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Pleuropulmonary blastomas are rare, potentially aggressive embryonal cancers of the lung parenchyma and pleural surfaces that account for 0.25%-0.5% of primary pulmonary malignancies in children. Pleuropulmonary blastomas are classified as cystic (type I), mixed cystic and solid (type II), and solid (type III). Pleuropulmonary blastoma occurs in the same age group (0-6 years) as other more common solid tumors such as neuroblastoma and Wilms tumor. Differential diagnosis includes metastasis from Wilms tumor and macrocystic congenital pulmonary airway malformation (CPAM). A key pathologic and genetic discriminator is the DICER1 germline mutation found in patients with pleuropulmonary blastoma. Imaging, histopathologic, and clinical data are important to use in conjunction in order to determine the diagnosis and risk stratification of pleuropulmonary blastomas. Survival varies from poor to good, depending on type. However, the spectrum of pleuropulmonary blastoma is insufficiently understood due to the variable presentation of this rare disease. We present a current review of the literature regarding pleuropulmonary blastomas in this article.

Penetrating injuries of the thorax and abdomen, such as gunshot and stabbing, are rare in children. We present the case of a pediatric patient with a history of remote gunshot injury presenting with a late aneurysm in the left ventricle. (Level of Difficulty: Intermediate.)

Stent angioplasty of patent ductus arteriosus has been shown to be a viable alternative to operative shunt placement in cyanotic neonates. With broader implementation of this strategy, novel complications are bound to arise. We present a series of cases evaluated for ductal stent angioplasty in which a dilated and torturous ductus arteriosus compressed the left mainstem bronchus. After reviewing our recent experience with ductal stenting and isolated Blalock-Taussig shunts, our best estimate of the incidence of bronchial compression by the dilated ductus is 4.6% (3/64, 95% confidence interval 1.0-12.9%). Awareness of the airway and other nonvascular contents of the thorax is an important consideration prior to ductal stenting.

ObjectiveAlthough PU.1/Spi1 is known as a master regulator for macrophage development and function, we have reported previously that it is also expressed in adipocytes and is transcriptionally induced in obesity. Here, we investigated the role of adipocyte PU.1 in the development of age-associated metabolic syndrome.MethodsWe generated mice with adipocyte specific PU.1 knockout, assessed metabolic changes in young and aged PU.1fl/fl (control) and AdipoqCre PU.1fl/fl(aPU.1KO) mice, including body weight, body composition, energy expenditure and glucose homeostasis. We also performed transcriptional analyses using RNA-Sequencing of adipocytes from these mice.ResultsaPU.1KO mice have elevated energy expenditure at a young age and decreased adiposity and increased insulin sensitivity in later life. Corroborating these observations, transcriptional network analysis indicated the existence of validated, aPU.1-modulated regulatory hubs that direct inflammatory and thermogenic gene expression programs.ConclusionsOur data provide evidence for a previously uncharacterized role of PU.1 in the development of age-associated obesity and insulin resistance.

Objective: To assess the value of lateral neck radiographs in quantifying adenoid hypertrophy to help guide treatment decisions in patients with symptoms of nasal obstruction. Study Design: Retrospective review. Methods: Quantitative radiologic grading of adenoids was correlated with the intraoperative grading to select cases in agreement between the two methods. The percent airway obstruction was calculated as a ratio of adenoid size to the size of the nasopharyngeal airway near the level of the choanae on the lateral neck radiographs for adenoidectomy cases in which radiographic and intraoperative grading of adenoid size were in agreement. Results: A total of 426 adenoidectomy cases with preoperative lateral neck radiographs were reviewed (M:F = 254:172 for age range 9 months to 16 years), and only cases in agreement between radiographic and intraoperative adenoid grading were included in radiographic analysis (N = 234). The percent airway obstruction values were significantly different between “severely obstructive” (N = 137, mean = 94.71, SD = 6.55, range [72.00; 100.00]) and “moderately obstructive” adenoid categories (N = 97, mean = 78.53, SD = 6.91, range [63.67; 98.08]), not only within clinically relevant age groups (1-3 years, 4-7 years, 8-15 years), but also for the entire data set (95% CI [14.41; 17.95], P Conclusion: Lateral neck radiographs can provide useful supplemental information on the degree of nasopharyngeal airway obstruction when other clinical findings do not clearly point toward adenoid hypertrophy as a primary cause of nasal obstruction. In our data set, a 65% nasopharyngeal airway obstruction represents a value two standard deviations below the mean for “moderately” obstructive adenoid category, and can be viewed as a simplified cut-off to indicate that the degree of adenoid enlargement is clinically relevant. This cut-off value can assist in evaluation of patients with symptoms of nasal obstruction. Level of Evidence: 4

