Occipital emissary vein existence and its impact on the diagnosis of idiopathic intracranial hypertension in pediatric patients.

PURPOSE: This study aimed to investigate the efficacy of occipital emissary vein (OEV) detection in the diagnosis of idiopathic intracranial hypertension (IHH) in the pediatric age group, and to compare the prevalence and luminal diameter of OEV in patients with IHH and in healthy control subjects. METHODS: Conventional magnetic resonance imaging findings were assessed in the patients with IHH and in healthy control subjects who were under the age of 18, by two observers. The presence and luminal dimension of OEV and transverse sinus stenosis were also evaluated and compared between these two groups with magnetic resonance venography techniques. RESULTS: The rate of OEV existence was 7 times higher in the IIH group compared to the control group based on the second observer outcome (p = 0.010, OR = 7.0), with a very good interobserver agreement (Ƙ = 0.85). The dimension of OEV ranged between 0.6 and 2.5 mm. There was no correlation found between the opening pressure and the dimension of OEV (p = 0.834). CONCLUSION: In conclusion, OEV existence could be an additional radiological finding for diagnosing IHH among pediatric patients, alongside other conventional findings.

Coats plus syndrome: a rare cause of severe gastrointestinal tract bleeding in children - a case report.

BACKGROUND: Coats plus syndrome, cerebroretinal microangiopathy with calcifications and cysts, is a rare disease with autosomal recessive pattern occurring due to a mutation in CTC1, encoding conserved telomere maintenance component 1, gene. Besides retinal involvement, abnormalities in brain and osteopenia, serious life-threatening bleeding in gastrointestinal tract and portal hypertension can be observed. CASE PRESENTATION: A 6-year-old girl with Coats plus syndrome presented to the pediatric emergency department with vomiting blood and blood in stool. An upper and lower gastrointestinal endoscopy revealed esophageal varices and vascular telangiectasia in the pyloric antrum, duodenum, and colon. She received palliative care and the bleeding was stopped after receiving intravenous octreotide. She then was followed in the pediatric gastroenterology, neurology, and ophthalmology clinics. She was later hospitalized and admitted to the intensive care unit as she continued to have intermittent gastrointestinal system bleeding. She eventually died due to severe gastrointestinal system bleeding. CONCLUSIONS: Coats plus syndrome can lead to life-threatening gastrointestinal bleeding and portal hypertension. As Coats plus syndrome is quite rare, there is little published data on this syndrome. This report presents a case of Coats plus syndrome as a rare cause of gastrointestinal bleeding and portal hypertension.

Assessment of Liliequist membrane by 3D-SPACE technique at 3 T.

INTRODUCTION: Liliequist membrane (LM) is the most important anatomic structure for the success of endoscopic third ventriculostomy (ETV). Identification of this membrane is difficult with conventional MRI techniques. The purpose of this retrospective study is to determine the impact of three-dimensional sampling perfection with application-optimized contrasts using different flip-angle evolutions (3D-SPACE) sequence with variant flip-angle mode (VFAM) in the assessment of LM at 3-T MRI devices. METHODS: 3D-SPACE with VFAM images were obtained in 445 patients. LM visibility and integrity were scored as 0 (good), 1 (moderate), and 2 (poor) on these images for each parts (sellar, diencephalic, and mesencephalic) and overall of the membrane. RESULTS: According to the LM overall integrity scores, 11 % (48 cases) of the patients had perforated membrane. According to subsegmental integrity scores, sellar part was completely intact in 63 % of patients, diencephalic segment was completely intact in 60 % of the patients, and mesencephalic segment was completely intact in 95 % of the patients. Visibility scores of the third ventricle inferior wall were significantly higher in the patients with intact LM (p = 0.001). There was not any statistically significant relationship between LM pattern and overall integrity (p = 0.352). LM attachment sites could be detected easier in the patients who had better visibility of third ventricle inferior wall or intact LM (p < 0.001 for both). CONCLUSION: 3D-SPACE technique is a useful alternative for the evaluation of morphology, integrity, individual variations, topographic relationships, and visibility of LM since it has some advantages including lower SAR values, fewer artifacts, and high-resolution images.

