Outcome after prenatal diagnosis of congenital anomalies of the kidney and urinary tract.

UNLABELLED: Congenital anomalies of the kidney and urinary tract are common findings on fetal ultrasound. The aim of this prospective observational study was to describe outcome and risk factors in 115 patients born 1995-2001. All prenatally diagnosed children were stratified into low- and high-risk group and followed postnatally clinically and by imaging at defined endpoints. Risk factors were evaluated using odds ratios. Neonatal diagnosis included pelvi-ureteric junction obstruction (n = 33), vesicoureteral reflux (n = 27), solitary mild pelvic dilatation (postnatal anteroposterior diameter 5-10 mm; n = 25), and further diagnosis as primary obstructive megaureter, unilateral multicystic dysplastic kidney, renal dysplasia and posterior urethral valves. In 38 children with prenatal isolated hydronephrosis, ultrasound normalized at median age of 1.2 years (range 0.1-9). Surgery was performed in 34 children at median age of 0.4 years (0.1-10.8). Persistent renal anomalies without surgery were present in 43 children and followed in 36 for median time of 16 years (12.2-18). Oligohydramnios and postnatal bilateral anomalies were significantly associated with surgery and impaired renal function. CONCLUSION: The majority of children had a favourable postnatal outcome, in particular children with prenatally low risk, i.e. isolated uni- or bilateral hydronephrosis. Oligohydramnios and postnatal bilateral anomalies were risk factors for non-favourable outcome. WHAT IS KNOWN: • In congenital anomalies of the kidney and urinary tract significantly poorer outcome is known in patients with bilateral renal hypoplasia or solitary kidney associated with posterior urethral valves. • Other factors as proteinuria and vesicoureteral reflux were associated with a higher risk of progression to chronic renal failure in these patients. What is New: • Unlike other studies giving us above-mentioned information, we included all patients with any kind of prenatally diagnosed congenital anomalies of the kidney and urinary tract. Our study shows long-term follow up (median 16 years, range 12.2-18 years), especially in patients not needing surgery, but with persistent anomalies. • During postnatal long-term follow up (median 2.2 years, range 0.1-18 years) one third each showed normalization, need of surgery or persistence of anomalies without need of surgery. Our study revealed a good prognosis in the majority of these children, in particular with prenatally low risk, i.e. isolated uni- or bilateral hydronephrosis, and revealed oligohydramnios and postnatal bilateral anomalies as risk factors for a non-favourable outcome, defined as need of surgery, persistent anomalies with impaired renal function, end stage renal failure or death.

ESPR Uroradiology Taskforce--imaging recommendations in paediatric uroradiology, part VIII: retrograde urethrography, imaging disorder of sexual development and imaging childhood testicular torsion.

Three new consensus-based recommendations of the European Society of Paediatric Radiology Uroradiology Taskforce and the European Society of Urogenital Radiology Paediatric Working Group on paediatric uroradiology are presented. One deals with indications and technique for retrograde urethrography, one with imaging in the work-up for disorders of sexual development and one with imaging workflow in suspected testicular torsion. The latter is subdivided to suggest a distinct algorithm to deal with testicular torsion in neonates. These proposals aim to outline effective imaging algorithms to optimise diagnostic accuracy and to harmonize diagnostic imaging among institutions and practitioners.

ESPR uroradiology task force and ESUR Paediatric Work Group--Imaging recommendations in paediatric uroradiology, part VI: childhood renal biopsy and imaging of neonatal and infant genital tract. Minutes from the task force session at the annual ESPR Meeting 2012 in Athens on childhood renal biopsy and imaging neonatal genitalia.

