Reduced gyrification in fetal growth restriction with prenatal magnetic resonance images.

Placental-related fetal growth restriction, resulting from placental dysfunction, impacts 3-5% of pregnancies and is linked to elevated risk of adverse neurodevelopmental outcomes. In response, the fetus employs a mechanism known as brain-sparing, redirecting blood flow to the cerebral circuit, for adequate supply to the brain. In this study we aimed to quantitatively evaluate disparities in gyrification and brain volumes among fetal growth restriction, small for gestational age and appropriate-for gestational-age fetuses. Additionally, we compared fetal growth restriction fetuses with and without brain-sparing. The study encompassed 106 fetuses: 35 fetal growth restriction (14 with and 21 without brain-sparing), 8 small for gestational age, and 63 appropriate for gestational age. Gyrification, supratentorial, and infratentorial brain volumes were automatically computed from T2-weighted magnetic resonance images, following semi-automatic brain segmentation. Fetal growth restriction fetuses exhibited significantly reduced gyrification and brain volumes compared to appropriate for gestational age (P < 0.001). Small for gestational age fetuses displayed significantly reduced gyrification (P = 0.038) and smaller supratentorial volume (P < 0.001) compared to appropriate for gestational age. Moreover, fetal growth restriction fetuses with BS demonstrated reduced gyrification compared to those without BS (P = 0.04), with no significant differences observed in brain volumes. These findings demonstrate that brain development is affected in fetuses with fetal growth restriction, more severely than in small for gestational age, and support the concept that vasodilatation of the fetal middle cerebral artery reflects more severe hypoxemia, affecting brain development.

Sarcopenia is a Predictor for Adverse Clinical Outcome in Pediatric Inflammatory Bowel Disease.

OBJECTIVES: Inflammatory bowel disease (IBD) has a high impact on nutritional status. Sarcopenia is related to higher risk of surgery and rescue therapy in adults with IBD; however, comparable data in pediatric populations are scarce. We evaluated muscle mass as a predictor of disease outcome in pediatric IBD. METHODS: All pediatric IBD patients who underwent magnetic resonance enterography (MRE) during 2008 to 2019 were included. Muscle mass was assessed by measuring the area of the psoas muscle at the upper level of L3 on MRE. The psoas area divided by the body surface area (BSA) yielded the psoas index. Clinical and radiological data, including disease location, activity, course, and medications were documented. The control group included non-IBD children who underwent an MR imaging study. RESULTS: We enrolled 101 IBD patients, 69 (68.3%) with Crohn disease (CD) and 32 (31.7%) with ulcerative colitis (UC) (mean age 15.03 ±â€Š3.27 years). The psoas index was significantly lower in the IBD patients compared with the 87 controls (326 vs 528, respectively, P < 0.001). Patients with a psoas index in the lowest quartile had significantly higher risk for biologic therapy (multivariate analysis, hazard ratio [HR] = 12.1, P = 0.046) and disease exacerbation (HR = 9, P = 0.047) independently of body mass index, compared with patients with a psoas index in the uppermost quartile. CONCLUSIONS: Sarcopenia correlates with the radiological severity of pediatric IBD and serves as a predictor for adverse clinical disease outcome. Muscle mass measurement in MRE studies may serve as a possible marker for disease outcome in this population.

Fetal Ventriculomegaly and Hydrocephalus - What Shouldn't be Missed on Imaging?

Fetal ventriculomegaly is one of the most frequently diagnosed abnormalities detected prenatally. The finding of additional subtle abnormalities can facilitate accurate prognoses, which may range from normal outcomes to significant neurodevelopmental sequelae. Pathogenesis and imaging patterns of ventriculomegaly and hydrocephalus in the fetus based on the pattern-recognition approach using fetal MRI are reviewed in this paper. This radiological approach may shed light on clinical course prediction and therapeutic efficacy of hydrocephalus in the fetus.

Semiautomatic segmentation and follow-up of multicomponent low-grade tumors in longitudinal brain MRI studies.

PURPOSE: Tracking the progression of low grade tumors (LGTs) is a challenging task, due to their slow growth rate and associated complex internal tumor components, such as heterogeneous enhancement, hemorrhage, and cysts. In this paper, the authors show a semiautomatic method to reliably track the volume of LGTs and the evolution of their internal components in longitudinal MRI scans. METHODS: The authors' method utilizes a spatiotemporal evolution modeling of the tumor and its internal components. Tumor components gray level parameters are estimated from the follow-up scan itself, obviating temporal normalization of gray levels. The tumor delineation procedure effectively incorporates internal classification of the baseline scan in the time-series as prior data to segment and classify a series of follow-up scans. The authors applied their method to 40 MRI scans of ten patients, acquired at two different institutions. Two types of LGTs were included: Optic pathway gliomas and thalamic astrocytomas. For each scan, a "gold standard" was obtained manually by experienced radiologists. The method is evaluated versus the gold standard with three measures: gross total volume error, total surface distance, and reliability of tracking tumor components evolution. RESULTS: Compared to the gold standard the authors' method exhibits a mean Dice similarity volumetric measure of 86.58% and a mean surface distance error of 0.25 mm. In terms of its reliability in tracking the evolution of the internal components, the method exhibits strong positive correlation with the gold standard. CONCLUSIONS: The authors' method provides accurate and repeatable delineation of the tumor and its internal components, which is essential for therapy assessment of LGTs. Reliable tracking of internal tumor components over time is novel and potentially will be useful to streamline and improve follow-up of brain tumors, with indolent growth and behavior.

