Establishing a standard protocol for the voiding cystourethrography.

The voiding cystourethrogram (VCUG) is a frequently performed test to diagnose a variety of urologic conditions, such as vesicoureteral reflux (VUR). The test results determine whether continued observation or an interventional procedure is indicated. VCUGs are ordered by many specialists and primary care providers, including pediatricians, family practitioners, nephrologists, hospitalists, emergency room physicians, and urologists. Current protocols for performing and interpreting a VCUG are based on the International Reflux Study in 1985. However, more recent information provided by many national and international institutions suggests a need to refine those recommendations. The lead author of the 1985 study, R.L. Lebowitz, agreed to and participated in the current protocol. In addition, a recent survey directed to the chairpersons of pediatric radiology of 65 children's hospitals throughout the United States and Canada showed that VCUG protocols vary substantially. Recent guidelines from the American Academy of Pediatrics (AAP) recommend a VCUG for children between 2 and 24 months of age with urinary tract infections but did not specify how this test should be performed. To improve patient safety and to standardize the data obtained when a VCUG is performed, the AAP Section on Radiology and the AAP Section on Urology initiated the current VCUG protocol to create a consensus on how to perform this test.

Multi-institutional Study Comparing the Height of Contrast During Performance of Voiding Cystourethrogram in Children.

OBJECTIVE: To assess the effect of contrast height during the voiding cystourethrogram (VCUG). The VCUG is the gold standard diagnostic test for vesicoureteral reflux (VUR). Variation in parameters may affect detection and grade of reflux. MATERIALS AND METHODS: In a multicenter, prospective, nonrandomized, observational study, patients undergoing VCUG were selected. VCUG was performed per study protocol except for a change in contrast height. The initial fill was performed at 50 cm and the second at 100 cm. Data collected included presence and grade of VUR and volume filled. The actual bladder volume filled was normalized to the estimated bladder capacity (EBC) as a percentage. A Cohen's kappa coefficient of agreement was used to test for difference in the incidence of reflux and grade between contrast heights. A Wilcoxon signed-rank test was used for differences in the percent EBC filled between heights. RESULTS: From May 2012 to November 2013, 184 patients were enrolled. Seventy-one patients (39%) exhibited VUR at 50 cm and 80 patients (43%) at 100 cm. The kappa coefficient of agreement between 50 cm and 100 cm showed substantial agreement, with no significant difference in VUR grade. The percent of EBC filled at each height was significantly different: %EBC filled at 50 cm: 101 ± 46 (range 9.2-228.3), and %EBC filled at 100 cm: 130 ± 56 (range 37.8-280.6) (P < .0001). CONCLUSION: No significant difference was noted in the detection of VUR with different contrast heights. A significantly larger bladder volume was instilled at 100 cm.

Changes in cardiac function and cerebral blood flow in relation to peri/intraventricular hemorrhage in extremely preterm infants.

OBJECTIVE: To investigate whether changes in cardiac function and cerebral blood flow (CBF) precede the occurrence of peri/intraventricular hemorrhage (P/IVH) in extremely preterm infants. STUDY DESIGN: In this prospective observational study, 22 preterm infants (gestational age 25.9 ± 1.2 weeks; range 23-27 weeks) were monitored between 4 and 76 hours after birth. Cardiac function and changes in CBF and P/IVH were assessed by ultrasound every 12 hours. Changes in CBF were also followed by continuous monitoring of cerebral regional oxygen saturation (rSO2) and by calculating cerebral fractional oxygen extraction. RESULTS: Five patients developed P/IVH (1 patient grade II and 4 patients grade IV). Whereas measures of cardiac function and CBF remained unchanged in neonates without P/IVH, patients with P/IVH tended to have lower left ventricular output and had lower left ventricle stroke volume and cerebral rSO2 and higher cerebral fractional oxygen extraction during the first 12 hours of the study. By 28 hours, these variables were similar in the 2 groups and myocardial performance index was lower and middle cerebral artery mean flow velocity higher in the P/IVH group. P/IVH was detected after these changes occurred. CONCLUSIONS: Cardiac function and CBF remain stable in very preterm neonates who do not develop P/IVH during the first 3 postnatal days. In very preterm neonates developing P/IVH during this period, lower systemic perfusion and CBF followed by an increase in these variables precede the development of P/IVH. Monitoring cardiac function and cerebral rSO2 may identify infants at higher risk for developing P/IVH before the bleeding occurs.

