Monochorionic Twin Complications and Fetoscopic Interventions.

Monochorionic twins are at risk for complications due to the presence of placental vascular anastomoses, including twin-twin transfusion syndrome, twin anemia-polycythemia sequence, selective fetal growth restriction, and twin reversed arterial perfusion sequence. While ultrasound is the primary modality to screen for the development of these complications, MRI plays an important role in assessing monochorionic twin pregnancies for the development of other complications, such as neurologic injury. In this article, the authors review the ultrasound imaging findings associated with monochorionic twin complications, management options, and the role for MRI in these pregnancies.

Dysfunctional Postnatal Mitochondrial Energy Metabolism in a Patient with Neurodevelopmental Defects Caused by Intrauterine Growth Restriction Due to Idiopathic Placental Insufficiency.

We report the case of a four-year-old male patient with a complex medical history born prematurely as the result of intrauterine growth restriction due to placental insufficiency. His clinical manifestations included severe neurodevelopmental deficits, global developmental delay, Pierre-Robin sequence, and intractable epilepsy with both generalized and focal features. The proband's low levels of citrulline and lactic acidosis provoked by administration of Depakoke were evocative of a mitochondrial etiology. The proband's genotype-phenotype correlation remained undefined in the absence of nuclear and mitochondrial pathogenic variants detected by deep sequencing of both genomes. However, live-cell mitochondrial metabolic investigations provided evidence of a deficient oxidative-phosphorylation pathway responsible for adenosine triphosphate (ATP) synthesis, leading to chronic energy crisis in the proband. In addition, our metabolic analysis revealed metabolic plasticity in favor of glycolysis for ATP synthesis. Our mitochondrial morphometric analysis by transmission electron microscopy confirmed the suspected mitochondrial etiology, as the proband's mitochondria exhibited an immature morphology with poorly developed and rare cristae. Thus, our results support the concept that suboptimal levels of intrauterine oxygen and nutrients alter fetal mitochondrial metabolic reprogramming toward oxidative phosphorylation (OXPHOS) leading to a deficient postnatal mitochondrial energy metabolism. In conclusion, our collective studies shed light on the long-term postnatal mitochondrial pathophysiology caused by intrauterine growth restriction due to idiopathic placental insufficiency and its negative impact on the energy-demanding development of the fetal and postnatal brain.

Clinical spectrum of orbital and ocular abnormalities on fetal MRI.

BACKGROUND: Fetal magnetic resonance imaging (MRI) may reveal sonographically occult ocular abnormalities. When discovered, acquired causes and genetic associations must be sought. OBJECTIVE: We aim to evaluate a fetal cohort with orbit and/or globe malformations to determine whether there are imaging patterns that suggest the underlying cause. MATERIALS AND METHODS: We searched all fetal MRI reports performed at an academic children's hospital over 9 consecutive years for orbit and/or globe abnormalities. Each positive exam and all follow-up MRIs were evaluated for interocular distance, globe size, shape and signal, and brain malformations. Genetic and clinical diagnoses were recorded from the medical record. RESULTS: Seventy-six of 3,085 fetuses (2.5%) were diagnosed with ocular and/or globe abnormalities; 50% had postnatal follow-up MR exams, all confirming the fetal MRI findings. Ninety-two percent (70/76) had concurrent brain malformations. Sixty-seven percent (51/76) were diagnosed with an underlying disorder and 39% of these were genetically proven. The most common diagnoses with ocular globe abnormalities included CHARGE (coloboma of the eye, heart anomaly, choanal atresia, retardation and genital and ear anomalies) syndrome, trisomy 13 syndrome, dystroglycanopathy, holoprosencephaly and diencephalic-mesencephalic junction dysplasia. Genetic diagnoses were more likely with ocular globe abnormalities than isolated orbital abnormalities (P=0.04). Sixty-seven percent of fetuses with ocular calcifications, hemorrhage and/or lens abnormalities had potential maternal risk factors (P=0.03). CONCLUSION: Malformed ocular globes are associated with brain malformations and genetic abnormalities. Ocular calcifications, hemorrhage and/or lens abnormalities may be associated with maternal risk factors. Genetic work-up should be considered when an ocular globe size or shape abnormality is detected.

Prevalence and Risk Factors for Pharmacoresistance in Children With Focal Cortical Dysplasia-Related Epilepsy.

