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.

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.

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.

The Impact of Gd-Eob-Dtpa-Enhanced MR Cholangiography in Biliary Diseases: Comparison with T2-Weighted MR Cholangiopancreatography.

BACKGROUND: Contrast enhanced magnetic resonance cholangiography is a novel technique and promising method in demonstrating biliary tree anatomy and evaluating biliary disorders. However, to date, there are a limited number of studies that have focused on the impact of this technique. AIMS: We aimed to evaluate the additional role of contrast enhanced MR cholangiography (MRC) and compare contrast enhanced MRC with T2-weighted (w) magnetic resonance cholangiopancreatography (MRCP) in the diagnosis of biliary disorders. STUDY DESIGN: Diagnostic accuracy study. METHODS: The T2w-MRCP and contrast enhanced MRC sequences of 31 patients whose gold standard test results were available were scored visually for the existence of pathological findings with regard to any of the biliary diseases. Gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid (Gd-EOB-DTPA) was used as the contrast agent. The correlation values were determined according to the statistical analysis made from those scores and the sensitivity, specificity and accuracy values of each sequence were detected as well. RESULTS: We detected that the correlation values with gold standard methods of contrast enhanced MRC sequences were significantly higher than the ones of T2w-MRCP sequences. The correlation ratios of T2w-MRCP sequences were between 26 and 34%, while those for contrast enhanced MRC sequences were between 81 and 83% for the first reader and the correlation ratios of T2w-MRCP sequences were between 10 and 61%, whereas those of contrast enhanced MRC were between 79 and 81% for the second reader The mean sensitivity, specificity and accuracy values of T2w-MRCP sequences were 14.3-42.5%, 85-89.2% and 59.3-72.5%, respectively, while the mean sensitivity, specificity and accuracy values of contrast enhanced MRC sequences were 100%, 86.7% and 93.2-93.3%, respectively. CONCLUSION: We suggest that obtaining of contrast enhanced MRC sequences in addition to the T2w-MRCP can be useful in the diagnosis of many diseases in relation with biliary tree.

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.

The efficacy of diffusion weighted imaging and apparent diffusion coefficients mapping for liver metastasis of colonic adenocarcinomas.

OBJECTIVES: To establish retrospectively the relation between the histopathologic grade of colorectal liver metastasis and apparent diffusion coefficient (ADC) values of hepatic metastases of colorectal adenocarcinomas. METHODS: The diagnoses of liver metastases were confirmed with biopsy, surgery, and follow-up imaging findings. Twenty-six patients with 94 liver metastasis were included in the study. Of 94 masses, 59 were poorly-differentiated adenocarcinoma, 18 were moderately-differentiated adenocarcinoma, and 17 were well-differentiated regarding the diameters, ADC values, and ratio index (RI) values. Kolmogorov-Smirnov normality test, Kruskal-Wallis analysis of variance, Mann-Whitney U test with Bonferroni correction, Spearman correlation analysis, and receiver operating characteristics curve methods were applied to evaluate the statistical relations. RESULTS: There was a statistically significant difference in terms of ADC values and RI between poorly-differentiated adenocarcinoma and moderately-differentiated adenocarcinoma plus well-differentiated adenocarcinomas. Poorly-differentiated adenocarcinomas have the lowest ADC values and highest RI values among other groups. CONCLUSION: Use of ADC values alone can be executed for the diagnosis of focal hepatic masses and also can aid in the differentiation of benign and malignant hepatic lesions.

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.

Ectopic anterior cerebellum (ala lobule centralis).

In this case report we present an adolescent girl who was referred to our radiology department for assessment with advanced magnetic resonance (MR) imaging on suspicion of low-grade quadrigeminal cistern neoplasm on 1.5 Tesla MR examination. We were able to evaluate detailed cerebellar anatomy more clearly, and detected that the lesion was compatible with ectopic cerebellar tissue (a very rare developmental variation) on submillimetric 3-dimensional (3D) images from a 3 Tesla MR unit which has a 32-channel head coil. Our findings were further supported by diffusion tensor imaging which clearly indicated that the lesion was a part of the cerebellum. Furthermore, MR spectroscopic metabolite ratios were in accordance with the characteristics of normal neuronal tissue. As far we know there is no published report that contains similar findings to those of our patient. In conclusion, cranial MR images, if possible in 3D format (with very small isotropic voxels) should be obtained for the precise diagnosis of the lesions located in this region; in addition, the differential diagnostic list should be well known and advanced imaging techniques should be used when necessary.

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.

Assessment of third ventriculostomy patency with the 3D-SPACE technique: a preliminary multicenter research study.

OBJECT: The goal of this study was to determine the value of the 3D sampling perfection with application-optimized contrasts using different flip-angle evolutions (3D-SPACE) technique in the evaluation of endoscopic third ventriculostomy (ETV) patency. METHODS: Twenty-six patients with ETV were examined using 3-T MRI units. Sagittal-plane 3D-SPACE with variant flip-angle mode, 3D T1-weighted (T1W), and 3D heavily T2-weighted (T2W) images were obtained with isotropic voxel sizes. Also, sagittal-axial plane phase-contrast cine (PC)-MR images were obtained. The following findings were evaluated: diameters of stoma and third ventricle, flow-void sign on 3D-SPACE and PC-MR images, integrity of the third ventricle on heavily T2W images, and quantitative PC-MRI parameters of the stoma. Obtained sequences were evaluated singly, in combination with one another, and all together. RESULTS: The mean area, flow, and velocity values measured at the level of stoma in patients with patent stoma were significantly higher than those measured in patients with closed stoma (p < 0.05). There was significant correlation among PC-MRI, 3D-SPACE, and 3D heavily T2W techniques regarding assessment of ETV patency (p < 0.001). The 3D-SPACE technique provided the lowest rate of ambiguous results. CONCLUSIONS: The 3D-SPACE technique seems to be the most efficient one for determination of ETV patency. The authors suggest the use of 3D-SPACE as a stand-alone first-line sequence in addition to routine brain MRI protocols in assessing patients with ETV, thereby decreasing scan time and reserving the use of a combination of additional sequences such as PC-MRI and 3D heavily T2W images in suspicious or complex cases.

