Pearls of Temporal Bone Imaging in Children with Hearing Loss.

Hearing loss is one of the most common indications for temporal bone imaging in children. Hearing loss may be congenital or acquired, and it may be conductive, sensorineural, or mixed audiologically. Temporal bone imaging plays an important role in the assessment and management of this condition. An understanding of the embryology of ear structures better enables the radiologist to interpret abnormalities on imaging of the temporal bone. Here, we provide a general review of ear development and a description of known genetic defects that contribute to congenital ear anomalies associated with hearing loss. We provide appropriate imaging techniques for the temporal bone depending on the clinical presentation and a systematic approach to imaging for children with hearing loss. Diagnostic imaging for developmental anomalies of the ear and cholesteatoma will be discussed.

Multimodality Imaging Evaluation of Fetal Spine Anomalies with Postnatal Correlation.

Congenital anomalies of the spine are associated with substantial morbidity in the perinatal period and may affect the rest of the patient's life. Accurate early diagnosis of spinal abnormalities during fetal imaging allows prenatal, perinatal, and postnatal treatment planning, which can substantially affect functional outcomes. The most common and clinically relevant congenital anomalies of the spine fall into three broad categories: spinal dysraphism, segmentation and fusion anomalies of the vertebral column, and sacrococcygeal teratomas. Spinal dysraphism is further categorized into one of two subtypes: open spinal dysraphism and closed spinal dysraphism. The latter category is further subdivided into those with and without subcutaneous masses. Open spinal dysraphism is an emergency and must be closed at birth because of the risk of infection. In utero closure is also offered at some fetal centers. Sacrococcygeal teratomas are the most common fetal pelvic masses and the prognosis is variable. Finally, vertebral body anomalies are categorized into formation (butterfly and hemivertebrae) and segmentation (block vertebrae) anomalies. Although appropriate evaluation of the fetal spine begins with US, which is the initial screening modality of choice, MRI is increasingly important as a problem-solving tool, especially given the recent advances in fetal MRI, its availability, and the complexity of fetal interventions. Online supplemental material is available for this article. ©RSNA, 2021.

The spectrum of brain malformations and disruptions in twins.

Twins have an increased risk for congenital malformations and disruptions, including defects in brain morphogenesis. We analyzed data on brain imaging, zygosity, sex, and fetal demise in 56 proband twins and 7 less affected co-twins with abnormal brain imaging and compared them to population-based data and to a literature series. We separated our series into malformations of cortical development (MCD, N = 39), cerebellar malformations without MCD (N = 13), and brain disruptions (N = 11). The MCD group included 37/39 (95%) with polymicrogyria (PMG), 8/39 (21%) with pia-ependymal clefts (schizencephaly), and 15/39 (38%) with periventricular nodular heterotopia (PNH) including 2 with PNH but not PMG. Cerebellar malformations were found in 19 individuals including 13 with a cerebellar malformation only and another 6 with cerebellar malformation and MCD. The pattern varied from diffuse cerebellar hypoplasia to classic Dandy-Walker malformation. Brain disruptions were seen in 11 individuals with hydranencephaly, porencephaly, or white matter loss without cysts. Our series included an expected statistically significant excess of monozygotic (MZ) twin pairs (22/41 MZ, 54%) compared to population data (482/1448 MZ, 33.3%; p = .0110), and an unexpected statistically significant excess of dizygotic (DZ) twins (19/41, 46%) compared to the literature cohort (1/46 DZ, 2%; p < .0001. Recurrent association with twin-twin transfusion syndrome, intrauterine growth retardation, and other prenatal factors support disruption of vascular perfusion as the most likely unifying cause.

"It's Not a Tumor": A Rare Case of Symptomatic Cerebellar Developmental Venous Anomaly.