Voiding cystourethrogram--are our protocols the same?

PURPOSE: Voiding cystourethrogram is the gold standard for evaluating and diagnosing vesicoureteral reflux. Reflux detection can potentially be affected by many parameters during voiding cystourethrogram. MATERIALS AND METHODS: A 29-item survey was sent via e-mail through SurveyMonkey® to the chairperson of pediatric radiology at 65 national pediatric hospitals. This survey included questions on institutional protocols for performing voiding cystourethrogram. RESULTS: Responses were received from 41 institutions from across North America, including 17 of 19 Randomized Intervention for Children with Vesicoureteral Reflux study sites. Many aspects of the reports of voiding cystourethrogram protocols were similar with 90% or greater agreement in allowing parents in the room, contrast infusion by gravity, catheter or feeding tube use without balloons, no contrast dilution and voiding without a catheter in place. The height at which contrast medium was raised for infusion was 40, 60, 80, 100 and greater than 100 cm at 2.4%, 17.1%, 17.1%, 39.0% and 12.2% of sites, respectively, while the height was not measured or it varied at 12.2%. The infilling phase stopped when the bladder appeared full at 2.4% of sites, infusion stopped itself at 12.2%, patient voided at 61.0%, volume attained age expected capacity at 12.2%, the patient was uncomfortable at 4.9% and results varied at 7.3%. CONCLUSIONS: Data reveal that voiding cystourethrogram is performed differently across North America and no standard protocol exists for the procedure. These differences could significantly impact voiding cystourethrogram results among institutions and taint our ability to compare results in the literature.

Sonographic evaluation of pediatric localized scleroderma: preliminary disease assessment measures.

BACKGROUND: Our earlier work in the ultrasonograpy of localized scleroderma (LS) suggests that altered levels of echogenicity and vascularity can be associated with disease activity. Utrasound is clinically benign and readily available, but can be limited by operator dependence. We present our efforts to standardize image acquisition and interpretation of pediatric LS to better evaluate the correlation between specific sonographic findings and disease activity. METHODS: Several meetings have been held among our multi-center group (LOCUS) to work towards standardizing sonographic technique and image interpretation. Demonstration and experience in image acquisition were conducted at workshop meetings. Following meetings in 2007, an ultrasound measure was developed to standardize evaluation of differences in echogenicity and vascularity. Based upon our initial observations, we have labeled this an ultrasound disease activity measure. This preliminary measure was subsequently evaluated on over 180 scans of pediatric LS lesions. This review suggested that scoring levels should be expanded to better capture the range of observed differences. The revised levels and their definitions were formulated at a February 2009 workshop meeting. We have also developed assessments for scoring changes in tissue thickness and lesion size to better determine if these parameters aid evaluation of disease state. RESULTS: We have standardized our protocol for acquiring ultrasound images of pediatric LS lesions. A wide range of sonographic differences has been seen in the dermis, hypodermis, and deep tissue layers of active lesions. Preliminary ultrasound assessments have been generated. The disease activity measure scores for altered levels of echogenicity and vascularity in the lesion, and other assessments score for differences in lesion tissue layer thickness and changes in lesion size. CONCLUSIONS: We describe the range of sonographic differences found in pediatric LS, and present our efforts to standardize ultrasound acquisition and image interpretation for this disease. We present ultrasound measures that may aid evaluation of disease state. These assessments should be considered a work in progress, whose purpose is to facilitate further study in this area. More studies are needed to assess their validity and reliability.

Management of urachal remnants in early childhood.

