Nephron Sparing Surgery in Bilateral Wilms Tumors With Botryoid Growth Pattern.

Botryoid growth pattern (BGP) is a polypoid mass growing into the renal pelvis, rarely seen in bilateral Wilms tumors where it represents a surgical challenge. We report our experience of nephron sparing surgery in 3 patients with BGP in bilateral Wilms tumor. Surgical en bloc removal was performed after calyx opening with no complications. The histology of the BGP was Intralobar Nephrogenic Rest in all cases while all Wilms tumors were of intermediate risk. One patient early recurred. At a follow-up of 9 months, 22 and 23 years, all patients were alive with a moderate renal insufficiency and hypertension.

Harmonisation of imaging protocols, radiation doses and image quality in gastrointestinal fluoroscopy examinations - multicentre study.

BACKGROUND: Paediatric gastrointestinal fluoroscopy examinations can impart varying amounts of radiation for the same patient size and exam type. OBJECTIVE: To investigate the variability of imaging protocol, radiation dose and image quality in paediatric fluoroscopy examinations in order to provide recommendations for the harmonisation and optimisation of local practices. MATERIALS AND METHODS: Five paediatric radiology departments performing fluoroscopically-guided contrast enema, micturating cystourethrography and upper gastrointestinal tract examinations participated in this study. Information on imaging protocols and radiation doses was retrospectively collected for more than 2,400 examinations. Image quality was analysed on clinical and phantom images. RESULTS: Patient doses showed great variability among centers with up to a factor of 5 for similar fluoroscopy times. The five departments had imaging protocols with major differences in fluoroscopy dose regulation curves and additional filtration. Image quality analysis on phantoms and patients images showed no major improvement in contrast, spatial resolution or noise when increasing the radiation dose. Age-based diagnostic reference levels using both dose area product and fluoroscopy time were proposed per procedure type. CONCLUSION: Disparities between centers and no correlation of radiation dose with image quality criteria create margins for optimisation. These results highlight the need for guidelines on fluoroscopy image quality and dose reference levels in paediatric gastrointestinal examinations to harmonise practices and optimise patient dose.

Assessment of an artificial intelligence aid for the detection of appendicular skeletal fractures in children and young adults by senior and junior radiologists.

BACKGROUND: As the number of conventional radiographic examinations in pediatric emergency departments increases, so, too, does the number of reading errors by radiologists. OBJECTIVE: The aim of this study is to investigate the ability of artificial intelligence (AI) to improve the detection of fractures by radiologists in children and young adults. MATERIALS AND METHODS: A cohort of 300 anonymized radiographs performed for the detection of appendicular fractures in patients ages 2 to 21 years was collected retrospectively. The ground truth for each examination was established after an independent review by two radiologists with expertise in musculoskeletal imaging. Discrepancies were resolved by consensus with a third radiologist. Half of the 300 examinations showed at least 1 fracture. Radiographs were read by three senior pediatric radiologists and five radiology residents in the usual manner and then read again immediately after with the help of AI. RESULTS: The mean sensitivity for all groups was 73.3% (110/150) without AI; it increased significantly by almost 10% (P<0.001) to 82.8% (125/150) with AI. For junior radiologists, it increased by 10.3% (P<0.001) and for senior radiologists by 8.2% (P=0.08). On average, there was no significant change in specificity (from 89.6% to 90.3% [+0.7%, P=0.28]); for junior radiologists, specificity increased from 86.2% to 87.6% (+1.4%, P=0.42) and for senior radiologists, it decreased from 95.1% to 94.9% (-0.2%, P=0.23). The stand-alone sensitivity and specificity of the AI were, respectively, 91% and 90%. CONCLUSION: With the help of AI, sensitivity increased by an average of 10% without significantly decreasing specificity in fracture detection in a predominantly pediatric population.

Early life ionizing radiation exposure and cancer risks: systematic review and meta-analysis.