Cerebral developmental venous anomalies (DVAs) are benign anatomical variants of the venous system and are commonly described as an incidental finding without clinical significance. Neurologic symptoms or abnormal examination findings are rare and usually attributed to hemorrhagic complications related to coexisting cavernous malformations. There have been limited case reports of symptomatic, uncomplicated DVAs described in the literature. The following case describes a previously healthy child who presented to the emergency department with an acute onset of altered mental status, headache, and focal neurologic examination abnormalities. Magnetic resonance imaging revealed a prominent cerebellar DVA. There was no evidence of a cavernous angioma, hemorrhage, or acute parenchymal injury. This case report illustrates a clinically symptomatic, uncomplicated posterior fossa DVA. It provides additional evidence regarding the potential for a cerebral venous malformation in causing focal neurologic deficits.

Recurrent bloody stools associated with visceral infantile haemangioma in a preterm twin girl.

A premature twin infant girl was transferred to a level IV neonatal intensive care unit for recurrent bloody stools, anaemia and discomfort with feeds; without radiographic evidence of necrotising enterocolitis. Additional imaging after transfer revealed a large retroperitoneal mass in the region of the pancreas compressing the inferior vena cava and abdominal aorta, raising suspicion for neuroblastoma. Abdominal exploration and biopsy unexpectedly revealed that the lesion was an infantile capillary haemangioma involving the small bowel, omentum, mesentery and pancreas. The infant was subsequently treated with propranolol, with a decrease in the size of the lesion over the first year of her life and a drastic improvement in feeding tolerance. While cutaneous infantile haemangiomas are common, visceral infantile haemangiomas are less so and may present a significant diagnostic challenge for clinicians. This interesting case demonstrates that such lesions should be considered in the differential diagnosis for unexplained gastrointestinal bleeding or abdominal symptoms in newborns.

Establishment of normative values for the fetal posterior fossa by magnetic resonance imaging.

OBJECTIVE: Suspected Dandy-Walker continuum anomalies constitute a significant percentage of prenatal cases evaluated by magnetic resonance imaging (MRI). To unify the description of posterior fossa malformations, we sought to establish objective measurements for the posterior fossa in normal fetuses between 18 and 37 weeks gestation. METHODS: T2-weighted images of normal fetal brains in sagittal projection were obtained from fetal magnetic resonance (MR) studies of normal brains performed from 2009 to 2017.121 fetal brains were included in the analysis. Three radiologists reviewed images and recorded the following for each case: superior posterior fossa angle (SPFA), posterior fossa perimeter, and tegmento-vermian angle (TVA). RESULTS: For each feature, the mean of the measurements, the percentage of absolute difference of the reader measurement compared with mean measurement, and the interclass correlation (ICC) were calculated. Values are reported as mean ± standard deviation. Perimeter increases linearly with age, whereas the SPFA and the TVA are independent of gestational age. For all included cases, the SPFA averaged 100.9° ± 8° and the TVA averaged 2.5° ± 2.3°. CONCLUSION: The superior posterior fossa angle, a novel measurement, and the posterior fossa perimeter can be used for establishing the expected size of the posterior fossa in second- and third-trimester fetuses by MRI.

Diagnosis, histopathologic correlation and management of hepatoblastoma: What the radiologist needs to know.

Hepatoblastomas are complex pediatric tumors with several pathological subtypes, some of which demonstrate differing imaging features and portend varying prognosis. The radiologist plays a major role not only in the pre-surgical evaluation and baseline staging of the tumor, but also in guiding management and evaluating prognosis based on the PRETEXT (pretreatment extent of tumor) classification. We discuss the pathology, imaging features, and baseline evaluation, with a focus on the role of the radiologist in the management of these tumors.

Perforated jejunitis in a child with acute lymphoblastic leukemia treated with pegaspargase.

Survival rates of children with acute lymphoblastic leukemia have improved since the incorporation of asparaginase in the treatment protocol, but the medication has potential serious complications, including vascular thrombosis. Here, we describe the case of a 13-year-old boy with pre-T-cell acute lymphoblastic leukemia whose treatment course was complicated by perforated jejunitis requiring resection of a portion of his small bowel. Pathologic assessment showed transmural ischemia, mesenteric venous and arterial thrombi, and scattered cytomegalovirus inclusion bodies. Pediatric mesenteric ischemia is rare, and its consideration in patients treated with asparaginase is discussed.

