Congenital Hepatic Cyst in Patients With Patau Syndrome: A Rare Clinical Finding.

Trisomy 13 (T13), frequently referred to as Patau syndrome, is a rare autosomal aneuploidy most commonly due to nondisjunction in meiosis. Frequently seen characteristics include cleft lip, cleft palate, cerebral defects, anophthalmia, and polydactyly among many more. We report a rare case of a newborn female with T13, demonstrating several known anomalies associated with the syndrome and an associated large congenital hepatic cyst, exhibiting a significant mass effect on vital organs. Based on a literature review conducted in August 2023, we found no previous documentation of a congenital hepatic cyst reported with T13.

Left-Sided Sensorineural Hearing Loss and Facial Weakness in a 35-Year-Old Patient: A Diagnostic Challenge and Case Report.

We present the case of a 35-year-old patient who presented with a three-month history of left-sided sensorineural hearing loss and left-sided facial weakness. Initial imaging suggested a schwannoma, and the patient underwent ten treatments of intra-tympanic steroid injections and antibiotics, and was scheduled for surgery. However, the planned schwannoma removal surgery with gamma-knife was aborted due to the absence of the previously identified mass on the pre-procedure MRI. Subsequent imaging revealed continued enhancement of the left internal auditory canal (IAC), leading to considerations of lymphoma, sarcoidosis, IgG4 disease, or other inflammatory condition. The patient's symptoms have significantly improved since and are currently being conservatively managed and monitored. However, the patient continues to show persistent findings on MRI. This case highlights the diagnostic challenges faced in identifying the underlying etiology of this patient and emphasizes the need for further investigations and multidisciplinary management in patients with similar presentations.

False Positives in Artificial Intelligence Prioritization Software for Intracranial Hemorrhage Identification in the Postoperative Period: A Report of Two Cases.

The implementation of artificial intelligence (AI) in radiology has shown significant promise in the identification of acute intracranial hemorrhages (ICHs). However, it is crucial to recognize that AI systems may produce false-positive results, especially in the postoperative period. Here, we present two cases where AI prioritization software erroneously identified an acute ICH on a postoperative non-contrast CT. These cases highlight the need for a more careful radiology review of AI-flagged images in postoperative patients to avoid further unnecessary imaging and unwarranted concerns from radiologists, clinicians, and patients.

Brain MRI segmentation of Zika-Exposed normocephalic infants shows smaller amygdala volumes.

BACKGROUND: Infants with congenital Zika syndrome (CZS) are known to exhibit characteristic brain abnormalities. However, the brain anatomy of Zika virus (ZIKV)-exposed infants, born to ZIKV-positive pregnant mothers, who have normal-appearing head characteristics at birth, has not been evaluated in detail. The aim of this prospective study is, therefore, to compare the cortical and subcortical brain structural volume measures of ZIKV-exposed normocephalic infants to age-matched healthy controls. METHODS AND FINDINGS: We acquired T2-MRI of the whole brain of 18 ZIKV-exposed infants and 8 normal controls on a 3T MRI scanner. The MR images were auto-segmented into eight tissue types and anatomical regions including the white matter, cortical grey matter, deep nuclear grey matter, corticospinal fluid, amygdala, hippocampus, cerebellum, and brainstem. We determined the volumes of these regions and calculated the total intracranial volume (TICV) and head circumference (HC). We compared these measurements between the two groups, controlling for infant age at scan, by first comparing results for all subjects in each group and secondly performing a subgroup analysis for subjects below 8 weeks of postnatal age at scan. ZIKV-exposed infants demonstrated a significant decrease in amygdala volume compared to the control group in both the group and subgroup comparisons (p<0.05, corrected for multiple comparisons using FDR). No significant volume differences were observed in TICV, HC, or any specific brain tissue structures or regions. Study limitations include small sample size, which was due to abrupt cessation of extramural funding as the ZIKV epidemic waned. CONCLUSION: ZIKV-exposed infants exhibited smaller volumes in the amygdala, a brain region primarily involved in emotional and behavioral processing. This brain MRI finding may lead to poorer behavioral outcomes and warrants long-term monitoring of pediatric cases of infants with gestational exposure to Zika virus as well as other neurotropic viruses.

Malignant and Nonmalignant Sinonasal Tumors.