The European Society of Paediatric Radiology Uroradiology Task Force and the ESUR Paediatric Work Group jointly publish guidelines for paediatric urogenital imaging. Two yet unaddressed topics involving patient safety and imaging load are addressed in this paper: renal biopsy in childhood and imaging of the neonatal genital tract, particularly in girls. Based on our thorough review of literature and variable practice in multiple centers, procedural recommendations are proposed on how to perform renal biopsy in children and how to approach the genital tract in (female) neonates. These are statements by consensus due to lack of sufficient evidence-based data. The procedural recommendation on renal biopsy in childhood aims at improving patient safety and reducing the number of unsuccessful passes and/or biopsy-related complications. The recommendation for an imaging algorithm in the assessment of the neonatal genital tract focuses on the potential of ultrasonography to reduce the need for more invasive or radiating imaging, however, with additional fluoroscopy or MRI to be used in selected cases. Adherence to these recommendations will allow comparable data and evidence to be generated for future adaptation of imaging strategies in paediatric uroradiology.

ESPR uroradiology task force imaging recommendations in paediatric uroradiology, part VII: standardised terminology, impact of existing recommendations, and update on contrast-enhanced ultrasound of the paediatric urogenital tract.

Our purpose is to harmonise and standardise terminology in paediatric uroradiology, to provide and update recommendations for contrast-enhanced US to standardise imaging and encourage further research, and to assess the impact of the existing recommendations in paediatric urogenital imaging. Based on thorough review of literature and variable practice at several centres and after discussion within urogenital imaging groups as well as with other subspecialties, we propose a standardisation of terminology in urogenital imaging. An update with recommendations on paediatric contrast-enhanced US has been issued based on available literature and reports. Finally, a questionnaire has been used to assess the knowledge, applicability and usefulness of, and the adherence to existing recommendations of the European Society of Paediatric Radiology (ESPR) Uroradiology Task Force. In conclusion, the ESPR is working to improve patient safety and optimise paediatric urogenital imaging. Standardisation of terminology and provision of updated knowledge on contrast-enhanced US in childhood will contribute to this task, ideally reducing the need for invasive or radiating imaging. Not all existing recommendations are commonly known, which limits adherence to these recommendations and the availability of comparable data and evidence for future adaptation of imaging strategies in paediatric uroradiology.

ESPR uroradiology task force and ESUR paediatric working group: imaging and procedural recommendations in paediatric uroradiology, part III. Minutes of the ESPR uroradiology task force minisymposium on intravenous urography, uro-CT and MR-urography in childhood.

The ESPR working groups have addressed intravenous urography (IVU), uro-CT and MR-urography (MRU) in childhood as the last in the series of recommendations for paediatric uroradiology. The aim of this process was again to standardise paediatric uroradiologic imaging and to reduce invasiveness and radiation dose. As for the existing recommendations, the new proposals are consensus-based because evidence is lacking, use and indications have changed, or approaches on how to perform the examination in children differ in the literature (MRU). As in the previous recommendations, a thorough review of the literature and existing guidelines and recommendations has been performed. The proposals were discussed within the group and with non-member experts and colleagues from other partner disciplines. These recommendations aim to serve as a quality measure in order to standardise the procedures and thus grant comparable good quality results throughout different institutions.

Randomised trial of oral versus sequential intravenous/oral cephalosporins in children with pyelonephritis.

