Objective To describe the clinical features of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children. Method Data from 11 pediatric patients with autoimmune GFAP astrocytopathy were retrospectively analyzed. Results All of the patients showed encephalitis and meningoencephalitis or meningoencephalomyelitis with or without myelitis. 45.4% of the patients had fever, 27.3% headaches, 18.2% dizziness, 18.2% drowsiness, and 18.2% mental disorders. Cerebrospinal fluid (CSF) was detected in all patients. The white blood cell counts (WBC) (90.9%), lactic dehydrogenase levels (72.7%), protein level (36.4%), and adenosine deaminase activity (ADA) level (27.3%) were elevated, and the CSF glucose levels (72.7%) were slightly reduced. Nine patients (90%) were found to have brain abnormalities, of which five (50.0%) patients had abnormal symmetrical laminar patterns or line patterns hyperintensity lesions on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images in the basal ganglia, hypothalamus, subcortical white matter and periventricular white matter. The linear radial enhancement pattern of the cerebral white matter was only seen in two patients, with the most common being abnormal enhancement of leptomeninges (50%). Five patients had longitudinally extensive spinal cord lesions. Conclusion The findings of pediatric patients with autoimmune GFAP astrocytopathy are different from previous reports.

Background Cranial fasciitis (CF) is a rare benign fibroproliferative lesion of the skull. To date, the summarized radiologic characteristics and the subtype of the disease have not been reported. our purpose was to summarize the characteristic clinicoradiology features and management of CF and to improve the knowledge of radiologists and clinicians. Methods We searched our institution’s database and retrieved the clinical and radiologic data of CF patients confirmed by histopathological examination. The clinicoradiology features and management of CF were analysed retrospectively. Results A total of 14 CF patients were included. A total of 85.7% of the patients presented with a painless, firm, nonmobile and single mass. Tenderness and multiple masses were found in 14.3% of the patients. The mass was clearly increased in 2 patients and gradually increased in 5 patients in the short term. We divided these patients into three types based on the CT characteristics. The characteristic features of type I (9 patients) presented as an expansive and osteolytic bone destruction with a soft tissue mass. Type II (2 patients) presented as a scalp mass with mild erosion of the outer skull plate. Type III (3 patients) presented as a scalp mass without skull destruction. All patients underwent surgical resection. For type I patients, craniectomy and cranioplasty were performed. For type II patients, complete excision of the scalp mass with local skull curettage was performed. For type III patients, complete excision of the scalp mass was performed. There were no cases of recurrence after follow-up. Conclusions CF usually presents as a painless, firm, nonmobile and single mass with a clear boundary. There are generally three types of MSCT findings: bone destruction with a soft tissue mass, a scalp mass with erosion of the skull and a scalp mass. Different management strategies should be utilized for the various types of CF.

Background: Hydronephrosis is a common condition, and the correct diagnosis of hydronephrosis is necessary to improve the early diagnosis rates of pediatric hydronephrosis. Objective: To explore and analyze the diagnostic value of dynamic contrast-enhanced magnetic resonance imaging [DCE-MRI] analyzed using the Tofts model in children with unilateral hydronephrosis. Methods: We retrospectively selected data from 88 children with unilateral hydronephrosis treated in our hospital from September 2018 to October 2020. Routine and DCE-MR renal image indexes were collected and their pharmacokinetic variables were calculated based on the Tofts model to compare kinetic parameters of affected and normal kidney. We compared the renal parenchymal thickness and other renal function indexes in children with different degrees of hydronephrosis, and drew receiver operating characteristic[ROC] curves to evaluate the diagnostic value of this approach in children with hydronephrosis. Results: The Ktrans, Kep, and Ve values in the diseased kidneys were lower than those in the normal ones [P<0.05]. The thickness of the healthy renal parenchyma in children with severe hydronephrosis was higher than in children with moderate and mild hydronephrosis, but the renal parenchyma thickness and the thickness ratio of renal parenchyma in the affected side were lower than those in children with moderate and mild hydronephrosis [P<0.05]. Sensitivity, specificity and accuracy of DCE-MRI and Tofts model in the diagnosis of hydronephrosis in children were higher than those of single DCE-MRI [P<0.05]. The area under the ROC curve for the DCE-MRI and Tofts model approach for the diagnosis of hydronephrosis in children was 0.789 [95% CI, 0.72-0.859], and the sensitivity and specificity were 86.36% and 71.59%, respectively. Conclusions: DCE-MRI and Tofts model can provide clear picture of renal morphology, and renal function evaluation parameters. They have high sensitivity and specificity in the diagnosis of hydronephrosis in children.

