The rudimentary uterine buds associated with MRKH syndrome always maintained caudal relationship with ovary. Ovaries or rudimentary uterine buds can be ectopic and should be recognized with MRI before undergoing fertility treatment. Following MRI diagnosis, surgery allows patients to have sexual function with possible attainment of reproduction after assisted reproduction technique or surrogacy.
Objective:The aim of this study was to evaluate the magnetic resonance imaging (MRI) findings of caudal regression syndrome (CRS) and concomitant anomalies in pediatric patients.Materials and Methods:A hospital-based cross-sectional retrospective study was conducted. The study group comprised 21 pediatric patients presenting to the Departments of Radiodiagnosis and Pediatric Surgery in a tertiary care hospital from May 2011 to April 2016. All patients were initially evaluated clinically followed by MRI.Results:In our study, 21 pediatric patients were diagnosed with sacral agenesis/dysgenesis related to CRS. According to the Pang's classification, 2 (9.5%) patients were Type I, 5 (23.8%) patients were Type III, 7 (33.3%) patients were Type IV, and 7 (33.3%) patients were of Type V CRS. Clinically, 17 (81%) patients presented with urinary incontinence, 6 (28.6%) with fecal incontinence, 9 patients (42.9%) had poor gluteal musculatures and shallow intergluteal cleft, 7 (33.3%) patients had associated subcutaneous mass over spine, and 6 (28.6%) patients presented with distal leg muscle atrophy. MRI showed wedge-shaped conus termination in 5 (23.8%) patients and bulbous conus termination in 3 (14.3%) patients above the L1 vertebral level falling into Group 1 CRS while 7 (33.3%) patients had tethered cord and 6 (28.6%) patients had stretched conus falling into Group 2 CRS.Conclusion:MRI is the ideal modality for detailed evaluation of the status of the vertebra, spinal cord, intra- and extra-dural lesions and helps in early diagnosis, detailed preoperative MRI evaluation and assessing concomitant anomalies and guiding further management with early institution of treatment to maximize recovery.
BACKGROUND Methotrexate (MTX) is a chemotherapeutic agent commonly used in haematological malignancy. Its complication in childhood Acute Lymphoblastic Leukaemia (ALL) is well known to cause leukoencephalopathy. However, its complications in adults are uncommon. High doses of MTX is used to prevent CNS recurrence and haematological relapse. Diffusion weighted magnetic resonance imaging (DWI) plays an important role in early diagnosis. Aim of our study was to evaluate role of diffusion weighted MR imaging in Methotrexate-induced Leukoencephalopathy in adult acute lymphoblastic Leukaemia patients. METHODS A hospital based cross-sectional retrospective study was conducted. The study group comprised of 7 adult acute lymphoblastic leukaemia (ALL) patients presenting to the Departments of Radio-diagnosis, Medicine and Radiotherapy in a tertiary care hospital from April 2012 to May 2015. RESULTS In our study, 7 adult patients of acute lymphoblastic leukaemia (ALL), four patients (57.1%) developed MTX toxicity in interim maintenance phase and 3 patients (42.9%) developed in second maintenance phase of multidrug BFM-95 Regimen. Diffusion weighted images showed patchy multifocal restriction in 2 patients (28.6%) of acute MTX neurotoxicity and no diffusion restriction in rest 5 patients (71.4%). Acute MTX neurotoxicity in two patients (28.6%) had low apparent diffusion coefficient (ADC) value while 3 patients (42.9%) of chronic MTX neurotoxicity had high ADC value and 2 patients (28.6%) of subacute MTX neurotoxicity had normal ADC value. CONCLUSIONS Prompt recognition of MTX neurotoxicity with diffusion weighted imaging and ADC value are essential to institute early treatment to improve the outcome after MTX overdose in adult ALL patients.
Context:Ectopic neurohypophysis (EN) refers to an interrupted, nonvisualized, and thinned out pituitary stalk with ectopic location of the posterior pituitary gland. Concurrent extra-pituitary cerebral and extra-cranial anomalies have been rarely reported in patients of EN.Aim:The aim of this study was to evaluate the magnetic resonance imaging (MRI) findings of extra-pituitary cerebral anomalies in pediatric patients of EN.Settings and Design:A hospital-based cross-sectional study was conducted in a tertiary care center.Subjects and Methods:The study group comprised eight pediatric patients of EN associated with extra-pituitary cerebral or vascular anomalies. Clinical and biochemical assessment was done in all patients.Results:Out of the total eight patients with EN, MRI showed interrupted pituitary stalk in five patients (62.5%) and nonvisible pituitary stalk in three patients (37.5%). Ectopic posterior pituitary bright spot was demonstrated in median eminence in six patients (75%), faintly visualized in one patient (12.5%) and nonvisualized in another one patient. Statistical significant association was noted between pituitary gland height and patient's body height with the pituitary gland volume (P < 0.001). Varied extra-pituitary cerebral anomalies encountered in our patients ranged from isolated anomalies such as optic nerve hypoplasia in three patients (37.5%), corpus callosum dysplasia in four patients (50%), agyria-pachygyria complex in two patients (25%), and intracranial vascular anomalies in two patients to syndromic association of tuberous sclerosis in one patient.Conclusion:Identifying and reporting of associated extra-pituitary cerebral anomalies in patients with EN are crucial in assessing the overall neurological outcome of such patients.
BACKGROUND Renal replacement lipomatosis is an uncommon benign entity where abundance of fibrofatty tissue proliferation occurs in renal sinus with further extension of proliferated fatty tissues into renal hilum, perinephric and periureteric spaces. It is usually associated with renal atrophy and marked renal parenchymal destruction. Aim of our study was cross-sectional imaging evaluation of Renal Replacement Lipomatosis (RRL). METHODS A hospital based cross-sectional retrospective study was conducted. The study group comprised of 16 patients presenting to the Departments of Radio-diagnosis, Surgery and Urology in a tertiary care hospital from May 2014 to April 2016. All patients were initially evaluated clinically and ultrasonographically followed by cross-sectional imaging modality like Computed Tomography (CT), Magnetic Resonance Imaging (MRI) or both. RESULTS Out of 16 patients of renal replacement lipomatosis, 15 patients (93.8%) had associated renal pelvic or ureteric calculus while 1 patient (6.2%) had left para-aortic mass without associated calculus. Out of fifteen patients of calculus related RRL, 8 patients (53.3%) had calculus size more than 40 mm, followed by 4 patients (26.7%) who had size from 20 to 40 mm and 3 patients (20%) had size less than 20 mm. The mean CT HU value of calculus was 1334±84.5 in our study. Three patients (18.8%) had only renal hilar fatty excessive deposition, 1 patient (6.2%) had renal hilar and perinephric space fat depositions, 3 patients (18.8%) had renal hilar, perinephric and periureteric spaces depositions and 9 patients (56.2%) had renal hilar and periureteric excessive fatty depositions. Delayed renal functioning was noted in 9 patients (56%), followed by non-functioning in 5 patients (31.2%) and 2 patients (12.5%) had normally functioning kidneys. CONCLUSION Cross-sectional imaging like CT and MRI scan helps in diagnosing RRL and proper delineation of extensions of excessive fatty tissue proliferation.
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