Marchand I.,University of Paris Descartes |
Garel C.,Service de Radiologie Pediatrique |
Polak M.,University of Paris Descartes |
Donadieu J.,Service de Radiologie Pediatrique
Journal of Clinical Endocrinology and Metabolism | Year: 2011
Context: Isolated central diabetes insipidus (CDI) can be the first manifestation of Langerhans cell histiocytosis (LCH), creating diagnostic dilemmas such as dysgerminoma and other inflammatory lesions. Method: In 2010, the French national LCH registry had enrolled 1236 LCH patients under 18 yr of age. Isolated CDI was the initial presentation of LCH in 26 patients. We reviewed their clinical and magnetic resonance imaging (MRI) features. Results: Median age at the diagnosis of CDI was 9.6 yr (1.8 -16.3), and median follow-up after CDI diagnosis was 9.9 yr (3.5-26.6). In addition to CDI, two patients had visual field defects, four had secondary amenorrhea, and 11 had anterior pituitary deficiency. Cerebral imaging (including computed tomography in two cases), performed in 22 patients within 3 months of CDI diagnosis, showed pituitary stalk thickening in 14 patients, which was moderate (3.0-7 mm) in nine cases and marked (>7 mm) in five cases. In eight cases, the lesion extended to the floor of the third ventricle. One child with LCH presented with a mild enlarged sellar content. During follow-up, 22 patients developed extrapituitary involvement, mainly of bone (n = 15), lung (n = 9), and skin (n = 9). Pituitary biopsy was performed in eight cases and was conclusive in six cases. Conclusions: Pituitary stalk thickening can be observed in LCH as well as lesions extending to the floor of the third ventricle. In all cases but one, the intrasellar content was not enlarged. Long-term follow-up with close attention to bone, skin, and lung disorders may lead to the diagnosis of LCH. Copyright © 2011 by The Endocrine Society.
PubMed | Unite de genetique clinique, Service de radiologie pediatrique, Unite de cytogenetique constitutionnelle and Service de chirurgie pediatrique
Type: Journal Article | Journal: European journal of medical genetics | Year: 2016
Terminal 7q deletion is rarely reported in the literature. Holoprosencephaly and sacral dysgenesis are found in association with this deletion, due to haploinsufficiency of SHH and HLBX9 genes respectively. We report on a 2-year-old boy with 7q35-36.3 deletion encompassing SHH identified by oligonucleotide array comparative genomic hybridization. In addition to other frequent features, the patient presented with esophageal atresia and tracheoeosophageal fistula diagnosed at birth. This case, together with two others previously described, one presenting with esophageal atresia, the other with congenital esophageal stenosis, confirms the possible association between congenital esophageal malformations and 7q terminal deletion including SHH.
PubMed | University Paris - Sud, Service de radiologie pediatrique, Service de Gynecologie Obstetrique et Diagnostique antenatal, Reanimation pediatrique and 3 more.
Type: | Journal: Annals of neurology | Year: 2017
While vein of Galen aneurysmal malformations (VGAM) can be diagnosed in the fetus, the challenge is predicting the occurrence of its two major complications: cardiopulmonary failure and encephalomalacia. This study attempts to determine which fetal brain MRI features might be used to predict the development of these complications at birth.The cohort was extracted from a prospectively-assembled database of VGAM cases managed at a single referral centre from 2000 to 2014. Of 251 patients with VGAM, 83 cases were diagnosed prenatally. A total of 58 patient charts having at least one fetal MRI were reviewed. Patterns of brain parenchyma, hydrocephalus and so-called middle cerebral artery (MCA) pseudo-feeders were correlated with cardiac failure, pulmonary hypertension and encephalomalacia at birth.The median gestational age at fetal MRI was 32.3 WP (2.3). Nine fetuses (16%) had encephalomalacia. Thirty-one fetuses (53%) had MCA pseudo-feeders. Twenty-six fetuses (45%) had prenatal hydrocephalus. Prenatal MCA pseudo-feeders were a risk factor for encephalomalacia at birth (p=0.001). MCA pseudo-feeders and hydrocephalus were risk factors for both severe cardiac failure (p=0.01 and p=0.04, respectively) and severe pulmonary hypertension (p=0.014 and p=0.05, respectively) at birth.MCA pseudo-feeders are the result of impaired cerebral blood flow, and are thus a risk factor for further brain-melting at birth. Their presence can be used for informing parents and as an aid in management decisions. This article is protected by copyright. All rights reserved.
Fuchsmann C.,Service dOtorhinolaryngologie Pediatrique |
Fuchsmann C.,University Claude Bernard Lyon 1 |
Quintal M.-C.,University of Montréal |
Giguere C.,University of Montréal |
And 7 more authors.