Primary pericardial synovial sarcoma in an adolescent patient: magnetic resonance and diffusion-weighted imaging features.

Primary synovial sarcomas of the pericardium are extremely rare tumors, especially in pediatric population. As far as we know, only few cases have been reported in the literature. This uncommon location for synovial sarcomas could lead to misdiagnosis. Radiologists and clinicians should be aware of the imaging findings and differential diagnosis of pericardial synovial sarcoma. Herein we presented a 15-year-old boy who had primary pericardial synovial sarcoma with imaging features.

MRI of Residual Red Bone Marrow in the Distal Femur of Healthy Subjects.

BACKROUND: The purpose of our study is to examine the correlation of the residual red bone marrow areas of distal femoral metaphysis with the age, gender, weight and hemoglobin (hgb) values; evaluate the results, and comprehend the importance of these residual areas in the light of the results. MATERIAL/METHODS: 140 nonsmoking patients between the ages of 26 and 72 (92 women, 48 men) who had knee MR examinations were included in the study. The residual red bone marrow areas in the distal femoral metaphysis in MR images were examined by a radiologist. The areas were separated into grades according to their sizes. The hemoglobin values of the cases were measured. The size of the residual red bone marrow area and the age, gender, weight and hemoglobin values of the cases were compared by using the Tukey and Chi-Square Tests. RESULTS: Although no significant differences were observed between the mean ages, weights and hemoglobin values of the grades, a significant difference was detected between the gender distribution The male group had less residual red bone marrow in the distal femoral metaphysis than the female group (p=0.003). CONCLUSIONS: We observed that the hypointensities due to residual red bone marrow observed in the T1WS of the distal femoral metaphysis are not related with the age, weight and hemoglobin values. No grade 2 and grade 3 patient was detected in male group. We observed that these hypointense areas showed difference according to the gender variable; however, were not affected by the hemoglobin values over certain levels.

A comparison of two artificial intelligence-based methods for assessing bone age in Turkish children: BoneXpert and VUNO Med-Bone Age.

PURPOSE: This study aimed to evaluate the validity of two artificial intelligence (AI)-based bone age assessment programs, BoneXpert and VUNO Med-Bone Age (VUNO), compared with manual assessments using the Greulich-Pyle method in Turkish children. METHODS: This study included a cohort of 292 pediatric cases, ranging in age from 1 to 15 years with an equal gender and number distribution in each age group. Two radiologists, who were unaware of the bone age determined by AI, independently evaluated the bone age. The statistical study involved using the intraclass correlation coefficient (ICC) to measure the level of agreement between the manual and AI-based assessments. RESULTS: The ICC coefficients for the agreement between the manual measurements of two radiologists indicate almost perfect agreement. When all cases, regardless of gender and age group, were analyzed, a nearly perfect positive agreement was observed between the manual and software measurements. When bone age calculations were separated and analyzed separately for girls and boys, there was no statistically significant difference between the two AI-based methods for boys; however, ICC coefficients of 0.990 and 0.982 were calculated for VUNO and BoneXpert, respectively, and this difference of 0.008 was significant (z = 2.528, P = 0.012) for girls. Accordingly, VUNO showed higher agreement with manual measurements compared with BoneXpert. The difference between the agreements demonstrated by the two software packages with manual measurements in the prepubescent group was much more pronounced in girls compared with boys. After the age of 8 years for girls and 9 years for boys, the agreement between manual measurements and both AI software packages was equal. CONCLUSION: Both BoneXpert and VUNO showed high validity in assessing bone age. Furthermore, VUNO has a statistically higher correlation with manual assessment in prepubertal girls. These results suggest that VUNO may be slightly more effective in determining bone age, indicating its potential as a highly reliable tool for bone age assessment in Turkish children. CLINICAL SIGNIFICANCE: Investigating the most suitable AI program for the Turkish population could be clinically significant.

Splenic cysts: clues in sonographic differential diagnosis and a new role for twinkling artifact.

