Juvenile Xanthogranuloma of the Head and Neck: Imaging Findings in 11 Cases.

BACKGROUND: Juvenile Xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis, occurring mainly in infancy. With an extracutaneous lesion, its diagnosis is difficult, because of a wide clinical spectrum. Here we demonstrate and characterize imaging features of 11 patients with JXG of the head and neck in various locations. MATERIAL AND METHODS: We recorded clinical data and reviewed all imaging studies of 11 patients with JXG of the head and neck. Ultrasonography (US) alone was performed in 1 patient; MRI alone in 6 patients; US and MRI in 1 patient; and US, CT, and MRI in 3 patients. We evaluated the following characteristics in all studies: location and number of lesions, echogenicity and vascularization on US, density on CT, signal intensity on T1- and T2-weighted images, ADC and enhancement on MRI, and tumor boundaries and bone involvement. RESULTS: Lesions were well-defined in 9 cases, and bone erosion was present in 2. On US, lesions were hypoechoic or hyperechoic and with or without vascularization. On CT, lesions were hyper-dense, with no calcification. On MRI, lesions were mildly hyper-intense or iso-intense on T1-weighted images in 8 of 9 patients, hypo-intense on T2-weighted images in 7 of 10, low ADC in 7 of 9, and enhancement in 7 of 7. CONCLUSIONS: The diagnosis of extra cutaneous JXG may be proposed, with the following suggestive criteria: age < 1 year, well-defined lesion, mild hyper-intensity on T1-weighted images, hypo-intensity on T2-weighted images, low ADC, enhancement, and possible adjacent bone involvement.

Imaging characterization of paediatric tumours with the neurotrophic tyrosine receptor kinase fusion transcript.

OBJECTIVES: The neurotrophic tyrosine receptor kinase (NTRK) fusion transcript (FT) is a major genetic landmark of infantile fibrosarcoma (IFS) and cellular congenital mesoblastic nephroma (cCMN) but is also described in other tumours. The recent availability of NTRK-targeted drugs enhances the need for better identification. We aimed to describe the anatomic locations and imaging features of tumours with NTRK-FT in children. CASE SERIES: Imaging characteristics of NTRK-FT tumours of 41 children (median age: 4 months; 63% <1 year old; range: 0-188) managed between 2001 and 2019 were retrospectively analysed. The tumours were located in the soft tissues (n = 24, including 19 IFS), kidneys (n = 9, including 8 cCMN), central nervous system (CNS) (n = 5), lung (n = 2), and bone (n = 1). The tumours were frequently deep-located (93%) and heterogeneous (71%) with necrotic (53%) or haemorrhagic components (29%). Although inconstant, enlarged intratumoural vessels were a recurrent finding (70%) with an irregular distribution (63%) in the most frequent anatomical locations. CONCLUSION: Paediatric NTRK-FT tumours mainly occur in infants with very variable histotypes and locations. Rich and irregular intra-tumoural vascularization are recurrent findings. ADVANCES IN KNOWLEDGE: Apart from IFS of soft tissues and cCMN of the kidneys, others NTRK-FT tumours locations have to be known, as CNS tumours. Better knowledge of the imaging characteristics may help guide the pathological and biological identification.

Analysis of the rate of force development reveals high neuromuscular fatigability in elderly patients with chronic kidney disease.

