Robben, S.G.F.

The lumbosacral angle does not reflect progressive tethered cord syndrome in children with spinal dysraphism (Article)

2010-12-01T00:00:00Z

Purpose: Our goal was to validate the hypothesis that the lumbosacral angle (LSA) increases in children with spinal dysraphism who present with progressive symptoms and signs of tethered cord syndrome (TCS), and if so, to determine for which different types and/or levels the LSA would be a valid indicator of progressive TCS. Moreover, we studied the influence of surgical untethering and eventual retethering on the LSA. Methods: We retrospectively analyzed the data of 33 children with spinal dysraphism and 33 controls with medulloblastoma. We measured the LSA at different moments during follow-up and correlated this with progression in symptomatology. Results: LSA measurements had an acceptable intra- and interobserver variability, however, some children with severe deformity of the caudal part of the spinal column, and for obvious reasons those with caudal regression syndrome were excluded. LSA measurements in children with spinal dysraphism were significantly different from the control group (mean LSA change, 21.0° and 3.1° respectively). However, both groups were not age-matched, and when dividing both groups into comparable age categories, we no longer observed a significant difference. Moreover, we did not observe a significant difference between 26 children with progressive TCS as opposed to seven children with stable TCS (mean LSA change, 20.6° and 22.4° respectively). Conclusions: We did not observe significant differences in LSA measurements for children with clinically progressive TCS as opposed to clinically stable TCS. Therefore, the LSA does not help the clinician to determine if there is significant spinal cord tethering, nor if surgical untethering is needed.

Structural lung changes, lung function, and non-invasive inflammatory markers in cystic fibrosis (Article)

2010-05-01T00:00:00Z

Cystic fibrosis (CF) lung disease is characterized by chronic airway inflammation and recurrent infections, resulting in (ir)reversible structural lung changes and a progressive decline in lung function. The objective of this study was to investigate the relationship between non-invasive inflammatory markers (IM) in exhaled breath condensate (EBC), lung function indices and structural lung changes, visualized by high resolution computed tomography (HRCT) scans in CF. In 34 CF patients, lung function indices (forced expiratory volume in 1 s, forced vital capacity [FVC], residual volume, and total lung capacity [TLC]) and non-invasive IM (exhaled nitric oxide, and condensate acidity, nitrate, nitrite, 8-isoprostane, hydrogen peroxide, interferon-gamma) were assessed. HRCT scans were scored in a standardized and validated way, a composite score and component scores were calculated. In general, the correlations between non-invasive IM and structural lung changes, and between IM and lung function were low (correlation coefficients <0.40). Patients with positive sputum Pseudomonas cultures had higher EBC nitrite levels and higher parenchymal HRCT subscores than patients with Pseudomonas-negative cultures (p < 0.05). Multiple linear regression models demonstrated that FVC was significantly predicted by hydrogen peroxide in EBC, and the scores of bronchiectasis and mosaic perfusion (Pearson correlation coefficient R = 0.78, p < 0.001). TLC was significantly predicted by 8-isoprostane, nitrate, hydrogen peroxide in EBC, and the mucous plugging subscore (R = 0.92, p < 0.01). Static and dynamic lung function indices in this CF group were predicted by the combination of non-invasive IM in EBC and structural lung changes on HRCT imaging. Future longitudinal studies should reveal whether non-invasive monitoring of airway inflammation in CF adds to better follow-up of patients.

Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements. (Article)

2004-05-01T00:00:00Z

PURPOSE: To retrospectively compare thin-section computed tomographic (CT) scores obtained with five scoring systems for assessment of pulmonary disease in children with cystic fibrosis and to determine additional value of bronchial and arterial dimension measurements. MATERIALS AND METHODS: Scores obtained with five thin-section CT scoring systems were compared. A score of 0 indicated the absence of abnormalities; a higher score meant that more structural abnormalities were seen. Three observers assigned scores and then reassigned scores after intervals varying from 1-2 weeks to 1-2 months at review of thin-section CT scans obtained in 25 children with cystic fibrosis. Interobserver and intraobserver reliability was calculated with intraclass correlation coefficients. Quantitative measurements of bronchial and arterial dimensions were obtained. Thin-section CT scores were correlated (Spearman correlation) with bronchial and arterial dimensions and with results of pulmonary function tests (PFTs), such as forced expiratory volume in 1 second (FEV(1)). RESULTS: Scores with all five scoring systems were reproducible, with intraclass correlation coefficients of 0.74 and higher (P <.05), and showed significant correlations with FEV(1) (R = -0.73 to -0.69, P <.01). Ratio of bronchial diameter to accompanying pulmonary arterial diameter was correlated with thin-section CT scores but not with FEV(1). Ratio of bronchial wall thickness to accompanying pulmonary arterial diameter was not correlated with thin-section CT scores or PFT results. CONCLUSION: Thin-section CT scores were reproducible and were correlated with PFT results. Measurements of bronchial dimensions were not significantly related to scores or PFT results.