AIM: Our aim was to investigate the frequency of various splenic cysts, to define the sonographic differential diagnostic clues and to introduce the value of twinkling artefact in the diagnosis of epidermoid splenic cysts. MATERIAL AND METHODS: All the splenic cysts imaged by ultrasound in 3 university hospitals during the period of 2005 to 2022 were recorded, followed-up and analyzed. RESULTS: One hundred seventy-one patients with splenic cysts were detected and these were classified and 73% of the cysts were simple. Ten cysts were epidermoid cysts as proven by post-operative final histology. CONCLUSION: Cystic splenic lesions are rare. Most of them are small simple cysts. Epidermoid cysts are larger in volume, constitute 6% of the total and can be differentiated by the help of twinkling artefact by Doppler ultrasound.

Tortuous occipital emissary vein combined with dural venous sinus stenosis in contrast-enhanced MRV for evaluation of idiopathic intracranial hypertension.

PURPOSE: Venous outflow impediment is increasingly recognized in idiopathic intracranial hypertension (IIH). We aim to search for the value of tortuous occipital emissary vein (OEV) in IIH by integrating measurable transverse sinus (TS) stenosis in contrast-enhanced MR venography (CE-MRV). METHODS: Twenty-one IIH patients were evaluated with CE-MRV. Each patient had high LP opening pressure (> 25 cm.H2O), and presented papilledema. Age- and sex-matched 21 control subjects who underwent cranial CE-MRV were selected. The OEV and the following features: intraosseous diameter of more than 3 mm, twisted course, and continuous with prominent sub-occipital extracranial veins were named tortuous OEV. TS stenosis was measured by utilizing the coronal T1-VIBE series to calculate quantitative metrics such as TS max./min.. The tortuous OEV and TS max./min. were registered to create a bivariate logistic regression model to assess the performance of tortuous OEV for IIH when accompanied by TS stenosis. RESULTS: Six (29%) tortuous OEVs were observed in the IIH group, while no tortuous OEV was identified in the control group (p = 0.021). The mean TSmax./min. was 2.48 ± 1.19 in patients with IIH and 1.23 ± 0.33 in the control group (p < 0.001). According to regression analysis, tortuous OEV is not predictive of IIH (p = 0.999), while the higher TSmax./min. (> 1.69) is predictive of IIH (p = 0.022, OR: 8.9; %95 CI; 1.4-59.0) when accompanied together. CONCLUSION: Tortuous occipital emissary vein is more frequently seen in patients with IIH. However, the tortuous appearance alone does not predict idiopathic intracranial hypertension unless associated with measurable transverse sinus narrowing in CE-MRV.

A novel biphasic oral contrast solution for enterographic studies.

PURPOSE: To compare the efficacy and patients' tolerance of a new mixed biphasic oral contrast solution with routine biphasic oral contrast agent in magnetic resonance (MR) enterography (MRE). MATERIALS AND METHODS: Thirty-seven patients (group 1) had MRE with the new mixture, whereas 14 patients (group 2) had MRE with biphasic oral contrast agent (lactulose). Magnetic resonance enterography images were evaluated by 2 experienced radiologists. Each intestinal segment was evaluated for luminal distension (LD), distinction from the surrounding tissue (wall conspicuity), and the confidence of radiologist for evaluation of the specified segment (radiological evaluation confidence). Comparisons between the 2 groups were performed using the Mann-Whitney U test. Interrater and intrarater agreement values were obtained. In addition, patients' acceptability and tolerance were assessed. RESULTS: The new mixture was more effective than the oral contrast agent used in group 2 for LD, wall conspicuity, and radiological confidence. The values of interrater and intrarater agreement in scoring LD, wall conspicuity, and radiological confidence were generally moderate. CONCLUSION: Our new mixture allowed good-quality enterographic images, and this solution was well tolerated by patients. In addition, this mixture is useful for evaluation of small bowels and colonic segments. We suggest the use of it for enterographic examinations.

Evaluation of spontaneous third ventriculostomy by three-dimensional sampling perfection with application-optimized contrasts using different flip-angle evolutions (3D-SPACE) sequence by 3T MR imaging: preliminary results with variant flip-angle mode.