BACKGROUND: Chronic kidney disease (CKD) induces muscle wasting and a reduction in the maximum voluntary force (MVF). Little is known about the neuromuscular fatigability in CKD patients, defined as the reduction of muscle force capacities during exercise. Neuromuscular fatigability is a crucial physical parameter of the daily living. The quantification of explosive force has been shown to be a sensitive means to assess neuromuscular fatigability. Thus, our study used explosive force estimates to assess neuromuscular fatigability in elderly CKD patients. METHODS: Inclusion criteria for CKD patients were age ≥ 60 years old and glomerular filtration rate (GFR) < 45 mL/min/1.73 m2 not on dialysis, and those for controls were GFR > 60 mL/min/1.73 m2 , age and diabetes matched. The fatigability protocol focused on a handgrip task coupled with surface electromyography (sEMG). Scalars were extracted from the rate of force development (RFD): absolute and normalized time periods (50, 75, 100, 150 and 200 ms, RFD50 , RFD75 , RFD100 , RFD150 and RFD200 , respectively), peak RFD (RFDpeak in absolute; NRFDpeak normalized), time-to-peak RFD (t-RFDpeak ) and the relative force at RFDpeak (MVF-RFDpeak ). A statistical parametric mapping approach was performed on the force, impulse and RFD-time curves. The integrated sEMG with time at 0-30, 0-50, 0-100 and 0-200 ms time intervals relative to onset of sEMG activity was extracted and groups were compared separately for each sex. RESULTS: The cohort of 159 individuals had a median age of 69 (9IQR ) years and body mass index was 27.6 (6.2IQR ) kg/m2 . Propensity-score-matched groups balanced CKD patients and controls by gender with 66 males and 34 females. In scalar analysis, CKD patients manifested a higher decrement than controls in the early phase of contraction, regarding the NRFDpeak (P = 0.009; η2 p  = 0.034) and RFD75 and RFD100 (for both P < 0.001; η2 p  = 0.068 and 0.064). The one-dimensional analysis confirmed that CKD males manifest higher and delayed neuromuscular fatigability, especially before 100 ms from onset of contraction. sEMG was lower in CKD patients than controls in the 0-100 ms (at rest: P = 0.049, Cohen's d = 0.458) and 0-200 ms (at rest: P = 0.016, Cohen's d = 0.496; during exercise: P = 0.006, Cohen's d = 0.421) time windows. Controls showed greater decrease of sEMG than CKD patients in the 0-30 ms (P = 0.020, Cohen's d = 0.533) and 0-50 ms (P = 0.010, Cohen's d = 0.640) time windows. As opposite to females, males showed almost the same differences between groups. CONCLUSIONS: Our study is the first to show that CKD patients have higher fatigability than controls, which may be associated with an impaired motor-unit recruitment, highlighting a neural drive disturbance with CKD. Further studies are needed to confirm these findings.

Generating High-Resolution Synthetic CT from Lung MRI with Ultrashort Echo Times: Initial Evaluation in Cystic Fibrosis.

Background Lung MRI with ultrashort echo times (UTEs) enables high-resolution and radiation-free morphologic imaging; however, its image quality is still lower than that of CT. Purpose To assess the image quality and clinical applicability of synthetic CT images generated from UTE MRI by a generative adversarial network (GAN). Materials and Methods This retrospective study included patients with cystic fibrosis (CF) who underwent both UTE MRI and CT on the same day at one of six institutions between January 2018 and December 2022. The two-dimensional GAN algorithm was trained using paired MRI and CT sections and tested, along with an external data set. Image quality was assessed quantitatively by measuring apparent contrast-to-noise ratio, apparent signal-to-noise ratio, and overall noise and qualitatively by using visual scores for features including artifacts. Two readers evaluated CF-related structural abnormalities and used them to determine clinical Bhalla scores. Results The training, test, and external data sets comprised 82 patients with CF (mean age, 21 years ± 11 [SD]; 42 male), 28 patients (mean age, 18 years ± 11; 16 male), and 46 patients (mean age, 20 years ± 11; 24 male), respectively. In the test data set, the contrast-to-noise ratio of synthetic CT images (median, 303 [IQR, 221-382]) was higher than that of UTE MRI scans (median, 9.3 [IQR, 6.6-35]; P < .001). The median signal-to-noise ratio was similar between synthetic and real CT (88 [IQR, 84-92] vs 88 [IQR, 86-91]; P = .96). Synthetic CT had a lower noise level than real CT (median score, 26 [IQR, 22-30] vs 42 [IQR, 32-50]; P < .001) and the lowest level of artifacts (median score, 0 [IQR, 0-0]; P < .001). The concordance between Bhalla scores for synthetic and real CT images was almost perfect (intraclass correlation coefficient, ≥0.92). Conclusion Synthetic CT images showed almost perfect concordance with real CT images for the depiction of CF-related pulmonary alterations and had better image quality than UTE MRI. Clinical trial registration no. NCT03357562 © RSNA, 2023 Supplemental material is available for this article. See also the editorial by Schiebler and Glide-Hurst in this issue.