Cerebral palsy and seizures in a child with tubulinopathy pattern dysgenesis and focal cortical dysplasia.

A 7-year-old boy with a history of spasticity, global developmental delay, and seizures was given the general diagnosis of cerebral palsy at an early age. Chromosomal array analysis performed at an outside center was normal. The patient's family sought neurodevelopmental pediatric care at a new institution following a move out of state. Electroencephalography confirmed abnormal epileptogenic activity. Brain magnetic resonance imaging showed findings consistent with a tubulin gene defect (tubulinopathy) and of focal cortical dysplasia, as well as evidence of a remote occipital lobe injury. This case report describes the various brain magnetic resonance findings suggestive of a tubulin gene defect and raises the possibility of focal cortical dysplasia manifesting as a result of tubulin dysfunction.

Contrast-enhanced CT features of hepatoblastoma: Can we predict histopathology?

BACKGROUND: Hepatoblastoma is the most common hepatic malignancy occurring in the pediatric population. Intratumoral cellular behavior varies, and the small-cell undifferentiated histopathology carries a poorer prognosis than other tissue subtypes. Neoadjuvant chemotherapy is recommended for this tumor subtype prior to surgical resection in most cases. Early identification of tumors with poor prognosis could have a significant clinical impact. Objective The aim of this work was to identify imaging features of small-cell undifferentiated subtype hepatoblastoma that can help distinguish this subtype from more favorable tumors and potentially guide the clinical management. We also sought to characterize contrast-enhanced CT (CECT) features of hepatoblastoma that correlate with metastatic disease and patient outcome. MATERIALS AND METHODS: Our study included 34 patients (24 males, 10 females) with a mean age of 16months (range: 0-46months) with surgically confirmed hepatoblastoma and available baseline abdominal imaging by CECT. Clinical data and CT abdominal images were retrospectively analyzed. RESULTS: Five tumors with small-cell undifferentiated components were identified. All of these tumors demonstrated irregular margins on CT imaging. Advanced PRETEXT stage, vascular invasion and irregular margins were associated with metastatic disease and decreased survival. Capsular retraction was also significantly associated with decreased survival. Irregular tumor margins demonstrated statistically significant association with the presence of small-cell undifferentiated components. No other imaging feature showed statistically significant association. CONCLUSION: Tumor margin irregularity, vascular invasion, capsular retraction, and PRETEXT stage correlate with worse patient outcomes. Irregular tumor margin was the only imaging feature significantly associated with more aggressive tumor subtype.

Prenatal diagnosis of Chudley-McCullough syndrome.

Chudley-McCullough syndrome (CMS) is an autosomal-recessive disorder characterized by a complex brain malformation and profound congenital sensorineural hearing loss. Postnatal brain imaging findings include ventriculomegaly, partial agenesis of corpus callosum, inferior cerebellar dysplasia, arachnoid cysts, and malformations of cortical development including frontal subcortical heterotopia and polymicrogyria. Prenatal diagnosis of CMS is important due to the markedly less severe neurodevelopmental prognosis compared to disorders with similar brain imaging findings. We report prenatal imaging features that help distinguish CMS from other disorders, including slit-like frontal horns, agenesis of the corpus callosum, frontal subcortical heterotopia, arachnoid cysts, and cerebellar dysplasia. © 2016 Wiley Periodicals, Inc.

Ultrasound of pediatric breast masses: what to do with lumps and bumps.