PURPOSE: This prospective study aimed to evaluate the use of three-dimensional sampling perfection with application-optimized contrasts using different flip-angle evolutions (3D-SPACE) in the diagnosis of spontaneous third ventriculostomy (STV) and to compare it with phase-contrast cine magnetic resonance imaging (PC-MRI). METHODS: PC-MRI and 3D-SPACE images were obtained in 15 patients with hydrocephalus for evaluation of STV as well as in 10 control cases. The presence of STV was evaluated visually by two experienced radiologists on both PC-MRI and 3D-SPACE images, and the results were statistically compared. RESULTS: There was a strong correlation between PC-MRI and SPACE scores for both readers (correlation coefficient [r]=0.784; P=0.001). There was also a good correlation between PC-MRI scores and consensus-based results. Interobserver reliabilities were strong for all STV scores. In addition, there was excellent correlation between 3D-SPACE scores and consensus-based results (first reader's kappa value: 0.87; second reader's kappa value: 1). CONCLUSION: 3D-SPACE can provide morphological-physiological information for the evaluation of STV with no need for additional PC-MRI analysis or other sequences. As a non-invasive test, it can also be included among the first line of choices of MRI sequences for patients with obstructive hydrocephalus.

Radiologic manifestations of colloid cysts: a pictorial essay.

Colloid cysts are among rare benign tumours of the third ventricle. Although the most frequent symptoms are headache and syncope, arrest hydrocephalus or sudden death could appear with colloid cysts. The aim of this pictorial essay was to increase awareness of the clinical presentation, computed tomography (CT) and magnetic resonance (MR) imaging spectrum, and treatment options of the colloid cysts. The data of 11 patients with histopathologically and/or clinically proven colloid cyst were analysed, retrospectively; and the neuroradiologic appearances of the cysts were evaluated. The CT and MR appearance of colloid cysts may change, depending on the viscosity or the cholesterol content of the cysts. However, the cystic content is the most important factor that could affect the success of treatment. Cysts that are especially rich in protein and cholesterol tend to be hyperdense on CT, hypointense on T2-weighted sequences and hyperintense on T1-weighted sequences. These cysts are viscous, and the success of aspiration is significantly low. In the diagnosis and evaluation of small-sized cysts that have an ingredient similar to cerebrospinal fluid, 3-dimensional sequences might be useful. The radiologic appearances of colloid cysts could play an important role in directing these patients to alternative surgical modalities, including resection.

Bouveret syndrome: evaluation with multidetector computed tomography and contrast-enhanced magnetic resonance cholangiopancreatography.

Cholecystenteric fistula is one of the rarest complications of biliary lithiasis, with a frequency of less than 1%. Bouveret syndrome is a gastric outlet obstruction produced by gallstone(s) located in the distal stomach or proximal duodenum. The route of gallstone migration to the bowel is most commonly via a cholecystoduodenal fistula; however, fistulization of the stomach is a rarer variation. Early diagnosis of this situation is crucial to reduce morbidity and mortality. In this report, we present a patient with cholecystogastric fistula and Bouveret syndrome. To our knowledge, there is no published paper in the literature related to the diagnosis of Bouveret syndrome with multidetector computed tomography (MDCT) (64 detectors) and/or contrast-enhanced magnetic resonance cholangiopancreatography (CE-MRCP). Our aim was to discuss the efficacy of MDCT and CE-MRCP in the detection and evaluation of cholecystenteric fistulas. We showed the exact localization and relation of biliary stones and the fistula by MDCT and CE-MRCP. We also evaluated the biliary system with CE-MRCP physiologically. In conclusion, when biliary lithiasis and ileus are detected in plain radiography, the first-line diagnostic tool should be MDCT. In complicated cases or when biliary obstruction is suspected, CE-MRCP can give important morphological and physiological information regarding the whole abdomen and biliary system.

Magnetic resonance imaging of vulvar dermatofibrosarcoma protuberans - report of a case.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) of the vulva is a rare low-grade soft tissue sarcoma. Magnetic resonance imaging (MRI) findings of vulvar DFSP were essentially unreported in the literature. CASE REPORT: We report a DFSP of vulva with its clinical, histological and MRI features. As far we know this is the first case of histologically confirmed vulvar DFSP presenting with MR images. The diagnosis of DFSP is usually made by histopathologic and clinical findings. CONCLUSIONS: MRI is useful both for the diagnosis of DFSP and following up the patients since it has high soft tissue resolution and no risk of radiation exposure. With MRI the relation to the adjacent anatomical structures, extension and depth of the tumour and possible lymph node involvement can also be demonstrated.