BACKGROUND: Ionizing radiation use for medical diagnostic purposes has substantially increased over the last three decades. Moderate to high doses of radiation are well established causes of cancer, especially for exposure at young ages. However, cancer risk from low-dose medical imaging is debated. OBJECTIVE: To review the literature on cancer risks associated with prenatal and postnatal medical diagnostic ionizing radiation exposure among children and to assess this risk through a meta-analysis. MATERIALS AND METHODS: A literature search of five electronic databases supplemented by a hand search was performed to retrieve relevant epidemiological studies published from 2000 to 2019, including patients younger than 22 years of age exposed to medical imaging ionizing radiation. Pooled odds ratio (ORpooled) and pooled excess relative risk (ERRpooled) representing the excess of risk per unit of organ dose were estimated with a random effect model. RESULTS: Twenty-four studies were included. For prenatal exposure (radiographs or CT), no significant increased risk was reported for all cancers, leukemia and brain tumors. For postnatal exposure, increased risk was observed only for CT, mostly for leukemia (ERRpooled=26.9 Gy-1; 95% confidence interval [CI]: 2.7-57.1) and brain tumors (ERRpooled=9.1 Gy-1; 95% CI: 5.2-13.1). CONCLUSION: CT exposure in childhood appears to be associated with increased risk of cancer while no significant association was observed with diagnostic radiographs.

Neurological Involvement in Childhood Evans Syndrome.

PURPOSE: Immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) are associated in the definition of Evans syndrome (ES). The occurrence of neurological involvement in this population is poorly described and suggests an underlying primary immunodeficiency (PID). We aimed to describe the clinical manifestations, evolution, and PID profiles of these patients. METHODS: OBS'CEREVANCE is a French, nationwide prospective cohort that includes children with chronic ITP, AIHA, and ES. Patients with a neurological involvement were described. Centralized radiological and pathological reviews and genetic analyses were performed. RESULTS: On October 2016, eight patients (7/181 ES, 1/371 AIHA, and 0/615 ITP) were identified, all male, with a median age (range) at cytopenia onset of 11.5 years (1.6-15.8). Neurological symptoms appeared with a median delay of 6 years (2.5-18) after cytopenia and were polymorphic: seizures (n = 4), cranial nerve palsy (n = 2), Brown-Sequard syndrome (n = 2), intracranial pressure (n = 2), vertigo (n = 1), and/or sensory neuropathy (n = 1). Magnetic resonance imaging (MRI) showed inflammatory lesions, confirmed by pathology for five patients with macrophagic or lymphoplasmocytic infiltrates. All patients had other relevant immunopathological manifestations: pulmonary nodules (n = 6), lymphoproliferation (n = 4), abnormal immunophenotype (n = 8), and hypogammaglobulinemia (n = 7). Treatment consisted of steroids that improved symptomatology and MRI. Five patients relapsed and three had an asymptomatic radiological progression. A PID was identified in 3/8 patients: 22q11.2 microdeletion (n = 1) and CTLA deficiency (n = 2). CONCLUSION: Neurological involvement is a rare and severe late event in the course of childhood ES, which can reveal an underlying PID. Imaging and pathology examination highlight a causative immune dysregulation that may guide targeted therapeutic strategies.

Ultrasonography and Computed Tomographic Manifestations of Abdominal Sarcoidosis in Children.

OBJECTIVES: Sarcoidosis is a multisystem, granulomatous inflammatory disease affecting both pediatric and adult patients. So far in children, very few radiological descriptions of abdominal sarcoidosis manifestations have been reported. The present study describes the frequency and the appearance of abdominal radiologic manifestations in pediatric patients with histologically proven sarcoidosis. METHODS: We reviewed retrospectively all of the radiological examinations of 22 patients ages 1 to 15 years at diagnosis with proven sarcoidosis evaluated in a university pediatric hospital between 1994 and 2014. The locations of biopsies and the angiotensin-converting enzyme level were reported. The size, shape, and parenchymal homogeneity of the liver and spleen, the presence of abdominal lymph nodes, and abnormalities of the gastrointestinal tract were tabulated. RESULTS: The study included 22 children (mean age: 9.9 ±â€Š2.8 years). The liver was the most frequent location of biopsy (12/22), even without radiological involvement. Abdominal manifestations were present in 11 of 22 children with sarcoidosis. Hepatomegaly was the most frequent abnormality, reported in 8 of 11 cases either homogeneous (n = 7) or nodular (n = 1). Homogeneous lymph node enlargement was noted in 6 of 11 cases and splenomegaly in 4 of 11 cases. No calcification was observed. All patients with initial abdominal sarcoidosis had simultaneous thoracic involvement. CONCLUSIONS: Abdominal manifestations in children sarcoidosis are frequent but often nonspecific. Nodular hepatosplenomegaly is rare. All of our patients with abdominal abnormalities had a more specific associated thoracic involvement. Awareness of this association could assist the clinicians in assessing the initial diagnosis of abdominal sarcoidosis in children.