To evaluate the safety of power injectors for contrast-enhanced computed tomography (CT) in children, namely: the prevalence and injury severity of contrast extravasations related to power injectors and the factors associated with these events.The need to obtain informed consent was waived for this HIPAA-compliant and IRB approved retrospective study. Around 2429 contrast-enhanced CT performed with a power injector were identified during a 3-year period. Data collected included patient demographic, power injector, and contrast agent information. The patients' symptoms, severity of injury and treatment with contrast extravasation were recorded. Around 1496 cases (823 boys, 673 girls) were included in the analysis. Independent-sample t test and Chi-square were used. For a sub-analysis using the extravasation cases, nonparametric tests were used.The mean age was 9.5 ± 6.1 years. The most common access site, catheter site, and contrast agent used were the antecubital fossa, 22 gauge and Iohexol. The mean peak pressure was 68.9 ± 62.3 psi and the flow rate was 1.7 ± 0.9 mL/s. Eighteen cases of contrast extravasation were identified with a mean age of 11.2 ± 6.2 years. There were seven mild, six moderate, and five severe. Cases with extravasation had significantly higher peak pressure (p0.001) and flow rate (p0.001) compared to those without extravasation. Patients who received Iohexol-350 had significantly more contrast extravasation compared to those who used Iohexol-300 (p = 0.03). However, after post-hoc correction, only peak pressure (p0.01) and flow rate (p = 0.01) remained significant.The use of power injectors in children undergoing contrast-enhanced CT is associated with a low rate of extravasation and of long-term injury.

Partial liquid ventilation is proposed as an alternative ventilation strategy to reduce surface tension, increase alveolar recruitment, and decrease inflammation. Studied in acute respiratory distress and other indications, liquid ventilation is being revisited for infants with bronchopulmonary dysplasia. Perfluorooctyl bromide used for liquid ventilation is radiopaque, allowing radiographic visualization of lung liquid ventilation patterns that may provide additional insight into pulmonary pathophysiology. Current protocols utilize reduced liquid dosing, resulting in unique imaging features. We discuss optimal radiographic technique and report initial ultrasound evaluation results. With renewed interest in partial liquid ventilation, it may be helpful for pediatric radiologists to familiarize themselves with the clinical use and radiographic appearance of liquid ventilation material.

To describe the technique and report on our initial experience with the use of intrahepatic dynamic contrast magnetic resonance lymphangiography (IH-DCMRL) for evaluation of the lymphatics in patients with hepatic lymphatic flow disorders. This is a retrospective review of the imaging and clinical findings in six consecutive patients undergoing IH-DCMRL. The technique involves injection of a gadolinium contrast agent into the intrahepatic lymphatic ducts followed by imaging of the abdomen and chest with both heavily T2-weighted imaging and dynamic time-resolved imaging. In six consecutive patients, IH-DCMRL was technically successful. There were four patients with protein-losing enteropathy (PLE) and two with ascites in this study. In the four patients with PLE, IH-DCMRL demonstrated hepatoduodenal connections with leak of contrast into the duodenal lumen not seen by conventional lymphangiography. In one patient with ascites, IH-DCMRL demonstrated lymphatic leakage into the peritoneal cavity not seen by intranodal lymphangiography. In the second patient with ascites, retrograde lymphatic perfusion of mesenteric lymphatic networks and nodes was seen. Venous contamination was seen in two patients. No biliary contamination was identified. There were no short-term complications. IH-DCMRL is a cross-sectional technique which successfully evaluated hepatic lymphatic flow disorders and warrants further investigation. • Intrahepatic dynamic contrast magnetic resonance lymphangiography (IH-DCMRL) is a new imaging technique to evaluate hepatic lymphatic flow disorders such as protein-losing enteropathy. • In comparison to conventional liver lymphangiography, IH-DCMRL offers a 3D imaging technique and better distal lymphatic contrast distribution and does not use ionizing radiation.

