Lynn Ansley Fordham

Observer variability assessing US scans of the preterm brain: the ELGAN study

BackgroundNeurosonography can assist clinicians and can provide researchers with documentation of brain lesions. Unfortunately, we know little about the reliability of sonographically derived diagnoses.ObjectiveWe sought to evaluate observer variability among experienced neurosonologists.Materials and methodsWe collected all protocol US scans of 1,450 infants born before the 28th postmenstrual week. Each set of scans was read by two independent sonologists for the presence of intraventricular hemorrhage (IVH) and moderate/severe ventriculomegaly, as well as hyperechoic and hypoechoic lesions in the cerebral white matter. Scans read discordantly for any of these four characteristics were sent to a tie-breaking third sonologist.ResultsVentriculomegaly, hypoechoic lesions and IVH had similar rates of positive agreement (68–76%), negative agreement (92–97%), and kappa values (0.62 to 0.68). Hyperechoic lesions, however, had considerably lower values of positive agreement (48%), negative agreement (84%), and kappa (0.32). No sonologist identified all abnormalities more or less often than his/her peers. Approximately 40% of the time, the tie-breaking reader agreed with the reader who identified IVH, ventriculomegaly, or a hypoechoic lesion in the white matter. Only about 25% of the time did the third party agree with the reader who reported a white matter hyperechoic lesion.ConclusionObtaining concordance seems to be an acceptable way to assure reasonably high-quality of images needed for clinical research.

Safety of contrast-enhanced ultrasound in children for non-cardiac applications: a review by the Society for Pediatric Radiology (SPR) and the International Contrast Ultrasound Society (ICUS)

The practice of contrast-enhanced ultrasound in children is in the setting of off-label use or research. The widespread practice of pediatric contrast-enhanced US is primarily in Europe. There is ongoing effort by the Society for Pediatric Radiology (SPR) and International Contrast Ultrasound Society (ICUS) to push for pediatric contrast-enhanced US in the United States. With this in mind, the main objective of this review is to describe the status of US contrast agent safety in non-cardiac applications in children. The five published studies using pediatric intravenous contrast-enhanced US comprise 110 children. There is no mention of adverse events in these studies. From a European survey 948 children can be added. In that survey six minor adverse events were reported in five children. The intravesical administration of US contrast agents for diagnosis of vesicoureteric reflux entails the use of a bladder catheter. Fifteen studies encompassing 2,951 children have evaluated the safety of intravesical US contrast agents in children. A European survey adds 4,131 children to this group. No adverse events could be attributed to the contrast agent. They were most likely related to the bladder catheterization. The existing data on US contrast agent safety in children are encouraging in promoting the widespread use of contrast-enhanced US.

Early lung disease in young children with primary ciliary dyskinesia

Primary ciliary dyskinesia (PCD) is an autosomal recessive disease in which ciliary dysfunction leads to chronic lung, sinus, and middle ear disease. PCD is often not diagnosed until late childhood due to its presumed rarity and the technical expertise necessary for diagnosis; as such, little is known about lung disease in young children with PCD. We report on 3 young children with PCD who had evidence of lung disease on infant pulmonary function testing, bronchoscopy, and/or computed tomography (CT) of the chest before 3 years of age. Pediatr Pulmonol. 2008; 43:514–516.

Diagnostic Ionizing Radiation Exposure in a Population-Based Sample of Children With Inflammatory Bowel Diseases

