Isolated neonatal MRI punctate white matter lesions in very preterm neonates and quality of life at school age.

OBJECTIVE: To study the quality of life at school age of very preterm infants presenting isolated punctate periventricular white matter lesions (IPWL) on late-preterm or term magnetic resonance imaging (MRI). METHODS: In 1996-2000, 16 of the 131 very preterm neonates explored by MRI were found to have IPWL. At the age of 9-14, 12 children from the IPWL group were compared with 54 children born preterm but with a normal MRI (no lesion). Quality of life (Health Status Classification System Pre School questionnaire), school performance, and motor outcome were investigated. RESULTS: Overall quality of life did not differ between the groups (classified as perfect in 2/12 of the IPWL vs 20/54 in the no-lesion). The sub-items mobility and dexterity differed significantly between the two groups, with impairment in the IPWL group (p < 0.001 and p < 0.05). This group also displayed higher levels of motor impairment: they began walking later [20(4) vs. 15(3) months), p < 0.01], had higher frequencies of cerebral palsy (6/12 vs. 2/54, p < 0.05), and dyspraxia (4/12 vs. 0/54, p < 0.001). The rate of grade retention did not differ between the groups (3/12 in the IPWL group vs. 17/54 in the no-lesions group) but, as expected, was higher than that of the French general population (17.4%) during the study period. CONCLUSION: This long-term follow-up study detected no increase in the risk of subsequent cognitive impairment in very preterm infants with IPWL, but suggests that these children may have a significantly higher risk of dyspraxia, and motor impairment.

Arterial spin labeling in clinical pediatric imaging.

Arterial spin labeling (ASL) perfusion-weighted magnetic resonance imaging is the only approach that enables direct and non-invasive quantitative measurement of cerebral blood flow in the brain regions without administration of contrast material and without radiation. ASL is thus a promising perfusion imaging method for assessing cerebral blood flow in the pediatric population. Concerning newborns, there are current limitations because of their smaller brain size and lower brain perfusion. This article reviews and illustrates the use of ASL in pediatric clinical practice and discusses emerging cerebral perfusion imaging applications for children due to the highly convenient implementation of the ASL sequence.

Pubo-femoral distance: an easy sonographic screening test to avoid late diagnosis of developmental dysplasia of the hip.

OBJECTIVES: To propose an easy and reproducible sonographic screening test able to avoid late presentation of developmental dysplasia of the hip (DDH). METHODS: The pubo-femoral distance (PFD) cut-off point was determined on 980 infants who underwent ultrasonography of the hips in comparison with the final diagnosis, based on clinical, ultrasound data and follow-up. PFD reproducibility was tested on 52 hips by both an experienced and an inexperienced radiologist. After teaching this screening method to general radiologists, its impact was evaluated by analysing the rate of late diagnosis in an entire administrative area. RESULTS: An abnormal PFD threshold above 6 mm gave a sensitivity of 97.4 % for the diagnosis of DDH (271 hips) and a specificity of 84 %. Sensitivity increased to 100 % when taking into consideration children of 1 month or older. The PFD measurement stayed reproducible even in inexperienced hands (k = 0.795). For 3 years, following the extension of screening to all female infants, there was no late diagnosis of DDH in girls in amongst a catchment area of 1 million inhabitants. CONCLUSIONS: PFD measurement with a threshold for abnormality of 6 mm at the age of 1 month, without asymmetry above 1.5 mm, could eradicate DDH late diagnosis.

Idiopathic club foot treated with the Ponseti method. Clinical and sonographic evaluation of Achilles tendon tenotomy. A review of 221 club feet.

The Ponseti method applied to treating idiopathic club foot consists in placing successive corrective casts, possibly an Achilles tendon tenotomy, then derotation braces, a method that has proven its efficacy. This study compared 221 cases of club foot treated with this method between 2002 and 2007 divided into two groups, based on whether or not Achilles tendon tenotomy was performed. Assessment was both clinical and sonographic. We observed clear improvement of the results in the group that underwent Achilles tendon tenotomy and a significant difference in the rate of secondary surgery. The sonographic evaluation also showed improvement of the morphological results. We now systematically propose Achilles tendon tenotomy however severe club foot may be.

