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Infection during pregnancy is common and the developing fetal brain is vulnerable to vertical transmission due to immaturity of the fetal immune system. Infection is a major cause of multiple organ abnormalities, including the neuraxis, due to the neurotropism of the infectious agents. This review sets out to give an overview of fetal infection, review the general principles of the nature and timing of the infectious insult with respect to outcomes, review the neuroimaging of infection by ultrasound and magnetic resonance imaging (MRI), and review the various pathogens involved, including the two most common, cytomegalovirus (CMV) and Toxoplasma, and also other common viral and nonviral infections.

We report the first case of sporadic hypochondroplasia diagnosed in utero by computed tomography (CT) three-dimensional (3D) imaging and molecular analysis at 38 weeks’ gestation. Prenatal sonographic examinations performed at 32 and 35 weeks’ gestation revealed a rhizomelic shortness of the long bones (femur and humerus) with macrocephaly. Based on these findings, a nonlethal form of skeletal dysplasia was suspected and a multislice CT imaging with 3D reconstruction was performed depicting skeletal abnormalities which suggested hypochondroplasia. The prenatal diagnosis was confirmed by DNA mutation analysis of the fibroblast growth receptor 3 gene.

Resume La radiographie du thorax constitue l'exploration de base des poumons et du mediastin chez l'enfant. La connaissance precise de l'anatomie est necessaire pour permettre une interpretation pertinente. La lecture du cliche comporte successivement l'analyse des structures osseuses, des tissus mous, du mediastin, des champs pulmonaires. La realisation d'une incidence de profil n'est pas systematique et doit etre faite « a la demande ». Les differents aspects en rapport avec le thymus doivent egalement etre connus, en particulier dans les 2 premieres annees de vie.

We report the 1st confirmed case of an extradural hematoma diagnosed in utero by ultrasonography and magnetic resonance imaging at 31 weeks’ gestation. No signs of intracranial hemorrhage were found. During pregnancy, the expecting mother suffered from sustained cough, and a blood sample test revealed acute pertussis. The presence of fetal hydrocephaly, brain parenchymal deviation, and other abnormalities suggested a poor outcome, and based on these findings pregnancy termination was recommended and carried out. Postmortem examination confirmed the diagnosis of an extradural hematoma in the posterior fossa.

Resumen En la edad adulta se observan escoliosis idiopaticas (o primarias) y escoliosis degenerativas que, en los casos tipicos, solo aparecen una vez finalizado el periodo de crecimiento. Si bien la frecuencia de las escoliosis idiopaticas graves disminuye debido a una mejor atencion medica, las escoliosis degenerativas aumentan a causa del envejecimiento de la poblacion. Desde el punto de vista anatomopatologico, las curvaturas principales idiopaticas se vuelven rigidas y las contracurvaturas o zonas de union se ven afectadas por los mecanismos de la artrosis. Las escoliosis degenerativas esencialmente lumbares se agravan poco desde el punto de vista angular, pero a menudo se complican con compresiones neurologicas. El envejecimiento natural muscular y neurologico agrava las lesiones osteoarticulares. El equilibrio sagital, actualmente bien estudiado teniendo en cuenta la posicion de la pelvis, de las caderas y de las rodillas, puede verse seriamente alterado con desplazamiento de la persona hacia delante. Las consecuencias clinicas son de orden estetico, y dolorosas con raquialgias de multiples causas; a veces se trata de complicaciones neurologicas, sobre todo en el marco de las escoliosis degenerativas; las complicaciones respiratorias son mucho menos frecuentes. En estos cuadros complejos se deben aplicar puntuaciones de evaluacion que tengan en cuenta la calidad de vida y la repercusion funcional. Desde el punto de vista radiologico, las radiografias en carga de pie y de perfil permiten medir la escoliosis y su repercusion en el plano sagital. Las radiografias de correccion ayudan a tomar la decision terapeutica, particularmente la decision de intervenir quirurgicamente. Se propone la radiculografia asociada con escaner o resonancia magnetica para identificar una causa compresiva en una eventual radiculalgia. Las pruebas de infiltracion radicular y la exploracion electrica realizada correctamente permiten localizar la radiculalgia. Desde el punto de vista terapeutico, el tratamiento conservador tiene siempre su papel: incluye el tratamiento medico aplicado a una lumbalgia comun, la rehabilitacion y, en los casos mas graves, la ortopedia conservadora. La cirugia, practicada tras un estudio anestesico completo y despues de informar claramente al paciente sobre sus ventajas y riesgos, incluye un tiempo de liberacion entre las vertebras anteriores y/o posteriores y una instrumentacion tambien anterior y/o posterior con injerto oseo. Las indicaciones dependen esencialmente de la edad del adulto, de la topografia de la deformacion y de los signos de alarma sobre los que se desee actuar (raquialgia, desequilibrio, radiculalgias). Los resultados deben evaluarse mediante puntuaciones que se encuentran en curso de validacion. La tasa de complicaciones es considerable; predominan la infeccion, la seudoartrosis y los dorsos planos postoperatorios, que pueden llevar a cirugias complejas de reintervencion con osteotomias.