We report severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spike ΔH69/V70 in multiple independent lineages, often occurring after acquisition of receptor binding motif replacements such as N439K and Y453F, known to increase binding affinity to the ACE2 receptor and confer antibody escape. In vitro, we show that, although ΔH69/V70 itself is not an antibody evasion mechanism, it increases infectivity associated with enhanced incorporation of cleaved spike into virions. ΔH69/V70 is able to partially rescue infectivity of spike proteins that have acquired N439K and Y453F escape mutations by increased spike incorporation. In addition, replacement of the H69 and V70 residues in the Alpha variant B.1.1.7 spike (where ΔH69/V70 occurs naturally) impairs spike incorporation and entry efficiency of the B.1.1.7 spike pseudotyped virus. Alpha variant B.1.1.7 spike mediates faster kinetics of cell-cell fusion than wild-type Wuhan-1 D614G, dependent on ΔH69/V70. Therefore, as ΔH69/V70 compensates for immune escape mutations that impair infectivity, continued surveillance for deletions with functional effects is warranted., Graphical abstract, Meng et al. report that the SARS-CoV-2 spike ΔH69/V70 has arisen multiple times. The deletion increases entry efficiency associated with increased cleaved spike in virions and can compensate for loss of infectivity. The B.1.1.7 spike requires ΔH69/V70 for efficient cell entry and cell-cell fusion activity.

We report on 2 different types of left pulmonary artery sling (LPAS), types IA and IIB, diagnosed by fetal magnetic resonance imaging (MRI). We suggest that fetal MRI is an effective tool for accurately diagnosing LPAS and helping guide its perinatal management. Fetal MRI is relatively unaffected by the conditions that limit visualization by echocardiography. When prenatal ultrasound detects either a possible anomalous origin of the left pulmonary artery or a tracheobronchial anomaly, fetal MRI may provide additional information to confirm the LPAS diagnosis and classify its type. To our knowledge, these are the first reports of prenatally diagnosed LPAS by fetal MRI.

A 16-year-old girl presented to the emergency department with orthopnea and exercise intolerance for 1 week. Electrocardiogram at that time showed rightward deviation of the cardiac axis, with nonspecific T-wave inversion in the lateral precordial leads and flattening in the inferior leads. She was discharged from the emergency department with cardiology follow-up. Subsequent echocardiogram demonstrated that "the heart is in a funny position in the chest," and computed tomography of the chest was requested to evaluate for a mediastinal mass causing leftward displacement of the heart.

Generalized arterial calcification of infancy (GACI) is a rare genetic disease that affects the circulatory system and the large- and medium-sized arteries throughout the body. GACI usually occurs during fetal development. Babies with GACI are diagnosed early, generally soon after birth and in some cases before birth by fetal ultrasound.

