Aggregatibacter actinomycetemcomitans infection in children: two case reports and a review of the literature.

Aggregatibacter actinomycetemcomitans (Aa), a Gram-negative coccobacillus commonly associated with endocarditis, poses a rare diagnostic challenge in pediatric cases. The presentation of two pediatric cases-myositis and chest mass-highlights novel aspects, including unusual symptom presentations in children which can be mistaken for malignancy. The limited sensitivity of standard blood tests complicates diagnosis, leading to delayed diagnosis and treatment. Representative samples must be taken, especially if blood cultures are negative. Despite advances in detection methods, diagnosing Aa infection remains difficult due to its rarity in children and variable clinical presentation. In conclusion, a comprehensive understanding of Aa infection in children is essential for early and effective diagnostic and therapeutic management.

The neuropsychology and neuroanatomy of reduplicative paramnesia.

Reduplicative paramnesia refers to the delusional belief that there are identical places in different locations. In this case-control study we investigated the clinical, phenomenological, neuropsychological and neuroanatomical data of eleven patients with reduplicative paramnesia and compared them against a control group of eleven patients with severe spatial disorientation without signs of reduplicative paramnesia. We show that most patients with reduplicative paramnesia report that a current place is reduplicated and/or relocated to an other familiar place. Patients with reduplicative paramnesia show a higher prevalence of deficits in the executive functions compared to the control patients, while mnestic and visuo-spatial deficits were both frequent in patients with reduplicative paramnesia and the control group. Patients with reduplicative paramnesia mostly suffer from right hemispheric lesions with a maximal overlap in the dorsolateral prefrontal cortex. Using lesion network mapping we show that lesions causing reduplicative paramnesia are connected to bilateral anterior insula and the right cingulate cortex. We argue that patients with reduplicative paramnesia fail to integrate the actual context with visuo-spatial memories and personal relevant emotional information due to a disruption of the neural network within the anterior temporal lobe, the cingulate cortex and the anterior insula. Also patients with reduplicative paramnesia are not able to resolve this conflict due to the lesion of the dorsolateral prefrontal cortex and executive dysfunction.

New insights in cerebral findings associated with fetal myelomeningocele: a retrospective cohort study in a single tertiary centre.

OBJECTIVE: To investigate cerebral anomalies other than Chiari type 2 malformation in fetuses with myelomeningocele (MMC). DESIGN: A retrospective cohort study in a single tertiary centre. SETTING: A review of associated cerebral anomalies in cases with prenatal diagnosis of myelomeningocele. POPULATION: Seventy cases of fetal myelomeningocele. METHODS: Ultrasound and MRI images were blindly reviewed. Postnatal imaging and results of the postmortem results were also reviewed. The association between cerebral anomalies and the following ultrasound findings was measured: level of the defect, ventriculomegaly, microcephaly and fetal talipes. MAIN OUTCOME MEASURES: A microcephaly was observed in 32/70 cases (46%) and a ventriculomegaly was observed in 39/70 cases (56%). Other cerebral anomalies were diagnosed in 47/70 (67%). RESULTS: Other cerebral anomalies were represented by 42/70 cases with abnormal CC (60%), 8/70 cases with perinodular heterotopia (PNH; 11%), 2/70 cases with abnormal gyration (3%). MRI performed only in fetal surgery cases confirmed the ulltrasound findings in all cases and provided additional findings in two cases (PNH). Risk ratios of fetal cerebral anomalies associated with MMC did not reach significance for microcephaly, ventriculomegaly, talipes or the level of the defect There was an overall good correlation between pre- and postnatal findings with a Kappa value of 0.79 [95% CI 0.57-1] and 82% agreement. CONCLUSION: Fetal brain anomalies other than Chiari type 2 malformation are frequently observed in fetuses with myelomeningocele, predominantly represented by CC anomalies. Whether these associated cerebral anomalies have an impact on selecting cases eligible for fetal surgery needs further evaluation. TWEETABLE ABSTRACT: Fetal cerebral anomalies other than Chiari type 2 malformation, microcephaly, and ventriculomegaly may be associated with MMC in up to 67% of the cases.

Added value of MRI for the diagnosis of adnexal torsion in children and adolescents after inconclusive ultrasound examination.

