There are numerous reports that patients with thalassemia are faced with hypercoagulability leading to vascular disorders. One of these complications is known as a silent infarct, defined as a small infarct detected by cerebral imaging but without any neurological symptoms. Since it has a progressive nature, it is of vital importance because it may lead to symptomatic cerebrovascular accidents in the future. Twenty-two children with thalassemia intermedia were enrolled into the study and MRI scans were performed. All demographic data and clinical features of the patients were obtained during the follow-up period. In addition to the patients, 13 healthy controls were included to compare serum anticoagulant levels with those of the thalassemia intermedia patients. Four of the patients were found to have silent cerebral infarcts (SCIs). The lesions involved varying amounts of the deep cerebral white matter and sub-cortical areas. One patient showed 'net line' filling defects within the ambient cistern on MRI images corresponding to moyamoya vessels. Three patients had undergone splenectomy, and three were transfused irregularly and had less than six transfusions per year. More importantly, protein C levels were lower and platelet levels were significantly higher in the patient group compared with controls. We were not able to find any association between SCI and transfusion number or splenectomy. However, of the total patients four thalassemia intermedia patients had SCI in early childhood and this is an unusual finding. In order to verify the findings, further studies must be conducted involving larger numbers of patients.
Horseshoe lung is a congenital pulmonary malformation that is usually associated with scimitar syndrome. This malformation consists of fusion of both pulmonary lobes from the posterobasal segments. The fusion appears in the retrocardiac area, in front of the esophagus and thoracic aorta. Pleural separation of pulmonary lobes distinguishes pseudohorseshoe appearance from a true horseshoe lung. Scimitar syndrome known as hypogenetic lung syndrome is a part of the congenital pulmonary venolobar syndrome. It is a partial anomalous pulmonary venous return with pulmonary hypoplasia. Scimitar vein is an anomalous drainage vessel between the right pulmonary lobe vessels and the inferior vena cava. The appearance of the vessel resembles Turkish scimitar; therefore, the syndrome is called scimitar syndrome. We hereby report a 61-year-old woman with adult form congenital scimitar syndrome and will describe the imaging findings of pseudohorseshoe lung appearance.
Objectives: The purpose of this study was to detect the types and prevalences of vena cava variations, as well as looking for gender differences and associating anomalies. Patients and Methods: The computed tomography (CT) images of 5763 patients who had undergone thorax and abdominal CT examinations were evaluated retrospectively for superior vena cava (SVC), inferior vena cava (IVC), and left renal vein (LRV) variations. Results: Vena cava variations were detected in 637 (11%) patients. SVC variations were present in 15 (0.26%) patients, while 36 (0.64%) patients had IVC, and 596 patients (10.34%) had LRV variations. It was also shown that there was no difference between the prevalences of the variations in terms of gender difference. When associating anomalies were evaluated, it was seen that there was a significant increase in the risk of observing the horseshoe kidney anomaly in patients with retroaortic left renal vein (RALRV) variation. Conclusion: Vena cava variations are not rare. When present, they may coexist with other vena cava variations or other variations. These variations can easily be detected on CT. Properly characterizing and classifying IVC variations is crucial for proper planning of surgical interventions and transvenous interventions to prevent serious complications and failures.
Osteopetrosis is a clinical syndrome characterized by the failure of osteoclasts to resorb bone. Excessive bone density can interfere with vital tissues and structures, causing serious problems of the body. Hematopoietic insufficiency, disturbed tooth eruption, nerve entrapment syndromes, and growth impairment may develop in a patient with osteopetrosis. Herein, we present an adolescent girl diagnosed with non-infantile type of osteopetrosis with rare complications of the disease like mandibular osteomyelitis and portal hypertension (PHT) without liver cirrhosis. To our knowledge, this is the first pediatric case with osteopetrosis related PHT.
Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency disorder that is characterized by a triad of microthrombocytopenia, severe immunodeficiency, and eczema. We report the case of a 7-year-old male patient with chronic thrombocytopenia that was diagnosed as WAS after dilatation of the ascending aorta was noticed. WAS is rare, and it is a disease that requires high suspicion for diagnosis. We recommend periodic echocardiography and magnetic resonance imaging examinations to evaluate aortic aneurysms in children with WAS and that surgical intervention should not be delayed when aneurysm is detected.
