Fetal magnetic resonance imaging, ultrasound, and echocardiography findings in twin reversed arterial perfusion sequence.

BACKGROUND: Twin reversed arterial perfusion (TRAP) sequence is a rare complication of monochorionic multiple gestation pregnancies, in which the pump twin provides hemodynamic support to a nonviable co-twin (acardius). Fetal magnetic resonance imaging (MRI) is used to detect pump twin abnormalities, particularly brain ischemia, prior to fetal intervention to interrupt umbilical blood flow to the acardius. OBJECTIVE: To summarize the imaging findings of TRAP sequence pregnancies in a large series. MATERIALS AND METHODS: A single-center retrospective review was performed of all TRAP sequence pregnancies referred for fetal MRI (2004-2021). Fetal MRI, ultrasound, and echocardiography data were collected. RESULTS: Eighty-eight TRAP sequence pregnancies with MRI were included (mean gestational age, 19.8±2.8 weeks). Demise of the pump twin was noted in two pregnancies at the time of MRI. By MRI, 12% (10/86) of live pump twins had abnormalities, including 3% (3/86) with brain abnormalities and 9% (8/86) with extra-cranial abnormalities. By echocardiography, 7% (6/86) of pump twins had structural cardiac abnormalities. Three acardius morphological subtypes were identified by MRI: acephalus (55%, 48/88), anceps (39%, 34/88), and amorphous (7%, 6/88). The mean ultrasound acardius to pump twin ratio A/P ratio, calculated for each twin pair as the ratio of the acardius trunk (and head, if present) plus limb volume to the pump twin estimated fetal weight) differed among the three acardius subtypes (P=.03). The mean A/P ratio moderately correlated with pump twin cardiothoracic ratio and combined cardiac output (Pearson's r=0.45 and 0.48, respectively, both P<.001). CONCLUSION: Fetal MRI of TRAP sequence pregnancies found anomalies in a substantial number of pump twins. The three acardius subtypes differed in A/P ratio, which moderately correlated with the pump twin cardiothoracic ratio and combined cardiac output.

A Prospective Multi-Institutional Study Comparing the Brain Development in the Third Trimester between Opioid-Exposed and Nonexposed Fetuses Using Advanced Fetal MR Imaging Techniques.

BACKGROUND AND PURPOSE: While the adverse neurodevelopmental effects of prenatal opioid exposure on infants and children in the United States are well described, the underlying causative mechanisms have yet to be fully understood. This study aims to compare quantitative volumetric and surface-based features of the fetal brain between opioid-exposed fetuses and unexposed controls by using advanced MR imaging processing techniques. MATERIALS AND METHODS: This is a multi-institutional IRB-approved study in which pregnant women with and without opioid use during the current pregnancy were prospectively recruited to undergo fetal MR imaging. A total of 14 opioid-exposed (31.4 ± 2.3 weeks of gestation) and 15 unexposed (31.4 ± 2.4 weeks) fetuses were included. Whole brain volume, cortical plate volume, surface area, sulcal depth, mean curvature, and gyrification index were computed as quantitative features by using our fetal brain MR imaging processing pipeline. RESULTS: After correcting for gestational age, fetal sex, maternal education, polysubstance use, high blood pressure, and MR imaging acquisition site, all of the global morphologic features were significantly lower in the opioid-exposed fetuses compared with the unexposed fetuses, including brain volume, cortical volume, cortical surface area, sulcal depth, cortical mean curvature, and gyrification index. In regional analysis, the opioid-exposed fetuses showed significantly decreased surface area and sulcal depth in the bilateral Sylvian fissures, central sulci, parieto-occipital fissures, temporal cortices, and frontal cortices. CONCLUSIONS: In this small cohort, prenatal opioid exposure was associated with altered fetal brain development in the third trimester. This adds to the growing body of literature demonstrating that prenatal opioid exposure affects the developing brain.

Imaging Similarities Between Oral-Facial-Digital Syndrome Type 1 and Aicardi Syndrome: Prenatal and Postnatal Magnetic Resonance Imaging (MRI) Findings in 4 Patients.

