Clinical and magnetic resonance imaging spectrum of complex perianal fistulizing Crohn's disease: A cohort study from northern India.

BACKGROUND: Fistulizing perianal Crohn's disease (CD) is a debilitating condition associated with significant morbidity and reduction in the quality of life. Magnetic resonance imaging (MRI) of the pelvis is the preferred imaging modality for the comprehensive assessment of the perianal fistula. There is a paucity of data from India on the MRI spectrum of complex perianal fistula in CD. METHODS: A single-centre cross-sectional analysis of patients with fistulizing perianal CD, who underwent pelvic MRI between January 2020 and December 2021, was performed. The clinical (age, sex, disease duration, disease location and behavior, disease activity [Perianal Disease Activity Index, PDAI] and treatment received) and radiological (number and location of fistulae, extensions, number and location of internal and external openings, fistula activity, presence or absence of perianal abscess and associated proctitis) characteristics of complex perianal fistula (defined according to the American Gastroenterological Association classification) were recorded. RESULTS: Of total 175 patients with CD who attended the gastroenterology clinic during the study period, 27 (15.42%) (mean age 42±15.5 years, 62.96% females and median disease duration four years) had complex perianal fistula and were included in the analysis. The mean PDAI was 5.48±2.53. The median Van Assche Index was 17 (interquartile range [IQR] 13-19). A majority (96.29%) of the fistulae were trans-sphincteric and four (14.81%) fistulae extended into the supralevator space. All fistulae were active on MRI. Concomitant perianal abscess and proctitis were seen in 59.26% (n=16) and 62.96% (n=17) of patients, respectively. Combination therapy with biologics and antibiotics/immune-suppressants were the most commonly prescribed medical therapy. Six (22.22%) patients underwent combined medical and surgical (non-cutting seton, fistulectomy, fecal diversion) treatment. CONCLUSION: The cumulative risk of the development of fistulizing perianal CD in a northern Indian cohort was similar to the western populations. Complex perianal fistulae were predominantly trans-sphincteric and commoner in females. MRI evaluation is pivotal for the delineation of fistula anatomy, assessment of disease extent and activity and the evaluation of concomitant perianal abscess and other complications.

Fibrolamellar hepatocellular carcinoma presenting as cholestatic jaundice: An unusual presentation of a rare disease.

Fibrolamellar hepatocellular carcinoma is a rare primary hepatic tumor that usually occurs in youth. The common presenting features are vague abdominal pain, nausea, vomiting and weight loss. We present a case report of a young male who presented with cholestatic jaundice and on evaluation was diagnosed to have fibrolamellar hepatocellular carcinoma. He underwent successful surgical resection of the tumor. In young individuals presenting with unexplained cholestasis, fibrolamellar hepatocellular carcinoma should be considered.

Evaluation of Nutritional Characteristics Reveals Similar Prevalence of Malnutrition in Patients with Ulcerative Colitis and Crohn's Disease.

BACKGROUND: Inflammatory bowel disease (IBD) is associated with an increased risk of malnutrition and sarcopenia. AIMS: To evaluate the nutritional status of patients with IBD and determine the threshold values of different parameters of nutritional assessment to identify malnutrition. METHODS: This was a single-centre cross-sectional analysis of adult patients with IBD [ulcerative colitis (UC) and Crohn's disease (CD)] who underwent anthropometry [body mass index (BMI), mid upper arm circumference (MUAC) and triceps-fold thickness (TSF)], body composition analysis and assessment for sarcopenia [hand-grip strength and skeletal muscle index (SMI) at L3 vertebral level)]. Age- and gender-matched healthy adults served as controls. Malnutrition was defined according to the European Society of Clinical Nutrition and Metabolism (ESPEN) criteria. RESULTS: A total of 406 patients [336 (82.76%) UC and 70 (17.24%) CD; mean age 40.56 ± 13.67 years; 215 (52.95%) males] with IBD and 100 healthy controls (mean age 38.69 ± 10.90 years; 56 (56%) males) were enrolled. The mean BMI, MUAC, TSF thickness, fat and lean mass, hand-grip strength, and SMI at L3 vertebral level were lower in patients with IBD compared to controls. The prevalence of malnutrition was similar in UC and CD [24.40% (n = 82) and 28.57% (n = 20), respectively (p = 0.46)]. Thresholds for fat mass in females (15.8 kg) and visceral fat index in males (0.26) were both sensitive and specific to detect malnutrition. The cutoff values of MUAC and TSF thickness to identify malnutrition were 23.25 cm and 25.25 cm, and 16.50 mm and 8.50 mm, in females and males, respectively. CONCLUSION: Malnutrition and sarcopenia were common in patients with IBD, with the prevalence being similar in patients with both UC and CD.