BACKGROUND AND OBJECTIVES: Focal cortical dysplasia is the most common cause of surgically-remediable epilepsy in children. Little is known about the risk factors for the timing and development of pharmacoresistance in this population. This study sought to evaluate the prevalence and risk factors for pharmacoresistance in pediatric FCD-related epilepsy. METHODS: In this retrospective single-center cohort design, patients were identified from search of centralized radiology report database and a central epilepsy surgical database. Inclusion criteria consisted of: 3T MRI-confirmed FCD from January, 2011 to January, 2020; ages 0 days to 22 years at MRI; at least 18 months of documented follow-up after MRI, unless had single seizure or incidentally discovered FCD. Records were excluded if there was dual pathology (except for mesial temporal sclerosis), hemimegalencephaly, or tuberous sclerosis complex present in imaging or history. RESULTS: One hundred forty-three patients with confirmed FCD met inclusion criteria. One hundred twenty-four children had epilepsy (87% of FCD patients) with median age of seizure onset 2.7 years (IQR 0.75-6 years, range 0 to 17 years). Twelve children (8.5%) had a single lifetime seizure (provoked or unprovoked) or recurrent provoked seizures. Seven children (4.9%) had incidental FCD. Ninety-two patients (74%) of those with epilepsy met criteria for pharmacoresistance. Of children with epilepsy of all types, 93 children (75%) were seizure-free at the last visit; Eighty-two patients underwent epilepsy surgery, of whom 59 (72%) achieved seizure freedom. 7% (9/124) achieved seizure freedom with a second ASM, and 5.6% (7/124) with a third or more ASMs. Failure of only one antiseizure medication is associated with enormous increased incidence and earlier development of pharmacoresistance (OR 346, 95% CI 19.6-6100). Cox regression showed FCD lobar location, pathologic subtype, and age of seizure onset are not. CONCLUSIONS: Failure of one antiseizure medication is associated with substantial risk of pharmacoresistance. These data support an operational re-definition of pharmacoresistance, for surgical planning, in FCD-related epilepsy to the failure of one antiseizure medication, and support early, potentially curative surgery to improve outcomes in this patient population.

Networks Underlie Temporal Onset of Dysplasia-Related Epilepsy: A MELD Study.

OBJECTIVE: The purpose of this study was to evaluate if focal cortical dysplasia (FCD) co-localization to cortical functional networks is associated with the temporal distribution of epilepsy onset in FCD. METHODS: International (20 center), retrospective cohort from the Multi-Centre Epilepsy Lesion Detection (MELD) project. Patients included if >3 years old, had 3D pre-operative T1 magnetic resonance imaging (MRI; 1.5 or 3 T) with radiologic or histopathologic FCD after surgery. Images processed using the MELD protocol, masked with 3D regions-of-interest (ROI), and co-registered to fsaverage_sym (symmetric template). FCDs were then co-localized to 1 of 7 distributed functional cortical networks. Negative binomial regression evaluated effect of FCD size, network, histology, and sulcal depth on age of epilepsy onset. From this model, predictive age of epilepsy onset was calculated for each network. RESULTS: Three hundred eighty-eight patients had median age seizure onset 5 years (interquartile range [IQR] = 3-11 years), median age at pre-operative scan 18 years (IQR = 11-28 years). FCDs co-localized to the following networks: limbic (90), default mode (87), somatomotor (65), front parietal control (52), ventral attention (32), dorsal attention (31), and visual (31). Larger lesions were associated with younger age of onset (p = 0.01); age of epilepsy onset was associated with dominant network (p = 0.04) but not sulcal depth or histology. Sensorimotor networks had youngest onset; the limbic network had oldest age of onset (p values <0.05). INTERPRETATION: FCD co-localization to distributed functional cortical networks is associated with age of epilepsy onset: sensory neural networks (somatomotor and visual) with earlier onset, and limbic latest onset. These variations may reflect developmental differences in synaptic/white matter maturation or network activation and may provide a biological basis for age-dependent epilepsy onset expression. ANN NEUROL 2022;92:503-511.

Proton and Multinuclear Spectroscopy of the Pediatric Brain.

Magnetic resonance spectroscopy (MRS) is a valuable adjunct to structural brain imaging. State-of-the-art MRS has benefited greatly from recent technical advancements. Neurometabolic alterations in pediatric brain diseases have implications for diagnosis, prognosis, and therapy. Herein, the authors discuss MRS technical considerations and applications in the setting of various pediatric disease processes including tumors, metabolic diseases, hypoxic/ischemic encephalopathy/stroke, epilepsy, demyelinating disease, and infection.