Resumen Los sindromes neurocutaneos o facomatosis comprenden un grupo de enfermedades responsables de lesiones de tipo hamartomatoso o tumoral de los tejidos derivados esencialmente del neuroectodermo, aunque tambien de las otras hojas embrionarias. La mayoria de estas enfermedades son de origen genetico. Los estudios de imagen desempenan un papel capital en el diagnostico y la evaluacion de las lesiones. Las neurofibromatosis se dividen en dos tipos principales. La neurofibromatosis de tipo 1 asocia manchas «cafe con leche», lesiones del sistema nervioso y una afectacion osea displasica. El pronostico esta determinado por la aparicion de tumores cerebrales, en particular, de gliomas. Los tumores nerviosos perifericos suelen ser multiples y quiescentes, pero pueden evolucionar espontaneamente. Las lesiones del aparato locomotor estan dominadas por la afectacion raquidea y las lesiones fibrosas intraoseas. La neurofibromatosis de tipo 2 asocia un neurinoma bilateral del nervio cocleovestibular y otros tumores del sistema nervioso central. La esclerosis tuberosa o enfermedad de Bourneville se caracteriza por la existencia a nivel cerebral de tuberosidades corticales, nodulos subependimarios, calcificaciones y astrocitomas intraventriculares de celulas gigantes. Las otras lesiones son los quistes y angiomiolipomas renales y los rabdomiomas cardiacos. La angiomatosis de Sturge-Weber asocia un angioma plano del trigemino, un angioma coroideo y un angioma pial, responsable de una atrofia secundaria del parenquima cerebral subyacente. La enfermedad de von Hippel-Lindau se caracteriza por la existencia de hemangioblastomas a nivel del sistema nervioso central, asociados a un riesgo incrementado de carcinoma renal de celulas claras y de feocromocitoma. La nevomatosis basocelular comprende quistes mandibulares, anomalias oseas, intracraneales y genitales.

Pseudocysts, lipomas, infarcts and simple cysts of the calcaneus are described as different entities in the medical literature. However, some evolutions or associations may suggest a relationship which is not yet demonstrated in all cases. The aim of this article is to describe each lesion and emphasize their different characteristics that may suggest a relationship between them.

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.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.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.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.

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.

Scoliosis is frequent, especially during the peripubertal period, which corresponds to the period of greatest activity. About 75% of scoliosis are idiopathic, while the remaining 25% can be divided into neuromuscular, congenital and miscellaneous etiologies. When it is associated with pain, a comprehensive work up should be undertaken to identify the underlying etiology of the scoliosis. Idiopathic scoliosis is typically evaluated using standard radiographs. The number of follow-up radiographs and radiation exposure must be maintained to a minimum. Radiation safety guidelines and protocols must be implemented. Large cassettes can be replaced by digital acquisitions using units with reconstruction software. The technique of evaluation of these scoliosis series radiographs as well as their results will be reviewed. Recent data regarding the evaluation of spinal and pelvic balance parameters in the standing position will be introduced. Atypical idiopathic and other scoliosis require additional evaluation. Computed tomography, with 2D and 3D reformations, is ideal for evaluation of bony anomalies. MRI is ideal for evaluation of the spinal canal and cord, from the skull base down to the sacrum, for all patients with surgical scoliosis or patients with neurological symptoms. The increased availability of MR imaging has resulted in an increased number of so-called idiopathic asymptomatic scoliosis where an underlying anomaly can be identified.

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;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.

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.