Petit-Morel method for hip development dysplasia in toddlers: A retrospective observational study in 34 patients.

BACKGROUND: The Petit-Morel method allows the treatment of developmental hip dysplasia in toddlers by combining gradual traction to achieve reduction followed by immobilisation during which pelvic osteotomy is performed. The objective of this study was to assess the radiographic and clinical outcomes in a retrospective cohort of patients. HYPOTHESIS: The Petit-Morel method is associated with low rates of avascular necrosis and residual acetabular dysplasia at skeletal maturity, as well as with satisfactory medium-term clinical outcomes. MATERIAL AND METHODS: We conducted a single-centre retrospective study of 34 patients (35 hips) treated between 1997 and 2014. The radiological assessment criteria included an evaluation for avascular necrosis classified according to Kalamchi and MacEwan, the vertical centre edge (VCE) angle, femoral head sphericity according to Mose, and acetabular dysplasia at skeletal maturity according to Severin. Hip function was assessed by determining the Postel-Merle d'Aubigné (PMA) score. RESULTS: Mean age at treatment was 19±4 months (range, 14-29). Mean follow-up was 11 years (range, 5-20). There were two failures including one case of recurrent dislocation requiring surgical reduction. Group II avascular necrosis occurred in 1 (3%) patient. Tönnis Grade IV dysplasia was significantly associated with resolving irregularity of the ossification centre, seen in 19 (54%) cases (p=0.002). In the 18 patients followed-up to skeletal maturity, with a mean follow-up of 15 years (range, 12-20 years), 17 hips were Severin Class I. The mean VCE angle was 29° (range, 15°-38°), and the head was spherical for 34 (98%) hips. The PMA score at last follow-up was excellent (17-18). The mean VCE angle was greater in all 5 patients who experienced pain during long walks (35° [range, 32°-37°]) than in the asymptomatic patients (28° [range, 15°-38°]) (p=0.009). DISCUSSION: The Petit-Morel method is a reliable treatment that provides good clinical and radiological outcomes. Overcorrection of the VCE angle was noted in the patients who experienced walking-related pain in adulthood. LEVEL OF EVIDENCE: IV, retrospective observational cohort study.

Sirolimus (Rapamycin) for Slow-Flow Malformations in Children: The Observational-Phase Randomized Clinical PERFORMUS Trial.