PURPOSE: Generally, it is recommended that all urachal remnants be excised to avoid recurrent disease and possible malignant transformation later in life. However, spontaneous resolution with no need for further intervention has been reported. We reviewed the experience with urachal remnants at a single institution and evaluated which patients could be treated nonoperatively and which required surgical intervention. MATERIALS AND METHODS: We reviewed the medical records and radiographic studies of all patients with urachal remnants from January 1999 to January 2007. Patients were analyzed according to initial presentation, imaging findings and treatment. Serial clinical examinations and radiographic imaging were used to follow patients. RESULTS: An external urachal sinus was found in 9 patients (39.1%) and 1 was surgically excised. Of the 12 urachal cysts (52.2%) 9 ultimately required surgical excision, of which 6 were infected initially. A patent urachus was found in 2 patients, which resolved during an observation period. Radiographic imaging and/or physical examination diagnosed all remnants initially and confirmed complete resolution during followup. Eight of the 10 urachal remnants (80.0%) that resolved developed in patients younger than 6 months. Various accompanying urogenital anomalies were found in 8 patients (34.8%). CONCLUSIONS: A small urachal remnant, especially at birth, may be viewed as physiological. Urachal remnants in patients younger than 6 months are likely to resolve with nonoperative management. However, if symptoms persist or the urachal remnant fails to resolve after 6 months of age, it should be excised to prevent recurrent infections.

A favorable outcome following 32 vesicocentesis and amnioinfusion procedures in a fetus with severe prune belly syndrome.

Patients with severe prune belly syndrome rarely survive beyond the first days of life. We present a case of prune belly syndrome that initially presented with severe oligohydramnios, megacystis and associated poor urine biochemistries. Due to an anteriorly located placenta the patient was referred to three major centers, but was turned down because of the unfavorable prognostic findings. Therefore, fetal intervention was performed with 32 vesicocentesis and amnioinfusion procedures. Despite the unfavorable prenatal findings, and having undergone numerous fetal interventions, the birth resulted in a viable infant.

De novo three-way chromosome translocation 46,XY,t(4;6;21)(p16;p21.1;q21) in a male with cleidocranial dysplasia.

Cleidocranial dysplasia (CCD) is an autosomal dominant skeletal dysplasia associated with cranial, clavicular, and dental anomalies. It is caused by mutations in the RUNX2 gene, which encodes an osteoblast-specific transcription factor and maps to chromosome 6p21. We report clinical and molecular cytogenetic studies in a patient with clinical features of CCD including wormian bones, delayed fontanel closure, hypoplastic clavicles and pubic rami, and supernumerary dentition. Additional abnormalities of bone growth and connective tissue, including easy bruisability, scarring, bleeding, joint hypermobility, and developmental delay were also observed. Molecular cytogenetic studies identified a de novo apparently balanced three-way translocation 46,XY,t(4;6;21)(p16;p21.1;q21). Further mapping revealed the breakpoint on 6p21 to be approximately 50 kb upstream of exon 1 of the RUNX2 gene, with RUNX2 being intact on the derivative chromosome 6. We hypothesize that the proband's CCD has arisen from disruption of the developmentally regulated gene RUNX2 at the 6p21 breakpoint, due to a position effect mutation which may have altered the expression of the gene. Further studies might unravel a new regulatory element for RUNX2.

Inguinal herniation of the bladder in an infant.

Inguinal herniation of the bladder is an uncommon finding with fewer than 200 cases reported in the literature. It is found most commonly in older, obese men with lower urinary tract symptoms. We report a case of inguinal herniation of the bladder in a premature infant.

Qualitative assessment of cervical spinal stenosis: observer variability on CT and MR images.

BACKGROUND AND PURPOSE: Several studies have been undertaken to validate quantitative methods of evaluating cervical spinal stenosis. This study was performed to assess the degree of interobserver and intraobserver agreement in the qualitative evaluation of cervical spinal stenosis on CT myelograms and MR images. METHODS: Cervical MR images and CT myelograms of 38 patients were evaluated retrospectively. Six neuroradiologists with various backgrounds and training independently assessed the level, degree, and cause of stenosis on either MR images or CT myelograms. Unknown to the evaluators, 16 of the patients were evaluated twice to determine intraobserver variability. RESULTS: Interobserver agreement among the radiologists with regard to level, degree, and cause of stenosis on CT myelograms showed kappa values of 0.50, 0.26, and 0.32, respectively, and on MR images showed kappa values of 0.60, 0.31, and 0.22, respectively. Intraobserver agreement with regard to level, degree, and cause of stenosis on CT myelograms showed mean kappa values of 0.69, 0.41, and 0.55, respectively, and on MR images showed mean kappa values of 0.80, 0.37, and 0.40, respectively. CONCLUSION: MR imaging and CT myelographic evaluation of cervical spinal stenosis by using current qualitative methods results in significant variation in image interpretation.