An unusual case of metastatic trophoblastic neoplasm presenting with diffuse cystic lung disease and pulmonary artery pseudoaneurysms in a teenager.

Diffuse cystic lung diseases (DCLDs) are a diverse group of lung disorders characterized by the presence of multiple air filled cysts within the lung tissue. These cysts are thin walled and surrounded by normal lung tissue. In adults, DCLD can be associated with various conditions such as lymphangioleiomyomatosis (LAM), Langerhans cell histiocytosis, cancers, and more. In children, DCLD is often linked to lung developmental abnormalities, with bronchopulmonary dysplasia being a common cause. Patients with pulmonary cysts are typically asymptomatic, but some may experience mild symptoms or pneumothorax. While DCLD in children is rarely due to malignancy, metastatic lung disease can be a cause. It is important for clinicians to be aware of the possibility of metastatic lung disease when encountering DCLD.

Imaging of Fontan-Associated Liver Disease.

ABSTRACT: The Fontan procedure is the definitive treatment for patients with single-ventricle physiology. Surgical advances have led to a growing number of patients surviving into adulthood. Fontan-associated liver disease (FALD) encompasses a spectrum of pathologic liver changes that occur secondary to altered physiology including congestion, fibrosis, and the development of liver masses. Assessment of FALD is difficult and relies on using imaging alongside of clinical, laboratory, and pathology information. Ultrasound, computed tomography, and magnetic resonance imaging are capable of demonstrating physiologic and hepatic parenchymal abnormalities commonly seen in FALD. Several novel imaging techniques including magnetic resonance elastography are under study for use as biomarkers for FALD progression. Imaging has a central role in detection and characterization of liver masses as benign or malignant. Benign FNH-like masses are commonly encountered; however, these can display atypical features and be mistaken for hepatocellular carcinoma (HCC). Fontan patients are at elevated risk for HCC, which is a feared complication and has a poor prognosis in this population. While imaging screening for HCC is widely advocated, no consensus has been reached regarding an optimal surveillance regimen.

Normative Femoral and Tibial Lengths in a Modern Population of Twenty-First-Century U.S. Children.

BACKGROUND: The Green-Anderson (GA) leg-length data remain the gold standard for the age-based assessment of leg lengths in children despite their methodologic weaknesses. We aimed to summarize current growth trends among a cross-sectional cohort of modern U.S. children using quantile regression methods and to compare the median femoral and tibial lengths of the modern U.S. children with those of the GA cohort. METHODS: A retrospective review of scanograms and upright slot-scanning radiographs obtained in otherwise healthy children between 2008 and 2020 was completed. A search of a radiology registry revealed 3,508 unique patients between the ages of 2 and 18 years for whom a standard-of-care scanogram or slot-scanning radiograph had been made. All patients with systemic illness, genetic conditions, or generalized diseases that may affect height were excluded. Measurements from a single leg at a single time point per subject were included, and the latest available time point was used for children who had multiple scanograms made. Quantile regression analysis was used to fit the lengths of the tibia and femur and overall leg length separately for male patients and female patients. RESULTS: Seven hundred patients (328 female and 372 male) met the inclusion criteria. On average, the reported 50th percentile tibial lengths from the GA study at each time point were shorter than the lengths in this study by 2.2 cm (range, 1.4 to 3.3 cm) for boys and 2 cm (range, 1.1 to 3.1 cm) for girls. The reported 50th percentile femoral lengths from the GA study at each time point were shorter than the lengths in this study by 1.8 cm (range, 1.1 to 2.5 cm) for boys and 1.7 cm (range, 0.8 to 2.3 cm) shorter for girls. CONCLUSIONS: This study developed new growth charts for femoral and tibial lengths in a modern U.S. population of children. The new femoral and tibial lengths at nearly all time points are 1 to 3 cm longer than traditional GA data. The use of GA data for epiphysiodesis could result in underestimation of expected childhood growth. LEVEL OF EVIDENCE: Prognostic Level IV . See Instructions for Authors for a complete description of levels of evidence.

Pilot study to determine whether reduced-dose photon-counting detector chest computed tomography can reliably display Brody II score imaging findings for children with cystic fibrosis at radiation doses that approximate radiographs.

