Biomedical subjects
Publications and source records attributed to F Diard.
The authors review the mechanism and imaging findings of intraarticular, juxtaarticular, and intraosseous ganglion cysts. They specially emphasize particular features according to the most frequent locations.
UNLABELLED: Sickle cell anemia is one of the qualitative hemoglobinopathies which are the most widespread in the world. In a retrospective study on 11 drepanocytary patients (10 SS and 1 SC) observed at the BORDEAUX children's hospital. 3 Processus were identified: Hemolysis: 3 hepatomegaly, 3 splenomegaly, 1 hematochromatosis and 4 pigmentary lithiasis; Thrombosis: 1 infarctus case of spleen, kidney, lung, 1 papillary necrosis and 1 brain vascular ischemic appearance; INFECTION: 1 spleen abscess and 1 pneumopathy. In 15% of instances, visceral appearances are allowed to discoverer sickle cell anemia. Hemolysis type appearances were currently observed (63.6%) like pigmentary lithiasis and hepatomegaly. The authors analyze the major radiological appearances of this hemoglobinopathy and recall the interest in knowing this disease even in low endemicity countries.
Drepanocytosis is one of the qualitative hemoglobinopathies which are the most widespread in the word. In a retrospective study on 16 drepanocytary patients observed at the Bordeaux Children's hospital, 7 patients showed medullar hyperplasia, 10 patients showed vaso-occlusive, and 4 patients showed infections. Medullar hyperplasia: bone demineralisation was the current appearance (47%), cortical thinning down (40.5%). They touch long bone. Thrombosis: bone demineralisation was the current appearance (86.3%) and touch long bone, 1 first appearance right femoral head infarct. Some particular appearances we found. 2 cases of "hand foot syndrome", 1 case ischemic aseptic arthritis and 1 case of vertebra "step staircase". Three salmonella pandiaphysite and 1 tuberculosis spondylodiscite were observed. The authors analyse the major radiological appearances of this hemoglobinopathy and recall the interest in knowing this disease even in low endemicity countries.
We report five adolescent girls with secondary breast tumours. All were imaged by US and the imaging findings have been analysed. Four girls had rhabdomyosarcoma as the primary malignant disease and one had leukaemia. US features were variable, but most of the cases showed heterogeneous nodules which were quite different from the usual benign lesions (fibroadenoma, abscess, cyst) encountered at this age. The metastases produced a variety of echo characteristics: masses with well-defined margins, hyperechoic foci and sonolucent or hypoechoic nodules with posterior attenuation or lacking posterior enhancement. One lesion was round and heterogeneous with a hyperechoic centre. These US findings in an adolescent girl suggest the need for fine-needle aspiration. Mammograms, when performed, never revealed microcalcifications and were quite difficult to analyse at this age, due to the dense glandular breast tissue.
Numerous diseases can lead to multilocular lesions of the liver. The authors report a rare pediatric case of hepatic granulomas due to Pasteurella multocida: a 7-year-old girl with chronic fever was investigated by sonography and CT scan, demonstrating mesenteric lymph node enlargement and numerous small hepatic lesions. After surgical biopsy, histopathology of the liver specimens showed pyogenic granuloma, with serologic testing positive for Pasteurella multocida. Treatment with a tetracycline and corticosteroids was successful. Pasteurella multocida infection, despite its habitual benign course, should be suspected among differential diagnoses of lymphogranulomatous affections with hepatic involvement. No case of liver and lymph node foci in a child has been previously described.
Abdominal wall defects include a broad spectrum of structural malformations with variable severity and prognosis. The purpose of prenatal ultrasound examination is to correctly diagnose and classify these malformations according to their localization (particularly their relation to the umbilical cord insertion), their contents, their size and associated malformations or karyotypic abnormalities. Based on this examination, two groups can be distinguished: gastroschisis or omphalocele (when the latter is isolated, in particular without karyotypic abnormalities) which can be surgically corrected at birth, and for which predictive criteria of outcome must be evaluated (vitality of herniated bowel, size and contents of omphalocele); severe malformations (ectopia cordis, cloacal exstrophy, Beckwith-Wiedemann syndrome, short umbilical cord, amniotic band syndrome) for which pregnancy termination could be proposed.
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Aneurysmal bone cysts in the skull vault are reported in two patients 7 and 9 years old, who presented with vomiting and headaches caused by raised intracranial pressure. CT showed the cystic lesion in the diploë, with predominantly inward expansion. Fluid-fluid levels were seen on both CT and MRI; the latter also showed bleeding within the cyst. MR angiography showed compression of the superior sagittal sinus in the second case.
A case of acute torsion of an accessory spleen is reported. In a 15-year-old girl was admitted to hospital with acute abdominal pain. Ultrasonography showed a round, hypoechoic, solid mass. Computed tomography demonstrated a low-density mass with peripheral enhancement after intravenous contrast medium. These findings suggest the presence of a capsule. At laparotomy, the patient was found to have torsion and infarction of an accessory spleen. Sonographic and computed tomographic findings are discussed.