Round window anatomy predicts ease of cochlear implantation in children.

OBJECTIVES: We aim to evaluate the utility of the Round Window Angle (RWA) as a predictor of difficulty and operative time in cochlear implantation. METHODS: A retrospective study of pediatric patients that underwent cochlear implantation and CT temporal bone imaging from January 2008 to November 2019. Correlation, univariate, and multivariate analysis were conducted. RESULTS: 347 implantations met inclusion criteria. We found a difference in RWA for difficult (median: 101°, n = 5) and non-difficult (median: 74, n = 317) implantations (p < 0.0001). There was also a difference in RWA in patients with round windows visualized intra-operatively (p < 0.0197). When controlling for age and intraoperative round window visualization, logistic regression showed RWA was significantly associated with difficult insertion (OR: 1.687; p = 0.0246). Further, there was positive correlation between RWA and operative time (r = 0.1779, p = 0.0013) with patients with acute RWAs having shorter operative times (mean 115.7 ± 32.1 min) than those with obtuse RWA (mean 183.5 ± 97.0 min) (p = 0.0035). When accounting for surgeon and patient age, multivariate linear regression showed round window visualization (ß = 3.456, p = 0.0006) and obtuse RWA (ß = 6.172, p < 0.0001) was associated with an increase in operative time. CONCLUSION: Further research is needed to identify difficult cochlear implantations to increase the success and reduce risks associated with the surgery. Our study reports the possibility that an obtuse RWA both significantly increases difficulty and time of operation due to decreased round window visualization.

Spatiotemporal distribution and age of seizure onset in a pediatric epilepsy surgery cohort with cortical dysplasia.

OBJECTIVE: Focal Cortical Dysplasias (CD) are a common etiology of refractory pediatric epilepsy and are amenable to epilepsy surgery. We investigated the association of lesion volume and location to age of seizure onset among children with CD who underwent epilepsy surgery. METHODS: A retrospective study of epilepsy surgery patients with pathologically-confirmed CD. Regions of interest (ROI) determined preoperative lesion volumes on 1.5 T and 3 T T2 and SPGR MRIs, and location in 7 distributed neural networks. Descriptive and inferential statistics were used. RESULTS: Fifty-five patients were identified: 35 girls (56.5 %). Median age of seizure onset: 19.0 months (range 0.02 months - 16.0 years). Median age of surgery: 7.8 years (range 2.89 months - 24.45 years). CD were frontal (n = 21, 38 %); temporal (n = 15, 27 %); parietal (n = 10, 18 %); occipital (n = 3, 5%); multilobar (n = 6, 11 %). Frontal FCD had seizure onset < 1-year-old (P = 0.10); temporal lobe CD seizure onset was more likely > 5-years-old (P= 0.06). Median lesion volume for CD was 23.23 cm3 (range: 1.87-591.73 cm3). Larger CD lesions were associated with earlier epilepsy (P = 0.01, r = -0.16). We did not find that lesions proximal to early maturing cortical regions were associated with earlier seizure onset. We found an association with CD location in the default mode network (DMN) and age onset < 5years old (P = 0.03). Age of seizure onset was negatively correlated with percent of CD overlapping motor cortex (P = 0.001, r =-0.794) but not with CD overlap of the visual cortex (P = 0.35). There was no effect of CD type on age of epilepsy onset. SIGNIFICANCE: Larger CD lesions are associated with earlier onset epilepsy. CD most commonly occurs within the DMN and Limbic network, and DMN is associated with seizure onset before 5-years-old. Percent of CD overlapping motor cortex correlates with earlier seizure onset. These observations may reflect patterns of brain maturation or regional differences in clinical expression of seizures.

Taenia-tela choroidea complex and choroid plexus location help distinguish Dandy-Walker malformation and Blake pouch cysts.