Diffusion-weighted MRI for differentiating Wilms tumor from neuroblastoma.

PURPOSE: Wilms tumor (WT) and neuroblastoma (NB) are the most common pediatric abdominal malignant neoplasms of the kidney and adrenal gland. Differentiating them from each other is essential since their treatments are different. Here, we aimed to show the diffusion characteristics of WT and NB for differentiation. METHODS: Diffusion-weighted imaging (DWI) of 17 histopathologically diagnosed lesions (10 NB and 7 WT in 8 female and 9 male patients) was evaluated retrospectively. The apparent diffusion coefficient (ADC) value for each tumor was calculated using region-of-interest (ROI) measurements by two observers. The mean ADC values were compared, and receiver operating characteristics (ROC) curve analysis was performed. Intraclass correlation was evaluated for the reliability of ADC measurement. RESULTS: The mean ADC values measured by two observers were 0.787±0.09 ×10-3 mm2/s and 0.768±0.08 ×10-3 mm2/s for WT, and 0.524±0.16 ×10-3 mm2/s and 0.529±0.16 ×10-3 mm2/s for NB, respectively (P = 0.006 and P = 0.011). Intraclass correlation coefficient was 0.955. Utilizing ROC curve analysis, a cutoff ADC value of ≤0.645 ×10-3 mm2/s was obtained to differentiate NB from WT. CONCLUSION: ADC values of NBs were significantly lower than WT with a perfect interobserver agreement. We suggest that DWI may have a role in differentiating the two tumors.

Synchronous Solid Pseudopapillary Tumor and Insulinoma in an Adolescent MEN1 Patient Presenting with Diagnostic Dilemmas.

Multiple endocrine neoplasia (MEN1) is a rare autosomal dominant disorder characterized by primary hyperparathyroidism, enteropancreatic neuroendocrine tumors, and anterior pituitary adenomas. A 16-year-old male presented to the emergency outpatient clinic with tonic convulsions. Physical examination in the postconvulsive period was unremarkable and revealed a muscular, postpubertal adolescent. Biochemical tests at admission were consistent with hyperinsulinemic hypoglycemia and remarkable for elevated levels of liver transaminases and creatine kinase. Work-up for a potential inborn error of metabolism and Doppler ultrasound for congenital portal-hepatic shunt were negative. When the patient was questioned, he reported using the anabolic steroid stanozolol to strengthen his muscles. His enzyme levels normalized after cessation of stanozolol. Hypoglycemia did not recur on diazoxide therapy. Magnetic resonance imaging showed two discrete lesions in the pancreas. Distal pancreatectomy revealed two masses 1.1 and 1.4 cm in diameter: a solid pseudopapillary tumor and an insulinoma. The patient also had asymptomatic primary hyperparathyroidism. DNA sequence analysis of the MEN1 gene in the index patient and his father and brother revealed a previously reported "pW183S" heterozygous mutation. This case further adds to the "pancreatic tumor" phenotype of MEN1 with the presence of a solid pseudopapillary tumor. This case report also confirms the need to meticulously question drug abuse in adolescents presenting to clinics with diagnostic challenges.

A rare cause of abdominal pain: Ectopic ovary and intestinal malrotation.

Altinay-Kirli E, Özcan R, Öncül M, Özmen E, Eliçevik M, Büyükünal C, Emir H, Topuzlu-Tekant G. A rare cause of abdominal pain: Ectopic ovary and intestinal malrotation. Turk J Pediatr 2017; 59: 699-703. Ectopic ovary is a rare anomaly that can be associated with unicornuate uterus and renal anomalies. Intestinal rotational anomalies are failure of normal rotation and this arrest in development can predispose to develop a malfixated midgut that is a risk factor for volvulus and significant morbidity and mortality especially in early childhood. Cyclic abdominal pain is a common symptom for both of two distinct pathologies in adolescent ages. Here, we report a case of unicornuate uterus together with right ectopic ovary and intestinal malrotation.

