Fertility, Pregnancy, and Clinical Outcomes after Uterine Arteriovenous Malformation Management.

STUDY OBJECTIVE: To evaluate fertility, pregnancy, and clinical outcomes after uterine arteriovenous malformation (UAVM) management. DESIGN: Single-center retrospective study (Canadian Task Force classification III). SETTING: One referral center. PATIENTS: Twenty-two patients with a UAVM diagnosed by magnetic resonance angiography or computed tomography angiography and managed by expectant management (EM) or uterine arterial embolization (UAE) during the study period were included. INTERVENTIONS: Nine of 22 patients underwent first-intention EM and 13 first-line UAE. Three of 9 EM patients (33.3%) required emergency second-intention UAE for nonresolution of the UAVM and severe genital bleeding. MEASUREMENTS AND MAIN RESULTS: To analyze fertility according to management approach, we defined 2 groups: EM only group (n = 6) and UAE group (n = 16; women who underwent first- or second-intention UAE). Overall, the median age was 29 years (range, 17-43). The mean follow-up after UAVM management was 39 months (range, 1-116). The success rate of the UAE procedure was 87.5% (14/16). Eight of 12 women (66.7%) who wished to conceive became pregnant: 2 of 5 (33.3%) in the EM group and 6 of 7 (85.7%) in the UAE group (p = 1). Overall, 11 patients (50%) conceived: 3 in the EM group (50%) and 8 in the UAE group (50%) (p = .9). The live birth rate was 36.4% (8/22) for the whole population. There were no miscarriages or ectopic pregnancies. The 8 women (72.7%) wishing to conceive who became pregnant all delivered: 1 by cesarean section and 7 by vaginal delivery. Median duration of pregnancy was 39 weeks and 5 days. The remaining 3 women (i.e., women who conceived but did not wish to become pregnant) had therapeutic abortions. One complication was experienced in the EM group (small for gestational age newborn) and none in the UAE group (p = .3). Limitations include retrospective design and small sample size. CONCLUSION: UAE for UAVM is an effective and safe technique. It does not impair fertility, and obstetric prognosis is good.

Ultrasound Presentation of a Disseminated Fetal and Neonatal Rhabdoid Tumor.

This is a case report of a disseminated fetal rhabdoid tumor discovered at 32 weeks of gestation in a 29-year-old woman on immunosuppressive therapy. The mother consulted for a decrease in fetal movement. Fetal ultrasound showed signs of a disseminated tumor affecting the left armpit, liver, spleen, and limbs. A caesarian section was performed because of signs of fetal distress. Immunohistochemical analysis of a fetal biopsy showed deletion of the SMARCB1 gene. Pathological analysis of the placenta showed a rhabdoid tumor invading both fetal and maternal compartments. The mother underwent a whole-body MRI, and no metastasis was found. To the best of our knowledge, this is the first report of a disseminated rhabdoid tumor invading both fetal and maternal compartments.

Fetal costello syndrome with neuromuscular spindles excess and p.Gly12Val HRAS mutation.

Costello syndrome (CS) is a rare multiple congenital disorder caused by activating germline mutations in HRAS gene and is characterized by coarse facial features, severe feeding difficulties, failure to thrive, mild to severe intellectual disability, severe postnatal growth retardation, cardiac abnormalities or cancer predisposition. Phenotypic spectrum associated with HRAS mutations is broad, ranging from attenuated CS phenotype to neonatal and lethal forms with limited genotype-phenotype correlations. Congenital myopathy with neuromuscular spindle excess has been rarely described in the literature. We report a new severe fetal case of CS with distal arthrogryposis due to neuromuscular spindle excess, confirmed by the detection of the p.Gly12Val mutation in HRAS gene. This case emphasizes the fact that HRAS is the only gene responsible for neuromuscular spindle excess, underlines a correlation between p.Gly12Val mutation and severe CS phenotype and points out the importance of a muscle biopsy performed according to the suitable procedure in neuromuscular disorders for any fetal arthrogryposis.

Early MRI in neonatal hypoxic-ischaemic encephalopathy treated with hypothermia: Prognostic role at 2-year follow-up.

UNLABELLED: The prognostic role of early MRI (≤ 6 days of life) is still uncertain in hypoxic-ischaemic encephalopathy (HIE) treated with hypothermia. OBJECTIVE: To compare the prognostic value of early (≤ 6 days) and late MRIs (≥ 7 days) in predicting adverse outcome at 2 years old in asphyxiated term neonates treated with hypothermia. METHODS: This retrospective study included all asphyxiated neonates eligible for hypothermia treatment between November 2009 and July 2012. Two MRI scans were performed at a median age of day 4 (early MRI) and day 11 (late MRI). Two radiologists analysed independently each MRI. Imaging was classified as normal/subnormal or abnormal, using a visual analysis. Apparent diffusion coefficient (ADC) values were measured within predefined areas and posterior limb of internal capsule (PLIC) signal intensity was analysed. Neurodevelopmental outcome was assessed at 18-41 months (median age 24 months) as favourable or adverse. RESULTS: Of the 38 neonates followed up, 8 had an adverse outcome, all related to abnormal MRIs. Twenty-nine neonates had both MRIs sequentially. Both early and late MRIs yielded 100% sensitivity for adverse outcome by using the visual analysis. Early MRI had a higher specificity than late MRI (96.3% versus 89.3%). ADC measurements did not provide further information than visual analysis. PLIC signal abnormalities were a good predictor of adverse outcome on both MRIs. CONCLUSION: Early MRI (≤ 6 days) was a good predictor of neurodevelopmental outcome at 2 years old. It could reliably guide intensive care decisions after the end of hypothermia treatment.