Liposarcoma of tongue: A case report.

ABSTRACT: Liposarcoma (LS) is one of the most common malignant tumors. However, oral LS is an extremely rare lesion that is often clinically misdiagnosed as a benign lesion because of its asymptomatic and indolent clinical course. In oral cavity, the tongue is the most frequent site for its occurrence.

Spectrum of magnetic resonance abnormalities in leigh syndrome with emphasis on correlation of diffusion-weighted imaging findings with clinical presentation.

Background: Leigh syndrome (LS) is a progressive neurodegenerative disorder of infancy/early childhood secondary to mitochondrial dysfunction. Imaging plays a pivotal role in the diagnosis of LS with certain typical magnetic resonance imaging (MRI) findings considered as a part of diagnostic criteria. We appraised various MRI findings on conventional MRI sequences and also assessed potential correlation between diffusion abnormalities and patient's clinical presentation. Aims: Our aim was to describe various patterns of central nervous system involvement in LS and to assess the correlation of diffusion-weighted imaging abnormalities with clinical presentation. Settings and Design: The design of the study was retrospective comprising 8 children with LS who had MRI between years 2014 and 2019. Subjects and Methods: Eight children between the age group of 4 months 8 years with LS based on clinical presentation, elevated lactate levels in CSF/Blood, and typical MRI findings were included in the study. Results and Conclusions: Brainstem was involved all (100%) patients while basal ganglia was affected in 5 (62.5%) children. Cerebral white matter involvement was present in 3 (37.5%) children, cerebellar in 2 (25%) children while spinal, corpus callosum, and thalamic involvement were observed in one (12.5%) patient each. Diffusion restriction was observed in 6 children, all of them presented with altered sensorium. Conventional MRI serves as an excellent tool for the diagnosis of LS in children with clinical suspicion. Acute encephalopathy frequently presents with diffusion restriction corresponding to active lesions. Hence, diffusion restriction on MRI predicts the activity of lesions in patients with LS.

Résumé Contexte: Le syndrome de Leigh (LS) est une maladie neurodégénérative progressive de la petite enfance/petite enfance secondaire à des maladies mitochondriales. dysfonctionnement. L'imagerie joue un rôle central dans le diagnostic du LS, certains résultats typiques de l'imagerie par résonance magnétique (IRM) étant considérés comme une partie des critères diagnostiques. Nous avons évalué divers résultats d'IRM sur des séquences d'IRM conventionnelles et également évalué la corrélation potentielle entre anomalies de diffusion et présentation clinique du patient. Objectifs: Notre objectif était de décrire divers modèles d'atteinte du système nerveux central dans le LS et pour évaluer la corrélation des anomalies d'imagerie pondérées en diffusion avec la présentation clinique. Paramètres et conception: la conception de l'étude était rétrospective comprenant 8 enfants atteints de LS qui ont eu une IRM entre les années 2014 et 2019. Sujets et méthodes: Huit enfants entre le groupe d'âge de 4 mois 8 ans avec LS basé sur la présentation clinique, les niveaux élevés de lactate dans le LCR/le sang, et typique Les résultats de l'IRM ont été inclus dans l'étude. Résultats et conclusions : Le tronc cérébral a été impliqué chez tous les patients (100 %) tandis que les ganglions de la base ont été impliqués. touché chez 5 (62,5%) enfants. Une atteinte cérébrale de la substance blanche était présente chez 3 (37,5%) enfants, cérébelleuse chez 2 (25%) enfants alors que Une atteinte de la colonne vertébrale, du corps calleux et du thalamus a été observée chez un patient (12,5 %) chacun. Une restriction de diffusion a été observée chez 6 enfants, tous présentaient un sensorium altéré. L'IRM conventionnelle est un excellent outil pour le diagnostic du LS chez les enfants présentant des soupçon. L'encéphalopathie aiguë se présente fréquemment avec une restriction de diffusion correspondant à des lésions actives. Par conséquent, la restriction de diffusion sur L'IRM prédit l'activité des lésions chez les patients atteints du LS. Mots-clés : ganglions de la base, tronc cérébral, imagerie pondérée en diffusion, hypotonie, lactate sérique.

