Short-term growth was positively associated with LOS and birth GA. A higher preterm formula proportion, but not DM proportion, was associated with slower rates of decline in short-term growth trajectories, but feeding type was unrelated to long-term growth. In conclusion, controlling for total human milk fed, DM did not affect short- or long-term growth.

ACR Appropriateness Criteria® Acutely Limping Child Up To Age 5.

Imaging plays in important role in the evaluation of the acutely limping child. The decision-making process about initial imaging must consider the level of suspicion for infection and whether symptoms can be localized. The appropriateness of specific imaging examinations in the acutely limping child to age 5 years is discussed with attention in each clinical scenario to the role of radiography, ultrasound, nuclear medicine, computed tomography, and magnetic resonance imaging. Common causes of limping such as toddler's fracture, septic arthritis, transient synovitis, and osteomyelitis are discussed. 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.

ACR Appropriateness Criteria® Right Lower Quadrant Pain-Suspected Appendicitis.

Appendicitis remains the most common surgical pathology responsible for right lower quadrant (RLQ) abdominal pain presenting to emergency departments in the United States, where the incidence continues to increase. Appropriate imaging in the diagnosis of appendicitis has resulted in decreased negative appendectomy rate from as high as 25% to approximately 1% to 3%. Contrast-enhanced CT remains the primary and most appropriate imaging modality to evaluate this patient population. MRI is approaching CT in sensitivity and specificity as this technology becomes more widely available and utilization increases. Unenhanced MRI and ultrasound remain the diagnostic procedures of choice in the pregnant patient. MRI and ultrasound continue to perform best in the hands of experts. 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.

Radioaerosol Imaging Predicts the Utility of Aerosolized Antifungal Therapy in Allergic Bronchopulmonary Aspergillosis.

A 10-year-old girl with a history of complicated cystic fibrosis was hospitalized for pulmonary exacerbation with allergic bronchopulmonary aspergillosis and severe right upper lobe bronchiectasis diagnosed with chest radiograph and CT. She was started on itraconazole during the hospitalization in attempt to decrease her systemic steroid dose, but she had ongoing coughing and wheezing. The possibility was raised that the right upper lobe bronchiectasis may be a nidus for ongoing aspergillosis. Radioaerosol imaging was performed to determine whether the aerosolized antifungals would reach the area of bronchiectasis.

The Effect of Electroencephalography Leads on Image Quality in Cerebral Perfusion SPECT and 18F-FDG PET/CT.

Cerebral perfusion SPECT and 18F-FDG PET/CT are commonly performed diagnostic procedures for patients with epilepsy. Individuals undergoing these tests are often inpatients with electroencephalography leads. We have routinely removed these leads because of concern that they would lead to imaging artifacts. The leads would then be replaced at the conclusion of the scan. The goal of our study was to determine whether the electroencephalography leads actually do cause artifacts that can lead to erroneous scan interpretation or make the scan uninterpretable. Methods: 18F-FDG PET/CT and 99mTc-pertechnetate SPECT were performed on a 2-dimensional brain phantom. The phantom was scanned with standard leads, CT/MR-compatible leads, and no leads. The quality of the images was ranked by 3 experienced nuclear medicine physicians, who then determined whether they could differentiate each of the scans from a scan in which it was known that no leads were present. Results: No differences could be detected between scans obtained without leads and scans obtained with either set of leads. The standard electroencephalography leads did create artifacts in the CT portion of the PET/CT images, whereas the CT/MR-compatible leads did not. Conclusion: This phantom study suggests that electroencephalography leads, whether standard or CT/MR-compatible, do not need to be removed for SPECT or PET procedures. Further study evaluating the effect on actual patient scans would be of value to support this conclusion.

Detection of femoroplasty on pre- and post-arthroscopic comparison radiographs following treatment of femoroacetabular impingement syndrome: multi-reader accuracy and agreement study.

