Sylviane Hanquinet

Early alteration of structural and functional brain development in premature infants born with intrauterine growth restriction.

Placental insufficiency with fetal intrauterine growth restriction (IUGR) is an important cause of perinatal mortality and morbidity and is subsequently associated with significant neurodevelopmental impairment in cognitive function, attention capacity, and school performance. The underlying biologic cause for this association is unclear. Twenty-eight preterm infants (gestational age 32.5 ± 1.9 wk) were studied by early and term magnetic resonance imaging (MRI). An advanced quantitative volumetric three-dimensional MRI technique was used to measure brain tissue volumes in 14 premature infants with placental insufficiency, defined by abnormal antenatal Doppler measurements and mean birth weights <10th percentile (1246 ± 299 g) (IUGR) and in 14 preterm infants matched for gestational age with normal mean birth weights 1843 ± 246 g (control). Functional outcome was measured at term in all infants by a specialized assessment scale of preterm infant behavior. Premature infants with IUGR had a significant reduction in intracranial volume (mean ± SD: 253.7 ± 29.9 versus 300.5 ± 43.5 mL, p < 0.01) and in cerebral cortical gray matter (mean ± SD: 77.2 ± 16.3 versus 106.8 ± 24.6 mL, p < 0.01) when measured within the first 2 wk of life compared with control premature infants. These findings persisted at term with intracranial volume (mean ± SD: 429.3 ± 47.9 versus 475.9 ± 53.4 mL, p < 0.05) and cerebral cortical gray matter (mean ± SD: 149.3 ± 29.2 versus 189 ± 34.2 mL, p < 0.01). Behavioral assessment at term showed a significantly less mature score in the subsystem of attention-interaction availability in IUGR infants (p ± 0.01). Cerebral cortical gray matter volume at term correlated with attention-interaction capacity measured at term (r = 0.45, p < 0.05). These results suggest that placental insufficiency with IUGR have specific structural and functional consequences on cerebral cortical brain development. These findings may provide insight into the structural-functional correlate for the developmental deficits associated with IUGR.

Combination of event-related fMRI and diffusion tensor imaging in an infant with perinatal stroke.

Focal ischemic brain injury, or stroke, is an important cause of later handicap in children. Early assessment of structure-function relationships after such injury will provide insight into clinico-anatomic correlation and potentially guide early intervention strategies. We used combined functional MRI (fMRI) with diffusion tensor imaging (DTI) in a 3-month-old infant to explore the structure-function relationship after unilateral perinatal stroke that involved the visual pathways. With visual stimuli, fMRI showed a negative BOLD activation in the visual cortex of the intact right hemisphere, principally in the anterior part, and no activation in the injured hemisphere. The functional activation in the intact hemisphere correlated clearly with the fiber tract of the optic radiation visualized with DTI. DTI confirmed the absence of the optic radiation in the damaged left hemisphere. In addition, event-related fMRI (ER-fMRI) experiments were performed to define the characteristics of the BOLD response. The shape is that of an inverted gamma function (similar to a negative mirror image of the known positive adult BOLD response). The maximum decrease was reached at 5-7 s with signal changes of -1.7 +/- 0.4%.Thus, this report describes for the first time the combined use of DTI and event-related fMRI in an infant and provides insight into the localization of the fMRI visual response in the young infant and the characteristics of the BOLD response.

Extraosseous Langerhans Cell Histiocytosis in Children

Langerhans cell histiocytosis, a rare disease that occurs mainly in children, may produce a broad range of manifestations, from a single osseous lesion to multiple lesions involving more than one organ or system. The clinical course varies widely in relation to the patients age. Multisystem disease may demonstrate especially aggressive behavior in very young children, with the outcome depending largely on the stage of disease and the degree of related organ dysfunction at the time of diagnosis. Extraosseous manifestations are less commonly seen than osseous ones and may be more difficult to identify. To accurately detect extraosseous Langerhans cell histiocytosis at an early stage, radiologists must recognize the significance of individual clinical and laboratory findings as well as the relevance of imaging features for the differential diagnosis. The pattern and severity of pulmonary, thymic, hepatobiliary, splenic, gastrointestinal, neurologic, mucocutaneous, soft-tissue (head and neck), and salivary involvement in Langerhans cell histiocytosis generally are well depicted with conventional radiography, ultrasonography, computed tomography, and magnetic resonance imaging. However, the imaging features are not pathognomonic, and a biopsy usually is necessary to establish a definitive diagnosis.

Extra-osseous involvement of Langerhans' cell histiocytosis in children

The predominant clinical and radiological features of Langerhans’ cell histiocytosis (LCH) in children are due to osseous involvement. Extra-osseous disease is far less common, occurring in association with bone disease or in isolation; nearly all anatomical sites may be affected and in very various combinations. The following article is based on a multicentre review of 31 children with extra-osseous LCH. The objective is to summarise the diverse possibilities of organ involvement. The radiological manifestations using different imaging modalities are rarely pathognomonic on their own. Nevertheless, familiarity with the imaging findings, especially in children with systemic disease, may be essential for early diagnosis.

Reliability of color Doppler and power Doppler sonography in the evaluation of intussuscepted bowel viability.