BACKGROUND: The Brody II score uses chest CT to guide therapeutic changes in children with cystic fibrosis; however, patients and providers are often reticent to undergo chest CT given concerns about radiation. OBJECTIVE: We sought to determine the ability of a reduced-dose photon-counting detector (PCD) chest CT protocol to reproducibly display pulmonary disease severity using the Brody II score for children with cystic fibrosis (CF) scanned at radiation doses similar to those of a chest radiograph. MATERIALS AND METHODS: Pediatric patients with CF underwent non-contrast reduced-dose chest PCD-CT. Volumetric inspiratory and expiratory scans were obtained without sedation or anesthesia. Three pediatric radiologists with Certificates of Added Qualification scored each scan on an ordinal scale and assigned a Brody II score to grade bronchiectasis, peribronchial thickening, parenchymal opacity, air trapping and mucus plugging. We report image-quality metrics using descriptive statistics. To calculate inter-rater agreement for Brody II scoring, we used the Krippendorff alpha and intraclass correlation coefficient (ICC). RESULTS: Fifteen children with CF underwent reduced-dose PCD chest CT in both inspiration and expiration (mean age 8.9 years, range, 2.5-17.5 years; 4 girls). Mean volumetric CT dose index (CTDIvol) was 0.07 ± 0.03 mGy per scan. Mean effective dose was 0.12 ± 0.04 mSv for the total examination. All three readers graded spatial resolution and noise as interpretable on lung windows. The average Brody II score was 12.5 (range 4-19), with moderate inter-reader reliability (ICC of 0.61 [95% CI=0.27, 0.84]). Inter-rater reliability was moderate to substantial for bronchiectasis (0.52), peribronchial thickening (0.55), presence of opacity (0.62) and air trapping (0.70) and poor for mucus plugging (0.09). CONCLUSION: Reduced-dose PCD-CT permits diagnostic image quality and reproducible identification of Brody II scoring imaging findings at radiation doses similar to those for chest radiography.

Imaging of pre- and post-cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pediatric intraperitoneal malignancy.

Although rare, pediatric peritoneal carcinomatosis does occur in primary abdominopelvic tumors. Additionally, peritoneal carcinomatosis has been described to occur as metastatic disease where the primary tumor is outside the abdominopelvic cavity. Where amenable, cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) can be beneficial in disease management. However, favorable outcomes are predicated on specific tumor histology as well as proper patient selection, which significantly relies on preoperative imaging. This review gives a comprehensive, up-to-date summary on pediatric peritoneal carcinomatosis pre-surgical evaluation; where imaging is beneficial and limited; pediatric radiologists' role in helping to quantify disease; and how we, as pediatric radiologists, can help the surgeons and oncologists in the selection of patients for cytoreductive surgery and HIPEC.

NUT carcinoma of the thorax in a 7-year-old child.

We present a rare case of NUT midline carcinoma of the thorax in a 7-year-old-male who presented with nonspecific abdominal pain. The patient was initially evaluated with an abdominal ultrasound, which was negative, followed by an abdominopelvic CT that demonstrated a partially visualized infiltrative mediastinal mass. Subsequent, chest CT showed a large, aggressive appearing heterogenous middle mediastinal mass with pulmonary parenchyma, hilar, and posterior mediastinal invasion. Given its epicenter in the middle mediastinum and its irregular and invasive appearance, the primary consideration was NUT midline carcinoma, subsequently confirmed on biopsy.

Pediatric Abdominal Masses: Imaging Guidelines and Recommendations.

Pediatric abdominal masses are commonly encountered in the pediatric population, with a broad differential diagnosis that encompasses benign and malignant entities. The primary role of abdominal imaging in the setting of a suspected pediatric abdominal mass is to establish its presence, as nonneoplastic entities can mimic an abdominal mass, and to identify characteristic imaging features that narrow the differential diagnosis. In the setting of a neoplasm, various imaging modalities play an important role to characterize the mass, stage extent of disease, and assist in presurgical planning. The purpose of this article is to discuss a practical imaging algorithm for suspected pediatric abdominal masses and to describe typical radiological findings of the commonly encountered abdominal masses in neonates and children with emphasis on imaging guidelines and recommendations.

Chylolymphatic mesenteric cyst with midgut volvulus in an adolescent: a peculiar presentation.

Mesenteric cysts are rare intra-abdominal masses in the pediatric population, with the chylolymphatic variant comprising only 7.3% of all abdominal cysts. These cysts can have a varied clinical presentation, ranging from asymptomatic cystic masses to intestinal obstruction. We report a 16-year-old female, who presented with acute abdominal pain and vomiting, and was diagnosed with an intra-abdominal cystic mass with midgut volvulus accompanied by the classical "whirlpool sign" on imaging. She underwent laparoscopic abdominal exploration, which revealed a large chylolymphatic mesenteric cyst associated with feeder lymph vessels. This cyst had resulted in volvulus of the small bowel. The bowel was detorsed and found to be viable, and the cyst was removed en bloc after suction evacuation with ligation of the lymphatic feeder vessels. Midgut volvulus in the pediatric population is usually secondary to malrotation, but in this case the patient's small intestine was not found to be malrotated, and hence we present this case and urge physicians to consider a diagnosis of midgut volvulus even in the absence of malrotation in a child with a cystic abdominal mass presenting as intestinal obstruction.