Langerhans cell histiocytosis is classically considered as totally different from Chester-Erdheim's disease which consists in the infiltration of various parenchymas by macrophagic CD68-positive histiocytes. We report the case of a 46-year-old woman with a long history of diabetes insipidus who presented typical lesions of Langerhans cell histiocytosis on vulvar and skin biopsies as well as bony cellular infiltrates characteristic of Chester-Erdheim's disease. A few months later she presented cerebellar disorders and died after an 18-month course. At autopsy the pons was enlarged, due to numerous cellular infiltrates which were also scattered in the middle cerebellar pedoncles, dentate nuclei, midbrain and hypothalamus. There were S100-protein positive Langerhans cells intermingled with numerous ovoid CD68-positive histiocytes. There are a few reported cases of Chester-Erdheim's disease presenting foci of Langerhans cells histiocytosis in other parenchymas. In addition, there are 10 reported cases with diabetes insipidus and bilateral infiltration of the brain stem and cerebellum, considered as presenting either one type of histiocytosis or the other. Our case demonstrates that both histiocytoses may coexist in the brain and thus correspond in fact to the same pathology in certain particular cases.

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.

Diseases of the pancreas in children can be of congenital, inflammatory, or, more rarely, tumoral origin. The most frequent condition is pancreatitis regardless of its origin.

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.

We studied 10 patients with capsular tear of the glenohumeral joint in the rotator interval -- the triangular space separating the supraspinatus and subscapular tendons. The patients, manual laborers and athletic people, had no previous shoulder pain. Tears appeared after forced internal rotation caused by trauma. The pain was anterior and radiated distally. Pain was absent at rest but recurred immediately after activity was resumed. On examination, range of motion was normal. Hawkins' impingement sign and the palm-up test usually caused pain. Radiographic findings were normal. Arthrography showed leakage of contrast medium into the subscapularis fossa. The tear was not visualized with ultrasonography or magnetic resonance imaging. Eight patients underwent an operation consisting of minimal acromioplasty with coracoacromial ligament resection, coracoidplasty, and suture of the rotator interval. All patients had relief of pain and return of full function.

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.

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.

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.

Resume Des calcifications discales se retrouvent chez l’enfant entre 6 et 10 ans. Elles sont transitoires et benignes. Elles correspondent a une pâte molle contenant des cristaux d’hydroxyapatite de calcium, qui touche le nucleus pulposus, et plus rarement la totalite du disque. Elles siegent a l’etage cervical de C4 a C7 et a l’etage thoracique superieur. Elles peuvent etre uniques et multiples. Les calcifications thoraciques sont asymptomatiques. Les calcifications cervicales sont symptomatiques dans 75 % des cas. Le tableau le plus classique est celui d’un syndrome douloureux avec raideur (torticolis) qui s’accompagne d’une hyperthermie moderee avec un syndrome inflammatoire biologique, ce qui peut faire evoquer une spondylodiscite. Les radiographies simples permettent alors le diagnostic. Des complications peuvent survenir : des hernies anterieures avec erosion de l’angle antero-superieur de la vertebre sous-jacente, et parfois compression œsophagienne et dysphagie, des hernies foraminales avec compression radiculaire, ou posterieures avec migration intra-canalaire sous-ligamentaire sans compression. L’evaluation est faite en TDM ou IRM. L’evolution est toujours favorable avec regression des symptomes en 2 ou 3 semaines sous traitement antalgique et anti-inflammatoire. Les calcifications disparaissent sans sequelle en quelques mois.

Resume C’est une enthesopathie micro-traumatique de la jonction disco-vertebrale, survenant sur un rachis en croissance, entre 12 et 15 ans, responsable d’une cyphose douloureuse. Elle associe des irregularites des bords des plateaux vertebraux, une cuneiformation des vertebres thoraciques moyennes (T7-T8-T9), une degenerescence discale disseminee infraclinique (IRM), des deplacements de materiel discal qui se font rarement dans le canal rachidien et plus frequemment dans le corps vertebral en realisant des hernies intra-spongieuses, centrales, anterieures (retro-marginales) ou posterieures (pre-marginales). Le diagnostic repose sur les cliches simples du rachis de profil. En cas de cyphose severe (> 50°) le bilan pre-therapeutique necessite un test de reductibilite avec une radiographie du rachis de profil en appui sur un billot et une etude de la statique globale. L’IRM est indiquee dans les cas avec signes neurologiques. La TDM est utile en cas de hernie pre-marginale posterieure pour rechercher une fracture associee de la paroi posterieure. L’atteinte isolee du rachis lombaire est possible et survient chez des adolescents plus âges (15-18 ans). C’est le groupe ou les complications posterieures sont les plus frequentes. Les formes graves sont susceptibles d’induire des complications degeneratives precoces et des retrecissements canalaires chez l’adulte.