Purpose Patent ductus venosus (PDV) was a rare congenital portosystemic shunt, and few studies have been reported to date. The purpose of this study was to investigate the clinical and imaging findings to improve the recognition of PDV. Materials and Methods Medical records of 9 patients with PDV in Hunan Children’s Hospital from May 2013 to December 2020 were retrospectively reviewed. The clinical, and laboratory tests data were extracted from the electronic medical records. Two pediatric radiologists evaluated all imaging examinations of the patients.Results 9 patients with PDV were reviewed, including 7 males and 2 females, aged from 16 days to 16.5 years, median age was 1.6 years. PDV diameter ranged from 4.0mm to 17.5mm. The initial clinical presentations of PDV were varied, but jaundice and respiratory symptoms were the most common. Laboratory tests showed that 5/9 cases had hypoxemia, 2/9 had hyperammonemia, 7/9 had hyperbilirubinemia, 6/9 had abnormal coagulation function, 4/9 had abnormal myocardial enzymes, 8/9 had hepatic dysfunction, and 3/9 had renal dysfunction. The direct imaging sign of PDV was a vascular structure connecting the left branch of portal vein(LPV) to the inferior vena cava(IVC), running in the depth of the Arantius sulcus. The secondary imaging findings were as follows: All the patients had enlarged liver, especially the left lobe, and one of the patients presented with diffuse nodules in the liver. 3 patients presented with hypoperfusion in right lobe of liver. The spleen was enlarged in 8 cases but shrank in one. Dilated LPV and atrophic right branch of portal vein (RPV) were observed in all patients. The main portal vein (MPV) was dilated in 8 cases and shrank in one. Dilated right heart and pulmonary artery were observed in all cases. Abnormal renal imaging was observed in 2 patients. The complications and coexistent malformations were as follows: Brain MRI indicated hepatic encephalopathy in 4 cases. 7/9 patients were combined with other malformations, and the most common coexistent malformations were congenital heart disease (CHD) and vascular abnormalities, with 5 and 6 cases respectively. Conclusions PDV was a rare vascular malformation that can lead to multi-system lesions. The clinical presentations and laboratory findings were diverse. The diagnosis of PDV mainly depends on imaging examinations, and it is important to evaluate the secondary imaging changes. Complications and coexistent malformations were common, and we need to prevent omissions during the imaging evaluations.

Purpose The purpose of this study was to compare the differences between obstructed and unobstructed TAPVC using echocardiography, then to predict PVO by assessing clinical and echocardiographic parameters. Methods We conducted a retrospective study included 70 patients with TAPVC between 2014 and 2018. The morphological and hemodynamic echocardiographic parameters were observed and measured. The clinical and echocardiographic parameters that existed difference between obstructed and unobstructed TAPVC were selected to predict PVO by running ROC curve analysis. Results Between obstructed and unobstructed TAPVC, there were significant differences in ASD size, PA Vmax, MV VE, LVFS, LVEF, SV and the incidence of PDA, but there was no significant difference in birth weight. The first admission age of obstructed TAPVC was earlier than unobstructed type. The ROC curve analysis for the first admission age in predicting PVO showed the sensitivity and specificity were 76.7%, 80% respectively. The ROC curve analysis for multiple echocardiographic parameters showed the sensitivity and specificity were 82.6%, 100% respectively. Conclusions The presence of PVO led to anatomical and functional abnormity of patients with TAPVC, and then led to hospitalize earlier. The first admission age was a simple and feasible parameter in predicting PVO. The integrated use of multiple echocardiographic parameters had an excellent value in predicting PVO.