Changes in globus pallidus with (pre)term kernicterus (Article)

2003-01-01T00:00:00Z

OBJECTIVE: We report serial magnetic resonance (MR) and sonographic behavior of globus pallidus in 5 preterm and 3 term infants with kernicterus and describe the clinical context in very low birth weight preterm infants. On the basis of this information, we suggest means of diagnosis and prevention. METHODS: Charts and MR and ultrasound images of 5 preterm infants and 3 term infants with suspected bilirubin-associated brain damage were reviewed. Included were preterm infants with severe hearing loss, quadriplegic hypertonia, and abnormal hypersignal of globus pallidus on T2-weighted MR imaging (MRI). In 1 infant who died on day 150, the diagnosis was confirmed during the neonatal period. The others were picked up as outpatients and scanned at 12 or 22 months' corrected age. Three instances of term kernicterus were included for comparison of serial MRI in the neonatal period and early infancy: they were caused by glucose-6-phosphate dehydrogenase deficiency, urosepsis, and dehydration plus fructose 1-6 biphosphatase deficiency. RESULTS: Five preterm infants of 25 to 29 weeks' gestational age presented with total serum bilirubin (TSB) levels below exchange transfusion thresholds commonly advised. Mixed acidosis was present in 3 infants around the TSB peak. The bilirubin/albumin molar ratio was >0.5 in all, in the absence of displacing drugs. All failed to pass bedside hearing screen tests and had severe hearing loss on auditory brain response testing. Symmetrical homogeneous hyperechogenicity of globus pallidus was the alerting feature in 1 infant. Globus pallidus was hyperintense on T1-weighted MR images in this child. The other infants presented with severe developmental delay as a result of dyskinetic quadriplegia and hearing loss. Globus pallidus was normal on T1- but hyperintense on T2-weighted MR images at 12 or 22 months' corrected age. Subthalamic involvement was documented in coronal fluid attenuated inversion recovery MRI in 2 infants. The term infants with classical clinical presentation in the neonatal period had MR behavior similar to the preterms, but pallidal injury was not recognized with targeted sonographic examination. Their neonatal MR images demonstrated pallidal T1 hyperintensity and mild T2 hyperintensity. CONCLUSION: Acidotic very low birth weight preterm infants with low serum albumin levels develop MR-confirmed pallidal injury and hearing loss facing "accepted" TSB levels. Serial MRI documents a shift from acute mainly T1 hypersignal to permanent T2 hypersignal in globus pallidus within the late neonatal period. Subthalamic and not thalamic involvement helps to differentiate from ischemic or metabolic disorder. As newborns, these infants are rigid and have severe apnea, before developing hypertonic quadriplegia in infancy.

Prenatal prediction of pulmonary hypoplasia: clinical, biometric, and Doppler velocity correlates (Article)

2002-01-01T00:00:00Z

OBJECTIVES: To determine the value of pulmonary artery Doppler velocimetry relative to fetal biometric indices and clinical correlates in the prenatal prediction of lethal lung hypoplasia (LH) in prolonged (>1 week) oligohydramnios. METHODS: Forty-two singleton pregnancies with oligohydramnios associated with premature rupture of membranes ([PROM]; n = 31) or bilateral renal pathology (n = 11) were examined using color-coded Doppler ultrasound in a cross-sectional study design. Mean gestational age was 28.0 +/- 4.3 weeks (range: 20-36 weeks). Thoracic, cardiac, and abdominal circumference and the largest vertical amniotic fluid pocket were measured. Pulsed Doppler measurements of the arterial pulmonary branches were made at the level of the cardiac 4-chamber view. Diagnosis of LH was based on clinical, radiologic, and pathologic criteria. Clinicians were blinded to the prenatal measurements. RESULTS: The prevalence of lethal LH was 43%. In the PROM subset, combination of onset of PROM at or =8 weeks, and degree of oligohydramnios at < or =1 cm presented the highest clinical prediction rate for lethal LH. For both the total group and the PROM subset, the highest prediction rate for lethal LH was presented by thoracic circumference/abdominal circumference ratio, peak systolic velocity in the proximal branch, and time-averaged and end-diastolic velocity in the middle branch of the pulmonary artery. In the PROM subset, the combination of all 3 clinical, biometric, and Doppler parameters revealed the most favorable combination to predict lethal LH (positive predictive value: 100%; accuracy: 93%; and sensitivity: 71%). CONCLUSION: Doppler velocimetry may detect changes in pulmonary artery waveforms in the presence of LH but fails to be the ultimate test for the prenatal prediction of lethal LH. The best prediction can be achieved by combining clinical, biometric, and Doppler parameters.

Ultrasonography of the painful hip in childhood (Doctoral Thesis)

1999-06-16T00:00:00Z

There are many diseases in childhood that affect the hip joint. Some diseases are systemic in origin and initially may present themselves as hip disorders, such as rheumatoid arthritis. other diseases are localized specifically in the hip joint, such as transient synovitis and Perthes' disease, mostly unilateral. Neoplastic or infectious diseases around the hip joint may also manifest themselves as a painful hip. If both hips are affected the differential diagnosis should include skeletal dysplasias (multiple epiphyseal dysplasia) and metabolic diseases (hypothyroidism, Gaucher's disease, mucopolysaccharidoses and mucolipidoses).

Anterior joint capsule of the normal hip and in children with transient synovitis: US study with anatomic and histologic correlation (Article)

1999-01-01T00:00:00Z

PURPOSE: To study the anatomic components of the anterior joint capsule of the normal hip and in children with transient synovitis. MATERIALS AND METHODS: Six cadaveric specimens were imaged with ultrasonography (US) with special attention to the anterior joint capsule. Subsequently, two specimens were analyzed histologically. These anatomic findings were correlated with the US findings in 58 healthy children and 105 children with unilateral transient synovitis. RESULTS: The anterior joint capsule comprises an anterior and posterior layer, mainly composed of fibrous tissue, lined by only a minute synovial membrane. Both fibrous layers were identified separately at US in 98 of 116 (84%) hips of healthy subjects and in all hips with transient synovitis. Overall, the anterior layer was thicker than the posterior layer. In transient synovitis compared with normal hips, no significant thickening of both layers was present (P = .24 and .57 for the anterior and posterior layers, respectively). Normal variants include plicae, local thickening of the capsule, and pseudodiverticula. CONCLUSION: Increased thickness of the anterior joint capsule in transient synovitis is caused entirely by effusion. There is no US evidence for additional capsule swelling or synovial hypertrophy.