Archives of Otolaryngology - Head and Neck Surgery | Year: 2011
Objectives: To report the efficacy of propranolol as first-line treatment of head and neck hemangiomas in children and to present an optimized protocol for treating hemangiomas. Design: Multi-institutional retrospective study. Setting: Two tertiary care referral pediatric centers. Patients: Thirty-nine children with head and neck infantile hemangiomas were treated. Main Outcome Measures: Review of clinical records. Results: Propranolol was the sole treatment in 60% of patients and was started at a mean age of 4.1 months (age range, 1-11 months) for early interventions among 33 of 39 patients. Propranolol therapy resulted in lightening and reduction of hemangiomas at 37 of 39 locations within 2 days to 2 weeks. One subglottic hemangioma and 1 nasal tip hemangioma did not respond or showed only a partial response; in these patients, propranolol therapy was delayed and followed other treatment failures. After successful therapeutic regression, 6 recurrences occurred; when reintroduced, propranolol was again effective. Recurrences were avoided by prolonged treatment. Twenty-six hemangiomas occurring at locations for which corticosteroid treatment previously would not have been initiated (nose, lips, and parotid area) unless a complication had occurred were treated with propranolol and were rapidly controlled. The mean duration of propranolol therapy was 8.5 months. No instances of β-blocker discontinuation because of complications occurred, but propranolol was substituted by acebutolol in 5 patients because of trouble sleeping. Conclusions: Propranolol is an effective treatment of head and neck infantile hemangiomas, especially when started early within the rapid growth phase, and is first-line treatment of orbit and larynx hemangiomas. The efficacy and tolerability of propranolol led us to treat some hemangiomas in patientswhomwe previously would have observed rather than subject to corticosteroid therapy. Relapse was avoided if treatment was prolonged after theoretical involution (age 12 months). Questions remain about optimal dosing and age at treatment cessation. ©2011 American Medical Association. All rights reserved.
Scalabre A.,Service de Chirurgie Pediatrique |
Gorincour G.,Service de Radiologie Pediatrique |
Hery G.,Service de Chirurgie Pediatrique |
Gamerre M.,Hopital de la Conception |
And 2 more authors.
Journal of Pediatric Surgery | Year: 2012
Objective: The objective of this study is to describe the evolution of 8 cases of congenital malformations of the umbilical-portal-hepatic venous system diagnosed before the first month of life. Materials and methods: All cases of congenital malformation of the portal and hepatic venous system diagnosed prenatally or during the first month of life in our institution were systematically reviewed since November 2000. Clinical features, imaging, and anatomical findings were reviewed, focusing primarily on clinical and radiologic evolution. Results: Eight cases of congenital malformation of the umbilical-portal-hepatic venous system were studied. Fifty percent of these malformations were diagnosed prenatally. We report 4 portosystemic shunts. Three involuted spontaneously, and the fourth one required surgical treatment. We report a variation of the usual anatomy of portal and hepatic veins that remained asymptomatic, an aneurysmal dilatation of a vitelline vein causing portal vein thrombosis that needed prompt surgical treatment with good result, a complex portal and hepatic venous malformation treated operatively, and a persistent right umbilical vein that remained asymptomatic. Conclusion: Prenatal diagnosis of malformations of the umbilical-portal-hepatic venous network is uncommon. Little is known about the postnatal prognosis. Clinical, biologic, and radiologic follow-up by ultrasonography is essential to distinguish pathologic situations from normal anatomical variants. © 2012 Elsevier Inc. All rights reserved.
Fabre A.,Service de Pediatrie Multidisciplinaire |
Petit P.,Service de Radiologie Pediatrique |
Gaudart J.,Aix - Marseille University |
Mas E.,Unite de Gastroenterologie |
And 3 more authors.
Journal of Pediatric Gastroenterology and Nutrition | Year: 2012
Severity scores are used to predict the outcome of acute pancreatitis (AP). Several scores are used in adult patients, but none has been thoroughly validated for specific use in paediatric patients. We retrospectively collected data from 48 children with AP (13 severe and 35 mild). The main causes were trauma (23%), idiopathic (23%), lithiasis (12.5%), and virus (10.5%). We evaluated 3 clinical scores (Ranson, Glasgow modified, and DeBanto) and Balthazar computed tomography severity index. The clinical scores had a good specificity (approximately 85%) but a low sensitivity (approximately 55%) in predicting the severity of paediatric AP. The radiological score is better (sensitivity 80%, specificity 86%). The area under the receiver operator characteristic curve was 0.699 (95% CI 0.508%-0.891%, P=0.054) for the DeBanto score, 0.846 (95% CI 0.69%-1%, P=0.001) for the Ranson score, and 0.774 (95% CI 0.584%-0.964%, P=0.008) for the Glasgow and 0.898 (95% CI 0.73%-1%, P=0.011) for the Balthazar computed tomography severity index score. In our paediatric cohort, the severity of AP was best predicted by Balthazar computed tomography-based scoring scale. Our results confirm previously reported low sensitivity of adult-based clinical scoring scales. © 2012 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.