Perianal magnetic resonance imaging findings and their potential impact on outcome in children with perianal fistulizing Crohn disease.

BACKGROUND: Children with perianal fistulizing Crohn disease require intensive medical management but also have a higher risk for subsequent surgical interventions. OBJECTIVE: We performed a retrospective study to identify patient factors and perianal anatomical features by pelvic MR that are associated with surgical interventions in these children. MATERIALS AND METHODS: We included children with Crohn disease and perianal fistula who underwent pelvic MR with available, archived images and collected demographic, clinical and laboratory data. Radiologists reviewed pelvic MR exams and identified Park classification and additional anatomical features of perianal fistulas, including fistula branching, horseshoe ramifications, abscess, inflammatory mass, supralevator extension, anal sphincter damage, proctitis and posterior anal space involvement. We performed univariate and subsequent multivariate analysis to determine features associated with subsequent surgical intervention. RESULTS: Ninety-nine children with Crohn disease underwent pelvic MR. In this cohort, 69 children had no surgical interventions prior to baseline MRI, with subsequent median clinical follow-up of 5.5 years. Univariate analysis demonstrated that branching (P=0.009), supralevator extension (P=0.015) and anal sphincter damage (P=0.031) were significantly associated with subsequent surgical intervention. Age at baseline MRI was also associated with intervention (hazard ratio [HR] every 5 years: 2.13; 95% confidence interval [CI]: 1.18-3.83; P=0.012). A multivariable model identified only fistula branching (HR: 2.31; 95% CI: 1.28-4.15; P=0.005) and age (HR: 5.18; CI: 1.57-17.14; P=0.007) as independent predictors of subsequent surgery. No demographic, clinical or laboratory parameter predicted subsequent surgical intervention. CONCLUSION: Age and anatomical MR features indicating fistula complexity (branching, supralevator extension) and sphincter damage confer a higher risk of subsequent surgical intervention in children with perianal Crohn disease.

Bladder hemangioma: An arduous diagnosis of hematuria.

Urinary bladder hemangiomas are a rare cause of gross hematuria in young patients. Multimodality imaging with ultrasound, computed tomography, and magnetic resonance imaging help define the site and extent of hemangioma. We describe a case of an otherwise healthy 9-year-old male presenting with recurrent gross hematuria. The initial US did not demonstrate a bladder mass, but subsequent cystoscopies showed progressive enlargement of an extensive sessile and partially pedunculated vascular mass. Imaging with ultrasound, computed tomography, and magnetic resonance imaging persistently demonstrated a hypervascular bladder mass without extravesical extension. Ultimately, biopsy of the mass made the diagnosis of bladder hemangioma.

Procedure for optimal implementation of automatic tube potential selection in pediatric CT to reduce radiation dose and improve workflow.

It is important to employ radiation dose reduction techniques in pediatric computed tomography (CT) to reduce potential risks of radiation-induced malignancy. Automatic tube potential (kV) selection tools have been developed and become available on many CT scanners, which select the optimum kV based on the patient size and clinical task to improve the radiation dose efficiency. However, its use in pediatric CT has been mostly empirical, following manufacturer's default recommendation without solid demonstration for quality improvement. This study aimed to implement an automatic tube potential tool (CAREkV, Siemens Healthcare) into routine pediatric CT practice, using the "Plan-Do-Study-Act" quality improvement process, in place of an existing kV/mAs technique chart. The design of this quality improvement project involved Plan-Do-Study-Act stages. Plan and Do stages identified the criteria for optimal automatic kV selection; a range of phantoms representing typical pediatric groups were scanned on a dual-source 128-slice scanner using a fast-pitch scanning mode. The identified CAREkV settings were implemented into the CT protocol and evaluated after a 6-month period. In the Study stage, an objective evaluation of the image metrics and radiation dose for two similar patient cohorts using CAREkV and the technique-chart, respectively, were compared. The kV selected, image quality and radiation dose determined by CAREkV were comparable to those obtained while using the technique-chart. The CAREkV was successfully implemented into our pediatric abdominopelvic CT practice. By utilizing the "PDSA" process optimal image quality and radiation dose reduction were achieved with an automatic kV selection tool to improve CT workflow.