OBJECTIVES:The degree of diagnostic radiation exposure in children with inflammatory bowel diseases (IBD) is largely unknown. In this study, we describe this exposure in a population-based sample of children with IBD and determine the characteristics associated with moderate radiation exposure.METHODS:We ascertained radiological study use, demographic characteristics, IBD medication use, and the requirement for hospitalization, emergency department (ED) encounter, or inpatient gastrointestinal surgery among children with IBD within a large insurance claims database. Characteristics associated with moderate radiation exposure (at least one computed tomography (CT) or three fluoroscopies over 2 years) were determined using logistic regression models.RESULTS:We identified 965 children with Crohns disease (CD) and 628 with ulcerative colitis (UC). Over 24 months, 34% of CD subjects and 23% of UC subjects were exposed to moderate diagnostic radiation (odds ratio (OR) 1.71, 95% confidence interval (CI), 1.36 – 2.14). CT accounted for 28% and 25% of all studies in CD and UC subjects, respectively. For CD subjects, moderate radiation exposure was associated with hospitalization (OR 4.89, 95% CI 3.37 – 7.09), surgery (OR 2.93, 95% CI 1.59 – 5.39), ED encounter (OR 2.65, 95% CI 1.93 – 3.64), oral steroids (OR 2.25, 95% CI 1.50 – 3.38), and budesonide (OR 1.80, 95% CI 1.10 – 3.06); an inverse association was seen with immunomodulator use (OR 0.67, 95% CI 0.47 – 0.97). Except for oral steroids and immunomodulators, similar relationships were seen in UC.CONCLUSIONS:A substantial proportion of children with IBD are exposed to moderate amounts of radiation as a result of diagnostic testing. This high utilization may impart long-term risk, given the chronic nature of the disease.

Primary pulmonary lymphangiectasia in infancy and childhood

Primary pulmonary lymphangiectasia (PPL) is a rare disorder of unknown aetiology characterised by dilatation of the pulmonary lymphatics. PPL is widely reported to have a poor prognosis in the neonatal period and little is known about the clinical features of patients who survive the newborn period. The current authors report the outcome in nine patients diagnosed in infancy with PPL over a 15-yr period at a single university-based hospital clinic and followed for a median of 6 yrs. Although all of the patients initially experienced respiratory distress, respiratory symptoms improved in most patients after infancy and were notably better by the age of 6 yrs. Many patients had poor weight gain in the first years of life, which eventually improved. Radiological scans showed progressive resolution of neonatal infiltrates, but were characterised by hyperinflation and increased interstitial markings in older children. Most patients had evidence of bronchitis and grew pathogenic organisms from quantitative bronchoalveolar lavage culture. Pulmonary function tests showed predominantly obstructive disease that did not deteriorate over time. In conclusion, these results suggest that primary pulmonary lymphangiectasia does not have as dismal a prognosis as previously described and symptoms and clinical findings improve after the first year of life.

Magnetic resonance imaging of neuroblastoma using current techniques

We evaluated the ability of current magnetic resonance (MR) scanning techniques to detect and stage neuroblastoma in children, using surgical and histopathologic correlation. We prospectively and retrospectively reviewed 16 MR examinations from 14 patients with neuroblastoma (13 patients) or ganglioneuroblastoma (1 patient) and compared these to computed tomography (CT) (5 patients) and pathology (all patients). Sequences included: precontrast T1-weighted and T2-weighted images, and gadolinium-enhanced T1-weighted images. The study time for each MR exam was also calculated. Five primary tumors were intrathoracic paraspinous masses, eight were adrenal, and 1 was presacral. Neural foraminal invasion was demonstrated on MR in four of 14 patients. Three of the four patients had undergone CT and neural foraminal invasion was shown in one. Vascular encasement was demonstrated in five of 14 patients on MR images. Three of the five patients had undergone CT and vascular involvement was shown in two. All cases of neural foramina invasion and vascular encasement were proven at surgery. There were no false positive or false negative MR studies of neural foraminal invasion or vascular encasement. Bone marrow invasion was shown in two of 14 patients on MR images which were confirmed by bone marrow aspirate. No false negative cases of bone marrow invasion was shown. In one patient, CT considered one neuroblastoma to be adrenal in location which was correctly shown to be intrathoracic on MR. The mean study time for MR imaging was 49 min. Current MR techniques are accurate at detecting and staging neuroblastoma, and coverage of chest, abdomen, and pelvis can be performed in less than one hour.