Neonatal absolute nucleated red blood cell counts do not predict the development of cystic periventricular leukomalacia.

BACKGROUND: Infants with severe intraventricular-periventricular hemorrhage (IVH) have higher absolute nucleated red blood cell counts (aNRBC) at birth (a marker of intrauterine hypoxia) than controls. Periventricular leukomalacia (PVL) is known to be associated with prenatal and postnatal events. Whether PVL is also linked to intrauterine hypoxia is unknown. OBJECTIVES: To test the hypothesis that infants with PVL have higher aNRBC counts at birth than controls. METHODS: We studied 14 very low birth weight infants with PVL and compared them with 14 pair-matched controls without PVL. Head ultrasound scans were performed in all infants on days 3-5 and 21-25 of life. Paired tests, Fisher exact tests and stepwise logistic regression were performed for analysis. RESULTS: The groups were similar for gestational age (GA), birth weight (BW), prolonged rupture of membranes (PROM), Apgar scores, IVH, and aNRBC counts. PVL correlated significantly with low partial pressure of CO2 (PCO2) and IVH (P < 0.01). In logistic regression, when GA, gender, PROM, antenatal steroid therapy, 1 (or 5) minute Apgar scores, IVH grade, nosocomial sepsis, patent ductus arteriosus, necrotizing enterocolitis (NEC), need for pressors, aNRBC counts and lowest PCO2 were used as independent variables, PCO2 (P = 0.002), IVH grade (P= 0.001), GA (P = 0.038), NEC (P = 0.061) and use of dopamine (P = 0.010) remained in the analysis (total R2 = 68.2%). CONCLUSIONS: In contrast to severe IVH, aNRBC counts do not predict the development of PVL.

Brain diffusivity in infants with hypoxic-ischemic encephalopathy following whole body hypothermia: preliminary results.

Hypoxic-ischemic encephalopathy is an important cause of neuropsychological deficits. Little is known about brain diffusivity in these infants following cooling and its potential in predicting outcome. Diffusion tensor imaging was applied to 3 groups: (1) three infants with hypoxic-ischemic encephalopathy: cooled; (2) three infants with hypoxic-ischemic encephalopathy: noncooled; and (3) four controls. Diffusivity values at the corticospinal tract, thalamus, and putamen were correlated with Apgar scores and early neurodevelopmental outcome. While cooled infants exhibited lower Apgar scores than noncooled infants, their developmental scores at a mean age of 8 months were higher. All groups differed in their diffusivity values with the cooled infants showing better values compared with the noncooled, correlating with early neurodevelopmental outcome. These preliminary results indicate that diffusion tensor imaging performed at an early age in infants with hypoxic-ischemic encephalopathy may forecast clinical outcome and support the neuroprotective effect of hypothermia treatment.

Role of 18F-FDG PET/CT in staging and follow-up of lymphoma in pediatric and young adult patients.

OBJECTIVE: To assess the role of 18F-Fluorodeoxyglucose (18F-FDG) PET/CT in pediatric patients with Hodgkin disease (HD) and non-Hodgkin lymphoma (NHL). MATERIALS AND METHODS: 31 patients, mean age 12.9 +/- 5.1, HD (n = 24), and NHL (n = 7) underwent 18F-FDG PET/CT at diagnosis (n = 31 studies) and later in the course of the disease (n = 75 studies). The findings of PET/CT were correlated with diagnostic CT and clinical follow-up. RESULTS: PET/CT findings resulted in a change of disease staging in 10 patients (32.3%), upstaging in 7 (22.6%) and downstaging in 3 (9.6%). On a lesion analysis, 164 disease sites were detected by PET/CT of which 38 were overlooked by DCT. At mid-treatment, PET was negative in 28 out of 31 patients (90%) with negative predictive value of 96% as all latter patients except for 1, were disease free (mean 15.4 +/- 8.8 months). The positive predictive value of persistent increased 18F-FDG uptake was 100% as 3 patients with latter findings had active disease. On the CT part, 76 residual masses were identified in 22 patients. Increased 18F-FDG uptake was detected in 11 masses in 4 patients who had active disease. Remaining 65 PET negative masses were false positive findings. The positive predictive value of residual CT mass was 14%. CONCLUSIONS: PET/CT is associated with change in staging in approximately 1 out of 3 pediatric patients with HD and NHL. When used for monitoring response to treatment, a negative study is associated with disease-free period, even when residual mass is detected. A positive PET study indicates residual malignant disease.