To assess the diagnostic efficiency of CT angiography (CTA) to detect pulmonary vein stenosis in children. We retrospectively identify patients between 0 and 3 years old with confirmed pulmonary vein stenosis with conventional angiography or surgery and available CTA. Patients without confirmed stenosis of the pulmonary veins were included as controls. We excluded patients with previous surgery involving the pulmonary veins, exclusively right-heart conventional angiography or insufficient data in the operation note to confirm the status of the pulmonary veins. Two pediatric radiologists evaluated and determine the presence of stenosis and the pulmonary veins affected. Disagreement between the readers were solved by consensus with a third reader. A pediatric cardiologist reviewed the available angiographic images to determine the presence of stenosis. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy were calculated. Kappa statistics was performed. 26 patients (15 boys, 11 Girls) were included. Conventional angiography (n = 20) and cardiothoracic surgery (n = 6) confirmed the diagnosis of pulmonary vein stenosis in 13 children. The diagnostic performance at patient level showed the sensitivity, specificity, PPV, and NPV were 84.6%, 92.3%, 91.6%, 87.5%, and 88.4%, respectively. The interobserver was k = 0.76. The performance at pulmonary vein level showed the sensitivity, specificity, PPV, and NPV were 63.3%, 97.4%, 90.4%,85.7% and 87.9%, respectively. The interobserver agreement was k = 0.62. Computed Tomography Angiography is an excellent and reliable image technique for ruling in pulmonary vein stenosis in young children.

BACKGROUND: The Fontan operation is performed for surgical palliation of single ventricle physiology. This operation is usually preceded by a superior cavopulmonary connection (SCPC); lymphatic abnormalities after SCPC may be demonstrated at MRI and prior to the Fontan operation. PURPOSE: To determine if the degree of neck and thoracic lymphatic abnormalities at T2-weighted MRI in patients after superior cavopulmonary connection (SCPC) correlated with surgical outcomes from the Fontan procedure. MATERIALS AND METHODS: Patients for whom SCPC was performed for palliation of single ventricle disease who underwent chest MRI between July 2012 and May 2015 at a single institution were retrospectively reviewed. T2-weighted images were scored as lymphatic type 1 (little or no T2 mediastinal and supraclavicular signal) to type 4 (T2 signal into both the mediastinum and the lung parenchyma). Fontan takedown, duration of post-Fontan hospitalization and pleural effusion, postoperative plastic bronchitis, need for transplant, and mortality were tabulated. The relationship between lymphatic type and clinical outcomes was evaluated by using analysis of variance (ANOVA), the Kruskal-Wallis H test, and the Fisher exact test. RESULTS: A total of 83 patients (mean age, 7.9 years ± 2.6) were evaluated. Among these 83 patients, 53 (64%) were classified with type 1 or 2 lymphatic abnormalities, 17 (20%) with type 3, and 12 (16%) with type 4. The rate of failure of Fontan completion was higher in patients with type 4 than in type 1 or 2 (54% vs 2%, respectively; P = .004). Need for cardiac transplant (one of 13 [8%]) and death (three of 13 [23%]) occurred only in type 4. Median postoperative length of stay was longer for patients with type 4 than for those with types 1 or 2 (29 days vs 9 days, respectively; P < .01). CONCLUSION: Greater MRI-based severity of lymphatic abnormalities in patients prior to planned Fontan procedure was associated with failure of Fontan completion and longer postoperative stay. © RSNA, 2019 Online supplemental material is available for this article.