Objective: The aim of this study was to investigate percentages and gender distribution of the variations of the inferior vena cava (IVC), renal veins, and posterior lumbar tributaries of the left renal vein (LRV). Materials and Methods: For this cross sectional observational study, the computed tomography (CT) images of 1949 patients were evaluated retrospectively. Results: In the present study, percentages of double IVC, left IVC, and IVC interruption with azygos continuation were 0.5%, 0.2%, and 0.1%, respectively; circumaortic left renal vein (CLRV) was 6% and retroaortic left renal vein (RLRV) was 4.2%. Multiple renal vein variations were 24.1% on the right, but none on the left. Posterior lumbar tributaries of the renal veins were 0.15% on the right and 48% on the left. There was no difference between genders in terms of LRV and IVC variations. While the incidence of multiple right renal veins was statistically significantly higher in male patients compared to female patients (p = 0.045), the opposite was true for the posterior lumbar tributaries of the LRV (p = 0.035). Conclusion: The venous system has a wide variety of variations, and the renal venous circulation is supported by number variations on the right in men and collaterals on the left in women.
OBJECTIVE: This study aimed to investigate and compare the ultrasonography and contrast-enhanced magnetic resonance imaging characteristics of incidentally detected hyperechoic focal liver lesions.METHODS: Seventy-four patients (29 males and 45 females) who had undergone a B-mode ultrasonography and contrast-enhanced magnetic resonance imaging examination were included in this study. A total of 91 hyperechoic lesions detected on ultrasonography were evaluated. The ultrasonography features of these hyperechoic lesions were recorded, and the results were compared with those acquired from contrast-enhanced magnetic resonance imaging. The results were compared statistically using the Shapiro-Wilk, McNemar, and Wilcoxon signed-rank tests. RESULTS:A corresponding lesion was found on contrast-enhanced magnetic resonance imaging in 72 of the 91 (79.1%) hyperechoic lesions detected on ultrasonography. Forty-one (56.9%) of the magnetic resonance imaging-defined lesions were typical hemangiomas, while 10 (13.9%) were focal steatosis areas and 4 (5.6%) were diagnosed with hepatocellular carcinoma. In contrast, 6 lesions (8.3%) were diagnosed as simple hepatic cysts, 4 (5.6%) as sclerosing hemangioma, 2 (2.8%) as thrombosed hemangioma, 1 (1.4%) as focal nodular hyperplasia, 1 (1.4%) as hamartoma, 2 (2.8%) as hydatid cysts, and 1 (1.4%) as hepatic lipoma. No statistically significant differences were found between ultrasonography and magnetic resonance imaging in terms of the segmental classification of the true positive lesions based on contour structures and lesion area measurements (p=0. 558, p=0.375, and p=0.636, respectively).CONCLUSIONS: Incidentally detected hyperechoic zones may not necessarily be detected on magnetic resonance imaging. This may be secondary to focal hepatic steatosis or false interpretation of the radiologist. Lesions requiring therapy must be considered in the differential diagnosis.
Amaç: Serebral kitlesel lezyonu olan hastaların optimum klinik yönetimi için doğru tanı esastır. Konvansiyonel MR morfolojiyi değerlendiren yüksek rezolüsyonlu bir tekniktir. Ancak kesin tanı vermede yetersiz kaldığı durumlarda doku biyokimyası hakkında bilgi veren MR spektroskopi gibi fonksiyonel tekniklere ihtiyaç ortaya çıkmıştır. Bu çalışmanın amacı serebral lezyonların ayırıcı tanısında MR spektroskopinin rolünü ve neoplastik-nonneoplastik lezyon ayrımında sensitivite, spesifisite ve doğruluğunu değerlendirmektir. Gereç ve Yöntem: 1,5 Tesla cihazda PRESS lokalizasyon metodunda Probe-P puls sekansı ile orta TE (TE=144) ile çalışıldı. Tanısı patoloji veya klinik-radyolojik takip ile kesinleşmiş 55 olgudan 37'sinde tek ve 18'inde multivoksel inceleme yapıldı. 46 olguda lezyona ve 9 olguda lezyon periferine yönelik inceleme gerçekleştirildi. Bulgular: Olguların 20'sinde kesin tanı tümöral ve 35'inde nontümöral patoloji idi. MRS serebral lezyonlarda tümörü saptamada % 100 sensitivite,% 91,7 spesifisiteye sahip bulundu. Pozitif prediktif değer % 86,4 ve negatif prediktif değer % 100 idi. Neoplastik-nonneoplastik lezyon ayrımında NAA/Cho, NAA/Cr, Cho/Cr ve Cho/NAA oranlarının değerlendirildiği bu çalışmada en kullanışlı olan Cho/NAA oranı olup Cho/Cr oranı da hassas bulundu. Sonuç: MR Spektroskopinin serebral lezyonlarda neoplastiknonneoplastik lezyon ayrımında güvenilir bir yöntem olduğu sonucuna varılmıştır. Elde edilen spektral verilerden tümörü seçmede en hassas olanı Cho/ NAA oranı olup kolin artışı tümör lehine önemli bir bulgudur.
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