IMPORTANCE: Sirolimus is increasingly being used to treat various vascular anomalies, although evidence of its efficacy is lacking. OBJECTIVE: To assess the efficacy and safety of sirolimus for children with slow-flow vascular malformations to better delineate the indications for treatment. DESIGN, SETTING AND PARTICIPANTS: This multicenter, open-label, observational-phase randomized clinical trial included 59 children aged 6 to 18 years with a slow-flow vascular malformation who were recruited between September 28, 2015, and March 22, 2018, in 11 French tertiary hospital centers. Statistical analysis was performed on an intent-to-treat basis from December 4, 2019, to November 10, 2020. INTERVENTIONS: Patients underwent an observational period, then switched to an interventional period when they received oral sirolimus (target serum levels, 4-12 ng/mL). The switch time was randomized from month 4 to month 8, and the whole study period lasted 12 months for each patient. MAIN OUTCOMES AND MEASURES: The primary outcome was change in the volume of vascular malformations detected on magnetic resonance imaging scan (with centralized interpretation) per unit of time (ie, between the interventional period and the observational period). Secondary outcomes included subjective end points: pain, bleeding, oozing, quality of life, and safety. RESULTS: Among the participants (35 girls [59.3%]; mean [SD] age, 11.6 [3.8] years), 22 (37.3%) had a pure venous malformation, 18 (30.5%) had a cystic lymphatic malformation, and 19 (32.2%) had a combined malformation, including syndromic forms. Variations in the volume of vascular malformations detected on magnetic resonance imaging scans associated with the duration period were not overall significantly different between the interventional period and the observational period (all vascular malformations: mean [SD] difference, -0.001 [0.007]; venous malformations: mean [SD] difference, 0.001 [0.004]; combined malformations: mean [SD] difference, 0.001 [0.009]). However, a significant decrease in volume was observed for children with pure lymphatic malformations (mean [SD] difference, -0.005 [0.005]). Overall, sirolimus had positive effects on pain, especially for combined malformations, and on bleeding, oozing, self-assessed efficacy, and quality of life. During sirolimus treatment, 56 patients experienced 231 adverse events (5 serious adverse events, none life-threatening). The most frequent adverse event was an oral ulcer (29 patients [49.2%]). CONCLUSIONS AND RELEVANCE: This observational-phase randomized clinical trial allows for clarifying the goals of patients and families when starting sirolimus therapy for children older than 6 years. Pure lymphatic malformations seem to be the best indication for sirolimus therapy because evidence of decreasing lymphatic malformation volume per unit of time, oozing, and bleeding and increasing quality of life was found. In combined malformations, sirolimus significantly reduced pain, oozing, and bleeding. Benefits seemed lower for pure venous malformations than for the 2 other subgroups, also based on symptoms. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT02509468; clinicaltrialsregister.eu Identifier: 2015-001096-43.

Duration of mechanical ventilation is more critical for brain growth than postnatal hydrocortisone in extremely preterm infants.

Hydrocortisone is used in preterm infants. However, early disruption of growth velocities was observed in infants exposed to hydrocortisone. This retrospective study aimed to explore the postnatal brain growth of extremely preterm infants requiring hydrocortisone treatment as well as its association with perinatal factors. Extremely preterm infants exposed to hydrocortisone from 2011 to 2016 who survived up to 12 months were included. Each of them was matched with two infants not treated with hydrocortisone exhibiting similar gestational ages and nearly similar birth head circumferences. The outcome variables were brain tissue areas on MRIs performed at term-equivalent age and postnatal head circumference growth up to a corrected age of 12 months. Univariate and multiple regression analyses were performed. Infants treated with hydrocortisone (n=20) were matched with 40 infants not exposed to hydrocortisone. The infants exposed to hydrocortisone exhibited a lower birth weight (p=0.04) and a longer duration of mechanical ventilation (p<0.0001). Infants treated with hydrocortisone exhibited a smaller basal ganglia/thalamus area (p=0.04) at term-equivalent age and a smaller head circumference at a corrected age of 12 months (p=0.003). However, the basal ganglia/thalamus area and the postnatal brain growth were independently associated with the duration of mechanical ventilation and not with hydrocortisone. Interestingly, a significant interaction between hydrocortisone and sex was observed (p=0.04).Conclusion: This study supports previous data that indicated no obvious impact of hydrocortisone on brain growth and highlights the relationship between the severity of the neonatal course and postnatal brain growth in extremely preterm infants. What is Known: • Postnatal hydrocortisone disrupts transiently growth velocities including the head circumference growth. • Postnatal hydrocortisone has less impact on neurodevelopment than dexamethasone. What is New: • Hydrocortisone prescribed for infants in the most severe conditions did not show independent effect on brain growth up to the corrected age of 12 months. However, a different effect of hydrocortisone according to sex can't be excluded and needs further explorations. • Perinatal factors as birth weight and duration of mechanical ventilation were determinant for the subsequent brain growth.

Neuromuscular, Psychological, and Sleep Predictors of Cancer-Related Fatigue in Cancer Patients.