The approach to breast masses in children differs from that in adults in many ways, including the differential diagnostic considerations, imaging algorithm and appropriateness of biopsy as a means of further characterization. Most pediatric breast masses are benign, either related to breast development or benign neoplastic processes. Biopsy is rarely needed and can damage the developing breast; thus radiologists must be familiar with the imaging appearance of common entities so that biopsies are judiciously recommended. The purpose of this article is to describe the imaging appearances of the normally developing pediatric breast as well as illustrate the imaging findings of a spectrum of diseases, including those that are benign (fibroadenoma, juvenile papillomatosis, pseudoangiomatous stromal hyperplasia, gynecomastia, abscess and fat necrosis), malignant (breast carcinoma and metastases), and have variable malignant potential (phyllodes tumor).

Improving ultrasound quality to reduce computed tomography use in pediatric appendicitis: the Safe and Sound campaign.

BACKGROUND: Safety concerns about the use of radiation-based imaging such as computed tomography (CT) in children have resulted in national recommendations to use ultrasound (US) for the diagnosis of appendicitis when possible. We evaluated the trends in CT and US use in a statewide sample and the accuracy of these modalities. METHODS: Patients less than or equal to 18 years undergoing appendectomy in Washington State from 2008 to 2013 were evaluated for preoperative US/CT use, as well as imaging/pathology concordance using data from the Surgical Care and Outcomes Assessment Program. RESULTS: Among 3,353 children, 98.3% underwent preoperative imaging. There was a significant increase in the use of US first over the study period (P < .001). The use of CT at any time during the evaluation decreased. Despite this, in 2013, over 40% of the children still underwent CT imaging. Concordance between US imaging and pathology varied between 40% and 75% at hospitals performing greater than or equal to 10 appendectomies in 2013. Over one third (34.9%) of CT scans performed in the evaluation of children with appendicitis were performed after an indeterminate US. CONCLUSIONS: Although the use of US as the first imaging modality to diagnose pediatric appendicitis has increased over the past 5 years, over 40% of children still undergo a CT scan during their preoperative evaluation. Causality for this persistence of CT use is unclear, but could include variability in US accuracy, lack of training, and lack of awareness of the risks of radiation-based imaging. Developing a campaign to focus on continued reduction in CT and increased use of high-quality US should be pursued.

Diagnostic imaging of posterior fossa anomalies in the fetus and neonate: part 2, Posterior fossa disorders.

This second portion of a two-part review illustrates examples of posterior fossa disorders detectable on prenatal ultrasound and MRI, with postnatal or pathology correlation where available. These disorders are discussed in the context of an anatomic classification scheme described in Part 1 of this posterior fossa anomaly review. Assessment of the size and formation of the cerebellar hemispheres and vermis is critical. Diagnoses discussed here include arachnoid cyst, Blake's pouch cyst, Dandy-Walker malformation, vermian agenesis, Joubert syndrome, rhombencephalosynapsis, Chiari II malformation, ischemia, and tumors.

The fundamentals of fetal magnetic resonance imaging: Part 2.

Careful assessment of fetal anatomy by a combination of ultrasound and fetal magnetic resonance imaging offers the clinical teams and counselors caring for the patient information that can be critical for the management of both the mother and the fetus. In the second half of this 2-part review, we focus on space-occupying lesions in the fetal body. Because developing fetal tissues are programmed to grow rapidly, mass lesions can have a substantial effect on the formation of normal adjacent organs. Congenital diaphragmatic hernia and lung masses, fetal teratoma, and intra-abdominal masses are discussed, with an emphasis on differential etiologies and on fundamental management considerations.

Transient renal enlargement in pediatric hematopoietic cell transplant recipients.

Age-dependent renal length tables are routinely used when interpreting pediatric ultrasound. Standard renal length tables may not be accurate for HCT patients due to treatment effects on kidney size. The purpose of this study was to determine whether renal size changes from expected lengths based on age after HCT in the absence of other markers of renal disease. Four hundred and fifty renal measurements were made on 101 patients who underwent HCT between 2006 and 2010. Renal length was measured at 1-90 days pre-HCT and at 0-30, 31-90, 91-180, and 181+ days post-HCT. Values were compared with normal renal length tables. Average post-HCT renal lengths were greater than established normative renal length data within every age group. Age-adjusted average renal lengths measured at 0-30 and 31-90 days post-transplantation were significantly larger than pre-HCT renal lengths, with relative increases of 6.9% (4.5, 9.4; p < 0.001) and 3.9% (1.4, 6.4; p = 0.003), respectively. Average renal length did not differ significantly after 90 days post-transplantation. HCT patients may have larger kidneys in the absence of renal disease. Awareness of the potential phenomenon of transient renal enlargement following HCT can prevent misdiagnosis and eliminate unnecessary diagnostic evaluations, interventions, anxiety, resource allocation, and financial costs.