Lung radiology and pulmonary function of children chronically exposed to air pollution

We analyzed the chest radiographs (CXRs) of 249 clinically healthy children, 230 from southwest Mexico City and 19 from Tlaxcala. In contrast to children from Tlaxcala, children from southwest Mexico City were chronically exposed to ozone levels exceeding the U.S. National Ambient Air Quality Standards for an average of 4.7 hr/day and to concentrations of particulate matter (PM) with aerodynamic diameters ≤2.5 μm (PM2.5) above the annual standard. CXRs of Mexico City children demonstrated bilateral hyperinflation (151 of 230) and increased linear markings (121 of 230). Hyperinflation and interstitial markings were significantly more common in Mexico City children (p < 0.0002 and 0.00006 respectively). Mexico City boys had a higher probability of developing interstitial markings with age (p = 0.004). Computed tomography (CT) scans were obtained in 25 selected Mexico City children with abnormal CXRs. Mild bronchial wall thickening was seen in 10 of 25, prominent central airways in 4 of 25, air trapping in 8 of 21, and pulmonary nodules in 2 of 21. Only 7.8% of Mexico City children had abnormal lung function tests based on predicted values. These findings are consistent with bronchiolar, peribronchiolar, and/or alveolar duct inflammation, possibly caused by ozone, PM, and lipopolysaccharide exposure. The epidemiologic implications of these findings are important for children residing in polluted environments, because bronchiolar disease could lead to chronic pulmonary disease later in life.

Exposure to air pollution is associated with lung hyperinflation in healthy children and adolescents in Southwest Mexico City: a pilot study.

Air pollution produces adverse health effects. The consequences of lifelong daily exposures to atmospheric pollutants upon the respiratory apparatus of healthy children are of considerable clinical importance. We investigated the association between exposure to a highly polluted urban environment with a complex mixture of air pollutants ? ozone and particulate matter the predominant ones ? and chest x-ray abnormalities in 59 healthy Mexican children who are lifelong residents of Southwest Metropolitan Mexico City (SWMMC), with a negative history of tobacco exposure and respiratory illnesses. Their clinical results and x-ray findings were compared to those of 19 Mexican control children, residents of a low-pollution area, with a similar negative history of tobacco exposure and respiratory illnesses. Ozone concentrations in SWMMC exceeded the U.S. Environmental Protection Agency (U.S. EPA) National Ambient Air Quality Standard (NAAQS) for O3:0.08 ppm as 1-h maximal concentration, not to be exceeded more than 4 times a year, on 71% of days in 1986 and 95% in 1997, with values as high as 0.48 ppm. Ozone maximal peaks are usually recorded between 2 and 5 pm coinciding with children?s outdoor physical activities. Children in the control group reported no upper or lower respiratory symptomatology. Every SWMMC child complained of upper and/or lower respiratory symptoms, including epistaxis, nasal dryness and crusting, cough, shortness of breath, and chest discomfort. Children aged 7–13 yr had the most symptomatology, while 5? to 6-year olds and adolescents with the lowest number of statistically significant outdoor exposure hours had less respiratory symptoms. Bilateral symmetric mild lung hyperinflation was significantly associated with exposure to the SWMMC atmosphere (p=.0004). Chronic and sustained inhalation of a complex mixture of air pollutants, including ozone and particulate matter (PM), is associated with lung hyperinflation, suggestive of small airway disease, in a population of clinically healthy children and adolescents. Small airways are a target of air pollutants in SWMMC children, with ozone and PM being most likely responsible, based on experimental animal, controlled-chamber, and epidemiological data available. Our main concern is the potential likelihood for the development of chronic lung disease in this highly exposed population.Air pollution produces adverse health effects. The consequences of lifelong daily exposures to atmospheric pollutants upon the respiratory apparatus of healthy children are of considerable clinical importance. We investigated the association between exposure to a highly polluted urban environment with a complex mixture of air pollutants-ozone and particulate matter the predominant ones-and chest x-ray abnormalities in 59 healthy Mexican children who are lifelong residents of Southwest Metropolitan Mexico City (SWMMC), with a negative history of tobacco exposure and respiratory illnesses. Their clinical results and x-ray findings were compared to those of 19 Mexican control children, residents of a low-pollution area, with a similar negative history of tobacco exposure and respiratory illnesses. Ozone concentrations in SWMMC exceeded the U.S. Environmental Protection Agency (U.S. EPA) National Ambient Air Quality Standard (NAAQS) for O(3): 0.08 ppm as 1-h maximal concentration, not to be exceeded more than 4 times a year, on 71% of days in 1986 and 95% in 1997, with values as high as 0.48 ppm. Ozone maximal peaks are usually recorded between 2 and 5 pm coinciding with childrens outdoor physical activities. Children in the control group reported no upper or lower respiratory symptomatology. Every SWMMC child complained of upper and/or lower respiratory symptoms, including epistaxis, nasal dryness and crusting, cough, shortness of breath, and chest discomfort. Children aged 7-13 yr had the most symptomatology, while 5- to 6-year olds and adolescents with the lowest number of statistically significant outdoor exposure hours had less respiratory symptoms. Bilateral symmetric mild lung hyperinflation was significantly associated with exposure to the SWMMC atmosphere (p = .0004). Chronic and sustained inhalation of a complex mixture of air pollutants, including ozone and particulate matter (PM), is associated with lung hyperinflation, suggestive of small airway disease, in a population of clinically healthy children and adolescents. Small airways are a target of air pollutants in SWMMC children, with ozone and PM being most likely responsible, based on experimental animal, controlled-chamber, and epidemiological data available. Our main concern is the potential likelihood for the development of chronic lung disease in this highly exposed population.