The prevalence of pineal cyst in patients with cerebral palsy.

PURPOSE: Pineal cysts are common incidental findings during magnetic resonance imaging (MRI) examinations. The etiology of pineal cyst development is still unclear. We aimed to determine whether there is an association between periventricular leukomalacia and pineal cyst prevalence. METHODS: Clinical and MRI data of 201 patients with periventricular leukomalacia (110 female, 91 male; mean age, 6 years; range, 2-18 years) and 687 control patients (355 female, 332 male; mean age, 6 years¸ range, 2-18 years) who did not have any evidence of periventricular leukomalacia were independently evaluated by two radiologists for presence or absence of pineal cyst. RESULTS: Pineal cysts were detected in 32.3% of the study group (65/201) and 8.4% of the control group (58/687) (P < 0.001). Patients with periventricular leukomalacia were more likely to have a pineal cyst. In terms of pineal cyst detection on MRI, interobserver reliability was high between the two radiologists. CONCLUSION: The prevalence of pineal cysts is higher in patients with periventricular leukomalacia. We suggest that an ischemic process may have a role in the etiopathogenesis of pineal cyst development.

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.

Iatrogenic Superficial External Pudendal Artery Pseudoaneurysm: Treatment with Doppler US-Guided Compression.

Pseudoaneurysms rarely occur as a serious complication following incomplete hemostasis of an arterial puncture site. As a result of the increase in diagnostic and therapeutic angiography, the frequency of iatrogenic pseudoaneurysm has increased as well. Iatrogenic pseudoaneurysms associated with angiographic catheterization occur most commonly in the common femoral artery. Here we report a case of iatrogenic superficial external pudendal artery (SEPA) pseudoaneurysm following cardiac catheterization, which was diagnosed with Doppler ultrasound (US) and multidetector computed tomographic angiography (MDCTA) before Doppler US-guided compression therapy. To the best of our knowledge, iatrogenic SEPA pseudoaneurysm, which is an unusual vessel location for pseudoaneurysm occurrence, has not been reported in the literature. In patients in whom anticoagulant-thrombolytic therapy or therapeutic catheterization with larger sized sheath is planned, determination of the precise localization of arterial puncture site is important for the prevention of iatrogenic pseudoaneurysm development. Arterial puncture guided with Doppler US might reduce complications. When suspected, MDCTA is useful in the diagnosis and demonstration of iatrogenic pseudoaneurysms. Treatment of US-guided compression should be the first choice for iatrogenic pseudoaneurysms. Interventional radiologists and cardiologists should have enough experience about the catheterization complications and their treatment in order to decrease the morbidity and mortality related to the intervention.

The impact of share wave elastography in differentiation of hepatic hemangioma from malignant liver tumors in pediatric population.

OBJECTIVE: In children it is crucial to differentiate malignant liver tumors from the most common benign tumor, hepatic hemangiomas since the treatment strategies are quite different. We aimed to evaluate the efficiency of shear wave elastography (SWE) technique in differentiation of malignant hepatic tumors and hepatic hemangiomas. METHODS: Twenty patients with hepatic tumor were included in our study. Two radiologists performed SWE for 13 patients with malignant hepatic tumors including hepatoblastoma (n=7), hepatocellular carcinoma (n=3), metastasis (n=2), embryonal sarcoma (n=1) and 7 patients with hepatic hemangioma. All of our patients were between the age of 1 and 192 months (mean age: 56.88 months). Receiver operating characteristic analysis was achieved to evaluate the diagnostic accuracy of SWE and to determine the optimal cut-off value in differentiation hepatic hemangioma from malignant hepatic tumors. RESULTS: The mean SWE values (in kPa) for the first observer were 46.94 (13.8-145) and 22.38 (6.6-49.6) and those for the second observer were 57.91 (11-237) and 23.87 (6.4-57.5), respectively for malignant hepatic tumors and hepatic hemangiomas. The SWE values of malignant hepatic tumors were significantly higher than those of hepatic hemangioma (p=0.02). The inter-observer agreement was almost perfect (0.81). The area under the receiver operating characteristic curve of SWE for differentiating the hepatic hemangioma from malignant hepatic tumors was 0.77 with a sensitivity of 72.7% and a specificity of 66.7% at a cutoff value of 23.62 with 95% confidence interval. CONCLUSION: Shear wave elastography can be helpful in differentiation of malignant hepatic tumors and hepatic hemangioma.

Radiologic manifestation of a BCS1L-mutated patient.

There are various phenotypes of mutations in BCS1L which encode a mitochondrial inner membrane protein that facilitates the insertion of Rieske iron-sulfur protein into respiratory chain complex III. Herein we report the radiologic findings of a patient with bc1 synthesis-like (BCS1L) gene mutation who was presented with convulsions.