Evidence of a developmental cerebello-cerebral disorder.

The objective of this study was to examine the cognitive and neuroanatomical features of an adolescent with developmental hypoplastic left cerebellum who presented with executive and visuo-spatial deficits, nonverbal learning disabilities and interpersonal difficulties. He underwent a neuropsychological assessment, MRI and diffusion tensor imaging MRI. The neuropsychological impairments were primarily in executive functions, spatial and visual perception, graphomotor skills, arithmetic, social perception and comprehension. Fractional anisotropy, which is measured by diffusion tensor imaging and enables assessment of axonal integrity, was reduced in the right cerebral peduncle and right hemisphere white matter (p < 0.001). Based on the results, we hypothesize that disruption of neural circuits linking the hypoplastic left cerebellum to the right hemisphere may contribute to the evolution of a neurocognitive syndrome with characteristics of the developmental right hemisphere syndrome and suggestive of the cerebellar cognitive-affective syndrome.

Predominantly upper limb weakness, enlarged cisterna magna, and borderline intelligence in a child with de novo mutation of the skeletal muscle alpha-actin gene.

We present a 10-year-old boy from nonconsanguineous parents of Libyan (Sephardi) Jewish origin. Mild dysmorphism, hypotonia, and clubfoot deformities were noted at birth. On follow-up, he had borderline intelligence and nonprogressive muscle weakness, predominantly in the upper extremities. Physical examination revealed mild facial weakness, a bell-shaped chest cavity, kyphosis, winging of the scapula, and hypotonia of the shoulder girdle. Muscle biopsy demonstrated prominent variation in fiber size and central nuclei and numerous subsarcolemmal particles on modified Gomori trichrome stains. Electron microscopy depicted areas of disrupted sarcomeres with abnormal aggregates. Brain magnetic resonance imaging showed mild widening of the lateral ventricles and an enlarged cisterna magna. Molecular DNA analysis by polymerase chain reaction (PCR) and direct sequencing revealed a de novo heterozygous missense mutation in the skeletal muscle alpha-actin gene (ACTA1) changing codon 348 from TCG serine to TTG leucine.

Mass at the splenic hilum: a clue to torsion of a wandering spleen located in a normal left upper quadrant position.

Wandering spleen is an extremely rare condition in which the spleen is lacking its normal ligamentous attachments and therefore can move to an ectopic position in the abdomen or pelvis. Wandering spleen predisposes the patient to life-threatening complications due to torsion of the spleen's vascular pedicle, with resulting splenic infarction, portal hypertension, and bleeding. Because of the nonspecific symptoms, imaging plays an important role. To our knowledge, only a few case reports describing wandering spleen in the pediatric population have been published, including 2 cases in infancy. We report a case of splenic torsion in an infant with a preoperative diagnosis made on the basis of color and power Doppler sonography. The diagnosis was confirmed by contrast-enhanced helical computed tomography (CT) and proved at surgery. On sonography, a diffusely hypoechoic spleen and a mass at the splenic hilum representing the torsed splenic pedicle were shown. The splenic hilar mass correlates with the CT "whirl" sign indicative of torsion.

Fetal cytomegalovirus infection of the brain: the spectrum of sonographic findings.

BACKGROUND AND PURPOSE: Congenital cytomegalovirus (CMV) infection can cause a wide range of brain anomalies. These changes have been well described postnatally, but descriptions of their in utero evolution are scarce. The purpose of this study was to analyze the sonographic spectrum of intracranial abnormalities in fetuses with proved CMV infection and to determine characteristic patterns of this infection. METHODS: We reviewed the transabdominal and transvaginal sonograms of eight fetuses with proved CMV infection. The sonographic analysis searched for signs of ventriculitis, leukomalacia, calcification, vasculitis, and periventricular cyst formation. The gyral pattern, corpus callosum, and cerebellar morphology also were examined. Fetal MR imaging was performed in two cases. RESULTS: The sonographic diagnosis of suspected CMV infection was made at a mean gestational age of 27.5 weeks (range, 22-37 weeks). An abnormal pattern of periventricular echogenicity was found in all fetuses. Echogenic intraparenchymal foci and ventriculomegaly were present in five fetuses. Other signs of fetal infection were intraventricular adhesions, periventricular pseudocysts, sulcation and gyral abnormal patterns, hypoplastic corpus callosum, cerebellar and cisterna magna abnormalities, and signs of striatal artery vasculopathy. For all fetuses, transvaginal sonography provided additional information. Fetal MR imaging provided additional information in one case. CONCLUSION: The presence of the described sonographic findings, particularly if two or more are present in the same fetus, is an indication for CMV investigation.