The hypothesis was tested that oral antibiotic treatment in children with acute pyelonephritis and scintigraphy-documented lesions is equally as efficacious as sequential intravenous/oral therapy with respect to the incidence of renal scarring. A randomised multi-centre trial was conducted in 365 children aged 6 months to 16 years with bacterial growth in cultures from urine collected by catheter. The children were assigned to receive either oral ceftibuten (9 mg/kg once daily) for 14 days or intravenous ceftriaxone (50 mg/kg once daily) for 3 days followed by oral ceftibuten for 11 days. Only patients with lesions detected on acute-phase dimercaptosuccinic acid (DMSA) scintigraphy underwent follow-up scintigraphy. Efficacy was evaluated by the rate of renal scarring after 6 months on follow-up scintigraphy. Of 219 children with lesions on acute-phase scintigraphy, 152 completed the study; 80 (72 females, median age 2.2 years) were given ceftibuten and 72 (62 females, median age 1.6 years) were given ceftriaxone/ceftibuten. Patients in the intravenous/oral group had significantly higher C-reactive protein (CRP) concentrations at baseline and larger lesion(s) on acute-phase scintigraphy. Follow-up scintigraphy showed renal scarring in 21/80 children treated with ceftibuten and 33/72 with ceftriaxone/ceftibuten (p = 0.01). However, after adjustment for the confounding variables (CRP and size of acute-phase lesion), no significant difference was observed for renal scarring between the two groups (p = 0.2). Renal scarring correlated with the extent of the acute-phase lesion (r = 0.60, p < 0.0001) and the grade of vesico-ureteric reflux (r = 0.31, p = 0.03), and was more frequent in refluxing renal units (p = 0.04). The majority of patients, i.e. 44 in the oral group and 47 in the intravenous/oral group, were managed as out-patients. Side effects were not observed. From this study, we can conclude that once-daily oral ceftibuten for 14 days yielded comparable results to sequential ceftriaxone/ceftibuten treatment in children aged 6 months to 16 years with DMSA-documented acute pyelonephritis and it allowed out-patient management in the majority of these children.

Imaging recommendations in paediatric uroradiology: minutes of the ESPR workgroup session on urinary tract infection, fetal hydronephrosis, urinary tract ultrasonography and voiding cystourethrography, Barcelona, Spain, June 2007.

We present here a few basic proposals for algorithms and procedures for imaging the paediatric genitourinary tract based on initial discussion at a paediatric uroradiology symposium and proposals of the ESUR Paediatric Uroradiologic Guidelines Subcommittee. These recommendations were developed in the light of new knowledge that might influence existing guidelines. Regional, individual and local flexibility and variability should be preserved in order to make these recommendations applicable throughout Europe. They should help standardize dedicated imaging not only in terms of a quality measure to ensure state-of-the-art patient care, but also in forming a common basis for multi-institutional research. There is an urgent need for these guidelines in order to advance our understanding of the subject and to gain evidence and improve imaging efficacy. Our session worked towards establishing an agreement on imaging indications in common paediatric urological conditions, respecting the ALARA principle, and patient safety and care, and taking into account state of the art knowledge and efficacy aspects. We started the task with a reassessment of (1) imaging in urinary tract infection in infants and children, (2) postnatal imaging in mild-to-moderate neonatal hydronephrosis, (3) how to perform voiding cystourethrography, and (4) procedural recommendations for paediatric urosonography. This list is incomplete, and future recommendations will be developed, discussed and presented at forthcoming meetings.

Retroperitoneoscopically assisted repair of an abdominoscrotal hydrocele.

We present a 2-month-old boy with a primary obstructive megaureter and a giant abdominoscrotal hydrocele (ASH) on the left side. The ASH was initially misdiagnosed as a fornix rupture with infrarenal urinoma, but clinical and radiological examination led to the right diagnosis. Retroperitoneoscopically assisted mobilization combined with the inguinal approach allowed the successful resection of this large ASH.

Pre- and postnatal imaging of a girl with a cloacal variant.

We describe the prenatal MR findings in a 29-week fetus with a cloacal variant (urogenital sinus and anterior placed anus) in combination with an enlarged clitoris and urethral duplication and correlate them with postnatal imaging. Fetal MR imaging permits the diagnosis and characterization of cloacal and urogenital sinus malformations in utero. This information may guide pre-, peri- and postnatal management.

Tension gastrothorax.

