Iniencephaly is an uncommon and fatal neural tube defect involving the occiput and inion, this occurs together with rachischisis of the cervical and thoracic spine, and retroflexion of the head. We report the ultrasound (US) and magnetic resonance (MR) imaging findings of a case of iniencephaly with clubfeet and arthrogryposis. The diagnosis of iniencephaly is easy to make on ultrasound due to the typical star-gazing fetus. However, the details of the fetal brain and spinal cord may not be adequately delineated on US. We found MR imaging to be superior for depicting central nervous system abnormalities. MR imaging has evolved as an imaging modality and it is complementary to fetal US, yet US remains the screening modality of choice.
A significant sustained fall in annual blood transfusion requirement and a rise in platelet counts occurred post-splenectomy. Increase in annual blood transfusion requirement should be investigated to find the cause.
Background & objectives:Early atherosclerosis and vascular complication have been described in thalassaemia patients. There is lack of data or guidelines regarding monitoring of vascular health in thalassaemia. This study was conducted to compare carotid artery structural and functional indices such as carotid artery intima-media thickness (CIMT), stiffness index (SI) and Young's elastic modulus (YEM) in β-thalassemia patients with age and sex matched controls, and to correlate these parameters with serum ferritin, cardiac iron, and hepatic iron.Methods:This cross-sectional study included 53 β-thalassaemia patients receiving regular blood transfusions. Carotid artery indices such as CIMT, SI, and YEM were calculated by duplex ultrasound and colour Doppler. Serum ferritin levels were measured by chemiluminescence. Cardiac and hepatic iron estimation were done using MRI T2* sequences analyzed by a special thalassaemia software.Results:Mean CIMT of cases and controls were 0.48 ± 0.04 and 0.44±0.02 mm, respectively and these were significantly different (P<0.001). Similarly significant differences were noted in SI and YEM of cases (2.45±0.79 and 96.12±34.85, respectively) as compared to controls (1.98±0.54 and 68.60±24.29, respectively) (P<0.001). There was significant inverse correlation between stiffness index and cardiac iron overload assessed by MRI cardiac T2* (P=0.03). Mean SI and YEM of cases were (2.1736 ± 0.2986 and 107.3± 41.6, respectively) significantly higher among non-splenectomized patients compared to splenectomized patients (2.0136 ± 0.263 and 86.9 ± 25.2, respectively) (P<0.05).Interpretation & conclusions:CIMT and arterial stiffness indices were significantly increased in β-thalassaemia patients compared to controls which was indicative of early atherogenic changes. This study supports the hypothesis that iron overload is a risk factor for early atherosclerosis and cardiovascular disease.
Ultrasound (US) is a simple, non-invasive imaging modality which allows high-resolution imaging of the musculoskeletal (MSK) system. Its increasing popularity in pediatrics is due to the fact that it does not involve radiation, has an ability to visualize non-ossified cartilaginous and vascular structures, allows dynamic imaging and quick contralateral comparison. US is the primary imaging modality in some pediatric MSK conditions like infant hip in developmental dysplasia (DDH), hip joint effusion, epiphyseal trauma and evaluation of the neonatal spine. US is the modality of choice in infants with DDH, both in the initial evaluation and post-treatment follow-up. US has a sensitivity equivalent to MRI in evaluation of the neonatal spine in experienced hands and is a good screening modality in neonates with suspected occult neural tube defects. In other MSK applications, it is often used for the initial diagnosis or in addition to other imaging modalities. In trauma and infections, US can often detect early and subtle soft tissue abnormalities and a quick comparison with the contralateral side aids in diagnoses. Dynamic imaging is crucial in evaluating congenital instabilities and dislocations, soft tissue and ligamentous injuries, epiphyseal injuries and fracture separations. High-resolution imaging along with color Doppler (CD) is useful in the characterization of soft tissue masses. This article reviews the applications of US in pediatric MSK with emphasis on conditions where it is a primary modality. Limitations of US include inability to penetrate bone, hence, limited diagnosis of intraosseous pathology and operator dependency.
We report a case of bilateral ovarian torsion in a prepubertal girl with normal ovaries that occurred at an interval of 1 year. The diagnosis was made on gray-scale and color Doppler sonography, and ovarian salvage was performed. The role of preventive contralateral ovarian pexy in cases of ovarian torsion is emphasized.
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