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.

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.

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.

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.

Tracheobronchography and balloon dilatation in acquired neonatal tracheal stenosis.

Between 1988 and 1992, 18 mechanically ventilated newborn babies (mean weight 1300 g and gestational age 30 weeks) presented with deteriorating respiratory failure at a mean age of 29 days. All developed increased oxygen requirements, hypoxic and hypercapnic episodes, and radiological changes of fixed lobar emphysema or recurrent atelectasis which sometimes changed sides from one day to another. Tracheobronchography with iopydol-iopydone was normal in five (27%) cases, but in 13 showed tracheobronchial stenosis localised to the lower trachea (seven cases), to the right main bronchus (three cases), or including the left main bronchus (four cases). Eleven of these 13 patients underwent endoscopy and balloon dilatation of the stenotic area. Five patients died, one before endoscopy, one immediately after endoscopies, and three subsequently with severe bronchopulmonary dysplasia. The other six babies recovered without any sequelae after balloon dilatation.

Triphalangeal thumb and split foot in the same family.

The authors report a family with triphalangeal thumb with nail hypoplasia: one of them has also split feet. They believe that the existence of such families must make very circumspect with regard to genetic counseling for a minor problem such as triphalangeal thumb.

A case of Larsen syndrome with severe cervical malformations.

The authors report Larsen Syndrome in a male newborn with severe cervical spine malformations: segmentation abnormalities of the cervical spine and atlanto-axial dislocation. The severity of the cervical malformations occurring more often in the autosomal recessive form is emphasized.

Urinary fungal bezoars in children--report of two cases.

We report two cases of urinary obstruction by fungal bezoars in full-term neonates who presented a uropathy detected antenatally. Early percutaneous urinary diversion was performed to relieve renal impairment secondary to a primary megaureter in the first case and to bilateral pelvi-ureteral obstruction in the second. Acute fungal obstruction occurred first on the side of the primary megaureter and then on the healthy side in the first patient. Symptoms of infection and impaired renal function led to a diagnosis of fungal bezoar. In the second patient the development of the bezoar was more insidious and occurred after surgical correction of the obstructive pelvi-ureteral junction on the left side. Candiduria was the first sign in both cases. Ultrasonography is the best method to visualize fungal masses within the collecting system. In most cases, percutaneous nephrostomy allows relief of the obstruction, sampling of urine for culture and irrigation with amphotericin B. However, additional surgical intervention may be necessary. Systemic antifungal treatment using mainly 5-flucytosine is also given.

Late presenting Bochdalek hernia containing colon: misdiagnosis risk.

The case of an eleven-months-old girl is reported: she entered the intensive care unit for fever, vomiting and left pleural effusion. Abdominal echography, CT scan and colonic opacification led to the diagnosis of a colonic Bochdalek hernia. A review of the literature showed that colonic hernias are rare, with only five previous reported cases in which colon was found in the thorax. The authors emphasise that there is a high risk of misdiagnosis due to the fact that small intestine contrast studies are normal; only colonic contrasting may lead to the right diagnosis. The roles of echography and CT scan are discussed.

Renal cystic tumours in children--a diagnostic challenge.

A series of 8 cystic renal tumours is reported in seven-months to four-years-old children. The final diagnosis was cystic nephroma (multilocular cyst) in 4, cystic, partially differentiated nephroblastoma in 3 and partially cystic nephroblastoma in one. Pre-operative distinction between those three types is difficult and inadequate therapeutic approach may result from a wrong diagnosis. Progress in imaging techniques allows a better analysis of cysts and septa. If no solid part can be detected in the tumour, total nephrectomy is sufficient to obtain a favourable outcome.

[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.

[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.

[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.

[Acquired tracheobronchial stenosis of the premature infant under artificial respiration. Value of bronchography and endoscopic balloon dilatation. Apropos of 7 cases]. / Les sténoses trachéo-bronchiques acquises du prématuré ventilé. Intérêt de la bronchographie et de la dilatation endoscopique au ballonnet. A propos de 7 cas.

The authors report about a series of 7 cases of acquired tracheobronchial stenosis in premature infants who were intubated as soon as they were born and ventilated. Two important points can be pointed out: the merits of bedside bronchography, which allows diagnosing the lesions, the merits of balloon dilatation during tracheobronchoscopy on the ENT operating theatre to treat the stenosis. This technique allowed improving ventilation and removing the tube in 6 of 7 cases.

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.