BACKGROUND: Dandy-Walker malformation and Blake pouch cysts can have overlapping imaging features. The choroid plexus and associated taenia-tela choroidea complex are displaced inferolaterally in Dandy-Walker malformation and below the vermis in Blake pouch cysts. OBJECTIVE: To determine the normal fetal and postnatal MR appearance of the choroid plexus and taenia-tela choroidea complex, and whether their location can help distinguish Dandy-Walker malformation from Blake pouch cysts. MATERIALS AND METHODS: In this retrospective study, we evaluated brain MR exams from normal-appearing fetuses (gestational age 19-38 weeks) and infants, fetal and postnatal exams in Blake pouch cysts and Dandy-Walker malformation, and ambiguous cases equivocal for mild Dandy-Walker malformation and Blake pouch cysts. We documented choroid plexus and the taenia-tela choroidea complex location and axial and sagittal angles in each case. Then we contrasted and compared the original and updated fetal diagnoses based on taenia-tela choroidea complex and choroid plexus positions. RESULTS: The choroid plexus location and the taenia-tela choroidea complex location and angles varied significantly among normal exams, Blake pouch cyst exams and Dandy-Walker malformation exams (P<0.01). Dandy-Walker malformation showed inferolateral displacement of the taenia-tela choroidea complex and choroid plexus distant from the vermis. Adding the taenia-tela choroidea complex and choroid plexus into the assessment improved diagnostic accuracy, especially in ambiguous cases. CONCLUSION: The location of the taenia-tela choroidea complex and choroid plexus provided additional diagnostic neuroimaging clues that could be used in conjunction with other conventional findings to distinguish Dandy-Walker malformation and Blake pouch cysts. Normal, Blake pouch cyst, and Dandy-Walker malformation cases differed with regard to taenia-tela choroidea complex and choroid plexus position. Inferolateral taenia-tela choroidea complex displacement distant from the vermian margin was characteristic of Dandy-Walker malformation.

Prenatal diagnosis of diencephalic-mesencephalic junction dysplasia: Fetal magnetic resonance imaging phenotypes, genetic diagnoses, and outcomes.

OBJECTIVE: Report a single-center 12-year experience in the fetal diagnosis of diencephalic-mesencephalic junction dysplasia (DMJD) to expand the phenotype with Magnetic resonance imaging (MRI)-based classification, evaluate genetic etiologies, and ascertain outcomes. METHODS: Retrospective medical record and imaging review of all fetal MRI exams with DMJD were performed at our institution. RESULTS: Thirty-three pregnancies with fetal MRI findings of DMJD at 24 (18-37) weeks gestational age were studied; 70% were referred for fetal hydrocephalus. Three fetal MRI patterns were recognized. Type A (butterfly/hypothalamus-midbrain union) was seen in two cases (6%), Type B (partial thalamus-midbrain union) in 22 fetuses (70%), and Type C (complete/near complete midbrain-thalamic continuity) in nine fetuses (24%). L1CAM mutations were identified in four cases, and biallelic VRK1 variants in another. Among 14 live-born cases, 11 survived infancy, and 10 underwent postnatal brain MRI which confirmed the fetal MRI diagnosis in all but one case. Development was delayed in all surviving infants, most with additional neurological sequelae. CONCLUSIONS: DMJD may be identified by prenatal MRI as early as 18 weeks gestation. We propose three distinct phenotypic forms of DMJD, Types A-C. Next-generation sequencing provides an underlying molecular diagnosis in some patients, but further studies on associated genetic diagnoses and clinical outcomes are indicated.

The Role of Fetal MRI for Suspected Anomalies of the Posterior Fossa.

BACKGROUND: Posterior fossa anomalies can be diagnostic dilemmas during the fetal period. The prognosis for different diagnoses of the posterior fossa varies widely. We investigated whether fetal magnetic resonance imaging (MRI) and prenatal neurology consultation led to an alternate prognosis for fetuses referred due to concern for a fetal posterior fossa anomaly and concordance between pre- and postnatal diagnoses. METHODS: This is a retrospective study of cases referred to the Prenatal Pediatrics Institute at Children's National Hospital from January 2012 to June 2018 due to concern for posterior fossa anomaly. Each encounter was scored for change in prognosis based upon clinical and fetal MRI report. Postnatal imaging was compared with prenatal imaging when available. RESULTS: In total, 180 cases were referred for fetal posterior fossa anomalies based on outside obstetric ultrasound and had both fetal MRI and a neurology consultation. Fetal MRI and neurology consultation resulted in a change in fetal prognosis in 70% of cases. The most common referral diagnosis in our cohort was Dandy-Walker continuum, but it was not often confirmed by fetal MRI. In complex cases, posterior fossa diagnosis and prognosis determined by fetal MRI impacted choices regarding pregnancy management. Postnatal imaging was obtained in 57 (47%) live-born infants. Fetal and postnatal prognoses were similar in 60%. CONCLUSIONS: Fetal diagnosis affects pregnancy management decisions. The fetal-postnatal imaging agreement of 60% highlights the conundrum of balancing the timing of fetal MRI to provide the most accurate diagnosis of the posterior fossa abnormalities in time to make pregnancy management decisions.