Prenatal identification by magnetic resonance imaging (MRI) of callosal anomalies, particularly with accompanying intracranial abnormalities, poses a challenge for accurate prognostication and fetal counseling as outcome can vary widely depending on underlying etiology. In female patients, Aicardi syndrome is an important consideration, and prompt postnatal ophthalmologic assessment to identify ocular stigmata of Aicardi syndrome can aid with anticipatory guidance and greater vigilance for seizures. We present a case of a female with fetal and postnatal MRI findings of agenesis of corpus callosum and type 2b interhemispheric cysts, characteristically found in Aicardi syndrome, but was found to have oral-facial-digital syndrome type 1 (OFD1). We also present 3 other companion cases with pre- and postnatal imaging of patients with Aicardi syndrome. These cases highlight the importance of widening the differential diagnosis to also include OFD1 for female patients with callosal anomalies.

MRI Findings in Third-Trimester Opioid-Exposed Fetuses, With Focus on Brain Measurements: A Prospective Multicenter Case-Control Study.

BACKGROUND. The opioid epidemic has profoundly affected infants born in the United States, as in utero opioid exposure increases the risk of cognitive and behavioral problems in childhood. Scarce literature has evaluated prenatal brain development in fetuses with opioid exposure in utero (hereafter opioid-exposed fetuses). OBJECTIVE. The purpose of this study is to compare opioid-exposed fetuses and fetuses without opioid exposure (hereafter unexposed fetuses) in terms of 2D biometric measurements of the brain and additional pregnancy-related assessments on fetal MRI. METHODS. This prospective case-control study included patients in the third trimester of pregnancy who underwent investigational fetal MRI at one of three U.S. academic medical centers from July 1, 2020, through December 31, 2021. Fetuses were classified as opioid exposed or unexposed in utero. Fourteen 2D biometric measurements of the fetal brain were manually assessed and used to derive four indexes. Measurements and indexes were compared between the two groups by use of multivariable linear regression models, which were adjusted for gestational age (GA), fetal sex, and nicotine exposure. Additional pregnancy-related findings on MRI were evaluated. RESULTS. The study included 65 women (mean age, 29.0 ± 5.5 [SD] years). A total of 28 fetuses (mean GA at the time of MRI, 32.2 ± 2.5 weeks) were opioid-exposed, and 37 fetuses (mean GA at the time of MRI, 31.9 ± 2.7 weeks) were unexposed. In the adjusted models, seven measurements were smaller (p < .05) in opioid-exposed fetuses than in unexposed fetuses: cerebral frontooccipital diameter (93.8 ± 7.4 vs 95.0 ± 8.6 mm), bone biparietal diameter (79.0 ± 6.0 vs 80.3 ± 7.1 mm), brain biparietal diameter (72.9 ± 7.7 vs 74.1 ± 8.6 mm), corpus callosum length (37.7 ± 4.0 vs 39.4 ± 3.7 mm), vermis height (18.2 ± 2.7 vs 18.8 ± 2.6 mm), anteroposterior pons measurement (11.6 ± 1.4 vs 12.1 ± 1.4 mm), and transverse cerebellar diameter (40.4 ± 5.1 vs 41.4 ± 6.0 mm). In addition, in the adjusted model, the frontoocccipital index was larger (p = .02) in opioid-exposed fetuses (0.04 ± 0.02) than in unexposed fetuses (0.04 ± 0.02). Remaining measures and indexes were not significantly different between the two groups (p > .05). Fetal motion, cervical length, and deepest vertical pocket of amniotic fluid were not significantly different (p > .05) between groups. Opioid-exposed fetuses, compared with unexposed fetuses, showed higher frequencies of both breech position (21% vs 3%, p = .03) and increased amniotic fluid volume (29% vs 8%, p = .04). CONCLUSION. Fetuses with opioid exposure in utero had a smaller brain size and altered fetal physiology. CLINICAL IMPACT. The findings provide insight into the impact of prenatal opioid exposure on fetal brain development.

Extracallosal Structural Connectivity Is Positively Associated With Language Performance in Well-Performing Children Born Extremely Preterm.