Erdheim-Chester disease is a form of Histiocytosis which involves the adults and is distinct from Histiocytosis X. It is characterized by a constant diaphyseal and metaphyseal bone involvement predominating in the lower links. The diagnosis can readily be envisaged when the typical radiological findings are present. Bone involvement may be isolated and well tolerated, or can be associated with systemic involvement and a severe prognosis. We describe three cases of women aged 46, 50 and 73 years. One patient presented with isolated bone lesions, while the two others had a multiorgan localization. From the three cases and from an extensive review of the literature, we describe the spectrum of bone and visceral lesions that can be seen by imaging. The emphasis is put on lesions of the skeletal system, the retroperitoneum, the nervous system, and the pericardium. Furthermore, the relationships between Erdheim-Chester disease and Histiocytosis X are discussed.
An unusual case of infantile myofibromatosis with foci of calcifications was presented, with atypical age of onset and location. The radiological features of this tumor are not always evocative, and histological examination is necessary for diagnosis.
BACKGROUND: Lissencephaly (agyria-pachygyria) is a defect in migration of cerebral neurons resulting in failure of cortical gyri to develop. Progress in imaging techniques improves its diagnosis. POPULATION AND METHODS: The files of 17 patients (ten boys and seven girls), aged 7 months to 16 years, were retrospectively studied. The clinical picture consisted of mental retardation (17 patients), seizures (eight patients), facial dysmorphia (seven patients), axial hypotonia (four patients). CT scan was performed in 16 cases and MRI with T1 and T2 weighted images in all 17. RESULTS: The CT scan identified pachygyria in 12 cases. Cerebral calcifications were seen in four cases. MRI detected typical changes in all 17 cases: thickened cortex and gyri, loss of cortical white matter interdigitations, lack of operculisation of the sylvian fissure. Pachygyria was generalized (six patients) or localized (11 patients). Associated abnormalities were dysgenesis of corpus callosum in three patients, cerebellar hypoplasia in one, deep grey matter heterotopia in one; hypersignal of the white matter was identified on T2 weighted images in five patients. CONCLUSION: MR imaging permits precise analysis of abnormalities secondary to a defect in neuronal migration.
The majority of scoliosis (80%) and especially the idiopathic scoliosis are simply investigated by plain films of the entire spine with erect frontal and lateral views, and supine frontal view at the initial examination. Repeated erect frontal views are sufficient for the follow-up. The important radiation dose (average of 1.2 mG for the frontal view and 2.2 mG for the lateral views) imposes to reduce the frequency of these radiographs. This radiological study allows: to separate non structural and structural curves; the identification and the measurement of a structural curve, discussion for its etiology, appraisal of its progressiveness, discussion of the operative or non operative treatment, and radiological follow-up. Modern imaging (C.T, C.T myelography, MRI) is indicated only in very particular cases: idiopathic scoliosis with neurological symptoms, congenital scoliosis with structural vertebral abnormalities, especially these with posterior arch abnormalities, and progressive scoliosis in spinal dysplasiae. Painful scoliosis are unusual. If pain is present consideration should be given for diagnosis of an underlying lesion. In this case a relevant strategy using modern imaging becomes useful and will be discussed.
Having found three cases of ectasia of the umbilical recess in the antenatal period we decided to review the embryology and the ultrasound of the anatomy of the portal system. We consider that this is a normal anatomic variant of the portal system because the lesion is asymptomatic and regresses spontaneously.
Metaphyseal sclerotic bone changes associated with benign phaeochromocytoma are very rare in childhood. We report four cases, in each of which the radiographic changes returned to normal after removal of the tumour.
A 5-month-old boy presented with a right flank abdominal mass. Plain film of the abdomen showed a vertebral column within the mass. Surgery confirmed the diagnosis of fetus in fetu developed within an ectopic intraperitoneal right testicle. Fetus in fetu differs from teratoma by the presence of axial skeleton and organogenesis. When intratumoral axial skeleton is present, plain films are sufficient for the diagnosis.
Achalasia of the esophagus is rare in children. The authors report eight cases and emphasize two atypical clinical records: The first one is a 3 month-old infant with respiratory distress and "near miss" Sudden Death Infant Syndrome. The second one is a ten year-old boy with association of achalasia of the esophagus, ACTH insensibility and alacryma. Theses cases are discussed.
Pelizaeus-Merzbacher disease is a rare sex-linked leukodystrophy. Its metabolic basis has not been identified yet and diagnosis in mainly based on clinical findings and pathological investigation. Cerebral biopsy is not always performed because of lack of available therapy. Genetic counseling can be provided if the diagnosis is secured. Four cases are reported, in children with characteristic signs, who underwent magnetic resonance (MR) Imaging. MR Imaging appears to be more sensitive than computed tomography to visualize white matter disease: T1 weighted images suggest hypomyelination, in conformity with abnormal myelination. T2 weighted images show a non specific high signal in the white matter.