SUMMARY 13 cases of Tumoral Calcinosis in children from 6 Paediatric Institutions are presented. Rare localisations and importance of CT investigation are stressed. The youngest patient with Tumoral Calcinosis and another in whom Tumoral Calcinosis was associated with Acroosteolysis & Idiopathic Osteoporosis are described.

Twelve cases of Mucolipidosis II (I-cell disease) with a wide range of severity of skeletal involvement were studied. Pathological findings in two cases provided helpful information in understanding the radiographic features of dysostosis multiplex. Inhibition of the growth plate cartilage calcification and rickets-like lesions were observed in the metaphyses. Enhanced subperiosteal remodelling and paratrabecular fibrosis were also evident in the diaphyses. High levels of parathormone were found in one case. This finding supports the hypothesis that bone lesions may be secondary, at least in part, to damage in such viscera as the kidney and/or the liver and that they are mediated by vitamin D and parathormone.

Thigh pain is a serious condition in childhood and should be promptly and thoroughly investigated. Twenty-five cases of thigh pain lasting between 1 day and 7 months with mid-femoral periosteal thickening were analysed. There were 10 patients with Ewing's sarcoma, 7 with eosinophilic granuloma, 1 with osteoid osteoma, 1 with leukaemia, 3 with osteomyelitis, 1 with myositis and 2 with post-traumatic periosteal reaction. It was shown that patients with Ewing's sarcoma and eosinophilic granuloma have certain patterns of X-ray changes of high diagnostic value, which allow a proper diagnosis in most cases.

Retroperitoneal teratomas are rare in adults; they are malignant in 25.8% of cases. The authors report two benign cases in which radiologic examination was essential in establishing the diagnosis. Irregular calcifications, which are visible in 61.5% of reported cases, cannot be considered a sign of benignity, since 12.5% of calcified tumors were malignant. Because of the indeterminate course of the disease, surgical removal is indispensable.

Dysphagia of the newborn and the infant presents with many varied causes, neurological, malformative, turmoral or functional. Among the latter, cricopharyngeal achalasia is an exceptional entity open to discussion. Suspected as early as the first month, presenting with difficulty in swallowing with choking episodes and coughing up of food without any obvious etiology. Profile roetgengraphic studies will allow the diagnosis to be made. The characteristic sign is a round and regular posterior narrowing on the posterior wall of the esophagus (C4 - C5 level) with enlargement of the hypopharynx. This narrowing is a constant finding. This pathological entity, an absence of relaxation of the cricopharyngeus muscle during the third period of swallowing, well-known in the adult patient, has only exceptionally been described among newborn children. In the following case study, cure has only been obtained by an extra mucosal myotomy of the esophagus at the age of seven months. The discussion, considering the five cases reported in pediatric journals, covers the diagnostic elements, the physiopathology of the disease, the risks presented during evolution without treatment. Therapy must be precocious and surgical.

With the advent of routine fetal ultrasonography, the frequency of prenatal diagnosis of ovarian cysts has considerably increased. Based upon a personal experience of 8 cases and a review of published series, the authors discuss the management of perinatal ovarian cysts. Before birth punction of the cyst under sonographic guidance or cesarean delivery may be considered in order to avoid the rupture during normal delivery. After birth, surgery should be performed in case or voluminous or complicated cyst or uncertain diagnosis. Homogenuos medium sized cysts or cysts with functional cytology should be followed by echography, before surgery being considered.

SUMMARY Four cases of multifocal, non-staphylococcal osteomyelitis are reported. The organisms recovered were aspergillus and serratia in one patient and Klebsiella in two patients. In the fourth patient no organisms were recovered but the diagnosis of tuberculosis was established on the bases of radiographic, clinical and laboratory data and a therapeutic test. A non-staphylococcal aetiology should be suspected in all cases of osteomyelitis with unusual radiographic appearances.

The authors report a case of subperiosteal osteoid osteoma of the femoral neck in a 14 year-old girl presenting as chronic hip monoarthritis with narrowed coxofemoral joint space and non specific histological synovitis. They analyse the synovitis which is associated with some osteoid osteomas (hip, elbow, knee, ankle bones) emphasizing the "pseudo-arthritis of the hip" picture and insist that all cases of unexplained chronic monoarthritis of the hip should suggest subperiosteal osteoid osteoma of the neck.

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.

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.