PURPOSE: The purpose of this study was to assess the performance of magnetic resonance imaging (MRI) in children and adolescents with suspected adnexal torsion (AT) after inconclusive initial ultrasound examination. MATERIALS AND METHODS: Twenty-eight girls with a mean age of 12±4 (SD) years (range: 1 month to 18years) were included. All had clinically suspected AT and inconclusive initial ultrasound findings followed by pelvic MRI as a second-line imaging modality. The final diagnosis was obtained by surgery or follow-up. Two radiologists blinded to the clinical, ultrasound and surgical data, retrospectively and independently reviewed MRI examinations. Clinical and MRI features associated with AT were searched for using univariate analyses. RESULT: Among the 28 patients, 10/28 patients (36%) had AT and 22/28 (79%) had an ovarian or tubal mass. AT was associated with an age<13years (OR: 10.7; 95% CI: 1.3-148.2) (P=0.022) and a whirlpool sign at MRI (OR: 61.0; median unbiased estimate, 7.2) (P<0.0001). When a mass was present, the best quantitative MRI criteria for AT were mass volume and ovary-corrected volume≥30cm3 (κ=0.72 and 0.61, respectively), mass axis length≥5cm (κ=0.90), and mass surface area≥14 cm2 (κ=0.58), with moderate to almost perfect interobserver agreement. The overall sensitivity, specificity and accuracy of MRI for the diagnosis of AT were 100% (10/10; 95% CI: 69-100), 94% (17/18; 95% CI: 73-100) and 96% (27/28; 95% CI: 82-100) respectively, with perfect interobserver agreement (κ=1). CONCLUSION: In pediatric patients with suspected AT and inconclusive initial ultrasound examination, a strategy including MRI as a second-line imaging modality should be considered if MRI does not delay a potential surgery.

Prognostic value of diffusion-weighted magnetic resonance imaging of brain in fetal growth restriction: results of prospective multicenter study.

OBJECTIVE: To measure prospectively apparent diffusion coefficient (ADC) values between 28 and 32 weeks of gestation in different cerebral territories of fetuses with estimated fetal weight (EFW) ≤ 5th centile, and analyze their association with adverse perinatal outcome. METHODS: This was a prospective study involving six tertiary-level perinatal centers. In the period 22 November 2016 to 11 September 2017, we included singleton, small-for-gestational-age (SGA) fetuses with EFW ≤ 5th percentile, between 28 and 32 weeks of gestation, regardless of the umbilical artery Doppler and maternal uterine artery Doppler findings. A fetal magnetic resonance imaging (MRI) examination with diffusion-weighted sequences (DWI) was performed within 14 days following inclusion and before 32 weeks. ADC values were calculated in the frontal and occipital white matter, basal ganglia and cerebellar hemispheres. An ultrasound examination was performed within 1 week prior to the MRI examination. The primary outcome was a composite measure of adverse perinatal outcome, defined as any of the following: perinatal death; admission to neonatal intensive care unit with mechanical ventilation > 48 h; necrotizing enterocolitis; Grade III-IV intraventricular hemorrhage; periventricular leukomalacia. A univariate comparison of median ADC values in all cerebral territories between fetuses with and those without adverse perinatal outcome was performed. The association between ADC values and adverse perinatal outcome was then analyzed using multilevel logistic regression models to adjust for other common prognostic factors for growth-restricted fetuses. RESULTS: MRI was performed in 64 patients, of whom five were excluded owing to fetal movement artifacts on DWI and two were excluded for termination of pregnancy with no link to fetal growth restriction (FGR). One intrauterine death occurred secondary to severe FGR. Among the 56 liveborn neonates, delivered at a mean ± SD gestational age of 33.6 ± 3.0 weeks, with a mean birth weight of 1441 ± 566 g, four neonatal deaths occurred. In addition, two neonates required prolonged mechanical ventilation, one of whom also developed necrotizing enterocolitis. Overall, therefore, seven out of 57 (12.3%) cases had an adverse perinatal outcome (95% CI, 3.8-20.8%). The ADC values in the frontal region were significantly lower in the group with adverse perinatal outcome vs those in the group with favorable outcome (mean values of both hemispheres, 1.68 vs 1.78 × 10-3 mm2 /s; P = 0.04). No significant difference in ADC values was observed between the two groups in any other cerebral territory. A cut-off value of 1.70 × 10-3 mm2 /s was associated with a sensitivity of 57% (95% CI, 18-90%), a specificity of 78% (95% CI, 63-88%), a positive predictive value of 27% (95% CI, 8-55%) and a negative predictive value of 93% (95% CI, 80-98%) for the prediction of adverse perinatal outcome. A mean frontal ADC value < 1.70 × 10-3 mm2 /s was not associated significantly with an increased risk of adverse perinatal outcome, either in the univariate analysis (P = 0.07), or when adjusting for gestational age at MRI and fetal sex (odds ratio (OR), 6.06 (95% CI, 0.9-37.1), P = 0.051) or for umbilical artery Doppler (OR, 6.08 (95% CI, 0.89-41.44)). CONCLUSION: This first prospective, multicenter, cohort study using DWI in the setting of SGA found lower ADC values in the frontal white-matter territory in fetuses with, compared with those without, adverse perinatal outcome. To determine the prognostic value of these changes, further standardized evaluation of the neurodevelopment of children born with growth restriction is required. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Evaluation of septal insertion of atrioventricular valves in fetuses by postmortem 4.7 Tesla cardiac MRI: A feasibility study.