Irreducible developmental dysplasia of the hip due to acetabular roof cartilage hypertrophy. Diagnostic sonography in 15 hips.

INTRODUCTION: Irreducible developmental dysplasia of the hip (DDH) in newborns is a rare entity. The different obstacles preventing reduction have been described in the literature. HYPOTHESIS: A clinical form of DDH with hypertrophy of the cartilage of the acetabular roof (acetabular bulge) can be reliably identified on ultrasound and should probably be defined as a separate entity. MATERIALS AND METHODS: For the first time, the authors report their experience, a review of the literature and the radiographic description (ultrasound, arthrography MRI) of irreducible neonatal DDH due to hypertrophy of the cartilage of the acetabular roof (acetabular bulge) in 12 infants (15 hips). RESULTS: Neonatal sonography seems to be sufficient to identify this specific clinical entity without any additional work-up. This sonographic sign could help determine the therapeutic strategy earlier in this severe and complex form of DDH.

[Developmental dysplasia of the hip]. / Luxation congénitale de hanche chez l'enfant.

At one month, the acetabular depth from a standard lateral coronal section is 4.6 ± 1.0mm. The correlation between experienced and junior radiologists is high (kappa 0.795). A treshhold of 6mm with a Δ (difference between right and left) less than 1.5mm provides a sensitivity of 100% for the detection of DDH at one month. Universal US screening at one month of all high-risk infants in 2009 resulted in a reduction of delayed diagnosis of DDH (zero girls, two boys) in Ille-et-Vilaine due to continuous medical education of general radiologists.

Pediatric outcome of children with the prenatal diagnosis of isolated septal agenesis.

BACKGROUND: Isolated Septal Agenesis (SA) is a rare disease with clinical outcomes (especially neurological outcomes) that are unknown. The purpose of this study was to evaluate the clinical outcome of these children. METHODS: We conducted a retrospective multicenter study of 17 children with an isolated SA or SA combined with a moderate ventricular dilatation (VD) that was diagnosed antenatally and confirmed by a magnetic resonance imaging (MRI) performed in the antenatal period. RESULTS: Of the 17 children, 14 had normal neurological examinations, 2 had language development delay and visuo-spatial dyspraxia, and 3 of the 17 children had behavioral problems. Eight children had neuropsychological evaluations, and the results were normal in six cases. There were 3 cases of septo-optic dysplasia (SOD) diagnosed postnatally, which highlighted the difficulties in assessing the optic tract and hypothalamic-pituitary region in antenatal imaging. Language delay and behavioral disorders were the main abnormalities at follow-up. CONCLUSION: The discovery of an isolated SA reveals the difficulties of prenatal diagnosis to correlate the neurological and functional prognosis to morphological findings. The prognosis seemed to be good. It appears necessary to improve the diagnostic performance of fetal brain imaging and to follow-up these children prospectively to assess their long-term cognitive-behavioral outcomes.

[Echography of the hip and other imaging techniques in pediatrics]. / Echographie de hanche et autres techniques d'imagerie en pédiatrie.

OBJECTIVES: To standardize the ultrasound evaluation in congenital hip dysplasia according to the consensus meeting of 1991 in Paris. Detection of congenital anomalies is a daily pediatric challenge. The coronal plane at US of the hip is considered as the gold standard: its technique, its morphologic and dynamic results either normal or pathological, have been described. A standardized report and a simple terminology are proposed. 2. To explain the role of ultrasound, when performed in close collaboration with a pediatric orthopedic surgeon, for initial diagnosis and follow-up of anatomical anomalies. The role of arthrography and MR imaging will be discussed. 3. To describe the benefit of the ultrasound in cases of painful hip in children, including osteoarthritis, acute synovitis, osteochondritis. . . In these cases, the assessment of the hip is also done with radiographs, computer tomography, MR imaging and bone scan.

Patent ductus arteriosus and cystic periventricular leucomalacia in preterm infants.