BACKGROUND: Cancer-related fatigue (CRF) is the most reported side effect of cancer and its treatments. This distressing sense of exhaustion critically impairs quality of life and can persist for years after treatment completion. Mechanisms of CRF are multidimensional (eg, physical, psychological, or behavioral), suggesting the need for a complex assessment. Nevertheless, CRF remains assessed mainly with 1-dimensional questionnaires. The purpose of this study was to test whether neuromuscular parameters enhance a model including well-known predictors of CRF. PATIENTS AND METHODS: Forty-five participants with cancer history completed self-assessment questionnaires about quality of life, CRF, sleep disturbances, and emotional symptoms. They also completed a 5-minute handgrip fatiguing test composed of 60 maximal voluntary contractions to assess neuromuscular fatigability. Hierarchical linear regression analyses were performed to determine whether the neuromuscular fatigability threshold improved the FA12 score prediction beyond that provided by anxiety/depression and sleep disturbances. RESULTS: The hierarchical linear regression analysis evidenced that a model including anxiety/depression, sleep disturbances, and neuromuscular fatigability explained 56% of CRF variance. In addition, the results suggest that the mechanisms leading to CRF may be different from one person to another. CONCLUSION: Results revealed that sleep disturbances, emotional symptoms, and neuromuscular fatigability were the most important CRF predictors in cancer patients. This information could be useful for healthcare professionals offering tailored, individual support to patients with CRF.

Normal volumetric and T1 relaxation time values at 1.5 T in segmented pediatric brain MRI using a MP2RAGE acquisition.

OBJECTIVES: This study introduced a tailored MP2RAGE-based brain acquisition for a comprehensive assessment of the normal maturing brain. METHODS: Seventy normal patients (35 girls and 35 boys) from 1 to 16 years of age were recruited within a prospective monocentric study conducted from a single University Hospital. Brain MRI examinations were performed at 1.5 T using a 20-channel head coil and an optimized 3D MP2RAGE sequence with a total acquisition time of 6:36 min. Automated 38 region segmentation was performed using the MorphoBox (template registration, bias field correction, brain extraction, and tissue classification) which underwent a major adaptation of three age-group T1-weighted templates. Volumetry and T1 relaxometry reference ranges were established using a logarithmic model and a modified Gompertz growth respectively. RESULTS: Detailed automated brain segmentation and T1 mapping were successful in all patients. Using these data, an age-dependent model of normal brain maturation with respect to changes in volume and T1 relaxometry was established. After an initial rapid increase until 24 months of life, the total intracranial volume was found to converge towards 1400 mL during adolescence. The expected volumes of white matter (WM) and cortical gray matter (GM) showed a similar trend with age. After an initial major decrease, T1 relaxation times were observed to decrease progressively in all brain structures. The T1 drop in the first year of life was more pronounced in WM (from 1000-1100 to 650-700 ms) than in GM structures. CONCLUSION: The 3D MP2RAGE sequence allowed to establish brain volume and T1 relaxation time normative ranges in pediatrics. KEY POINTS: • The 3D MP2RAGE sequence provided a reliable quantitative assessment of brain volumes and T1 relaxation times during childhood. • An age-dependent model of normal brain maturation was established. • The normative ranges enable an objective comparison to a normal cohort, which can be useful to further understand, describe, and identify neurodevelopmental disorders in children.

Posterior fossa tumors in children: Radiological tips & tricks in the age of genomic tumor classification and advance MR technology.

Imaging plays a major role in the comprehensive assessment of posterior fossa tumor in children (PFTC). The objective is to propose a global method relying on the combined analysis of radiological, clinical and epidemiological criteria, (taking into account the child's age and the topography of the lesion) in order to improve our histological approach in imaging, helping the management and approach for surgeons in providing information to the patients' parents. Infratentorial tumors are the most frequent in children, representing mainly medulloblastoma, pilocytic astrocytoma and brainstem glioma. Pre-surgical identification of the tumor type and its aggressiveness could be improved by the combined analysis of key imaging features with epidemiologic data.

Detection of recurrent brain tumors in children: No significant difference in sensitivity between unenhanced and contrast-enhanced MRI.