Objectives Neonates in the neonatal intensive care unit often require considerable support with endotracheal tubes, umbilical arterial and venous catheters, and peripherally inserted central venous catheters. Support device evaluation with radiography exposes neonates to ionizing radiation. This study evaluated the effectiveness of sonographic localization for endotracheal tubes, umbilical arterial and venous catheters, and peripherally inserted central venous catheters. Methods This blinded prospective Institutional Review Board-approved, Health Insurance Portability and Accountability Act-compliant study with informed consent compared sonography to radiography for endotracheal tube, umbilical arterial and venous catheter, and peripherally inserted central venous catheter localization. Participants were consecutively recruited NICU patients of any weight, gestation, and chronologic age who had an endotracheal tube, umbilical arterial catheter, umbilical venous catheter, or peripherally inserted central venous catheter placed or adjusted and had subsequent radiographic confirmation within 24 hours. Sonographic evaluation was obtained as soon as possible, without prior review of the radiograph, and results were compared. Results Thirty sonographic studies were performed in 25 patients (14 male and 11 female), for a total of 50 lines and tubes: 18 umbilical venous catheters, 12 umbilical arterial catheters, 11 peripherally inserted central venous catheters, and 9 endotracheal tubes. Forty-nine support devices (98%) were visualized with sonography, all concordant with radiography. Forty-four were correctly positioned, and 6 were malpositioned. Sonography identified the location of umbilical venous catheters in all 18 cases (100%), umbilical arterial catheters in all 12 (100%), peripherally inserted central venous catheters in 10 (91%), and endotracheal tubes in 9 (100%). Conclusions The effectiveness of sonography was excellent for evaluation of umbilical arterial and venous catheters, endotracheal tubes, and peripherally inserted central venous catheters. These results support the goal of further point-of-care training and accreditation to use sonography as a primary modality for complete evaluation of NICU support devices.

Evaluate the feasibility and determinants of image quality of ECG-triggered High-Pitch Dual-Source Computed Tomography Angiography (CTA) for cardiovascular assessment in Children.All children that underwent ECG-triggered High-Pitch Dual-Source CTA between August 2014 and September 2017 were identified. Scanner parameters and patients' information were retrieved. Objective image quality was evaluated measuring the Hounsfield units (HU) and standard deviation of regions of interests in the left ventricle, ascending and descending aorta. Signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were calculated. Qualitative image quality was recorded independently by two pediatric radiologist blinded using a three-point scale: 1 - good image quality, 2 - mild artifacts, 3 - poor image quality. Continuous variables were presented as mean ± standard deviation. The interobserver agreement and non-parametric test were used.93 patients (mean age 5.6 ± 7.1 years) were selected. Average cardiovascular attenuation, SNR and CNR were 406.2 ± 146.3 HU, 24.2 ± 16.0 HU and 52.1 ± 38.6 HU, respectively. Average image quality was 1.51 ± 0.48 and the inter-observer agreement was excellent (k = 0.8). Worse subjective quality scores were associated with lower age, height, weight, BSA, lower contrast dose and slower injection rates (p0.05). Higher heart rate was associated with high attenuation (p0.05), however, SNR and CNR did not show an association with heart rate (p = 0.80).ECG-triggered High-Pitch Dual-Source cardiac CTA is feasible and provides good or excellent image quality for the evaluation of cardiovascular diseases in children.

Assess image quality and radiation dose of ECG-triggered High-Pitch Dual-Source CTA for the evaluation central vascular stents in children. We included all children ≤ 21 years old with one or more central vascular stents and available prospective ECG-triggered High-Pitch Dual-Source CTA performed at our institution between January 2015 and August 2017. Demographic and scanner information was retrieved. Two board-certified pediatric radiologists blinded to the clinical data, independently reviewed and scored each case using a four-point quality score. Scores 1, 2 and 3 were considered of diagnostic image quality. Inter-observer agreement and non-parametric test were used. 18 patients (10 girls, 8 boys) with a mean age of 9.47 ± 7.38 years (mean ± SD) met inclusion criteria. Thirty-two central vascular stents were evaluated. Mean quality score was 2.07 ± 0.94 with 12.5% (4/32) of the cases classified as unevaluable. Interobserver agreement was excellent (k = 0.86). There is no significant difference between quality score and stent location (p = 0.07). There is a significant difference with stent material as all non-diagnostic scores were only seen in covered stents made of platinum-iridium (p 0.001). There was no association between image quality and age, height, weight, BSA, heart rate, radiation dose or stent lumen size (p 0.05). ECG-triggered high-pitch spiral DS-CTA offers appropriate image quality for assessment of central vascular stents in children.