AIM: To test the association between early disturbances in hemodynamics induced by left-to-right shunting through the duct and cystic periventricular leucomalacia. PATIENTS: Forty-six preterm infants (27-32 wk) admitted to the neonatal intensive care unit with risk criteria. METHODS: Patent ductus arteriosus was evaluated on days 1 and 4, and was significant (sPDA) in cases of absent or reversed end diastolic flow in the subductal aorta. Resistance index was measured in the anterior cerebral artery and in the subductal aorta. MAIN OUTCOME: Diagnosis of cystic periventricular leucomalacia between day 10 and day 50. RESULTS: The 12 infants who developed cystic periventricular leucomalacia were compared with those who did not. On day 1, sPDA was more frequent (64% vs 26%; p = 0.03) in the cystic periventricular leucomalacia group, left ventricular output was higher (median = 341 vs 279 ml kg-1.min-1; p = 0.005), and rescue surfactant was more frequently used (83% vs 47%; p = 0.03). This latter association was confirmed by multivariate analysis. Resistance index in the anterior cerebral artery was increased in cases of significant patent ductus arteriosus (p < 0.01) and was correlated with resistance index in the subductal aorta. CONCLUSION: On day 1 in this selected population, sPDA has an effect on blood flow velocity waveform in cerebral arteries and is associated with an increase in the emergence of cystic periventricular leucomalacia. This association could be casual rather than causal.

Intratumoral consumption of indium-111-labeled platelets in a child with splenic hemangioma and thrombocytopenia.

The authors report Kasabach-Merritt syndrome (KMS) in a patient with thrombocytopenia and splenic hemangioma. A 13-month-old boy with a history of anemia, thrombocytopenia, and abdominal mass was admitted to the hospital. The scintigraphic studies showed that a large mass contiguous to the spleen was responsible for the platelet uptake. After partial splenectomy, the platelet count returned to normal. This report of KMS in a child with splenic hemangioma suggests that the scintigraphic studies are mandatory to confirm diagnosis. Indium-111-labeled platelets are useful in identifying hemangiomatous sequestration of platelets in patients with thrombocytopenia.

The RETAIN project: DICOM teleradiology over an ATM-based network. Radiological Examinations Transfer on an ATM Integrated Network.

The RETAIN project (Radiological Examinations Transfer on an ATM Integrated Network) has aimed at testing videoconferencing and DICOM image transfers to get advice about difficult radiological cases over an asynchronous transfer mode (ATM)-based network, which affords a more comfortable interface than narrow-band networks and allows exchange of complete image series using the DICOM format of studies. For this purpose, an experimental ATM network was applied between six university hospitals in four different countries. An assessment of the functionalities of the system was performed by means of log-file analysis, video recording of the sessions and forms filled out by the participants at the end of each session. Questionnaires were answered by the users at the end of the project to bring out perspectives of utilisation and added value. We discussed 43 cases during 20 sessions. For technical or organisational problems, only 20 of the 36 planned sessions took place. The throughput over ATM (10.5 Mbit/s, 20 times faster than six ISDN B-channels) was adequate. Despite the experimental configuration of the network, the system was considered as satisfactory by all the physicians. In 72 % of the sessions, the expected result (answer to the question) was gained. By common consent, videoconferencing was unanimously regarded as a prominent tool in improving the interaction quality. Asynchronous transfer mode is an efficient method for fast transferring of radiologic examinations in DICOM format and for discussing them through high-quality videoconferencing.

[Severe streptococcal group A infection complicating varicella]. / Infection sévère à streptocoque du groupe A compliquant une varicelle.

UNLABELLED: Varicella is a common viral infection which is generally benign in infancy and has a good outcome. It may sometimes be complicated by severe group A streptococcal superinfection. CASE REPORT: Three days after the beginning of varicella, a previously healthy 2-year-old girl presented with left leg pain, lameness and edema of all four limbs. Toxic shock syndrome occurred, due to beta-hemolytic group A Streptococcus grown from blood culture. Computerized tomography (CT) scan showed a mild effusion involving both hips. Cefotaxim was administered, but the week after magnetic resonance imaging (MRI) showed a necrotizing fasciitis and a lesion of the left leg leading to a patchy femoral diaphysis consistent with osteomyelitis. Joint aspirate culture did not grow. The left leg was immobilized in plaster for 6 weeks and the child was given cefotaxim and fosfomycin parenterally during 30 days, then followed by 45 days of oral amoxicillin. She recovered without sequelae. CONCLUSION: Group A Streptococcus infection is a dangerous complication of varicella. It must be considered in case of any joint pain occurring during or just after this disease. The choice of the best treatment needs full collaboration between surgeons, radiologists and pediatricians.