Veyrac C.,Service de Radiologie Pediatrique |
Blondiaux E.,Service de Radiologie Pediatrique |
Hadjigeorgi C.,Childrens Hospital Aghia Sophia
Pediatric Radiology | Year: 2011
Introduction: Abdominal injuries in abused children are less common than musculoskeletal and craniocerebral injuries; however they carry high mortality and morbidity rates. In every case of trauma, regardless of aetiology, radiologists are responsible for the documentation and evaluation of injuries. Injuries: Any abdominal injury pattern maybe observed following physical abuse and none is specific for abuse. However, a high index of suspicion should be maintained for every case of pancreatic, hollow viscous and other solid organ injuries, especially when there is delay in seeking help, a history of trauma to the child or siblings, young age, undernourishment, ecchymosis in non-ambulatory children or a non-plausible explanation for the injuries based on the provided history and the psychomotor condition of the child. Imaging modalities: CT with intravenous contrast material is the imaging modality of choice in every suspected inflicted abdominal injury. US could be the first imaging test for abused children with a low probability of abdominal injury and for follow-up. Upper gastrointestinal series could reveal acute or resolving mural haematomas in children with equivocal CT or US findings. Conclusion: Child abuse should be considered in the differential diagnosis of acute abdominal symptoms in young children. © 2010 Springer-Verlag.
Cherradi Lachhab I.,Service de radiologie pediatrique |
Dafiri R.,Service de radiologie pediatrique
Archives de Pediatrie | Year: 2014
The os odontoideum, or mobile odontoid apophysis, is a malformation of the cervico-occipital hinge caused by missing unity in the ossification center of the dens on the body of the axis. This malformation induces atloaxial instability and exposes the subject to the risk of bulbar-medullary compression. The true incidence of this condition is difficult to determine because many cases are asymptomatic. The pathogenesis is discussed. Congenital and traumatic theories have been advanced. Clinically, the defect can be discovered incidentally or revealed by neck pain, a stiff neck, dizziness, or deficient syndrome. CT and MRI imaging can detect the defects, and allow one to study the impact on the contents of the spinal canal and detect lesions. We report two new os odontoideum observations in children aged 5 and 14. years who were referred for equilibrium disorders and tetraparesis, respectively. © 2014 Elsevier Masson SAS.
Couture A.,Service de Radiologie Pediatrique |
Baud C.,Service de Radiologie Pediatrique |
Prodhomme O.,Service de Radiologie Pediatrique |
Saguintaah M.,Service de Radiologie Pediatrique |
Veyrac C.,Service de Radiologie Pediatrique
Journal de Radiologie | Year: 2011
Developmental dysplasia of the hip can arise in utero due to a dislocating posture, sometimes associated with predisposing genetic factors. The ideal time for diagnosis is during the neonatal period and adequate screening procedures must be in place. Indeed, the plasticity of hyaline cartilage and fibrocartilage combined with the growth potential at this age nearly always result in rapid complete resolution of the deformity. Ultrasound, when indicated, is the best imaging modality for diagnostic confirmation. It allows evaluation of the osteocartilaginous structures, joint space and soft tissues. Ultrasound provides the clinician with a reliable morphologic and dynamic evaluation tool improving the diagnostic accuracy and guiding orthopedic treatment. Our experience, dating back to 1985, is based on a population imaged between 2007 and 2009. From a total of 2480 neonates screened because of abnormal finding or risk factors, we identified 257 cases of dislocation (10%) in 191 neonates: 14 cases of nonreducible dislocation (10 neonates), 30 cases of reducible hip dislocation (24 neonates), 97 cases of dislocatable hip (73 neonates) and 116 cases of subluxable hip (84 neonates). Clinical and sonographic follow-up demonstrated therapeutic success in 237 cases (93%) and failure in 20 cases (one case of subluxable hip, two cases of dislocatable hip, three cases of dislocated hip, 14 cases of nonreducible hip dislocation). Imaging follow-up (6 to 24months) showed asymmetry in the size of the proximal femoral epiphyses in 20 cases (with resolution in 10 cases), three cases of dysplasia and one case of post-reduction osteochondritis. © 2011 Elsevier Masson SAS.
PubMed | Service de Radiologie Pediatrique
Type: | Journal: The Pan African medical journal | Year: 2016
Benign bone tumors are more common than malignant tumors in pediatrics. The exostosis (ostchondrome) is the most common. The different imaging techniques are pivotal in the study of tumors including its standard radiography. The aim of this work is to highlight the interest in imaging the diagnostic management of bone benign tumors in children through a retrospective study of 169 patients. All patients were investigated by plain radiography, scanner supplement with multiplanar reconstruction before and after injection of PDC and / or MRI 1. 5 Tesla was performed according to the indication. The average age is 6 years with a slight male predominance. Clinically, the swelling is present in 35% of cases. The pain in 29% of cases. The most common location is the metaphyseal long bone on: Femur: 25% of cases, humerus: 17% of cases, Tibia: 21% of cases. Main Benign tumors are found exostosis (20. 12%), bone cyst (31. 95%) and osteoblastoma (16, 57%). The imagery is used to specify the topography and extension of the lesion in the bone provide arguments in favor of benign and sometimes in favor of the cause of the injury. The only standard radiograph often provides a diagnosis of certainty in some cases.