BACKGROUND: Tension gastrothorax develops when the stomach herniated through a congenital diaphragmatic defect into the thorax is massively distended by trapped air. The authors present 5 cases and discuss the diagnostic and therapeutic management. CASE REPORTS: Four children, aged 3, 4, 6, and 13 months, presented with progressive respiratory distress. In only 1 child was the diagnosis of tension gastrothorax established initially, and immediate insertion of a nasogastric tube led to complete resolution of respiratory distress symptoms. In the remaining 3 children, the initial chest radiograph was misread as tension pneumothorax. One of them developed cardiac arrest and was successfully resuscitated. In 2 patients, thoracostomy resulted in immediate respiratory improvement. Only on follow-up radiographic evaluation was diaphragmatic hernia with herniation of the stomach into the left hemithorax diagnosed. One child underwent diagnostic thoracoscopy revealing the correct diagnosis. All 4 children underwent uneventful repair of a classic Bochdalek hernia. The fifth child, aged 5 months, had sudden infant death. At autopsy tension gastrothorax was found. CONCLUSION: Tension gastrothorax is a life-threatening condition leading to acute and severe respiratory distress. The condition exhibits distinct radiographic features. Emergency decompression of the distended stomach should first be attempted via nasogastric tube. If this maneuver fails, decompression must be achieved either by needle puncture or by chest tube insertion into the stomach.

Situs inversus, bronchiectasis, and sinusitis and its relation to immotile cilia: history of the diseases and their discoverers-Manes Kartagener and Bjorn Afzelius.

The relationship of Kartagener's syndrome to immobile cilia syndrome is a fascinating merging of clinical observations and basic science in Zurich, Stockholm, and Toronto. In 1933, Manes Kartagener, a Zurich pulmonary physician, reported four patients with the triad of sinusitis, bronchiectasis, and situs inversus. In the following decades, he reviewed reports of hundreds of cases, but the fact that the male patients with the condition never had offspring eluded his notice. In the 1970s, Bjorn Afzelius, a Ph.D. ultrastructuralist from Stockholm, reported cilia immotility in infertile males, some of the cases occurring in families. Half of the cases had Kartagener's triad. The observation of Afzelius was soon applied to children by Jennifer Sturgess, a Ph.D. ultrastructuralist, and her medical colleagues in Toronto. With over 500 MEDLINE references since 1966 on Kartagener's and over 1,000 references on immotile cilia, the causes of the pulmonary infections have become clearer as the patients demonstrate impaired clearance of mucus with resultant sinus and bronchial disease. The cause of the situs inversus remains elusive to this day. It is appropriate to call the condition Kartagener-Afzelius syndrome.

Solitary infantile choriocarcinoma of the liver: MRI findings.

Infantile hepatic choriocarcinoma is a rare, highly malignant germ-cell tumour believed to result from a choriocarcinoma of the placenta that spreads to the child. Most infants present with a characteristic clinical picture of anaemia, hepatomegaly and precocious puberty. Imaging findings, including conventional MRI, may be non-specific. To improve the accuracy of diagnosis, we present the imaging findings of contrast-enhanced dynamic MRI in a 4.5-month-old boy with infantile hepatic choriocarcinoma.

Renal dimensions measured by ultrasonography in children: variations as a function of the imaging plane and patient position.

The purpose of this study was to determine the effect of patient positioning on sonographic renal measurements and to test if the patient position alters the three-dimensional shape of the kidneys. The maximum longitudinal renal length and transverse renal width and depth were measured in the supine and prone position in 100 children (200 kidneys). Age ranged from 6 months to 16 years (mean age 5 years). The results were compared for statistically significant differences. The maximum measured longitudinal renal length was statistically significantly larger in the supine than in the prone position (supine position, left: 8.0 cm; right: 7.7 cm; prone position, left: 7.9 cm, right: 7.6 cm; P<0.001). There was no statistically significant change in the transverse diameters (width and depth, P>0.001) and renal volume ( P>0.001) in the supine vs. prone positions. Our results show that position-induced reshaping of the kidneys is unlikely to be responsible for the discrepancy in maximum longitudinal renal measurements comparing supine with prone positions. Position-dependent changes in the degree of filling of the renal calyces and pelvis as well as errors in caliper distance measurements for the different scan depths (supine vs. prone) are more likely to be responsible for the encountered differences. Consequently, we recommend to add prone renal length measurements in addition to the supine measurements. In follow-up examinations, renal length measurements should only be compared that have been collected in the same patient position.