OBJECTIVE: To assess diagnostic accuracy and agreement among radiologists in detecting femoroplasty on pre- and post-arthroscopic comparison frog lateral and anteroposterior (AP) pelvic radiographs after treatment of femoroacetabular impingement (FAI) syndrome. MATERIALS AND METHODS: In this retrospective, cross-sectional study, 86 patients underwent hip arthroscopy (52 with and 34 without femoroplasty) for treatment of FAI syndrome. Three radiologists blinded to clinical data and chronological order of the pre- and post-arthroscopic comparison radiographs independently examined AP pelvis and frog lateral radiographs to detect femoroplasty changes. Statistical analysis outputs included diagnostic accuracy parameters and inter- and intra-observer agreement. RESULTS: Identification of femoroplasty in the frog lateral projection has mean sensitivity 70%, specificity 82%, inter-observer agreement κ 0.74-0.76 and intra-observer agreement κ 0.72-0.85. Using the AP pelvis projection to detect femoroplasty has mean sensitivity 32%, specificity 71%, inter-observer agreement κ 0.47-0.65, and intra-observer agreement κ, 0.56-0.84. CONCLUSIONS: Radiologists are only moderately sensitive, though more specific, in femoroplasty detection in the frog lateral projection. The AP pelvis projection yields lower sensitivity and specificity. Both projections have moderate inter- and intra-observer agreement.

A Study of the Pharmacokinetic Properties and the In Vivo Kinetics of Erythrocytes Loaded With Dexamethasone Sodium Phosphate in Healthy Volunteers.

The objectives of this 2-phase study were to elucidate pharmacokinetics (PK), in vivo 24-hour recovery, and red blood cell (RBC) survival properties of RBC-encapsulated dexamethasone sodium phosphate (DSP) prepared using the EryDex System (EDS). The 24-hour RBC recovery and T50 survival phase studied subjects were randomized to receive autologous RBCs loaded with either 15-20 mg DSP (Group 1A) or sham saline (Group 2A). Loaded RBCs were radiolabeled with 51-Cr, and the labeled RBCs were followed over time in vivo. The PK phase evaluated dose levels of 2.5-5 mg (Group 1B) and 15-20 mg (Group 2B) DSP encapsulated in RBCs infused into healthy randomized subjects. The mean ± SD 24-hour RBC recovery was 77.9% ± 3.3% and 72.7% ± 10.5% for Groups 1A and 2A, respectively. The mean ± SD RBC life span was 84.3 ± 8.3 days in Group 1A and 88.9 ± 6.2 days in Group 2A. The PK phase actual DSP loading doses (mean ± SEM) were 4.2 ± 0.27 mg and 16.9 ± 0.90 mg in Groups 1B and 2B, respectively. Release of dexamethasone from RBCs in vivo peaked at 1 hour, and a sustained release of dexamethasone could be detected until 35 days after the single intravenous infusion in Group 2B. The mean RBC in vivo recovery for DSP-loaded processed cells compares similarly to the 24-hour recovery of regulated RBC products intended for transfusion. There was a minimal but acceptable adverse impact on the survival of EDS-processed RBCs. DSP-loaded autologous RBCs, prepared using the EDS, delivered a sustained dose of dexamethasone in vivo.

An Audio/Video Reporting Workflow to Supplement Standardized Radiology Reports.

Radiology studies are inherently visual and the information contained within is best conveyed by visual methodology. Advanced reporting software allows the incorporation of annotated key images into text reports, but such features may be less effective compared with in-person consultations. The use of web technology and screen capture software to create retrievable on-demand audio/visual reports has not yet been investigated. This approach may preempt potential curbside consultations while providing referring clinicians with a more engaged imaging service. In this work, we develop and evaluate a video reporting tool that utilizes modern screen capture software and web technology. We hypothesize that referring clinicians would find that recorded on-demand video reports add value to clinical practice, education, and that such technology would be welcome in future practice. A total of 45 case videos were prepared by radiologists for 14 attending and 15 trainee physicians from emergency and internal medicine specialties. Positive survey feedback from referring clinicians about the video reporting system was statistically significant in all areas measured, including video quality, clinical helpfulness, and willingness to use such technology in the future. Trainees unanimously found educational value in video reporting. These results suggest the potential for video technology to re-establish the radiologist's role as a pivotal member of patient care and integral clinical educator. Future work is needed to streamline these methods in order to minimize work redundancy with traditional text reporting. Additionally, integration with an existing PACS and dictation system will be essential to ensuring ease of use and widespread adoption.

Use of SPECT/CT to confirm inconclusive gastric emptying scintigraphy results.

A 70-year-old man with cramping, abdominal pain, and diarrhea for 5 months after revision of a Nissen fundoplication underwent further evaluation with solid gastric emptying scintigraphy. On sequential planar images, we were uncertain if activity was present within the stomach or within loops of small bowel. SPECT/CT performed at 4 hours was used to localize the tracer, confirming its presence within the stomach.