Children born extremely preterm (<28 weeks gestation) are at risk for language delay or disorders. Decreased structural connectivity in preterm children has been associated with poor language outcome. Previously, we used multimodal imaging techniques to demonstrate that increased functional connectivity during a stories listening task was positively associated with language scores for preterm children. This functional connectivity was supported by extracallosal structural hyperconnectivity when compared to term-born children. Here, we attempt to validate this finding in a distinct cohort of well-performing extremely preterm children (EPT, n = 16) vs. term comparisons (TC, n = 28) and also compare this to structural connectivity in a group of extremely preterm children with a history of language delay or disorder (EPT-HLD, n = 8). All participants are 4-6 years of age. We perform q-space diffeomorphic reconstruction and functionally-constrained structural connectometry (based on fMRI activation), including a novel extension enabling between-groups comparisons with non-parametric ANOVA. There were no significant differences between groups in age, sex, race, ethnicity, parental education, family income, or language scores. For EPT, tracks positively associated with language scores included the bilateral posterior inferior fronto-occipital fasciculi and bilateral cerebellar peduncles and additional cerebellar white matter. Quantitative anisotropy in these pathways accounted for 55% of the variance in standardized language scores for the EPT group specifically. Future work will expand this cohort and follow longitudinally to investigate the impact of environmental factors on developing language networks and resiliency in the preterm brain.

Clinical Applications of Fetal MRI in the Brain.

Fetal magnetic resonance imaging (MRI) has become a widely used tool in clinical practice, providing increased accuracy in prenatal diagnoses of congenital abnormalities of the brain, allowing for more accurate prenatal counseling, optimization of perinatal management, and in some cases fetal intervention. In this article, a brief description of how fetal ultrasound (US) and fetal MRI are used in clinical practice will be followed by an overview of the most common reasons for referral for fetal MRI of the brain, including ventriculomegaly, absence of the cavum septi pellucidi (CSP) and posterior fossa anomalies.

Value of pre- and postnatal magnetic resonance imaging in the evaluation of congenital central nervous system anomalies.

Fetal MRI and neonatal MRI of the central nervous system (CNS) are complementary tools that can help to accurately counsel and direct the management of children with anomalies of the central nervous system. Postnatal MRI can add to fetal MRI by allowing for monitoring of changes in the severity of disease, better delineation of a suspected prenatal anomaly, evaluation for secondary pathologies related to the primary diagnosis, and surgical management direction. In this review we discuss the roles of fetal and neonatal MRI in the diagnosis and treatment of congenital anomalies of the CNS through a series of case examples and how both are important in patient management.

Short- and Long-Term Outcomes of Prenatally Diagnosed Dandy-Walker Malformation, Vermian Hypoplasia, and Blake Pouch Cyst.

Dandy-Walker continuum, which includes Dandy-Walker malformation, vermian hypoplasia, and Blake pouch cyst, is among the most commonly diagnosed posterior fossa malformation by fetal magnetic resonance imaging (MRI). The objective of our retrospective study was to evaluate fetal and postnatal MRI scan and clinical outcomes. Seventy-two patients were identified; 40 patients had postnatal imaging and follow-up (7 Dandy-Walker malformation, 26 vermian hypoplasia, and 7 Blake pouch cyst). Although all patients with Dandy-Walker malformation required ventriculoperitoneal shunts and 66% were intubated at birth, none required tracheostomy tube and 2 of 5 surviving children had no neurologic deficits. Vermian hypoplasia was strongly associated with genetic conditions and cardiac malformations; odds of not ambulating normally were 12 times greater if a syndrome or injury was present. Echocardiogram and genetic screening are recommended with vermian hypoplasia. There is a risk for epilepsy in both Dandy-Walker malformation and vermian hypoplasia. Blake pouch cyst can be complicated by hydrocephalus, but outcome is favorable.

Imaging of orbital infectious and inflammatory disease in children.

Most acute nontraumatic periorbital and intraorbital pathologies in pediatric patients have an underlying infectious or inflammatory etiology, and imaging frequently plays a key role in the workup and management of these children. In this paper we review the clinical presentation and imaging findings in children with some of the most common infectious and inflammatory diseases involving the orbit. Basic relevant anatomy and imaging findings on various imaging modalities are also reviewed.

Utilization of 3-T fetal magnetic resonance imaging in clinical practice: a single-institution experience.