PURPOSE: The purpose of this study was to compare non-invasive high-spatial-resolution postmortem cardiac magnetic resonance imaging (MRI) and autopsy findings for evaluating the septal insertion of atrioventricular valves in fetuses. MATERIALS AND METHODS: Five fetal heart specimens including two normal hearts, one heart with complete atrioventricular septal defect (AVSD) and two hearts with linear insertion of atrioventricular valves (LIAVV; gestational age 17 to 34 weeks) were studied with cardiac MRI using a 4.7 T MRI scanner without sample preparation. Three (3D) and two-dimensional (2D) turbo-RARE (rapid imaging with refocused echoes) sequences in four-chamber and left-ventricular long-axis planes were obtained with a minimal isotropic/in-plane resolution of 156µm. Nonparametric tests were performed to compare the distance between insertions of medial leaflets of the atrioventricular valves and the inlet/outlet distance ratio between MRI and autopsy findings in normal, complete AVSD and with linear insertion of atrioventricular valves (LIAVV) fetal hearts. RESULTS: Despite apparent differences between LIAVV/normal hearts, no significant differences were found between differential insertion of medial leaflets and inlet/outlet distance ratios with both techniques. Very good to excellent reliability between both techniques was found for differential insertion (ICC: 87.2%; 95% CI: -21.7%, 99.1%) (P=0.963) and inlet/outlet distance ratio (ICC 98.3%; 95%CI: 85.2%, 99.8%) (P=0.537) measurements. CONCLUSION: Postmortem cardiac MRI could replace autopsy for assessing normal or abnormal septal insertion of atrioventricular valves in fetuses without requiring specific preparation of the heart.

Towards ontology-based decision support systems for complex ultrasound diagnosis in obstetrics and gynecology.

INTRODUCTION: We have developed a new knowledge base intelligent system for obstetrics and gynecology ultrasound imaging, based on an ontology and a reference image collection. This study evaluates the new system to support accurate annotations of ultrasound images. We have used the early ultrasound diagnosis of ectopic pregnancies as a model clinical issue. MATERIAL AND METHODS: The ectopic pregnancy ontology was derived from medical texts (4260 ultrasound reports of ectopic pregnancy from a specialist center in the UK and 2795 Pubmed abstracts indexed with the MeSH term "Pregnancy, Ectopic") and the reference image collection was built on a selection from 106 publications. We conducted a retrospective analysis of the signs in 35 scans of ectopic pregnancy by six observers using the new system. RESULTS: The resulting ectopic pregnancy ontology consisted of 1395 terms, and 80 images were collected for the reference collection. The observers used the knowledge base intelligent system to provide a total of 1486 sign annotations. The precision, recall and F-measure for the annotations were 0.83, 0.62 and 0.71, respectively. The global proportion of agreement was 40.35% 95% CI [38.64-42.05]. DISCUSSION: The ontology-based intelligent system provides accurate annotations of ultrasound images and suggests that it may benefit non-expert operators. The precision rate is appropriate for accurate input of a computer-based clinical decision support and could be used to support medical imaging diagnosis of complex conditions in obstetrics and gynecology.

CT and MR imaging in congenital cardiac malformations: Where do we come from and where are we going?