ACR Appropriateness Criteria ® on Developmental Dysplasia of the Hip—Child

Developmental dysplasia of the hip (DDH) affects 1.5 of every 1,000 caucasian Americans and less frequently affects African Americans. Developmental dysplasia of the hip comprises a spectrum of abnormalities, ranging from laxity of the joint and mild subluxation to fixed dislocation. Early diagnosis of DDH usually leads to low-risk treatment with a harness. Late diagnosis of DDH in children may lead to increased surgical intervention and complications. Late diagnosis of DDH in adults can result in debilitating end-stage degenerative hip joint disease. Screening decreases the incidence of late diagnosis of DDH. Clinical evaluation for DDH should be performed periodically at each well-baby visit until the age of 12 months. There is no consensus on imaging screening for DDH. Consideration for screening with ultrasound is balanced between the benefits of early detection of DDH and the increased treatment and cost factors. In addition, randomized trials evaluating primary ultrasound screening did not find significant decrease in late diagnosis of DDH. In the United States, hip ultrasound is selectively performed in infants with risk factors, such as family history of DDH, breech presentation, and inconclusive findings on physical examination. Ultrasound for DDH should be performed after 2 weeks of age because laxity is common after birth and often resolves itself. A pelvic radiograph can optimally be performed after the age of 4 months, when most infants will have ossification centers of the femoral heads.

The Fetal Dandy Walker Complex: Associated Anomalies, Perinatal Outcome and Postnatal Imaging

Objective: This study compares the diagnostic accuracy of prenatal ultrasound with postnatal imaging and evaluates for associated fetal anomalies and their impact on immediate neonatal outcome in fetal Dandy Walker complex (DWC). Methods: Cases of fetal DWC diagnosed in a single ultrasound unit from January 2000 through July 2004 were reviewed for associated fetal anomalies, fetal karyotype, immediate neonatal outcome and postnatal head imaging. Results: A total of 55 fetuses with DWC were identified. Of liveborn cases, postnatal imaging confirmed prenatal intracranial findings in 50% of Dandy Walker variant (DWV) and 100% of Dandy Walker malformation (DWM), with additional central nervous system findings noted in 21% of all cases. Additional fetal anomalies were seen in 26/40 (65%) cases of DWV and 15/15 (100%) cases of DWM. Immediate neonatal survival was predicted by karyotype and associated fetal anomalies. Conclusion: The sonographic diagnosis of fetal DWM is accurate. Significant discrepancies exist in prenatal and postnatal diagnosis of DWV. Comprehensive fetal ultrasound and karyotype should be offered for all fetuses with DWC. Postnatal imaging should be performed on all fetal DWC.