Sinonasal tumors are rare, diverse, complex lesions with overlapping demographic and clinical features. Malignant tumors are more common, with a grave prognosis, and require biopsy for accurate diagnosis. This article briefly reviews the classification of sinonasal tumors and provides imaging examples and imaging characteristics of each clinically important nasal and paranasal mass lesions. Although there are no true pathognomonic imaging features, it is important for the radiologist to have a broad knowledge of the various CT and MR imaging findings that can help narrow the differential diagnosis and aid in early diagnosis and mapping of tumor for treatment planning.

Maternal SARS-CoV-2, Placental Changes and Brain Injury in 2 Neonates.

Long-term neurodevelopmental sequelae are a potential concern in neonates following in utero exposure to severe acute respiratory syndrome coronavirus disease 2 (SARS-CoV-2). We report 2 neonates born to SARS-CoV-2 positive mothers, who displayed early-onset (day 1) seizures, acquired microcephaly, and significant developmental delay over time. Sequential MRI showed severe parenchymal atrophy and cystic encephalomalacia. At birth, neither infant was SARS-CoV-2 positive (nasopharyngeal swab, reverse transcription polymerase chain reaction), but both had detectable SARS-CoV-2 antibodies and increased blood inflammatory markers. Placentas from both mothers showed SARS-CoV-2-nucleocapsid protein and spike glycoprotein 1 in the syncytiotrophoblast, fetal vascular malperfusion, and significantly increased inflammatory and oxidative stress markers pyrin domain containing 1 protein, macrophage inflammatory protein 1 ßη, stromal cell-derived factor 1, interleukin 13, and interleukin 10, whereas human chorionic gonadotropin was markedly decreased. One infant (case 1) experienced sudden unexpected infant death at 13 months of age. The deceased infant's brain showed evidence of SARS-CoV-2 by immunofluorescence, with colocalization of the nucleocapsid protein and spike glycoprotein around the nucleus as well as within the cytoplasm. The constellation of clinical findings, placental pathology, and immunohistochemical changes strongly suggests that second-trimester maternal SARS-CoV-2 infection with placentitis triggered an inflammatory response and oxidative stress injury to the fetoplacental unit that affected the fetal brain. The demonstration of SARS-CoV-2 in the deceased infant's brain also raises the possibility that SARS-CoV-2 infection of the fetal brain directly contributed to ongoing brain injury. In both infants, the neurologic findings at birth mimicked the presentation of hypoxic-ischemic encephalopathy of newborn and neurologic sequelae progressed well beyond the neonatal period.

Utilization of computed tomography in pediatric temporal fractures: A dose reduction approach.

OBJECTIVES: METHODS: Retrospective chart review was performed of pediatric patients who underwent CT brain as part of a trauma pan-scan and dedicated temporal bone CT in the setting of head trauma. The original CT images were re-reviewed by two board certified Neuroradiologists in a blinded manner to determine the presence or absence of temporal bone fracture and if present, fracture line involvement of the 5 critical temporal bone anatomic structures. The dose length product (DLP), a measure of approximate total radiation dose delivered during CT scan (mGy-cm), was noted from the data available. RESULTS: There were 24 temporal bone fractures in a total of 29 patients (4 with bilateral fractures). There were 21 of 24 fractures that were correctly identified on trauma pan-scan CT with dedicated temporal bone CT considered as the diagnostic gold standard. There was a combined sensitivity and specificity of 91 % and 100 % respectively. The sensitivity and specificity in identifying involvement of critical structures were as follows: carotid canal (100 % and 100 %); ossicular chain (75 % and 100 %); tegmen tympani (60 % and 97.9 %); facial nerve canal (25 % and 100 %); otic capsule (N/A and 98.5 %). The median DLP for trauma pan-scan CT and temporal bone CT were 627 mGy-cm and 267 mGy-cm respectively. CONCLUSION: Dedicated TBCT is not required to accurately diagnose and characterize temporal bone fractures seen on trauma pan-scan CT. The radiation exposure of concurrent or subsequent dedicated temporal bone imaging is equal to approximately one half of the original trauma pan-scan CT.

Imaging of pediatric pancreas tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

Primary pancreatic tumors in children are rare with an overall age-adjusted incidence of 0.018 new cases per 100,000 pediatric patients. The most prevalent histologic type is the solid pseudopapillary neoplasm, followed by pancreatoblastoma. This paper describes relevant imaging modalities and presents consensus-based recommendations for imaging at diagnosis and follow-up.

Imaging of pediatric adrenal tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

Adrenal tumors other than neuroblastoma are uncommon in children. The most frequently encountered are adrenocortical carcinoma and pheochromocytoma. This paper offers consensus recommendations for imaging of pediatric patients with a known or suspected primary adrenal malignancy other than neuroblastoma at diagnosis and during follow-up.