BACKGROUND: Magnetic resonance imaging (MRI) with a gadolinium injection is currently used in the follow-up of children in remission of cerebral tumors (CTs). Intracerebral gadolinium deposition has been recently reported with unknown risks. The aim of this study was to evaluate the sensitivity of unenhanced brain MRI (U-MRI) in detection of tumor recurrence. METHODS AND MATERIALS: A set of 58 U-MRIs of children in remission was retrospectively evaluated by three seniors (a neuroradiologist, a pediatric and a general radiologist) and one junior to look for any recurrence. Clinical, tumoral and imaging data were collected. The final diagnosis was anatomopathological when available, or the clinicoradiological evolution. Sensitivity, specificity, predictive values and interobserver agreement were calculated. A Fisher test and Fleiss kappa coefficient were performed. RESULTS: For the seniors, the U-MRI had a sensitivity of 81% (95% confidence interval (CI): 0.56-0.90), and a negative predictive value (NPV) of 82% (95% CI: 0.63-0.94). The U-MRI sensitivity, regardless of the observer, was not significantly different from the contrast-enhanced MRI sensitivity (86%) according to a Fisher test (p > 0.05). No significant difference in sensitivity within the subgroups was found. The interobserver agreement of seniors was good (κ = 0.68). CONCLUSION: U-MRI brain was suboptimal for 80% of patients. Three-dimensional millimetric, fluid-attenuated inversion recovery, and diffusion would constitute helpful sequences in follow-up. Further specific studies depending on each tumor type are still required to determine whether a potential abstention of gadolinium intravenous injection should be discussed for children.

Treatment of voluminous and complicated superficial slow-flow vascular malformations with sirolimus (PERFORMUS): protocol for a multicenter phase 2 trial with a randomized observational-phase design.

BACKGROUND: Slow-flow superficial vascular malformations (VMs) are rare congenital anomalies that can be responsible for pain and functional impairment. Currently, we have no guidelines for their management, which can involve physical bandages, sclerotherapy, surgery, anti-inflammatory or anti-coagulation drugs or no treatment. The natural history is progressive and worsening. Mammalian target of rapamycin (mTOR) is a serine/threonine kinase that acts as a master switch in cell proliferation, apoptosis, metabolism and angio/lymphangiogenesis. Sirolimus directly inhibits the mTOR pathway, thereby inhibiting cell proliferation and angio/lymphangiogenesis. Case reports and series have reported successful use of sirolimus in children with different types of vascular anomalies, with heterogeneous outcomes. OBJECTIVE: The objective of this trial is to evaluate the efficacy and safety of sirolimus in children with complicated superficial slow-flow VMs. METHODS/DESIGN: This French multicenter randomized observational-phase, phase 2 trial aims to include 50 pediatric patients 6 to 18 years old who have slow-flow (lymphatic, venous or lymphatico-venous) voluminous complicated superficial VM. Patients will be followed up for 12 months. All patients will start with an observational period (no treatment). Then at a time randomly selected between month 4 and month 8, they will switch to the experimental period (switch time), when they will receive sirolimus until month 12. Each child will undergo MRI 3 times: at baseline, at the switch time, and at month 12. For both periods (observational and treatment), we will calculate the relative change in volume of the VM divided by the study period duration. This relative change weighted by the study period duration will constitute the primary endpoint. VM will be measured by MRI images, which will be centralized and interpreted by the same radiologist who will be blinded to the study period. Hence, each patient will be his/her own control. Secondary outcomes will include assessment of safety and efficacy by viewing standardized digital photographs and according to the physician, the patient or proxy; impact on quality of life; and evolution of biological makers (coagulation factors, vascular endothelial growth factor, tissue factor). DISCUSSION: The main benefit of the study will be to resolve uncertainty concerning the efficacy of sirolimus in reducing the volume of VMs and limiting related complications and the safety of the drug in children with slow-flow VMs. This trial design is interesting in these rare conditions because all included patients will have the opportunity to receive the drug and the physician can maintain it after the end of the protocol if is found efficient (which would not be the case in a classical cross-over study). TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT02509468 , first received: 28 July 2015. EU Clinical Trials Register EudraCT Number: 2015-001096-43.

Variable prenatal presentation of Pfeiffer syndrome: Suggested aids to prenatal sonographic diagnosis.

