MRI and clinical differences between optic pathway tumours in children with and without neurofibromatosis.

The purpose of this study was to evaluate the value of MRI in studying optic pathway tumours associated with neurofibromatosis, and to look for potentially helpful criteria for the management of such lesions. This retrospective study included 14 children with neurofibromatosis type 1 (NF-1) as well as a lesion of the optic pathway. Clinical data and MRI findings were analysed with regard to location, structure and course of the tumours, and were compared with 13 optic pathway tumours in patients without NF-1. The median age of onset was 4.1 years. 11 patients with NF-1 were asymptomatic. In the NF-1 group, the optic nerves were involved in 10 cases without a cystic component at the time of diagnosis. In the non-NF-1 group, the tumour was located in the chiasma in 11 cases; 12 cases had a cystic component. 10 of the NF-1 group had no tumour progression over an average follow-up of 3.2 years without treatment. These findings suggest that optic astrocytomas in association with NF-1 are distinct lesions from isolated optic gliomas. In NF-1, most such tumours show only slight progression, and may correspond to perineural gliomatosis rather than a true pilocytic astrocytoma. Among NF-1 patients, initial MRI provides no prognostic criteria in children who subsequently show tumour progression. Nevertheless, MRI can be useful in establishing the diagnosis of NF-1 and can serve as a baseline study.

Bone lesions secondary to benign phaeochromocytoma. Four cases in childhood.

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.

Abdominal cystic lymphangiomas in children: presurgical evaluation with imaging.

Cystic lymphangiomas are benign vascular tumors which are most often seen in young children. They are considered to be congenital malformations stemming from sequestration of lymphatic tissue. The authors report 15 cases of abdominal location and detail the findings of imaging in the etiologic and topographic diagnosis of these lesions. The initial incidents were essentially the discovery of a palpable abdominal mass, and more rarely, an acute gastrointestinal complication. There was also one case of prenatal diagnosis. Plain films provide only indirect signs related to the displacement of neighboring organs. Ultrasonography permits the etiologic diagnosis by showing an often voluminous, septated cyst. The intra- or retroperitoneal location of the lesion is sometimes difficult to determine by sonography, in which case CT scanning is usually adequate. For abdominal locations, percutaneous sclerosis is not available, and surgical removal is the only treatment for this disorder. The topography of the lesion and the involvement of retroperitoneal structures is important to determine.

[Total agenesis of the corpus callosum: ultrasonographic appearances. In two cases]. / Agénésie complète du corps calleux: aspect échographique. Deux observations.

Transfontanellar ultrasonography in two neonates demonstrated total agenesis of the corpus callosum: this was confirmed by computed tomography in one case, this not being necessary in the other infant. Diagnostic signs on pneumoencephalography and computed tomography are described, and those observed on supplementary ultrasonography are outlined: absence of the corpus callosum normally observed on ultrasonography, and the radial disposition, in a sagittal section, of the sulci of the internal surface of the cerebral hemispheres, which appears to be specific to this type of malformation. A frontal section shows a very typical "bull's head" deformity of the ventricular system.

[Is the rings and arcs pattern of mineralization specific for chondroid matrix tumors?]. / Les calcifications annulaires sont-elles spécifiques des matrices tumorales cartilagineuses?

The rings and arcs pattern of mineralization is considered pathognomonic for cartilaginous tumors. The authors report four cases of osteoblastomas in which the presence of such a pattern of mineralization mistakenly lead to a diagnosis of cartilaginous tumor. These densities are not explained by the histologic findings.

[Ultrasonography appearance of recessive hepatorenal polycystic disease in children (author's transl)]. / Aspects échographiques des polykystoses hépato-rénales récessives chez l'enfant.

Six cases of recessive hepatorenal polycystic disease in infants and young children were explored by intravenous urography and ultrasonography. Sufficient specific ultrasonography findings for making a diagnosis were obtained. The kidneys are large and produce strong echos, with inversion of the normal strength of medullary when compared to cortical echos, and poor definition of the renal sinus. Strong echos are also obtained from the liver, especially in the juvenile forms, because of the large amount of fibrosis. Large intrahepatic biliary ectasias were discovered in a 3-year-old child.

[Circumscribed non-tuberculous osteomyelitis in children. Apropos of 31 cases]. / Les ostéomyélites circonscrites non tuberculeuses de l'enfant. A propos de 31 cas.