Central nervous system malformations and early end-stage renal disease in oro-facio-digital syndrome type I: a review.

Intracerebral cysts and porencephaly or arachnoid cysts are rarely but are repeatedly reported in orofaciodigital (OFD) syndrome type 1. We report on 2 families in which OFD syndrome type 1 was observed with central nervous system (CNS) malformations and 3 sporadic cases of OFD with CNS defects, most likely representing fresh mutations for OFD 1. In one case, vermis hypoplasia was present; in another, periventricular heterotopiae were noted. We review the literature on CNS anomalies in OFD syndromes and stress the difficulties in genetic counseling and functional prognosis for children of OFD 1 female carriers prenatally diagnosed with a malformation of the brain. As for CNS malformations, renal cystic disease is an often overlooked complication specific to OFD 1. In 1 family, cystic medullary disease was noted in OFD 1 carriers, leading 1 patient to dialysis by age 35 years and the other to severe renal insufficiency by age 28 years. Longitudinal follow-up of OFD 1 carriers should be performed, and renal function should be assessed in those with cysts because the functional prognosis of this developmental anomaly may be worse than usually reported in the literature.

Prenatal detection of a congenital pancreatic cyst and Beckwith-Wiedemann syndrome.

We report a case of congenital pancreatic cyst detected prenatally by ultrasound in a fetus with evidence for a diagnosis of Beckwith-Wiedemann syndrome (BWS). Neonatal hypoglycaemia was prevented. The cyst was managed by internal drainage. This is the second reported case of BWS associated with pancreatic cystic dysplasia and the first time that this association has been detected prenatally. Differential diagnosis of cystic abdominal lesions occurring in utero should take pancreatic cysts into consideration. This case suggests that pancreatic cysts should be included in the BWS phenotype.

[Test of a teleradiology network based on ATM (asynchronous transfer mode)]. / Essai d'un réseau de téléradiologie basé sur ATM (asynchronous transfer mode).

The retain project (Radiological Examination Transfer on ATM Integrated Network) consisted in teleradiology trials focused on pediatric imaging between two university hospitals. Rennes (France) and Barcelona (Spain) using an integrated broadband communication network based on ATM (asynchronous transfer mode), as part of a European research program. The network used was a full 10 Mbits/s ATM network directly connected to local PACSs (medical imaging hospital networks). One important reason to explicitly consider ATM for medical imaging is that multimedia applications on such networks allow integration of digital data and person-to-person communication. The utility of broadband communication for teleradiology has been confirmed. High quality video and sound are important for both human communication and medical video transfer. The project led to guidelines regarding technical options still open to improvement.

[Contribution of post mortem skull scanning for the study of sudden infant death]. / Contribution du scanner crânien post-mortem à l'étude de la mort subite du nourrisson.

BACKGROUND: Despite post-mortem examination and autopsy, many cases of sudden infant death (SID) remain unexplained. The aim of this study was to assess usefulness of CT-scan in the Sudden Infant Death Syndrome (SIDS). POPULATION: Twenty-three cases of SIDS had a post-mortem CT-scan evaluation of skull and brain. The pictures were retrospectively reviewed by several independent radiologists who were unaware of the circumstances of death and results of autopsy. RESULTS: Aspects of pneumatocele, probably due to lumbar puncture were found in 6 cases. The subarachnoid spaces appeared inexplically hyperdense, as they were not correlated to the results of lumbar puncture and autopsy. The ventricles were normal in size or density. Density of the dural sinuses (superfical and deep) was often increased, an aspect possibly artefactual, due to post-mortem thrombosis. The cerebral parenchyma was often slighty hypodense; microcalcifications due to congenital toxoplasmosis were found in one case. CONCLUSION: There was no correlation between the CT-scan imaging and the delay of death and lumbar puncture. Infants with or without subarachnoid hemorrage had the same CT scan findings. The CT-scan has a poor value when autopsy is performed; in its absence, it could be useful for diagnosing post-traumatic intracerebral hematoma.