BACKGROUND: As the safety and efficacy of fetal magnetic resonance imaging (MRI) at 3 tesla (T) continues to evolve, understanding its potential benefits and limitations is becoming increasingly important. OBJECTIVE: We aim to compare the image quality of fetal MRI between 1.5 T and 3 T in routine clinical practice. MATERIALS AND METHODS: Fetal MRIs performed at 3 T between Jan. 1, 2019, and Dec. 31, 2019, at our institution were retrospectively reviewed by four fellowship-trained subspecialty radiologists. Imaging quality by system, sequence and artifacts were compared with matched controls at 1.5 T and rated using a modified Likert scale. RESULTS: Thirty-three fetal MRIs at 3 T were reviewed, and a control group of studies for the same clinical indication and equivalent gestational age were selected for comparison. Two of the four radiologists preferred 3-T image quality of the brain with slight agreement among the four reviewers (k=0.19, P=0.01). Three of the four radiologists had no preference for 1.5 T vs. 3 T in the majority of cases in evaluating the chest and abdomen. In the overall assessment, 3 T was preferred in less than half of cases by all four radiologists (k=0.07, P=0.26). In the evaluation of standing wave, moire fringe and magnetic susceptibility artifacts, 3 T was not preferred in the majority of studies by all four radiologists. Total exam time was significantly longer in the 3-T fetal MRIs (75.0±15.1 min) compared to the 1.5-T fetal MRIs (55.5±13.3 min, P<0.001). CONCLUSION: While 3 T is a feasible alternative to 1.5 T for fetal MRI, the increased artifacts and longer exam times observed at 3 T without clear improvement in overall image quality make 1.5 T preferable for fetal MRI in routine clinical practice.

Imaging in the presence of meroanencephaly.

Meroanencephaly occurs when there is an incomplete open median calvarial defect. This condition, which is in the spectrum of anencephaly, results in ectopic brain without skin covering and a normal foramen magnum. We present a rare case of a female fetus with meroanencephaly referred to our institution at 24 weeks and imaged with both prenatal ultrasound and MRI, demonstrating an open neural tube defect in the high parietal area and lack of visualization of the supratentorial ventricular system. Postnatal the child survived and went on to require antibiotic therapy and closure of the defect without cerebral spinal fluid diversion but demonstrates severe permanent neurologic deficits.

Imaging of open spinal dysraphisms in the era of prenatal surgery.

Over the last decade fetal surgery to repair open spinal dysraphisms has become an acceptable and in some cases desirable alternative to the traditional method of postnatal closure. Fetal MRI is an essential part of the workup in these patients, not only to select the appropriate candidates for fetal surgery but also to guide prenatal counseling and perinatal management. In this article we review current surgical techniques for prenatal repair, relevant imaging findings in the era of fetal surgery, and expected imaging findings of the brain and spine in the fetal and postnatal periods.

Fetal and postnatal MRI findings of Blake pouch remnant causing obstructive hydrocephalus.

Blake pouch remnant, also known as Blake pouch cyst or persistent Blake pouch, is a posterior fossa embryologic anomaly that is often seen in isolation with most affected patients being asymptomatic. However, even in isolation, Blake pouch remnant can result in obstructive hydrocephalus requiring early neurosurgical intervention making it an important diagnosis for the fetal radiologist to consider. We present a rare case of a patient with prenatally diagnosed "inferior vermian hypoplasia" on fetal MRI that went on to develop progressive obstructive hydrocephalus in infancy secondary to what was determined to be a Blake pouch remnant.

Magnetic resonance imaging of the fetal brain in monochorionic diamniotic twin gestation: correlation of cerebral injury with ultrasound staging and survival outcomes.

BACKGROUND: The current staging system of twin-twin transfusion syndrome (TTTS) is based on the ultrasound criteria and does not consider the cerebral injury. OBJECTIVE: To assess the incidence of cerebral damage on fetal MRI and correlate abnormal cerebral diffusion-weighted imaging (DWI) findings with survival outcome and the ultrasound staging of TTTS. MATERIALS AND METHODS: We conducted a retrospective review of MRI/DWI of fetal brains and the electronic medical records in monochorionic diamniotic twin gestations. Axial DWI of each fetal brain was performed on 1.5-tesla (T) clinical magnet with b values of 0 s/mm2 and 700 s/mm2. We correlated MRI/DWI abnormalities with the Quintero staging system and survival outcomes of the fetuses. RESULTS: Thirty-four pregnancies (68 fetuses) with 42 fetal cerebral abnormalities were identified by MRI/DWI alone. Of these 42 fetal cerebral abnormalities, 33 fetal brain lesions were visible only on DWI (n=25 donor, n=8 recipients; n=30 unilateral, n=3 bilateral; n=26 diffuse, n=7 focal). Quintero staging in these 34 pregnancies was as follows: 9 Stage I, 7 Stage II, 13 Stage III, 4 Stage IV, 1 Stage V. There was no significant correlation between the presence of cerebral infarction or hemorrhage using MRI/DWI and ultrasound staging (P=0.138). The overall survival rate was 63.2% (43/68). There was a significant correlation between the presence of cerebral infarction or hemorrhage on MRI/DWI and delivery status (P=0.009). CONCLUSION: Abnormal cerebral imaging findings on MRI/DWI from hypoxic-ischemic injury or hemorrhage can be seen at the beginning of the second trimester and do not correlate with the current ultrasound staging system; however, they do correlate with decreased survival. Fetal cerebral abnormalities could be incorporated into the TTTS staging system as an independent risk factor.