A rare case of pediatric Tolosa-Hunt syndrome.

Tolosa-Hunt syndrome is a rare disorder characterized by granulomatous inflammation involving the cavernous sinus, superior orbital fissure, and/or orbit with no additional underlying cause. Tolosa-Hunt syndrome most often presents with painful ophthalmoplegia involving one or multiple cranial nerves. Here we report the case of an 8-year-old girl who presented, atypically, without the hallmark finding of pain. This case of pediatric Tolosa-Hunt syndrome is the only reported example to date lacking what is considered its pathognomonic feature and thus brings to light the clinical variability of this already inconspicuous disorder.

The fetal falx cerebelli.

BACKGROUND: The falx cerebelli is a retrocerebellar dural reflection. The MR spectrum of the fetal falx cerebelli has not been described. OBJECTIVE: To determine the prevalence of falx cerebelli abnormalities in the context of posterior fossa malformations and compare them to age-matched normal fetal MRI exams. MATERIALS AND METHODS: We reviewed all consecutive fetal MRIs performed over 1 year at a children's hospital. We assessed the falx cerebelli in each examination for location, morphology, size and number. Exams were grouped into (1) normal or non-brain/head abnormalities or (2) abnormal brain or craniofacial structures. We used chi square, linear regression and logistic regression analyses; P<0.05 was considered significant. RESULTS: We included 424 examinations (223 controls, 201 malformations) from 378 patients (mean gestational age 27±6 weeks). In the control group, the mean falx size was 2.6±1.2 mm (anteroposterior) × 11.0±3.2 mm (craniocaudal), with 80% retrovermian centered; the falx was linear (23%), Y-shape (15%), V-shape (22%) or U-shape (21%); it was unusually multiplicated (17%) or absent (<2%). Falx cerebellar abnormalities were more common in abnormal exams (59%; 119/201) than in normal exams (19%; 43/223) (P<0.001). The falx was abnormal with Blake pouch cysts (9/9, 100%) and rhombencephalosynapsis (3/4, 75%), absent in all Chiari II (n=9) and most Dandy-Walker malformations (5/6, 83%), commonly multiplicated in mega cisterna magna (14/22, 64%), and deviated or absent in cases with arachnoid cysts (3/3, 100%) and adhesions (4/5, 80%). CONCLUSION: Structural alterations of the falx cerebelli are more prevalent in fetuses with brain and craniofacial abnormalities. Specific changes offer clues to posterior fossa diagnoses.

Chordoma with lung metastases at initial presentation of a pediatric patient.

Chordomas are rare, slow growing malignant tumors derived from notochord remnants that can arise anywhere along the neuronal axis. Chordomas are particularly rare in patients under 20 years of age and tend to be intracranial in location, as opposed to sacrococcygeal in adults. Metastasis at initial presentation is uncommon in all age groups and is exceedingly rare in the absence of local recurrence of the primary tumor. We report a case of advanced clival chordoma with marked nasopharyngeal disease extension and lung metastases at the time of presentation in a pediatric patient.

ACR Appropriateness Criteria® Headache.

Headache is one of the most common human afflictions. In most cases, headaches are benign and idiopathic, and resolve spontaneously or with minor therapeutic measures. Imaging is not required for many types of headaches. However, patients presenting with headaches in the setting of "red flags" such as head trauma, cancer, immunocompromised state, pregnancy, patients 50 years or older, related to activity or position, or with a corresponding neurological deficit, may benefit from CT, MRI, or noninvasive vascular imaging to identify a treatable cause. This publication addresses the initial imaging strategies for headaches associated with the following features: severe and sudden onset, optic disc edema, "red flags," migraine or tension-type, trigeminal autonomic origin, and chronic headaches with and without new or progressive features. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Prevalence of pituitary cysts in children using modern magnetic resonance imaging techniques.