Comparison of early and late MRI in neonatal hypoxic-ischemic encephalopathy using three assessment methods.

BACKGROUND: There is no consensus on the optimum timing of MRI in neonates with hypoxic-ischemic encephalopathy treated with hypothermia. Reliable early imaging assessment might help managing treatment. OBJECTIVE: To assess non-random differences between early and late MRI that might influence intensive-care decisions. MATERIALS AND METHODS: This single-center retrospective study included all asphyxiated term neonates eligible for hypothermia treatment November 2009-July 2012. MRI scans were systematically performed at day 4 (early MRI) and day 11 of life as part of routine protocol. Two experienced pediatric radiologists reviewed both scans according to three assessment methods: a pattern classification, a scoring system and a simplified classification. Agreement between early and late imaging findings was assessed using Cohen's kappa coefficients. RESULTS: Thirty-three neonates were included. Interobserver agreement was excellent. Early MRI detected all severe injuries. Agreement between early and late MRI was excellent for the simplified classification (κ = 0.82), good for the pattern classification (κ = 0.64), and good to excellent for 3 scores out of 4 in the scoring system (κ = 0.70-0.89). CONCLUSION: Early MRI may provide valuable information about brain injury to help parents and neonatologists in intensive-care decisions at the end of hypothermia treatment.

Imaging of musculoskeletal disorders related to pregnancy.

OBJECTIVE: This article provides an overview of the typical appearance of biomechanical and physiologic changes in pregnancy and an update on related pathophysiology. Conditions occurring during the childbearing, delivery and postpartum periods will be reported separately. CONCLUSION: Pregnancy causes biomechanical and physiologic changes that may be responsible for a wide spectrum of musculoskeletal disorders in the mother.

Whole-body post-mortem computed tomography compared with autopsy in the investigation of unexpected death in infants and children.

OBJECTIVES: To investigate the contribution of whole-body post-mortem computed tomography (PMCT) in sudden unexpected death in infants and children. METHODS: Forty-seven cases of sudden unexpected death in children investigated with radiographic skeletal survey, whole-body PMCT and autopsy were enrolled. For imaging interpretation, non-specific post-mortem modifications and abnormal findings related to the presumed cause of death were considered separately. All findings were correlated with autopsy findings. RESULTS: There were 31 boys and 16 girls. Of these, 44 children (93.6 %) were younger than 2 years. The cause of death was found at autopsy in 18 cases (38.3 %), with 4 confirmed as child abuse, 12 as infectious diseases, 1 as metabolic disease and 1 as bowel volvulus. PMCT results were in accordance with autopsy in all but three of these 18 cases. Death remains unexplained in 29 cases (61.7 %) and was correlated with no abnormal findings on PMCT in 27 cases. Major discrepancies between PMCT and autopsy findings concerned pulmonary analysis. CONCLUSIONS: Whole-body PMCT may detect relevant findings that can help to explain sudden unexpected death and is essential for detecting non-accidental injuries. We found broad concordance between autopsy and PMCT, except in a few cases of pneumonia. It is a non-invasive technique acceptable to relatives. KEY POINTS: • Whole-body post-mortem computed tomography (PMCT) is an effective non-invasive method. • Whole-body PMCT is essential for detecting child abuse in unexpected death. • There is concordance on cause of death between PMCT and autopsy. • Whole-body PMCT could improve autopsy through dissection and sampling guidance. • PMCT shows findings that may be relevant when parents reject autopsy.

Imaging of biliary disorders in children.

Biliary atresia and related disorders of the biliary tree, such as choledochal cyst, must be considered in the differential diagnosis of prolonged conjugated hyperbilirubinemia in infants and children. Pediatric biliary tract diseases include a variety of entities with a wide range of clinical presentations. Radiology plays an important role in the diagnosis and management of these pathologies. Unrecognized causes of biliary disease, like biliary atresia, can lead to liver transplantation during the first year of life. The aim of this article is to review the imaging of pediatric biliary disorders, including the implications of interventional radiology in some biliary diseases.

Coronary artery imaging during preoperative CT staging: preliminary experience with 64-slice multidetector CT in 99 consecutive patients.

The purpose of this study was to evaluate the clinical feasibility of coronary artery imaging during routine preoperative 64-slice MDCT scans of the chest. Ninety-nine consecutive patients in sinus rhythm underwent a biphasic multidetector-row spiral CT examination of the chest without the administration of beta-blockers, including an ECG-gated acquisition over the cardiac cavities, followed by a non-gated examination of the upper third of the thorax. Data were reconstructed to evaluate coronary arteries and to obtain presurgical staging of the underlying disease. The percentage of assessable segments ranged from 65.4% (972/1,485) when considering all coronary artery segments to 88% (613/693) for the proximal and mid segments, reaching 98% (387/396) for proximal coronary artery segments. The 387 interpretable proximal segments included 97 (97%) LM, 99 (100%) LAD, 96 (97%) LCX and 95 (96%) RCA with a mean attenuation of 280.70+/-52.93 HU. The mean percentage of assessable segments was significantly higher in patients with a heart rate