Over the last eight years (1975-1983) a total of 31 cases of circumscribed non-tuberculous osteomyelitis in children have been treated as against 21 cases of classical acute osteomyelitis, suggesting transformation of the mode of presentation of this disease. Lesions were located in the pelvis (8 cases), the calcaneum (3 cases) and the long bones (20 cases), the metaphysis being affected in the latter in 13 patients, the diaphysis in 4 and the epiphysis in three. Specific radiologic images for each location are defined. Clinical expression was subacute and signs of infection inconstant. Differential diagnosis is discussed in relation to each region involved, a positive diagnosis depending on clinical, biologic and radiologic findings, with the need for biopsy in case of doubt. The latter was necessary in 12 of the 31 children. Diagnosis was confirmed in all cases by the favorable outcome from treatment.

[Urethroperineal fistula in the left lateroposition (author's transl)]. / Fistule uréthro-périnéale latéralisée à gauche.

The authors describe a case of urethroperineal fistula in the lateropostion. It is a rare malformation, with a varied symptomatology, which requires radiological investigations for its morphological and topographical study. Surgical treatment varies as a function of the site and size of the fistula. Basing their comments on the studies by STEPHENS [12], the authors try to give an embryological explanation of this affection.

[Idiopathic achalasia of the esophagus in children. Report of 8 cases]. / Le méga-cesophage idiopathique de l'enfant. A propos de 8 observations.

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.

[Monostotic fibrous dysplasia of the calcaneus: two case reports]. / Dysplasie fibreuse monostotique du calcaneus: à propos de 2 cas.

The authors report two unusual cases of aggressive monostotic fibrous dysplasia involving the calcaneus. This lesion occurs on the 2th decade of life. The main symptom is talalgia. On plain films, differential diagnosis includes aggressive cystic or pseudocystic lesions of the calcaneus. On MR imaging, the tumor matrix is more suggestive of the diagnosis. Treatment of this aggressive form of fibrous dysplasia consists of a surgical curettage completed by cryotherapy and graft because of a high level of recurrence.

[Practical measurement of radiation dose in pediatric radiology: use of the dose surface product in digital fluoroscopy and for neonatal chest radiographs]. / Mesure pratique de l'irradiation en radiopédiatrie: utilisation du produit dose surface en fluorographie numérique et pour les radiographies pulmonaires néonatales.

PURPOSE: Control of radiation dose in pediatric radiology requires knowledge of the reference levels for all examinations. These data are useful for daily quality assessment, but are not perfectly known for some radiographic examinations. The purpose of our study was to evaluate the dose related to voiding cystourethrograms (VCUG), upper GI (UGI) and intravenous urography (IVU). Neonatal chest radiographs in the intensive care unit were also evaluated. MATERIAL AND METHODS: For examinations with contrast material (478VCUG, 220UGI, 80IVU), the children were divided in groups based on their weight, from 5 to 30 Kg. Measurements were performed using an ionization chamber and expressed with the dose-area product (DAP). For chest radiographs, a direct measurement of the entrance-skin dose was performed, with secondary calculation of the DAP. RESULTS: For VCUGs, the DAP ranged between 42.89cGy.cm2 and 125.41cGy.cm2. The range was between 76.43 and 150.62cGy.cm2 for UGIs and between 49.06 and 83.33cGy.cm2 for IVUs. For neonate chest radiographs, DAP calculations were between 0.29 and 0.99cGy.cm2. CONCLUSION: These values represent our reference doses. They allow continuous monitoring of our radiographic technical parameters and radiographic equipment and help to correct and improve them if necessary.

[Spinal localization of a solid aneurysmal cyst. Clinical, radiologic and histopathological aspects]. / Localisation rachidienne d'un kyste anévrysmal "solide". Aspects cliniques, radiographiques et histopathologiques.

An 8 year old child with a "solid" aneurysmal cyst of L4 posterior arch was investigated by straight films, bone scintigraphy and CT scans. The suggested diagnosis had been a benign tumor, probably an osteoblastoma. Initial histopathology results were in favor or a non ossifying fibroma or a chondromyxoid fibroma, this lacking concordance with the usual epidemiology of primary spinal tumors in children. A description is given of the histologic characteristics of this new histopathologic entity first described by Sanerkin in 1983.

[Contribution of ultrasonography to the study of retroperitoneal tumors in children (author's transl)]. / Apport de l'échographie dans l'étude des tumeurs rétro-péritonéales de l'enfant.