BACKGROUND: Our purpose was to describe and compare the cranial and extracranial abnormalities of Pfeiffer syndrome on prenatal imaging with postnatal or postmortem findings, which may help in prenatal diagnosis of Pfeiffer syndrome (PS). METHODS: Cases of fetuses with a confirmed diagnosis of PS over a 4-year period (2012-2016) were retrospectively reviewed. Prenatal imaging findings, postnatal, or postmortem investigations and genetic test results were analyzed. RESULTS: Four fetuses were ascertained, 3 with prenatal sonographic findings compatible with PS and one only diagnosed at postmortem. Cases were referred between 22 and 24 weeks' gestation. Three of the 4 cases were terminated, and details of postmortem/postnatal examination were available in all. There was variable presentation of features. Craniosynostosis was present in 3 cases, but only detected prenatally in 2. Extracranial signs included abnormalities of thumbs and/or big toes, detected prenatally in 3 of the 4 cases. A sacral appendage and vertebral or coronal clefts were present at postmortem in 3 cases but only detected prenatally in one. A cartilaginous tracheal sleeve was detected at postmortem in all 3 cases but not detected by prenatal ultrasound. Other findings included ventriculomegaly, posterior fossa, and facial anomalies. Molecular testing revealed mutations of the fibroblast growth factor receptor 2 (FGFR2) gene in all cases. CONCLUSION: Pfeiffer syndrome has a highly variable phenotype, and the absence of craniosynostosis on prenatal US does not exclude the diagnosis. Presence of abnormal thumbs and big toes, a sacral appendage, vertebral fusions, and coronal clefts should lead to prenatal molecular testing for PS.

Laparoscopic Pyloromyotomy: A Study of the Learning Curve.

INTRODUCTION: Laparoscopic pyloromyotomy (LPM) is a minimally invasive surgical technique used in pyloric stenosis treatment. This technique is safe, effective, and does not show more complications than laparotomy. Nevertheless, this technique requires an acquisition period to be optimally applied. This study analyses the learning curve of LPM. MATERIALS AND METHODS: Seven surgeons were retrospectively evaluated on their 40 first LPM. Patient data were recorded, including peroperative data (operation length and complications) and postoperative recoveries (renutrition, vomiting, and complications). The learning curves were evaluated and each variable was compared with the different moments of the learning curve. RESULTS: The mean operative time is 25 ± 11 minutes. It significantly decreases with the learning curve (p < 0.01). Ten procedures were necessary to acquire the operative technics. However, postoperative complications with a necessary redo procedure appear after the 10th patient. There is no significant difference concerning long-term postoperative complications according to the learning curve and to surgeons. The best results are recorded after the 20th patients. Hospital length of stay also decreases significantly after the 10th procedure. The recorded postoperative vomiting is independent to the operative time as the ad libitum feedings recovery. CONCLUSION: The learning curve of LPM is cut into three stages. Only 10 cases are needed to acquire the gesture. Complications appear after this acquirement period.

Polyurethane versus silicone port a cath: What's going on at removal?

BACKGROUND: Port-a-cath (PAC) is an essential device in the management of the patients of chronic illness, but despite theirs benefits there are many complications either at the time of insertion or at time of removal. Our aim of this study is to evaluate the fracture rate of the catheter at removal time in comparison with catheter type either polyurethane or silicone. METHODS: A retrospective monocentric study of all PACs which were removed at our university pediatric hospital between 1 January 2006 and 31 December 2016. Two groups were compared: polyurethane group and silicone group. RESULTS: Total of 216 central lines were removed, the mean age at the time of extraction was 9.7±4.9years and the mean time for both catheter was 2.7±1.6years, fracture occurred in 11 catheter of the polyurethane group (n=119), with no fracture of silicone group (n=86), in the polyurethane group, the risk of catheter fracture is significantly related to the duration of the PAC in place. CONCLUSION: We found that the polyurethane-based catheters are more vulnerable for rupture and retained fragment in the blood vessels, especially if left in place for long time, for this reason we have switched to silicone-based catheter and all catheters should be remove within duration maximal of 2years. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level II.