Prenatal US evaluation of the spinal cord using high-frequency linear transducers.

We illustrate the contribution of high-frequency linear abdominal transducers in the prenatal US examination of the spinal cord. After birth, such transducers are commonly used in US examination of the spinal cord. During the third trimester of gestation, the fetal spine is commonly facing anteriorly and US images of the spinal cord can be acquired using a high-frequency linear abdominal transducer. Images of the normal spinal cord, normal variants (ventriculus terminalis, cyst of filum terminale) and spinal cord abnormalities (myelomeningocele, meningocele, diastematomyelia, tethered spinal cord and caudal regression syndrome) are presented. In this pictorial essay, comparison between images acquired with low- and high-frequency transducers are provided as well as correlation with postnatal data. In the normal spine, anatomical details such as the conus medullaris, the filum terminale and the nerve root bundles are exquisitely depicted, making it possible to differentiate normal variants from abnormalities. In abnormal cases, the position of the conus medullaris, its shape and the nerve roots can be analyzed in detail. We describe the benefits of using high-frequency linear transducers in US examination of the spinal cord, which is common after birth but has not been hitherto reported in fetuses.

Prenatal evaluation of the middle ear and diagnosis of middle ear hypoplasia using MRI.

BACKGROUND: Analysis of the middle ear with fetal MRI has not been previously reported. OBJECTIVE: To show the contribution of fetal MRI to middle ear imaging. MATERIALS AND METHODS: The tympanic cavity was evaluated in 108 fetal cerebral MRI examinations (facial and/or cerebral malformation excluded) and in two cases, one of Treacher Collins syndrome (case 1) and the other of oculo-auriculo-vertebral (OUV) spectrum (case 2) with middle ear hypoplasia identified by MRI at 27 and 36 weeks' gestation, respectively. RESULTS: In all 108 fetuses (mean gestational age 32.5 weeks), the tympanic cavity and T2 hypointensity related to the ossicles were well visualised on both sides. Case 1 had micro/retrognathia and bilateral external ear deformity and case 2 had retrognathism with a left low-set and deformed ear. MRI made it possible to recognize the marked hypoplasia of the tympanic cavity, which was bilateral in case 1 and unilateral in case 2. Both syndromes are characterized by craniofacial abnormalities including middle ear hypoplasia, which cannot be diagnosed with US. CONCLUSION: The middle ear cavity can be visualized with fetal MRI. We emphasize the use of this imaging modality in the diagnosis of middle ear hypoplasia.

The role of computed tomography and magnetic resonance imaging for preoperative pediatric cochlear implantation work-up in academic institutions.

OBJECTIVE: The goal of the study is to investigate the association of pertinent preoperative temporal bone computed tomography (CT) and brain magnetic resonance imaging (MRI) results and intraoperative surgical findings and complications of pediatric cochlear implantation reported in academic settings. METHODS: This is a retrospective review of cochlear implant patients who received a pre-operative temporal bone CT and MRI of the brain between 2005 and 2012 at academic pediatric otolaryngology practices within children's hospitals in the United States and France. Scans were reviewed in a double-blind fashion and compared to intraoperative findings. RESULTS: 91 children were analyzed (mean age 5.54 +/- 0.58 years). A small facial recess identified on CT was associated with difficult insertion of electrodes (P = 0.0003). A prominent sigmoid sinus noted on CT was associated of difficult insertion of electrodes (P = 0.01), iatrogenic tegmen dehiscence (P = 0.005), as well as difficult round window access (P = 0.025). No specific CT finding was found to be associated with external auditory canal injury, perilymphatic gusher, or iatrogenic facial nerve injury. MRI brain and internal auditory canal findings were not predictive of surgical outcomes. CONCLUSIONS: Preoperative CT and MRI remain an important planning tool for pediatric cochlear implantation, particularly in academic institutions. The findings of our study demonstrate that a detailed assessment of both preoperative CT and MRI are valuable for teaching and surgical planning.