The management of patients with congenital heart disease was profoundly changed firstly by the advent of pediatric and prenatal ultrasound and then more recently by cardiac magnetic resonance imaging (MRI) and computed tomography (CT) of the heart and great vessels. The improved life expectancy of these patients has brought about new medical and imaging requirements. MRI and CT are increasing second line techniques in this group of patients. This article summarizes the advantages and limitations of CT and MRI in some frequently encountered situations in children and adults followed up for congenital heart disease.

Prenatal features of isolated subependymal pseudocysts associated with adverse pregnancy outcome.

OBJECTIVES: To identify at prenatal ultrasound (US) the features of apparently isolated subependymal pseudocysts (SEPC) that may indicate underlying pathology and should lead to further investigations. METHODS: This was a retrospective study of cases with SEPC detected on prenatal US and/or magnetic resonance imaging (MRI). Those with apparently isolated SEPC at US were classified into two groups as follows: Group 1 (n = 29): normal prenatal US and MRI (except for SEPC) and normal outcome; Group 2 (n = 12): normal prenatal cerebral US (except for SEPC) and abnormal prenatal cerebral MRI with or without abnormal outcome. A third group (n = 9) included cases with abnormal prenatal US and MRI. The latter cases with obvious cerebral abnormalities at US were excluded from the statistical analysis as they do not represent a diagnostic dilemma for clinicians. Groups 1 and 2 were analyzed, comparing them with respect to their SEPC characteristics (size, number, location in relation to the caudothalamic notch and the ventricular horns and morphology) and extracerebral abnormalities. RESULTS: The mean ± SD SEPC great axis was longer in Group 2 (11.67 ± 5.82 mm) than it was in Group 1 (8.00 ± 5.64 mm) (P = 0.021), suggesting an optimal cut-off for size of SEPC of ≥ 9 mm (sensitivity = 75%, specificity = 62%) to maximize sensitivity for predicting pathological outcome. SEPC adjacent to the temporal horns and SEPC located posterior to the caudothalamic notch were observed more frequently in Group 2, indicating their association with poor outcome (P = 0.003 and P = 0.003, respectively). Atypical morphology and extracerebral abnormalities were observed more frequently in Group 2 (P = 0.013 and P = 0.044, respectively). There was no statistically significant difference between groups for either number or location of cysts along the inferior wall or adjacent to the lateral wall of the frontal horns (P = 0.591 and P = 0.156, respectively). CONCLUSION: When apparently isolated SEPC are observed at prenatal US, further investigations should be performed under the following circumstances: (1) SEPC great axis ≥ 9 mm; (2) SEPC adjacent to the occipital and temporal horns; (3) SEPC located posterior to the caudothalamic notch; (4) SEPC with atypical morphology.

Periventricular nodular heterotopia on prenatal ultrasound and magnetic resonance imaging.

OBJECTIVES: To describe the prenatal ultrasound and magnetic resonance imaging (MRI) findings suggestive of periventricular nodular heterotopia (PNH). METHODS: This retrospective case series included fetuses referred to our institution for brain MRI between 2007 and 2012, which were diagnosed with PNH and confirmed by postnatal MRI or autopsy. The type of PNH, associated ventriculomegaly and associated malformations are reported. RESULTS: We included 11 fetuses (nine female, two male) with a mean gestational age at diagnosis of 31 (range, 23-34) weeks. PNH lesions were small and diffuse (n = 7), large and multiple (n = 1) or single (n = 3). A targeted ultrasound examination performed before fetal MRI missed the diagnosis in four cases (one diffuse and three single); a further ultrasound examination performed after MRI diagnosed PNH in two of these four cases. Ventriculomegaly was present in six cases (four unilateral and two bilateral). PNH appeared in all cases as nodules of intermediate echogenicity protruding into the ventricular lumen. In all cases of diffuse PNH, the frontal horns and bodies of the lateral ventricles appeared square in shape on coronal view, with irregular borders on axial view. Associated cerebral malformations were observed in seven cases and included corpus callosal agenesis (n = 4, with additional malformations in two) and retrocerebellar cyst (n = 3). Extracerebral malformations were also present in two cases. Maternal MRI was performed in five of the six cases of isolated small and diffuse PNH in female fetuses, and demonstrated PNH in two of these. CONCLUSION: PNH is underdiagnosed at prenatal ultrasound, even on targeted scans. Irregular ventricular borders on axial view and irregular square-shaped lateral ventricles on coronal view are suggestive of PNH at prenatal ultrasound.