Radiographic and MRI features of deferiprone-related arthropathy of the knees in patients with beta-thalassemia.

OBJECTIVE: This study was undertaken to describe the radiographic and MRI appearances of arthropathy of the knees in 14 patients with beta-thalassemia major undergoing chelation therapy with deferiprone (L1). MATERIALS AND METHODS: All available radiographs and MRI studies of the knees in 14 beta-thalassemia major patients (mean age, 16.3 years; age range, 7-33 years) undergoing chelation therapy with L1 were retrospectively assessed for changes in the synovium, cartilage, and bone. Imaging findings and signs of knee arthropathy were correlated with chelation therapy and average serum ferritin concentration. RESULTS: Nine (64%) of the 14 patients developed arthralgia of the knees during treatment with L1. Abnormal imaging findings were present in all symptomatic and two asymptomatic patients (12/14, 86%) and included joint effusion, subchondral bone irregularity, and patellar beaks. Additional MRI findings were thickening and enhancement of the synovium; hypointense bands in the synovium; irregularly thickened epiphyseal and articular cartilage overlying subchondral bone defects; and, on T2-weighted sequences, hyperintense articular cartilage lesions. The degree of knee symptoms at the time of imaging did not reflect the severity of cartilage and subchondral bone changes. CONCLUSION: Radiologic changes can be seen in L1-related arthropathy and should be recognized. MRI of the knees should be considered in symptomatic children and young adults with thalassemia undergoing L1 chelation therapy for iron overload.

Burkitt's lymphoma with bilateral cavernous sinus and mediastinal involvement in a child.

We report a 12-year-old boy who presented with incomplete right ophthalmoplegia, exophthalmos and headache. Initial CT and MRI revealed a mass in the right cavernous sinus. During tumour work-up, CT identified additional tumour within the mediastinum. Biopsy of the mediastinal lesion identified Burkitt's lymphoma. We report on this case because radiologists and clinicians should be alerted to identify sites of primary Burkitt's lymphoma outside of the central nervous system if clinical symptoms indicate, or imaging shows, CNS lesions. Primary CNS involvement in Burkitt's lymphoma is rare.

Developmental nasal midline masses in children: neuroradiological evaluation.

Developmental nasal midline masses in children are rare lesions. Neuroimaging is essential to characterise these lesions, to determine the exact location of the lesion and most importantly to exclude a possible intracranial extension or connection. Our objective was to evaluate CT and MRI in the diagnosis of developmental nasal midline masses. Eleven patients (mean age 4.5 years) with nasal midline masses were examined by CT and MRI. Neuroimaging was evaluated for (a) lesion location/size, (b) indirect (bifid or deformed crista galli, widened foramen caecum, defect of the cribriform plate) and direct (identification of intracranially located lesion components or signal alterations) imaging signs of intracranial extension, (c) secondary complications and (d) associated malformations. Surgical and histological findings served as gold standard. Nasal dermoid sinus cysts were diagnosed in 9 patients. One patient was diagnosed with an meningocele and another patient with a nasal glioma. Indirect CT and MRI signs correlated with the surgical results in 10 of 11 patients. Direct CT findings correlated with surgery in all patients, whereas the direct MRI signs correlated in 9 of 11 patients. In 2 patients MRI showed an intracranial signal alteration not seen on CT. Neuroimaging corrected the clinical diagnosis in 1 patient. One child presented with a meningitis. In none of the patients was an associated malformation diagnosed. Intracranial extension is equally well detected by CT and MRI using indirect imaging signs. Evaluating the direct imaging signs, MRI suspected intracranial components in 2 patients without a correlate on CT. This could represent an isolated intracranial component that got undetected on CT and surgery. In 9 patients CT and MRI matched the surgical findings. The MRI did not show any false-negative results. These results in combination with the multiplanar MRI capabilities, the different image contrasts that can be generated by MRI and the lack of radiation favour the use of MRI as primary imaging tool in these young patients in which the region of imaging is usually centred on the radiosensitive eye lenses.