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Outcome prediction at the early sclerotic stage of Legg-Calvé-Perthes disease (LCPD) is valuable to select patients likely to benefit from early surgery. The metaphyseal apparent diffusion coefficient (ADC) ratio correlated significantly with Herring's classification of LCPD in a preliminary study of 49 MRIs, in which values greater than 1.63 were associated with poor outcomes. The objective of this study was to determine whether the femoral neck ADC ratio, with the 1.63 cut-off, determined at the initial stage of LCPD correlated with medium-term radiographic outcomes.The metaphyseal ADC ratio correlates significantly with medium-term radiographic outcomes of LCPD.A prospective study was performed in 27 children (mean age, 13 years; range, 9.5-16 years) who underwent 49 MRIs at the sclerosis or fragmentation stage of unilateral LCPD. ADCs measured bilaterally at the femoral head and neck were used to compute the corresponding ADC ratios between the affected and unaffected sides. The patients received regular follow-up for at least 5 years. The correlation between the ADC ratios and Stulberg grade at last follow-up was assessed.After a mean follow-up of 6.8 years (range, 5.2-8.4 years) from the date of the first MRI, 13 hips were Stulberg 1 or 2, 13 were Stulberg 3 or 4, and 1 was Stulberg 5. The metaphyseal ADC ratio increased significantly with the Stulberg grade (p0.01). When only MRIs obtained at the early stage of sclerosis were considered, the correlation remained significant (p=0.03). It was also significant in the subgroup of surgically treated patients (p0.0001) but was not significant in the subgroup without surgery (p=0.51). A metaphyseal ADC ratio greater than 1.63 was associated with a worse Stulberg grade (p=0.02).Diffusion-weighted MRI is a non-irradiating and non-invasive investigation that contributes to the management of LCPD when used in combination with morphological MRI sequences. Elevation of the femoral neck ADC is a finding of adverse prognostic significance that correlates with Herring's grade at the fragmentation stage and with Stulberg's grade at the healed stage. Early ADC elevation in the affected femoral neck can serve to select those patients most likely to benefit from early surgery before the fragmentation stage, i.e., before Herring's classification can be applied.III, prospective uncontrolled study 3.

Introduction The intervertebral disc (IVD) is made up of the annulus fibrosus (AF) and the nucleus pulposus (NP) – an inert hydrated complex. The ability of the IVD to deform is correlated to that of the NP and depends on its hydration. As the IVD ages, its hydration decreases along with its ability to deform. In adolescent idiopathic scoliosis, one of the etiological hypotheses pertains to the IVD, thus making its condition relevant for the diagnosis and monitoring of this pathology. Hypothesis IVD hydration depends on sex, age and spine level in an asymptomatic pediatric population. The corollary is data on a control group of healthy subjects. Material and methods A cohort of 98 subjects with normal spine MRI was enrolled; their mean age was 13.3 years. The disc volume and hydration of each IVD was evaluated on T2-weighted MRI sequences, using previously validated image processing software. This evaluation focused on the lumbar spine, from the thoracolumbar junction to the lumbosacral junction. It was assumed that IVD hydration was related to the ratio of NP and AF volumes. A mixed multivariate linear analysis was used to explore the impact of age, sex and spinal level on disc hydration. Results Disc hydration was higher overall in boys than in girls, but this difference was not significant. Hydration increased with age by +0.005 for each additional year (p = 0.0213). Disc hydration appears to be higher at the thoracolumbar junction than the lumbar spine, although this difference was not significant. Conclusion Through this MRI study, we established a database of non-pathological lumbar disc hydration as a function of age, sex and spinal segment along with 95% confidence intervals. Level of evidence IV.

To evaluate in children the agreement between femoral and tibial torsion measurements obtained with low-dose biplanar radiography (LDBR) and CT, and to study dose reduction ratio between these two techniques both in vitro and in vivo. Thirty children with lower limb torsion abnormalities were included in a prospective study. Biplanar radiographs and CTs were performed for measurements of lower limb torsion on each patient. Values were compared using Bland-Altman plots. Interreader and intrareader agreements were evaluated by intraclass correlation coefficients. Comparative dosimetric study was performed using an ionization chamber in a tissue-equivalent phantom, and with thermoluminescent dosimeters in 5 patients. Average differences between CT and LDBR measurements were –0.1° ±1.1 for femoral torsion and –0.7° ±1.4 for tibial torsion. Interreader agreement for LDBR measurements was very good for both femoral torsion (FT) (0.81) and tibial torsion (TT) (0.87). Intrareader agreement was excellent for FT (0.97) and TT (0.89). The ratio between CT scan dose and LDBR dose was 22 in vitro (absorbed dose) and 32 in vivo (skin dose). Lower limb torsion measurements obtained with LDBR are comparable to CT measurements in children and adolescents, with a considerably reduced radiation dose. • LDBR and CT lower-limb torsion measurements are comparable in children and adolescents. • LDBR considerably reduced radiation dose necessary for lower-limb torsion measurements. • LDBR can be used for evaluation of lower limb-torsion in orthopaediatric patients.