Unilateral Fourth Nerve Palsy due to Presumed Metastatic Melanoma.

ABSTRACT: An 81-year-old man with a history of metastatic melanoma presented with sudden onset of painless, binocular vertical diplopia. The clinical examination was consistent with a right fourth nerve palsy. An MRI of the head revealed a mass dorsal to the right tectum at the level of the inferior colliculus. An MRI just 4 months prior did not show a lesion in that location. An MRA of the head did not show an aneurysm. This is a rare case of an isolated fourth nerve palsy believed to be due to metastatic melanoma compressing the nerve along the dorsal midbrain.

ACR Appropriateness Criteria® Scoliosis-Child.

Scoliosis is frequently encountered in childhood, with prevalence of 2%. The majority is idiopathic, without vertebral segmentation anomaly, dysraphism, neuromuscular abnormality, skeletal dysplasia, tumor, or infection. As a complement to clinical assessment, radiography is the primary imaging modality used to classify scoliosis and subsequently monitor its progression and response to treatment. MRI is utilized selectively to assess for neural axis abnormalities in those at higher risk, including those with congenital scoliosis, early onset idiopathic scoliosis, and adolescent idiopathic scoliosis with certain risk factors. CT, although not routinely employed in the initial evaluation of scoliosis, may have a select role in characterizing the bone anomalies of congenital scoliosis and in perioperative planning. 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.

ACR Appropriateness Criteria® Suspected Spine Trauma-Child.

Choosing the appropriate imaging in children with accidental traumatic spine injuries can be challenging because the recommendations based on scientific evidence at this time differ from those applied in adults. This differentiation is due in part to differences in anatomy and physiology of the developing spine. This publication uses scientific evidence and a panel of pediatric experts to summarize best current imaging practices for children with accidental spine trauma. 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.

Utility of Hounsfield unit and hematocrit values in the diagnosis of acute venous sinus thrombosis in unenhanced brain CTs in the pediatric population.

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a recognized cause of childhood and neonatal stroke with high morbidity and mortality and a challenging diagnosis in the pediatric population. OBJECTIVE: We hypothesize that measuring Hounsfield units (HU) of blood in venous sinuses is a more reliable method to diagnose CVST and that normalizing the measured HU in relation to the patient's hematocrit levels may further improve detection of CVST in the pediatric population. MATERIALS AND METHODS: We performed a retrospective chart review of 15 pediatric patients with acute CVST and 31 control patients. Regions of interest (ROIs) were plotted to measure HU values within the venous sinuses of each patient. Hounsfield unit to hematocrit (HU:Hct) ratios were also calculated. In patients with CVST, HU values were determined in thrombosed and non-thrombosed venous sinuses. Statistical analysis was performed to calculate the differences between patient and control groups and to determine optimal cutoff values for HU and HU:Hct measurements in diagnosing CVST on non-contrast brain computed tomography (CT). RESULTS: A statistically significant difference in sinus attenuation and HU:Hct ratio was found between thrombosed (66.2±5.3 HU, 1.96±0.4) and non-thrombosed sinuses (47.2±4.5 HU, 1.38±0.25) in the patient group (P<0.0001), with the average attenuation difference being 19 HU. A statistically significant difference was also found between thrombosed sinuses in the patient group and sinuses (48.9±3.13 HU, 1.3±0.12) in the control group (P<0.0001). CONCLUSION: Optimal cutoff values of 58 HU and HU:Hct ratios of 1.4 lead to sensitivities of 100% in diagnosing CVST.

ACR Appropriateness Criteria® Sinusitis-Child.

Sinusitis is common in children that usually resolves spontaneously. Imaging is not part of the standard of care for initial diagnosis, however may be necessary in cases with persistent or chronic sinusitis to guide surgical intervention, or to rule out intracranial and vascular complications of sinusitis. Computed tomography (CT) and magnetic resonance imaging (MRI) are the leading imaging modalities. In this article, appropriateness in use of imaging modalities are discussed under common/clinically relevant scenarios. 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.

First Locally Acquired Congenital Zika Syndrome Case in the United States: Neonatal Clinical Manifestations.

