Study Design Retrospective single institutional observational study. Purpose Segmental spinal dysgenesis (SSD), a complex spinal dysraphic state caused by notochord malformation disorders, is named after its morphological presentation where a spine segment is dysgenetic, malformed or absent. This study’s objective was to examine and reassess SSD imaging findings and correlate them with an embryological explanation. Overview of Literature Scott and his colleagues defined SSD as segmental agenesis or dysgenesis of the lumbar or thoracolumbar vertebrae and underlying spinal cord. Tortori-Donati and his colleagues defined it as a morphologic continuum ranging from hypoplasia to an absent spinal cord segment. Methods Fifteen children, whose imaging findings and clinical features were consistent with SSD, were included in the study. Magnetic resonance imaging (MRI) was performed per institutional spine protocol. Results Five children (33.3%) presented with a high-ending bulbous cord with no caudal segment, six (40%) presented with a dorsal or lumbar segmental dysgenetic cord with a low-lying, bulky caudal cord but without significant spinal canal narrowing, and four (26.6%) presented with segmental caudal dysgenesis with severe kyphoscoliosis, gibbus deformity, and spinal canal narrowing with a normal distal segment (normal or low-lying). Conclusions SSD is a complex spinal anomaly in children requiring clinical-radiological assessment followed by multidisciplinary management based on the extent and severity of the dysgenetic cord and the type of SSD. MRI plays a crucial role in both diagnosing and classifying SSD prior to surgical treatment to prevent further impairment.

BACKGROUNDThe causes of non-acute neurological illness presenting as developmental delay and regression of attained milestones are frequently unknown, and clinicians and families can be frustrated by the lack of neuroimaging correlation especially when considering therapeutic options and long-term prognosis.The goal of our study is to determine if proton MR spectroscopy can depict abnormalities in patients with non-acute neurological illness with special reference to children with developmental delay and regression of attained milestones. MATERIALS AND METHODSThis is a retrospective descriptive study, where imaging of 615 children with non-acute neurological illness were included. Among those, 424 children's MR spectroscopy whose preliminary MRI showed predominantly normal or non-specific findings were analysed by two radiologists. MR spectroscopy was performed as per institutional protocol with multivoxel grid placed in bilateral subcortical white matter in the frontal and parieto-occipital regions, bilateral thalami and basal ganglia. The diagnosis was confirmed with biochemical, pathological, genetic studies and enzyme analysis. RESULTSSpectra of 198 children showed no specific findings in MRS to arrive at a specific diagnosis. Spectra of 153 children showed mild decrease in NAA and mild decrease in NAA/Cr ratio, which indicates neuronal depletion and is non-specific with no biochemical/genetic abnormality and were diagnosed as idiopathic developmental delay. 58 children were significantly different with smaller NAA peaks and decreased NAA/ Cr ratios were diagnosed as Neuronal Ceroid Lipofuscinosis, 3 children with elevated lipid peak at 0.9 and 1.3 ppm were diagnosed as Sjogren-Larsson Syndrome, 3 children with absent creatine peak were diagnosed as Cerebral creatine deficiency, 3 children with decreased NAA peak and mild elevated Cho/ Cr ratio were diagnosed as GM1 gangliosidosis. One child presented with non-specific white matter hyperintensity in MRI and elevated peak at 2 ppm similar to that of NAA was diagnosed as Salla disease. One child with broad based lipid peak at 0.9 -1.3 ppm and was diagnosed as disorder of beta-oxidation of fatty acid. Two children showed single peak noted at 3.5 ppm denoting glycine and was diagnosed as hyperglycinaemia. Phenylalanine peaks were noted in two children at 7.36 ppm and were diagnosed as Phenylketonuria.

BACKGROUND ADC (Apparent diffusion coefficient) value of the diffusion weighted imaging is a measure of the haemodynamics of the organ. Portal hypertension (PH) is one of the essential manifestations of chronic liver disease. ADC was used in previous studies to indirectly assess fibrosis of the liver. However, no studies have evaluated the ADC value of the spleen to assess the PH features in cirrhosis. Aims and Objectives-We did the study to analyse the correlation of ADC value of the spleen in cirrhotic patients, to the severity of PH in the form of clinically significant PH (CSPH) and also the surrogate markers of PH. MATERIALS AND METHODS We prospectively evaluated patients with chronic liver disease in our hospital from the month of June 2017 to October 2017 with MRI of the abdomen. 1.5-tesla diffusion-weighted imaging with ADC mapping was performed with b value of 0, 300 and 500 and the ADC value of the spleen and liver were calculated. They were correlated with the severity of the PH in terms of CSPH and PH surrogate markers. RESULTS In chronic liver disease/ cirrhotic patients, we found a significant correlation between ADC value of the spleen and CSPH as well as PH surrogate markers in the form of grade of oesophageal varices and symptomatic hypersplenism. CONCLUSION Our study showed that ADC value of the spleen correlated well with the severity of PH. ADC measurements may allow for noninvasive evaluation of portal pressure and even in assessment of treatment response.