OBJECTIVES: The prenatal finding of a cystic suprarenal mass (CSM) presents a wide differential diagnosis. The aim of this study was to present the natural course and outcome of antenatally diagnosed CSMs. METHODS: We reviewed the medical records of patients with prenatally detected CSMs that were assessed between January 1998 and December 2011. Retrospective data collection included the age at the time of diagnosis, the size of the mass, and the sonographic evolution of the mass. Surgical treatment was indicated in cases of malignant tumors and symptoms or when size increased. RESULTS: The observation period ranged from 1 month to 10 years. The data of 21 patients were analyzed. The median length of follow-up was 32 months (ranging from 2 to 131 months). A total of 13 masses were detected on the left side and 8 on the right side. In 13 patients, the lesions disappeared after a median of 7 months (ranging from 0 to 37 months). Surgery was performed in two neonates: one for a teratoma at 10 days postpartum, and one for a neuroblastoma at 17 days postpartum. Six patients had an extralobar pulmonary sequestration (ELPS), and in four patients, surgical resection was performed at an average of 9.2 months (ranging from 1 to 20 months) postpartum, because of an infection or increase in ELPS size. Histological examination confirmed the pulmonary sequestration. An association with congenital cystic adenomatoid malformation (CCAM) was found in one patient, and ectopic pancreatic tissue was discovered in another patient. Two patients conservatively managed remain under observation. CONCLUSIONS: During the neonatal period, the accurate assessment of CSMs is necessary to exclude the presence of malignant tumors. Most patients can be conservatively managed, with close follow-up, including radiological assessment. If the mass persists after 1 year of age, ELPS should be considered as a probable diagnosis, and minimally invasive laparoscopic surgery can be performed, as it provides both definitive diagnosis and treatment.

We reviewed our institutional experience with congenital mediastinal masses and compared the postnatal management and outcome of patients with or without prenatal diagnosis. Between January 1997 and August 2011, 24 patients underwent surgical procedures for congenital mediastinal mass. For eight patients, the mass was detected by prenatal ultrasonography at 27 weeks of gestation (range 22–35). Postnatal management consisted in open surgery for seven patients at a mean age of 9 months (range 1 day–20 months) and sclerotherapy for one lymphangioma at 5 months of life. Sixteen patients had postnatal diagnosis at 137 months (±194) of median age. Eight bronchogenic cysts, seven bronchopulmonary foregut malformations, five teratomas, three lymphangiomas and one haemangioma were operated on. The median age at resection was 28 months (1 day–15 years). There were four emergency procedures and no surgical mortality. The median follow-up was 45 months (3–144). The duration of mechanical ventilation and hospital stay was, respectively, 4.6 h and 7.5 days for antenatal patients and 24.3 h and 14.3 days for postnatal diagnosed patients. Prenatal diagnosis allows early management of congenital mediastinal malformations. Early resection can be performed prior to the occurrence of symptoms ∼1 year of life and is associated with an excellent outcome and less morbidity.

Congenital diaphragmatic hernia is a rare congenital malformation, as well as kidney ectopia. Among kidney ectopias, the intrathoracic one is the rarest. Those malformations concern more frequently boys, and affected more the left than the right side. Their association is poorly reported in the literature. We report the rare case of an early sonographic prenatal diagnosis of intrathoracic kidney at 22 weeks of gestation in a female fetus, completed on the follow-up by the diagnosis of an associated diaphragmatic hernia at 33 weeks of gestation. If chest mass is diagnosed prenatally or in neonate, ITK should be considered in a differential diagnosis, all the more if the ipsilateral renal fossa is empty. An associated DH should be searched if ITK is confirmed. Isolated ITK usually requires no specific treatment, in contrast with ITK associated with DH.

Antenatal detection of mesoblastic nephroma by US is possible. Reviewing the literature, we found 19 previously reported cases, only 1 of which underwent prenatal MRI. We present a further case diagnosed by US and confirmed with MRI. The imaging findings and differential diagnoses are discussed. Early and correct detection of this rare entity is of great interest, as it may facilitate prevention and management of severe obstetric and neonatal complications such as polyhydramnios and prematurity. MRI can help to evaluate the origin and the morphological features of a fetal abdominal mass.