A magnetic resonance imaging finding in children with cerebral palsy: Symmetrical central tegmental tract hyperintensity.

BACKGROUND: Central tegmental tract is an extrapyramidal tract between red nucleus and inferior olivary nucleus which is located in the tegmentum pontis bilaterally and symmetrically. The etiology of the presence of central tegmental tract hyperintensity on MRI is unclear. PURPOSE: In this study our aim is to evaluate the frequency of central tegmental tract lesions in patients with cerebral palsy and control group, as well as to determine whether there is an association between central tegmental tract lesions and cerebral palsy types. MATERIALS AND METHODS: Clinical and MRI data of 200 patients with cerebral palsy in study group (87 female, 113 male; mean age, 5.81years; range, 0-16years) and 258 patients in control group (114 female, 144 male; mean age, 6.28years; range, 0-16years) were independently evaluated by two reader for presence of central tegmental tract hyperintensity and other associated abnormalities. RESULTS: Central tegmental tract hyperintensities on T2WI were detected in 19% of the study group (38/200) and 3.5% of the control group (9/258) (p<0.0001). Among the total of 38 central tegmental tract lesions in study group, the frequency of central tegmental tract hyperintensity was 16% (24/150) in spastic cerebral palsy and 35% (14/40) in dyskinetic cerebral palsy (p=0.0131). CONCLUSION: The prevalence of central tegmental tract hyperintensity is higher in patients with cerebral palsy particularly in dyskinetic type. We suggest that there is an increased association of the tegmental lesions with dyskinetic CP. Patients with cerebral palsy and ischemic changes were more likely to have central tegmental tract lesions. According to our results we advocate that an ischemic process may have a role in the etiopathogenesis.

Cardiac veins: collateral venous drainage pathways in chronic hemodialysis patients.

Venous anomalies are diagnostic and therapeutic challenges. Subclavian or superior vena cava stenosis can be developed and venous return can be achieved via cardiac veins and coronary sinus in patients with central venous catheter for long-term hemodialysis. These types of abnormalities are not extremely rare especially in patients with a history of central venous catheter placement. Detection of these anomalies and subclavian vein stenosis before the surgical creation of hemodialysis fistulae or tunneled central venous catheter placement may prevent unnecessary interventions in those patients. Multidetector computed tomography (MDCT) technique can give further information when compared with fluoroscopy or digital subtraction angiography in the management of these patients. This case report describes interesting aspects of central vein complications in hemodialysis patients. As a conclusion, there are limited data about thoracic venous return, and further prospective studies with large patient number are required. MDCT with 3D reconstruction is particularly useful for the accurate evaluation of venous patency, variations, and collateral circulation. Also it is an excellent tool for choosing and planning treatment.

Fully automated morphological analysis of patients with obstructive sleep apnea.

BACKGROUND/AIM: Obstructive sleep apnea syndrome (OSAS) is a disease characterized by episodic hypoxia. We aimed to use the Freesurfer program for global evaluation of morphological changes in OSAS patients. MATERIALS AND METHODS: Three-dimensional T1-weighted images were obtained, and intracranial morphology was assessed in 18 patients with OSAS and 20 controls. Results of the volume and the cortical thickness analyses of both groups were compared statistically. RESULTS: The total cortical, left-right hemispheres gray matter (GM), corpus callosum, and total GM volumes were lower in OSAS patients when compared with the control group (P < 0.001). The average cortical thickness was lower in OSAS patients bilaterally in pars orbitalis, paracentral, rostral middle frontal, middle frontal, orbital, and superior frontal gyri when compared with the control group (P < 0.05). Furthermore, the volume and average cortical thickness of multiple anatomic regions, apart from the brain parts mentioned above, were decreased unilaterally (e.g., hippocampus, cingulum, putamen, thalamus) in OSAS patients (P < 0.05). CONCLUSION: Multiple morphologic changes occur in the cerebral structures of OSAS patients due to intermittent ischemia episodes. Detection of those areas with Freesurfer is easier. New studies with large series would be needed for these subjects.