Objective Assess the image quality of ECG-triggered High-Pitch Dual-Source CTA for the evaluation of native coronaries in children. Materials and methods Between August 2014 and September 2017, 45 children with morphologically normal cardiac chambers had cardiac prospective ECG-triggered High-Pitch Dual-Source CTA. Two pediatric radiologists blinded to clinical data, independently reviewed each case. The coronary arteries were evaluated using a four-point scale quality score according to the coronary segment. Attenuation, signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were measured using values from the air, inter-ventricular septum and ascending aorta at the level of the sinuses of Valsalva. Results 225 coronary segments were assessed showed a mean score of 2.40 ± 0.73, 94.2% had diagnostic image quality. The best and worst average quality were seen in segment 5 and 2, respectively. Inter-observer agreement was moderate for all segments except for segment 1, which was excellent. Worse quality scores were significantly associated with younger patients and low body mass index as well as with higher heart rates in all segments. The mean observed heart rate and BSA in patients with diagnostic image quality were below 77 bpm and over 1.4 m2 respectively. There is no significant association between attenuation, SNR and CNR with image quality. Conclusions Prospective ECG-triggered High-Pitch Dual-Source Computed Tomography Angiography achieves consistent and diagnostic image quality for coronary artery assessment at a low effective dose in pediatric patients.

Whether employed in a private practice, a community or academic setting, or even a hybrid practice, radiologists fill many roles in the course of a day. Teaching is one of the most important roles in the dissemination of knowledge and the continued renewal of the health, vibrancy and advancement of our field. In this article the author reviews some of the various facets of what radiology teaching and learning look like in the current era. Highlighted topics include workstation teaching, workshop and small group education, interdisciplinary activities and mentorship.

Congenital aberrant hepatic vascular communications result from intrahepatic or extrahepatic errors in vascular development or involution during the transition from fetal to newborn hepatic circulation. These hepatic vascular shunts can be fortuitously discovered and asymptomatic, or can cause symptoms of varying severity, often presenting diagnostic dilemmas. Some hepatic vascular shunts resolve spontaneously while others require interventional radiologic or surgical closure. Affected patients are often first studied with real-time and Doppler ultrasound, so radiologists should familiarize themselves with the expected ultrasound findings of these vascular shunts for effective diagnosis, triage and management. In this review, the authors focus on ultrasound and Doppler findings of hepatic vascular shunts with underlying embryology, clinical features and management strategies. Broadly, these aberrant hepatic vascular communications include portosystemic venous shunts (which can be intra- or extrahepatic and include persistent patent ductus venosus), arterioportal, arteriovenous or mixed shunts.

PRESENTATIONAn 18-year-old male adolescent presented with severe headache for 2 days. The pain was localized to the back of the head, radiated to the back of the neck, and was associated with photophobia. He denied fever, photophobia, nausea, vomiting, weakness/numbness/tingling of the extremities

Cardiovascular disease in children comprises a diverse collection of diseases involving multiple organ systems. Abnormality in children is predominately congenital but also may be acquired. Although noninvasive vascular imaging modalities such as magnetic resonance angiography and ultrasound lack ionizing radiation, with improving technology and an increased focus on radiation dose reduction, computed tomographic angiography (CTA) continues to have a role in evaluating cardiovascular disease in pediatric patients. This review focuses on specific considerations of CTA that the radiologist or ordering provider should consider when imaging the pediatric cardiovascular system.