Corrigendum to 'Anaplastic ependymoma metastases through a ventriculoperitoneal shunt' [Radiology Case Reports 15 (2020) 650-654].

[This corrects the article DOI: 10.1016/j.radcr.2020.02.036.].

Anaplastic ependymoma metastases though a ventriculoperitoneal shunt.

Ependymomas are rare glial tumors that comprise 10% of intracranial pediatric malignancies. Primary central nervous system malignancies can rarely metastasize extracranially. When metastases occur, it usually does so in the setting of surgical manipulation of the central nervous system and can spread through the blood, lymph, or artificial means, for example, a ventriculoperitoneal shunt. We describe the presentation and progression of an 18-month-old boy diagnosed with an ependymoma. Initially managed with surgery, radiation, and ventriculoperitoneal shunt placement for symptomatic hydrocephalus, the tumor later recurred with drop metastasis to the thoracic spinal cord. The patient subsequently developed extensive metastases within the abdominal cavity, which were seeded through a ventriculoperitoneal shunt. We present a case of a rare complication of intra-abdominal tumor seeding and carcinomatosis from an intracranial ependymoma through a ventriculoperitoneal shunt. This is a rare presentation of a possible complication, which requires awareness of both surgeons and radiologists.

Hepatobiliary MRI Contrast Agents: Pattern Recognition Approach to Pediatric Focal Hepatic Lesions.

OBJECTIVE. The purposes of this article are to review currently available hepatobiliary contrast agents, discuss techniques for optimization of pediatric liver MRI with hepatobiliary contrast agents, and review the imaging features of several pediatric hepatic lesions, focusing on their assessment with hepatobiliary contrast agents. CONCLUSION. MRI is the preferred imaging modality for complete assessment of focal liver lesions in the pediatric population. Imaging with gadolinium-based hepatobiliary contrast agents yields beneficial information about many focal liver lesions encountered in pediatric patients.

Subperiosteal hemorrhage: A rare complication in neurofibromatosis which may mimic malignant peripheral nerve sheath tumor.

Currently, there is little in the medical literature on subperiosteal hematomas in neurofibromatosis patients, particularly in those who have subperiosteal neurofibromas. Initial imaging and clinical presentation can be very worrisome and to the unaware may lead to early surgical intervention and/or anxiety in the patient and parents. This report presents the case of a 9-year-old child with neurofibromatosis type-1 and progressively increasing calf pain and swelling about a known plexiform neurofibroma. Imaging at initial presentation and at 1 month demonstrated evolving subperiosteal hemorrhage. This report adds to the small body of literature on subperiosteal hemorrhage as a complication of neurofibromatosis. Additionally, this report demonstrates an approach to noninvasive management of these lesions with the utility of initial and 1 month follow-up radiographs helpful in demonstrating hemorrhage evolution and possibly negating the need for biopsy.

RINT1 Bi-allelic Variations Cause Infantile-Onset Recurrent Acute Liver Failure and Skeletal Abnormalities.

Pediatric acute liver failure (ALF) is life threatening with genetic, immunologic, and environmental etiologies. Approximately half of all cases remain unexplained. Recurrent ALF (RALF) in infants describes repeated episodes of severe liver injury with recovery of hepatic function between crises. We describe bi-allelic RINT1 alterations as the cause of a multisystem disorder including RALF and skeletal abnormalities. Three unrelated individuals with RALF onset ≤3 years of age have splice alterations at the same position (c.1333+1G>A or G>T) in trans with a missense (p.Ala368Thr or p.Leu370Pro) or in-frame deletion (p.Val618_Lys619del) in RINT1. ALF episodes are concomitant with fever/infection and not all individuals have complete normalization of liver function testing between episodes. Liver biopsies revealed nonspecific liver damage including fibrosis, steatosis, or mild increases in Kupffer cells. Skeletal imaging revealed abnormalities affecting the vertebrae and pelvis. Dermal fibroblasts showed splice-variant mediated skipping of exon 9 leading to an out-of-frame product and nonsense-mediated transcript decay. Fibroblasts also revealed decreased RINT1 protein, abnormal Golgi morphology, and impaired autophagic flux compared to control. RINT1 interacts with NBAS, recently implicated in RALF, and UVRAG, to facilitate Golgi-to-ER retrograde vesicle transport. During nutrient depletion or infection, Golgi-to-ER transport is suppressed and autophagy is promoted through UVRAG regulation by mTOR. Aberrant autophagy has been associated with the development of similar skeletal abnormalities and also with liver disease, suggesting that disruption of these RINT1 functions may explain the liver and skeletal findings. Clarifying the pathomechanism underlying this gene-disease relationship may inform therapeutic opportunities.