BACKGROUND: Pituitary pars intermedia/Rathke cleft cysts or cyst-like structures are commonly encountered in children undergoing brain magnetic resonance imaging (MRI), especially when examinations include thin-section, high-resolution sequences. OBJECTIVE: To determine the prevalence of pituitary cystic lesions in children at our institution using modern MRI technique, to assess for associated endocrinopathy and to address the need for follow-up. MATERIALS AND METHODS: We retrospectively reviewed 232 consecutive 1.5- and 3-T brain MRIs in children ages 0-18 years (mean: 8.3±5.3 years). We evaluated 3-D volumetric T1 spoiled gradient echo (SPGR) and axial T2-weighted sequences. Pituitary glands were evaluated for the presence, size and signal characteristics of cysts. Cyst volumes were measured in three orthogonal planes. Endocrine abnormalities were documented from the medical record. RESULTS: Pituitary cysts were present in 57.7% of children (n=134), with a mean linear measurement of 3.6±1.17 mm (range: 0.4 to 12.3 mm). The overwhelming majority of cysts were hyopointense on T1-W images (n=121, 90%) and isointense on T2-W images relative to the adenohypophysis (n=106, 79%). T1 hyperintense and T2 hypointense signals were present in a minority, 6.7% and 8%, respectively. Most cysts were occult on post-contrast T1-W images (n=24, 77%). Endocrine abnormalities were present in 2/134 (1.5%) of children with cysts (these were unrelated to the pituitary gland) versus 1/98 (1%) children without cysts (P=0.7). CONCLUSION: More often than not, pituitary cysts/cyst-like structures can be found incidentally in children using modern MRI techniques. Follow-up is not typically required if there are no associated symptoms or excessive size.

"Laser and the Tuber": thermal dynamic and volumetric factors influencing seizure outcomes in pediatric subjects with tuberous sclerosis undergoing stereoencephalography-directed laser ablation of tubers.

PURPOSE: Tuberous sclerosis (TSC) is a well-known cause of medically refractory epilepsy (MRE). Stereoencephalography-directed magnetic resonance-guided laser interstitial thermal therapy (SEEG-directed MRgLITT) is an emerging minimally invasive technique that appears aptly suited for the surgical management of TSC. Our aims are to present our experiences with patients who had undergone SEEG-directed MRgLITT to identify and treat cortical tubers responsible for clinical seizures and to perform an in-depth analysis of volumetric and thermal dynamic factors that may be related to seizure outcomes. METHODS: We studied all pediatric patients with MRE due to TSC who underwent SEEG-directed MRgLITT, investigating seizure outcomes in relation to thermal dynamic and volumetric factors. RESULTS: Eight cortical tubers from three pediatric patients were analyzed. Two of three patients had Engel I outcomes at last follow-up (median 18 months). Average A/T (ablation volume/tuber volume) ratio for Engel I outcomes was 1.28 (variance, 0.16) and 0.84 (variance, < 0.01) for all other outcomes (P = 0.035). There was a moderate positive correlation when comparing ablation energy to ablation volume (R2 = 0.65) in cortical tuber tissue. When the calcified tuber is excluded, the correlation is stronger (R2 = 0.77). Thus, the calculated energy needed to ablate 1 cm3 of cortical tuber tissue is 1263.6 J (calcified tuber) or 1089.5 J (non-calcified tuber). CONCLUSIONS: SEEG-directed MRgLITT appears to be a safe and effective technique in the management of pediatric patients with MRE due to TSC. The A/T ratio may be a useful indicator in predicting seizure outcomes.

ACR Appropriateness Criteria® Neuroendocrine Imaging.

Neuroendocrine dysfunction includes suspected hyper- and hypofunction of the pituitary gland. Causative lesions may include primary masses of the pituitary such as pituitary microadenomas and macroadenomas, as well as extrinsic masses, typically centered in the suprasellar cistern. Clinical syndromes related to hormonal dysfunction can be caused by excessive hormonal secretion or by inhibited secretion due to mass effect upon elements of the hypothalamic-pituitary axis. Additionally, complications such as hemorrhage may be seen in the setting of an underlying mass and can result in hormonal dysfunction. MRI with high-resolution protocols is the best first-line test to evaluate the sella turcica and parasellar region. CT provides complementary information regarding bony anatomy, and may be appropriate as a first-line test in certain instances, but it provides less detail and lesion characterization when compared to MRI. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.