This paper presents 12 infants (9 boys, 3 girls) in whom the diagnosis of a suprarenal mass (10 left, 2 bilateral) was made on antenatal sonography. All were otherwise healthy neonates who were born at term after a normal pregnancy, labor and delivery. The masses ranged from 1 to 3.5 cm in diameter on initial scans at gestational ages of 19-35 weeks. Eleven masses were hyperechoic and 4 of these contained small, well-defined cysts. The 12th was hypoechoic. Follow-up sonography showed complete disappearance of the mass antenatally in 1 case and postnatally by 4-6 months in 5 cases; there was marked diminution in the size of the mass by 2 months of age in 4 infants, by 4 months in 1 case and by 15 months in 1 case. Eleven were managed nonoperatively. Laparotomy (after disappearance of the mass) in the 12th case revealed only some fibrous tissue. The 11 echogenic masses resemble previously reported imaging findings in infants with histologically proven intra-abdominal sequestrated lung. Conservative management with careful sonographic follow-up should, therefore, be considered in otherwise healthy fetuses or neonates with these imaging findings. We are less certain what the hypoechoic mass represented.

We report a rare example of anaemia and hypertension due to an incomplete Carney triad in a 14-year-old girl with no previous medical history. This rare non-familial syndrome generally involves two disparate tumours: gastrointestinal stromal tumour, paraganglioma and/or pulmonary chondroma. The complete triad is a syndrome that involves at least five loci: stomach, oeophagus, lung, the paraganglionic system, adrenal (cortex or medulla). The pathogenesis is unclear as these tumours have different embryological origins. Surgical treatment is necessary, and long-term follow-up is advisable as patients with Carney triad may re-present with tumour(s), even several years after the first presentation.

Hypophosphatasia is an inheritable disorder characterised by defective bone mineralisation due to the impaired activity of tissue-non-specific alkaline phosphatase (AP). Clinical presentation ranges from stillbirth without mineralised bone to pathological fractures in late adulthood. During childhood, the main manifestations include rickets, growth delay and dental problems. Fractures and bone pain usually characterise the adult form. A 9-year-old girl was referred for repetitive fractures after minimal trauma. She had normal growth, normal sclerae, no rickets and minimal dental abnormalities. Her sister had also presented fractures. The proband, her sister and mother had low total and bone-specific AP levels and E435K mutation in exon 12 of the liver/bone/kidney AP gene. Low AP levels must lead to genetic analysis. Bone fragility and repetitive fractures may be symptoms of hypophosphatasia in childhood, which must not be neglected. Associated factors such as vitamin D or calcium deficiency must be prevented. In conclusion, hypophosphatasia must not be forgotten as an aetiological factor of repetitive fractures or bone pain in children and AP activity should be checked accurately.

Background/Purpose Fetal ovarian cysts are frequently complicated by intracystic hemorrhage without associated clinical signs, which is often secondary to ovarian torsion leading to loss of the ovary. The aim of this study was to evaluate ovarian outcome and the place of prenatal management and surgery in the first few days of life in order to save the ovary. Methods Between January 1987 and June 2006, 82 fetal ovarian cysts in 79 patients were managed and clinically and ultrasonographically followed up for several months (median, 11 months; range, 6 months to 10 years) in all of the cases where the ovary was not removed. The ultrasonographic results regarding the ovarian parenchyma were broken down into 3 categories: follicular ovary, homogeneous ovary, and undetected ovary. Results Twenty-seven cysts remained simple throughout their evolution, and 55 were complicated by intracystic hemorrhage usually several weeks before birth. Overall, after disappearance of the cyst, a follicular ovary was detected in only 39% of the cases (32/82) and more often when the cyst was simple than when it presented an intracystic hemorrhage (85% vs 16.4%, χ 2 , P Conclusions A review of our series confirms the poor ovarian outcome linked to ultrasonographic signs of intracystic hemorrhage. Preventive action by puncture of "simple" cysts is still being studied. The presence of a bilateral cyst can, if pulmonary maturity has been reached, be an argument for inducement of premature birth with a view to performing conservative surgery. After birth, surgery in the first few days of life is only justified if the signs of intracystic hemorrhage appeared in the period very close to birth.

We analysed bone age radiographs in 102 girls with Turner syndrome and compared the findings with 93 control girls and nine girls with Leri-Weill syndrome. Various signs were analysed: radial bowing or Madelung deformity, maximal/minimal height of the radial epiphysis, brachymetacarpia of the 4th digit, carpal and epiphyseal angle, as well as a new sign the distal radio-ulnar physeal disparity. Two values differed significantly between the Turner group and the control group, the first being the epiphyseal angle which has already been reported to be greater in Turner syndrome, and the second being the new sign we have been able to describe. Turner patients had an increased distance between the ulnar and radial metaphysis, or “distal radio-ulnar physeal disparity”, the ulnar being shorter. Furthermore, in 27% of cases the medial extremity of the ulnar epiphysis was flattened and passed below the distal extremity of the radius, whose medial part projected over the distal extremity of the ulna, thus reproducing in reverse the characteristic feature of Leri-Weill syndrome. In the growth hormone-treated Turner patients, we found a significant correlation between distal radio-ulnar physeal disparity and growth velocity expressed in cm/year (r=0.28; P