Abstract In two similar cases of irreducible intussusception with wall necrosis and perforation, symptoms had begun 24 h before admission and the plain radiographs showed signs of small-bowel obstruction. The sonographic (US) appearances, however, were different: color Doppler (CD) US showed vascular flow in the intussuscepted bowel in one case and no flow in the other. After radio-clinical assessment, both children underwent surgery and an intestinal resection had to be performed, manual reduction being impossible. The reliability of the US findings and the prognostic value of CD and power Doppler US in determining the viability of the intussuscepted bowel are discussed with a review of the literature.

Diffusion-weighted imaging findings in Perthes disease with dynamic gadolinium-enhanced subtracted (DGS) MR correlation: a preliminary study

BackgroundLegg-Calvé-Perthes disease (LCP) is necrosis of the proximal femoral epiphysis of vascular origin. Clinical course and outcome in LCP disease varies considerably between different patients. Earlier prognostic criteria than those offered by conventional radiography are necessary to identify children who require prompt surgical treatment.ObjectiveTo assess the significance of signal alteration on diffusion-weighted MR imaging (DWI MR) in LCP.Materials and methodsTwelve boys with unilateral LCP disease (Catterall grade 2 and 3), at the initial sclerotic stage and early fragmentation phase, underwent dynamic gadolinium-enhanced subtracted (DGS) and DWI MR. For DGS MR, the lateral pillar enhancement was recorded. For DWI imaging, we measured ADC values in the diseased and the unaffected epiphyses and metaphyses. Receiver operating characteristic curves were performed to analyze the performance of DWI in establishing agreement with the results of DGS MR, which is the gold standard for prognosis.ResultsFemoral epiphysis increased diffusivity was observed in the affected hip in all cases. Increased metaphysis diffusivity in the affected side was observed in all cases with absent lateral pillar enhancement at DGS MR.ConclusionDWI seems to be a noninvasive means of distinguishing between Perthes disease with favourable and unfavourable prognosis.

Uterocervicoplasty with a bladder mucosa layer for the treatment of complete cervical agenesis

OBJECTIVE To create an endocervical canal in a patient with a complete cervical agenesis. DESIGN Case report. SETTING University hospital. PATIENT(S) A 12-year-old girl presented with lower abdominal pain. On examination, complete vaginal agenesis was noted, with a 2-cm vaginal dimple. A pelvic magnetic resonance imaging scan disclosed an hematometra and absence of the cervix and vagina. INTERVENTION(S) Initial surgical therapy consisted of a vaginoplasty with a sigmoid bowel segment and opening of the uterus by puncture and stenting. The cervical permeation failed, with immediate complete stenosis. A new attempt was made through a low sagittal hysterotomy by removing a central muscular cylinder and lining the channel with a free tubularized bladder mucosa graft. A stent was left in place. MAIN OUTCOME MEASURE(S) Hysteroscopy, hysterography, and clinical follow-up evaluation. RESULT(S) The cervical stent was removed after 5 months. A hysterography and hysteroscopy confirmed the permeability of the cervix, which was lined by a well-vascularized longitudinally folded mucosa. Regular menses had been noted for more than 3 years as of this report. CONCLUSION(S) Cervicoplasty with mucosal lining permits the creation of a patent cervical canal, even in the reputedly unfavorable forms of congenital cervical agenesis.

Noninflammatory fallopian tube pathology in children

Noninflammatory tubal abnormalities are rare in children and usually not well covered by traditional educational material. The presenting symptoms are nonspecific and are common to many other conditions, so its preoperative diagnosis is rarely made. The purpose of this study was to review the hospital charts and imaging findings in children and sexually inactive adolescents who showed fallopian tube pathology. Understanding of the pertinent findings of previous imaging examinations might assist radiologists in making the correct preoperative diagnosis and increase the likelihood of preserving the fallopian tubes. The clinical entities described in this article include isolated tubal torsion, paratubal cysts, hydrosalpinx, undescended/ectopic fallopian tube, and tubal inguinal hernia.

Generalised infantile myofibromatosis with intracranial involvement: imaging findings in a newborn

Abstract. Infantile myofibromatosis is a disorder of infancy and early childhood characterised by single or multiple nodular lesions localised to bone, muscle, viscera, subcutaneous tissue or central nervous system. These pseudotumours contain smooth muscle and fibroblasts. The prognosis depends on the amount, as well as location, of the lesions. We present the radiological findings on plain films, US and MRI of a newborn who presented at birth with multiple organ involvement, including the CNS. To our knowledge, the comprehensive imaging features of such severe and multifocal involvement have not previously been reported.

Magnetic resonance imaging helps in the early diagnosis of myositis ossificans in children

Abstract Two cases on myositis ossificans circumscripta (MOC) in the arm are reported. Plain X-ray films and magnetic resonance imaging (MRI) were performed in both cases. In the first, an intramuscular tumor-like mass without calcifications was found on MRI with soft-tissue edema extension. In the second, MRI disclosed additional bone-marrow edema. The diagnosis of MOC was confirmed by biopsy in one case and by follow-up in the other. MOC is a benign soft-tissue lesion that is rare in children, with an acute course and usually spontaneously favorable evolution. The differential diagnosis from an infection or a malignant tumor remains difficult. The best imaging modalities are conventional radiography and MRI. The MRI patterns of MOC are typical but not pathognomonic; typical MRI findings in conjunction with clinical symptoms during the early phase of MOC permit the postponement of a biopsy or aggressive surgical procedures. Surgery is indicated for cases not showing typical MOC calcifications at a later stage.