Ultrasonography investigations of retroperitoneal tumors in children were conducted in 25 cases: 11 nephroblastomas, 11 neuroblastomas, 2 ganglioneuromas, and 1 rhabdomyosarcoma. Ultrasonographic exploration of such tumors should include analysis of the mass: echostructure, limits, interfaces and dimensions, and analysis of its extension. The latter should determine the position in relation to the median line, the condition of the large vessels (inferior vena cava and aorta), the homolateral renal cavities and the contralateral kidney, and the presence or absence of glandular spread and hepatic metastases. The semiology of each of these elements is described and discussed, to determine their individual diagnostic value.

[Simple orthotopic ureteroceles in children]. / Urétérocèles orthotopiques simples de l'enfant.

A simple orthotopic ureterocele is a ureterocele developed on the terminal portion of a single ureter normally implanted into the bladder. This type of ureterocele is often bilateral and is less frequently observed in children than heterotopic ureteroceles complicating total duplication (27%-73%). The authors report six such cases, review their classical radiological signs, and emphasize three unusual findings:--A simple orthotopic ureterocele complicated by the presence of stones. --A prolapse of a ureterocele into the posterior urethra in a boy, with subsequent dysuria. --A simple orthotopic ureterocele in a blind ureter from a polycystic kidney, with a contralateral primary obstructive mega-ureter.

[Congenital toxoplasmosis and transfontanelle brain echography. Apropos of 8 cases observed in newborn infants and infants]. / Toxoplasmose congénitale et échographie encéphalique trans-fontanellaire. A propos de 8 cas observés chez des nouveau-nés et des nourrissons.

The undeniable value of brain ultrasound imaging in the detection of ventricular dilatation is emphasized, and a classification of intracranial calcifications proposed: hyperechogenic, nodular and irregular large images and those without a posterior shadow cone. Comparison between simple films and particularly computed tomography images was conducted in the cases studied.

[Nephroblastoma with egg-shell peripheral calcifications. Apropos of 3 cases]. / Les néphroblastomes avec calcifications périphériques "en coque". A propos de 3 observations.

Three cases of nephroblastoma presented "egg-shell" peripheral calcifications of pseudocystic appearance of radiology. These peripheral lesions are compared with the more commonly reported central calcifications. Their pathology is unknown and they lack prognostic significance of any importance. As for all nephroblastomas the diagnosis is based on straight abdomen images, intravenous urography, ultrasound and computed tomography. Differential diagnosis is basically from renal adenocarcinoma in children, the latter often exhibiting similar calcifications, the distinction between the two types of tumor depending on age of onset. These calcified lesions are non-specific findings and various diagnoses are discussed.

[Para-articular and intraosseous synovial cysts and articular mucoid cysts]. / Kystes synoviaux et kystes mucoïdes articulaires, para-articulaires et intra-osseux.

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.

[Retroperitoneal teratoma in children. Apropos of 2 cases]. / Les tératomes rétropéritonéaux de l'enfant. A propos de deux cas.

Retroperitoneal teratoma is infrequent (5% of all teratomas). The diagnosis is usually made within the first year of life. Two cases are reported. Case n. 1 is typical of mature benign teratoma with teeth, fat and water densities visible on the plain film of the abdomen. Cases n. 2 a malignant partly ossified immature teratoma. The ossification is in continuity of a congenital block of vertebral bodies L2 and L3; this association has not been reported in the literature. CT in both cases was contributive for delineation of the tumor but did not help to answer the question of malignancy or not; foeto-protein remains the only test to do that discrimination.

[Posterior hernia of a calcified disk in children. Apropos of 2 cases]. / Hernie postérieure d'un disque calcifié chez l'enfant. A propos de 2 observations.

Posterior herniation of a calcified disk was detected in two children. A 10-year-old boy presented with cervicobrachial neuralgia at the C7 level secondary to herniation of a calcified C6-C7 disk treated by surgery. A 13-year-old boy was seen with a post-traumatic painful stiffness in the thoracic region posteriorly, without neurologic complication, secondary to a herniated calcified T4-T5 disk. Surgery was not performed and the course was favorable with regression of clinical signs and spontaneous disappearance of the herniated calcified fragment. A literature review showed reports of 21 identical lesions, full details being provided in 9 cases. Epidemiologic data, clinical manifestations, diagnostic means and management of these patients are discussed.

[Agyria-pachygyria and pachygyria in children. Contribution of imaging]. / Agyries-pachygyries et pachygyries de l'enfant. Apport de l'imagerie.

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.