Functional MR urography (fMRU) provides comprehensive functional data that can be subject to variability. To interpret the results of fMRU, it is essential to know the intra- and inter-observer variability of the measured parameters. To define the range of variability in fMRU, particularly that of the differential renal function based on volume (volumetric differential renal function) and Patlak differential renal function measurements in children. We included 15 fMRU studies, 10 of non-duplicated and 5 of unilateral duplex kidneys. We recruited six observers with a range of fMRU experience, including two MRI technologists, one resident, one fellow, one pediatric radiologist and one pediatric urologist. The observers underwent intensive training in using the Children’s Hospital of Philadelphia (CHOP)-fMRU freeware for analysis. They conducted the fMRU analysis on each case twice, at least 1 week apart. Mean and standard deviation were calculated for each set of absolute volume, absolute Patlak, volumetric differential renal function and Patlak differential renal function. We calculated the statistical significance of these deviations using the student’s t-test. We also calculated interclass correlations for intra-observer and inter-observer agreement of both volume and Patlak measurements using SPSS software. Intra- and inter-observer variability did not differ significantly, measuring 6% and 4% for relative volume (volumetric differential renal function: P > 0.05) and 5% and 3% for relative function (Patlak differential renal function: P > 0.05). Absolute values of parameters showed more variability than the relative values. Intra- and inter-observer agreement was well above 0.90 (P

Hypoplastic left heart syndrome (HLHS) is the third most common cause of critical congenital heart disease in newborns, and one of the most challenging forms to treat. Secondary pulmonary lymphangiectasia has been recognized in association with HLHS, an appearance described on fetal MRI as the “nutmeg lung.” To investigate the association of fetal nutmeg lung with HLHS survival. A retrospective search of the fetal MRI database was performed. The nutmeg lung pattern was defined as T2 heterogeneous signal with tubular structures radiating peripherally from the hila. Postnatal echocardiograms and charts were reviewed. Forty-four fetal MR studies met inclusion criteria, of which 4 patients (9%) had the nutmeg lung pattern and 3 of whom also had restrictive lesions. Mortality in this nutmeg lung group was 100% by 5 months of age. Of the 40 patients without nutmeg lung, mortality/orthotopic heart transplant (OHT) was 35%. Of these 40 patients without nutmeg lung, 5 had restriction on echo, 3 of whom died/had OHT before 5 months of age (60% of patients with restriction and non-nutmeg lung). There was a significantly higher incidence of restrictive lesions (P = 0.02) and mortality/OHT (P = 0.02) in patients with nutmeg lung compared to those without. The nutmeg lung MR appearance in HLHS fetuses is associated with increased mortality/OHT (100% in the first 5 months of life compared to 35% with HLHS alone). Not all patients with restrictive lesions develop nutmeg lung, and outcome is not as poor when restriction is present in isolation. Dedicated evaluation for nutmeg lung pattern on fetal MR studies may be useful to guide prognostication and aid clinicians in counseling parents of fetuses with HLHS.

Background: There has been a growing recognition of the need for information about objective abnormalities in people with the fibromyalgia syndrome [FMS] and for an integrated approach to its diagn...

Sexual acts performed by a sleeping subject have been rarely reported. Two cases are now presented involving sexual behavior performed while asleep. The first case involves the hitherto unreported association of sleepsex with sleepeating. The second case concerns a rarely reported act of sexual battery by a known sleepwalker, and the use of somnambulism as a legal defense. Sexual behavior in sleep may be pleomorphic and more common than realized in both the patient and normal populations.

The recognition of spinal dysraphism as a potential cause of voiding dysfunction is of the utmost importance if appropriate treatment and followup are to be initiated. We present 2 women with prolonged voiding dysfunction who had spinal dysraphism, intradural lipoma and related urodynamic abnormalities. The importance of initial symptom recognition, physical examination, urodynamic evaluation, appropriate treatment and periodic re-examination to detect progressive neurological impairment is emphasized.

Whipple’s disease is a chronic bacterial infection caused by Tropheryma whipplei. It primarily affects the gastrointestinal (GI) tract but may affect other organs, such as lungs, joints, heart, kidney, central nervous system (CNS), and eyes. Severe abdominal disease leads to malabsorption and is associated with CNS co-morbidities, and can be potentially fatal. Ocular involvement (mainly keratitis, uveitis, and neuro-ophthalmic) occurs in 6–8%. It has a well-known yet unexplained propensity in middle-aged, white males.