[Prenatal intestinal volvulus: A life-threatening event with good long-term outcome]. / Volvulus prénatal du grêle : risque vital immédiat mais bon pronostic à long terme.

BACKGROUND AND AIMS: To describe the outcome of neonates with prenatal intestinal volvulus. PATIENTS AND METHODS: All neonates with prenatal intestinal volvulus managed in our institution between May 2004 and December 2010 were retrospectively studied. All neonates with prenatal or neonatal diagnosis of prenatal intestinal volvulus were included. We analyzed age at diagnosis, fetal ultrasound (US) scan and magnetic resonance imaging (MRI) findings, clinical signs at birth, surgical findings, management, and postoperative outcome. RESULTS: Ten neonates with prenatal intestinal volvulus were identified. Prenatal US scans or MRI demonstrated evidence of meconium peritonitis in one fetus and bowel dilatation in 2 others. The mean gestational age at birth was 36 weeks (range, 31-38 weeks) and the mean birth weight was 2811g (range, 2050-3700g). One premature neonate developed respiratory distress and required ventilatory support at birth. In 7 neonates, clinical examination showed distended abdomen and emesis, whereas plain abdominal radiographs showed intestinal obstruction. All neonates underwent surgery and all had normal intestinal rotation, except one with total intestinal volvulus secondary to malrotation. Other causes of volvulus were suspected in 4 neonates: mesenteric defect (n=1), intestinal atresia (n=2) and narrow mesentery (n=1). Detorsion of total volvulus, ileostomy, or intestinal resection with primary anastomosis was performed in 2, 5, and 3 neonates, respectively. One patient with total intestinal volvulus secondary to malrotation died, whereas all other neonates survived. In one patient, the postoperative course was complicated by intestinal dysmotility of the distal small bowel requiring a secondary jejunoileostomy. Stoma closure was subsequently performed at 1 year of age with good outcome. One patient developed angiocholitis treated successfully with antibiotics. Median time to initiate enteral feeds was 7 days (range, 4-16 days) and all patients were subsequently weaned from parenteral nutrition. Median duration of parenteral nutrition was 29 days (range, 6-667 days). None of the patients had cystic fibrosis. Unlike postnatal volvulus, most prenatal volvulus occurs without malrotation. Although prenatal volvulus is a life-threatening condition, our results suggest that good long-term outcome can be achieved in most cases.

Correlation between pre- and postnatal cerebral magnetic resonance imaging.

OBJECTIVES: To evaluate the diagnostic accuracy of fetal cerebral magnetic resonance imaging (MRI) on a large cohort and to compare pre- and postnatal MRI data. METHODS: This prospective study included all cases referred to our unit for fetal cerebral MRI examination between June 2006 and December 2009 and which underwent at least one postnatal MRI examination. Cases in which there was termination of pregnancy, fetal death or stillbirth were excluded. The pre- and postnatal diagnoses established by MRI were compared and divided into five subgroups: same diagnosis on pre- and postnatal MRI (Group 1); same diagnosis but different appearance related to the natural course of the disease (Group 2); different diagnosis (related to limitations of fetal MRI) (Group 3); same diagnosis but with additional findings discovered on postnatal MRI examination (Group 4); or same diagnosis but different appearance related to the natural course of the disease (as in Group 2) and associated with additional findings discovered on postnatal MRI examination (Group 5). The prognostic impact of a possible disagreement between pre- and postnatal findings was evaluated. RESULTS: One hundred fetuses were included. Fetal MRI was performed at a mean gestational age of 33 (range, 24-39) weeks and postnatal MRI at a mean age of 3.5 months. There were 53 cases classified as Group 1, 32 in Group 2, four in Group 3, 10 in Group 4 and one in Group 5. Thus, in 15 cases (Groups 3-5), there were discrepancies between pre- and postnatal findings (mostly related to corpus callosum anatomy, cortical and migration disorders). The discrepancy was judged to have a prognostic impact in 9/15 cases. Two postnatal MRI examinations were performed in eight cases, in one of which the second examination showed subependymal heterotopia which were not detectable on the first examination. CONCLUSION: Pre- and postnatal MRI data showed good agreement in 85% of cases. There was disagreement with a prognostic impact in 9% of cases.