Sonography of suspected acute appendicitis in children: Evaluation of the progress in performance of senior residents.

PURPOSE: The objective of this study was to evaluate the progress in performance of senior residents in diagnosing acute appendicitis. MATERIAL AND METHODS: Results were collected and compared of ultrasound examinations performed for suspected acute appendicitis by three senior residents and two faculty members over a six-month period in a university hospital setting. A grid with the sonographic findings was completed separately by the residents and the faculty members immediately after each examination. The duration of each examination was reported. The final ultrasound diagnosis was compared to the surgical and pathological results and to the clinical follow-up. RESULTS: The residents and faculty members performed 171 consecutive ultrasound examinations including 49 children with acute appendicitis and 122 with normal appendices. The accuracy of the diagnosis by the residents was 96%, and was similar to that of the faculty members (kappa=0.90) over the six months. The duration of the resident ultrasound examinations was significantly shorter during the second three-month period (p=0.01). No significant differences in diagnostic accuracy were demonstrated by the residents between the first and second three-month periods (p=0.06). CONCLUSIONS: The residents performed well when using sonography to diagnose acute appendicitis in children, and were faster during the second three-month period. LEVEL OF EVIDENCE: I.

Variability in Imaging Practices and Comparative Cumulative Effective Dose for Neuroblastoma and Nephroblastoma Patients at 6 Pediatric Oncology Centers.

The purpose of this study was to estimate the cumulative effective dose (CED) from diagnosis and posttherapy computed tomographic (CT) scans performed on children treated for neuroblastoma or nephroblastoma (Wilms tumor) and to examine the different imaging practices used in 6 regional pediatric oncology centers between January 2010 and December 2013. We analyzed retrospectively the CT scan acquisition data in children aged 10 years or younger at diagnosis. The use of nonionizing imaging modalities was reported. The CT examinations of 129 children, with a mean age at diagnosis of 36 months, treated for 66 neuroblastomas and 63 nephroblastomas, were analyzed. The mean follow-up period was 28 months (minimum, 8 months, maximum, 41 mo). There were 600 CT scans, with a total of 1039 acquisitions. The mean CED from CT scans was 27 mSv (minimum=18.25, maximum=45). Abdominal CT examinations contributed 85% of the total CED. A median of 4.6 CT scans, 10.3 sonograms, and 0.4 magnetic resonance imaging examinations per child were performed. Our results suggest a reduction in radiation exposure but variability in the imaging modality choice and acquisition protocols. We emphasize the need for consensus and standardization in oncologic pediatric imaging procedures. When feasible, we encourage the substitution of nonionizing examinations for CT.

Prenatal Sacral Anomalies Leading to the Detection of Associated Spinal Cord Malformations.

INTRODUCTION: Systematic analysis of the spine is recommended as part of the basic sonographic examination. The aim of our study is to assess the proportion of spinal cord anomalies diagnosed following detection of a sacral anomaly. MATERIAL AND METHODS: We analyzed retrospectively collected data in a prenatal tertiary center during a 9-year period. Patients were referred for second-line ultrasound in the context of diabetes mellitus or following detection of pelvic or lower spine anomalies. We included all cases of sacral anomalies with available postnatal or postmortem outcomes (imaging and/or pathologic data) and excluded all cases of open dysraphism (myelomeningocele). RESULTS: Nineteen patients were included. The mean gestational age was 28 weeks (21-39). Sacral anomalies included 2 cases of complete agenesis, 12 cases of partial agenesis, 4 segmentation anomalies, and 1 case of abnormal angulation of the sacral spine. Fourteen associated spinal cord malformations included 8 tethered spinal cords, 5 truncated spinal cords, and 1 lipoma of the filum terminale. All anomalies were confirmed by postnatal or postmortem examinations. CONCLUSIONS: Sacral anomalies detected during basic sonographic examination represent an important warning sign for associated spinal cord anomalies, with possible poor neonatal outcome.