In the spring of 2017, a full-term infant with microcephaly was delivered in South Florida. During first trimester, the mother presented with fever, nausea, and vomiting. She reported no foreign travel for herself or her partner. The infant's neurologic, ophthalmologic, neuroradiologic, and audiologic findings were highly suggestive of congenital Zika syndrome (CZS), confirmed by IgM antibodies and plaque reduction neutralization test. New observations, including peripheral temporal retinal avascularity and peripapillary retinal nerve fiber layer thinning, are presented from this first known case of non-travel-associated CZS in the United States. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:e93-e98.].

Neurologic Emergencies in Pediatric Patients Including Accidental and Nonaccidental Trauma.

This article summarizes common neurologic emergencies presenting in pediatric patients. Imaging techniques and appearances of specific conditions are detailed, including pearls and pitfalls for each presentation. Specific attention is given to differential diagnoses that can serve as mimickers of pediatric neurologic emergencies.

ACR Appropriateness Criteria® Headache-Child.

Headaches in children are not uncommon and have various causes. Proper neuroimaging of these children is very specific to the headache type. Care must be taken to choose and perform the most appropriate initial imaging examination in order to maximize the ability to properly determine the cause with minimum risk to the child. This evidence-based report discusses the different headache types in children and provides appropriate guidelines for imaging these children. 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.

Choledochal Malformations: Surgical Implications of Radiologic Findings.

OBJECTIVE: The purpose of this article is to review the relevant pathophysiologic features, appearances, and surgical implications of choledochal malformations. CONCLUSION: Choledochal malformations, colloquially called choledochal cysts, initially described in 1723, have been recategorized multiple times, the most widely accepted being the Todani classification based on morphologic features and location. Although readily applied to imaging findings, this classification system does not correlate well with clinical and surgical management. In 2004, Visser and colleagues proposed an alternative that emphasized the etiologic factors, imaging appearance, and treatment approach.

Pediatric patient with end-stage kidney disease secondary to Eagle-Barrett syndrome and metastatic unresectable hepatoblastoma treated successfully with chemotherapy and liver-kidney transplant.

HBL is the most common malignant liver neoplasm in children. The etiology of HBL is largely unknown but there are certain syndromes, such as Beckwith-Wiedemann syndrome, that have been clearly associated with an increased incidence of this malignancy. EBS, also known as prune belly syndrome, is a congenital anomaly characterized by lax abdominal musculature, bilateral cryptorchidism requiring, in some cases, hemodialysis due to significant kidney and urinary tract dysfunctions. Despite an improvement on the survival rates of patients with advanced-stage HBL, the presence of concomitant end-stage renal disease that occurs in patients with EBS constitutes a therapeutic challenge for the clinician not only due to the use of nephrotoxic chemotherapy but also due to the potential need for multi-organ transplant. We report case of a 2-year-old male patient with EBS diagnosed with stage IV, metastatic HBL successfully treated with multi-agent chemotherapy while on dialysis whom then underwent a simultaneous liver-kidney transplant followed by adjuvant chemotherapy. Ultimately, the patient achieved cancer remission with normalization of his renal function. Our report emphasizes that patients with HBL in the setting of EBS will not only require careful kidney function monitoring while receiving chemotherapy, but they might also need to undergo multi-organ transplantation in order to achieve adequate cancer control and also normalization of their kidney function. Awareness of this unusual association calls for further investigation to potentially establish a genetic association between these two disease processes.

Magnetic resonance imaging spectrum of intracranial tubercular lesions: one disease, many faces.

Tuberculosis is a devastating disease and has shown resurgence in recent years with the advent of acquired immunodeficiency syndrome. Central nervous system involvement is the most devastating form of the disease, comprising 10% of all tuberculosis cases. The causative organism, Mycobacterium tuberculosis, incites a granulomatous inflammatory response in the brain, the effects of which can be appreciated on magnetic resonance imaging (MRI), which can thus be used for diagnosis of the same. Neurotuberculosis can present in various patterns, which can be identified on MRI. The meningeal forms include leptomeningitis and pachymeningitis. Parenchymal forms of neurotuberculosis include tuberculoma in its various stages, tubercular cerebritis and abscess, tubercular rhombencephalitis, and tubercular encephalopathy. Each pattern has characteristic MRI appearances and differential diagnoses on imaging. Complications of neurotuberculosis, usually of tubercular meningitis, include hydrocephalus, vasculitis, and infarcts as well as cranial nerve palsies. Various MRI sequences besides the conventional ones can provide additional insight into the disease, help in quantifying the disease load, and help in differentiation of neurotuberculosis from conditions with similar imaging appearances and presentations. These can enable accurate and timely diagnosis by the radiologist and early institution of treatment in order to reduce the likelihood of permanent neurological sequelae.