Diagnostic imaging of fetal and pediatric orbital abnormalities.

OBJECTIVE: The orbit contains structures from which a wide spectrum of disease can arise. This article focuses on orbital anatomy and a simple compartmental approach to evaluating the orbit on diagnostic imaging. The characteristic findings of key fetal structural diseases and a wide spectrum of pediatric orbital disorders, including inflammatory disorders and developmental lesions, and the differential diagnosis of benign versus malignant masses will be discussed. CONCLUSION: Orbital abnormalities in fetuses may be recognized using ultrasound and MRI. Anophthalmia, hypertelorism, and hypotelorism either may be part of a genetic syndrome or may be related to a developmental abnormality of the fetal skull. In the pediatric population, cross-sectional imaging with CT and MRI offers a means to assess which compartments of the orbit are affected. Aggressive masses have characteristic features and must be evaluated for intracranial extension.

Voiding cystourethrography revisited: descriptive statistics for the detection of vesicoureteral reflux.

OBJECTIVE: To determine which factors might influence the detection of vesicoureteral reflux (VUR), we retrospectively reviewed factors including fluoroscopy time, number of true radiographic acquisitions, and patient characteristics from a large number of voiding cystourethrography (VCUG) examinations. MATERIALS AND METHODS: Nine hundred eighty-seven VCUG examinations performed between March 2006 and March 2009 were randomly selected for review. Data recorded were presence of VUR, patient age and sex, examination indication, follow-up status, presence of a diagnostic radiology trainee, fluoroscopy time, and number of true radiographic acquisitions. For initial examinations, descriptive comparison and logistic analyses were performed. To evaluate which variables related to reflux identification, we analyzed variables by logistic regression after stratifying by patient age (≤ 1 or > 1 year) for both the full sample and cases grouped by VUR severity (grades I and II [mild] or grades III-V [moderate to severe]). RESULTS: Nine hundred eighty-seven VCUG examinations were evaluated for the study (65.5% female; mean age, 3 years 2 months; age range, 2 weeks-16 years), and 761 cases met the inclusion criteria. VUR was detected in 101 of 349 infants (40 mild, 61 moderate to severe) and in 107 of 412 children older than 1 year (52 mild, 55 moderate to severe). A wide range of fluoroscopy times was similar between both positive and negative cases (0.033-4.233 minutes). The number of true radiographic acquisitions differed significantly between the negative and positive cases. CONCLUSION: Descriptive statistics and logistic regression analyses for a large number of VCUG examinations in a pediatric population are summarized. Our results showed that high fluoroscopy times were not associated with a higher likelihood of VUR. There may be a small benefit to the use of true radiographic acquisitions for detecting VUR.

Pediatric chest CT radiation dose reduction: protocol refinement based on noise injection for pulmonary nodule detection accuracy.

To establish appropriate chest computed tomography (CT) acquisition protocols that balance radiation dose and diagnostic capability in pediatric bone marrow transplant patients by assessing the accuracy of pulmonary nodule detection at simulated lower-radiation acquisitions, chest CT images from bone marrow transplant patients were reviewed by four pediatric radiologists at artificially reduced CT dose levels (0%, 30%, and 60%). Average accuracy for nodule detection in 31 randomly selected cases was 0.87 at 0% dose reduction, 0.90 at 30% reduction, and 0.86 at 60% reduction. We observed no clinically relevant difference in acceptability of images or accuracy levels with tested dose reductions to 60%.