Prenatal and postnatal MRI findings in open spinal dysraphism following intrauterine repair via open versus fetoscopic surgical techniques.

PURPOSE: The purpose of the study is to examine MRI findings of the brain and spine on prenatal and postnatal MRI following intrauterine repair of open spinal dysraphism (OSD) by open hysterotomy and fetoscopic approaches. MATERIALS AND METHODS: This study is a single-center HIPAA-compliant and IRB-approved retrospective analysis of fetal MRIs with open spinal dysraphism from January 2011 through December 2018 that underwent subsequent prenatal repair of OSD. RESULTS: Sixty-two patients met inclusion criteria: 47 underwent open repair, and 15 underwent fetoscopic repair, with an average gestational age of 22.6 ± 1.4 weeks at initial MRI. On postnatal MRI, spinal cord syrinx was seen in 34% (16/47) of patients undergoing open versus 33.3% (5/15) undergoing fetoscopic repair (P = 0.96). Postnatally, there was no significant difference in hindbrain herniation between the open versus fetoscopic repair groups (P = 0.28). Lateral ventricular size was significantly larger in the open (20.9 ± 6.7 mm) versus the fetoscopic repair (16.1 ± 4.9 mm) group (P = 0.01). CONCLUSION: Though lateral ventricular size in the open repair group was larger than the fetoscopic repair group, this can likely be explained by initial selection criteria used for fetoscopic repair. Other postoperative imaging parameters on postnatal MRI were not significantly different between the two groups.

Imaging diagnosis of ventriculomegaly: fetal, neonatal, and pediatric.

Ventriculomegaly is the term used to describe abnormal enlargement of ventricles in the brain. Neuroimaging, whether it is by ultrasound, computed tomography, or magnetic resonance imaging, is the key to its identification and can help to diagnose its cause and guide management in many cases. The implementation of the imaging modalities and potential differential considerations varies from the fetus, infant, and pediatric patient. Here we discuss how the imaging modalities can be used in these patient populations and review some of the differential considerations.

Prenatal evaluation of the Sakoda complex.

Sakoda complex is a rare but distinct combination of birth defects consisting of a basal cephalocele, agenesis of the corpus callosum, and midline cleft lip/palate. It has been reported in association with ophthalmologic abnormalities, cognitive deficits and severe epilepsy. Here we describe both prenatal and postnatal MRI findings of a classic case of Sakoda complex in a child with characteristic findings on fetal MRI; prenatal findings have not been described in the literature. Diagnosis of this entity has important implications for prenatal counseling and perinatal management, as is demonstrated in this case.

Fetal brain MRI findings and neonatal outcome of common diagnosis at a tertiary care center.

Fetal Magnetic Resonance Imaging (MRI) is increasingly used in prenatal evaluations. OBJECTIVE: Identify common brain malformations on fetal MRI and evaluate perinatal course. METHODS: Fetal consultations from 10/2016 to 12/2017 reviewed. RESULTS: Hundred consultations were requested; 94 were completed. Findings included: posterior fossa malformations (19%), agenesis/dysgenesis of corpus callosum (15%), congenital aqueductal stenosis (CAS) (14%), ventriculomegaly (11%), isolated cortical malformations (8.5%), and holoprosencephaly (6%). Posterior fossa malformations were more likely to be associated with genetic conditions and cardiac malformations. Patients with CAS all required intensive care unit admission. Overall, few patients with congenital brain malformations required feeding or respiratory support at discharge. None had seizures as neonates except two with early epileptic encephalopathy syndromes. CONCLUSIONS: Even though long term neurological prognosis is poor for many conditions including high lifetime risk of epilepsy, most are discharged with no feeding or respiratory support. Seizures are rarely seen in the neonatal period.