Microcephaly: a radiological review.

Microcephaly results from inadequate brain growth during development. It may develop in utero, and therefore be present at birth, or may develop later as a result of perinatal events or postnatal conditions. The aetiology of microcephaly may be congenital (secondary to cerebral malformations or metabolic abnormalities) or acquired, most frequently following an ischaemic insult. This distinct radiological and pathological entity is reviewed with a specific focus on aetiology.

Eye lens radiation exposure and repeated head CT scans: A problem to keep in mind.

OBJECTIVES: The deterministic character of radiation-induced cataract is being called into question, raising the possibility of a risk in patients, especially children, exposed to ionizing radiation in case of repeated head CT-scans. This study aims to estimate the eye lens doses of a pediatric population exposed to repeated head CTs and to assess the feasibility of an epidemiological study. METHODS: Children treated for a cholesteatoma, who had had at least one CT-scan of the middle ear before their tenth birthday, were included. Radiation exposure has been assessed from medical records and telephone interviews. RESULTS: Out of the 39 subjects contacted, 32 accepted to participate. A total of 76 CT-scans were retrieved from medical records. At the time of the interview (mean age: 16 years), the mean number of CT per child was 3. Cumulative mean effective and eye lens doses were 1.7mSv and 168mGy, respectively. CONCLUSION: A relatively high lens radiation dose was observed in children exposed to repeated CT-scans. Due to that exposure and despite the difficulties met when trying to reach patients' families, a large scale epidemiological study should be performed in order to assess the risk of radiation-induced cataracts associated with repeated head CT.

Longitudinal lung function and structural changes in children with primary ciliary dyskinesia.

BACKGROUND AND OBJECTIVES: Functional and structural lung evaluations are part of the follow-up of patients with primary ciliary dyskinesia (PCD). We aimed to evaluate transversal and longitudinal relationships between lung function test (LFT) and chest computed tomography (CT) in children with PCD, in stable clinical condition. MATERIALS AND METHODS: Data from children followed in the French National Center were retrospectively collected. Inclusion criteria were (i) definitive diagnosis of PCD, (ii) age less than 15 years at the beginning of follow-up, (iii) at least 8 years of follow-up, (iv) at least two couples of concurrent CT and LFT available in a phase of clinical stability of the lung disease without modification of the treatment regimen in the last 4 weeks. Twenty children (median age at entry 4.6 years, median follow-up 15.4 years) were included. Concurrent LFT (blood gas and spirometry) and CT (score) results were recorded. RESULTS: LFT indices (PaO(2) (n = 210), FVC, FEV(1) , FEF(2575%) (n = 195)) significantly decreased with age, and the mean annual decrease (z-score (% predicted)) was -0.17 (-0.49%), -0.09 (-0.50%), -0.10 (-0.89%), and -0.07 (-1.73%), respectively. First CT (median age 8.7 years) revealed bronchiectasis (70%), mucous plugging (70%), peribronchial thickening (90%), parenchymal abnormalities (65%), and hyperinflation (50%). CT scores (n = 74) significantly increased with age, and was negatively correlated to PaO(2), FVC, FEV(1), and FEF(2575%) longitudinal changes. CONCLUSION: In stable clinical condition, functional, and structural progressive impairments significantly correlated in children with PCD. Further prospective studies, including large populations of patients with various levels of disease severity, are needed to confirm whether lung function follow-up can be used to adjust CT frequency and help at minimizing the radiation burden in children with a good life expectancy.

Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: a nationwide retrospective study.

BACKGROUND: Vinblastine (VBL) is the standard treatment for systemic Langerhans cell histiocytosis (LCH), but little is known about its efficacy in central nervous system (CNS) mass lesions. METHODS: A retrospective chart review was conducted. Twenty patients from the French LCH Study Group register met the inclusion criteria. In brief, they had CNS mass lesions, had been treated with VBL, and were evaluable for radiologic response. RESULTS: The median age at diagnosis of LCH was 11.5 years (range: 1-50). Intravenous VBL 6 mg/m2 was given in a 6-week induction treatment, followed by a maintenance treatment. The median total duration was 12 months (range: 3-30). Eleven patients received steroids concomitantly. Fifteen patients achieved an objective response; five had a complete response (CR: 25%), ten had a partial response (PR: 50%), four had stable disease (SD: 20%) and one patient progressed (PD: 5%). Of interest, four out of the six patients who received VBL without concomitant steroids achieved an objective response. With a median follow-up of 6.8 years, the 5-year event-free and overall survival was 61% and 84%, respectively. VBL was well-tolerated and there were no patient withdrawals due to adverse events. CONCLUSION: VBL, with or without steroids, could potentially be a useful therapeutic option in LCH with CNS mass lesions, especially for those with inoperable lesions or multiple lesions. Prospective clinical trials are warranted for the evaluation of VBL in this indication.

Reliability analysis for manual measurement of coronal plane deformity in adolescent scoliosis. Are 30 x 90 cm plain films better than digitized small films?

For several years, digitized small radiographs are used to measure Cobb angle in idiopathic scoliosis. The interobserver and intraobserver Cobb angle measurement variability associated with small radiographs were compared with measurement variability associated with the long-cassette radiographs. Twenty adolescent patients with a double major idiopathic scoliosis had erect full-spine p-A radiographs and Cobb angle measurements performed by eight different observers on a 30 x 90 cm plain-film radiograph and a digitized 14 x 42 cm image. Inter-observer and intra-observer reliability using each techniques were assessed using a paired t-test, Spearman rank correlation study and intraclass correlation coefficients. The angle variability between small film and plain-film measurements was assessed using the same methods. Intra-observer and inter-observer study showed good reliability using both techniques. The comparison between small films and plain-films measurements showed very good agreement with an intraclass correlation coefficient of 95% and confidence interval between 0.962 and 0.972. In our study, Cobb angle determination was not found to vary significantly with film size. The small film image used for full-spine radiographs in our institution allows manual Cobb angle measurements to be performed. A study is currently conducted in our institution to determine if a computer-assisted measurement method significantly improves Cobb angle measurements reliability in routine practice compared with manual measurements of Cobb angles on small films.

Spinal fracture through the neurocentral synchondrosis in battered children: a report of three cases.

STUDY DESIGN: Retrospective case series. OBJECTIVE: To report 3 cases of unusual spinal fracture through the neurocentral synchondrosis. SUMMARY OF BACKGROUND DATA: Traumatic lesions of the spine or spinal cord due to physical abuse are rare. We report 3 cases of vertebral fractures involving the neurocentral synchondrosis in battered children. METHODS: The medical data of the 3 cases were retrospectively reviewed. We discuss the pathomechanism and treatment strategies. RESULTS: In 2 cases, the vertebral body was displaced anteriorly and, in 1 case, posteriorly, all without neurologic compromise. The lateral radiographs showed vertebral displacement, but the diagnosis could be suspected on frontal views because of interpedicular distance widening. Treatment was nonoperative with bracing in 2 cases. In the third case, a posterior epiphysiodesis was done to avoid development of progressive kyphosis of the injured spine. One child treated nonoperatively died from other traumatic lesions associated with the physical abuse. In the 2 other cases, outcome was good with a well-balanced spine in 1 case and a moderate, well-tolerated kyphosis in the other case. CONCLUSIONS: Such spinal lesions are rare but could be conservatively treated in case of anterior vertebral displacement without vertebral endplate impairment. A posterior displacement of vertebral body could be responsible for a neurologic compromise.