Correlation between magnetic resonance enterography and ileo-colonoscopy for assessment of disease activity in terminal ileal Crohn's disease.

BACKGROUND: Magnetic resonance enterography (MRE) has emerged as a novel tool for the assessment of disease activity in Crohn's disease (CD). Real world data from Indian subcontinent on performance of MRE in terminal ileal CD are lacking. METHODS: Retrospective analysis of patients with terminal ileal CD who underwent both ileo-colonoscopy and MRE was performed. Ileo-colonoscopy was considered the gold standard for assessment of disease activity. On ileo-colonoscopy, a simple endoscopic score for Crohn's disease (SES-CD) ≥2 was considered active disease; presence of ulcers indicated severe disease. MRE scoring of the disease activity was performed using magnetic resonance index of activity (MARIA) and simplified MARIA (MARIAs). The measure of agreement between ileo-colonoscopy and MRE and comparison of MARIA and MARIAs for assessment of disease activity and sensitivity of MRE to detect mucosal ulcerations were calculated. RESULTS: Seventy patients with terminal ileal CD (mean age 40.74±15.56 years; 71.4% males [n=50]) were evaluated. The sensitivities of MARIA and MARIAs scores to detect active disease were 0.76 and 0.84, respectively. The area under the receiver operating characteristic curve (AUROC) for detecting severe disease was 0.836 (p<0.0001) for MARIA and 0.861 (p<0.0001) for MARIAs. For mild active disease, there was no agreement between SES-CD and MARIA or MARIAs; however, for severe disease, the agreement was fair and moderate for MARIA and MARIAs, respectively. MARIA and MARIAs were comparable for identification of active and severe disease (κ 0.759, p<0.0001 and κ 0.840, p<0.0001, respectively). MRE was 68.18% sensitive to detect mucosal ulcers. CONCLUSION: MRE is a reliable and sensitive tool for detection of endoscopically severe, but not mild, terminal ileal CD.

Case 300: Ruptured Mucinous Cystadenoma of the Pancreas.

History Part one of this case appeared 4 months previously and may contain larger images. A 40-year-old woman presented to the outpatient department of our hospital with a 2-year history of abdominal pain, which had worsened in the past few days. There were no other constitutional symptoms; in particular, there was no weight loss or loss of appetite. The serum amylase and serum lipase levels were always within normal limits, even during the current episode. There was no history of pancreatitis, alcohol abuse, drug use, or trauma. The patient was evaluated at an outside clinic 2 years ago and underwent imaging, including US and dedicated pancreatic CT, which revealed a complex multiseptated noncalcified pancreatic cystic lesion. After CT, the patient underwent endoscopic US, which showed the lesion was suspected to be an infected parasitic cyst; fine-needle aspiration and cytology were not attempted due to the risk of iatrogenic rupture. The patient was advised to undergo surgical resection but declined the procedure and was lost to follow-up for 2 years before her current visit to our facility. On clinical examination, there were no clinically important findings, aside from mild to moderate pain on deep palpation and some shifting dullness that was suggestive of ascites. The patient always remained hemodynamically stable. US-guided ascitic fluid analysis was performed, which revealed amylase levels of 869 IU/L (normal range, 4-234 IU/L). Considering prior CT and endoscopic US reports, the patient was further evaluated with a pancreatic MRI protocol for lesion characterization and to explain the new-onset abdominal pain.

Diagnostic Accuracy of Calculated Tumor Volumes and Apparent Diffusion Coefficient Values in Predicting Endometrial Cancer Grade.

Background: Magnetic resonance imaging (MRI) has been shown to be an accurate imaging technique for the preoperative assessment of local staging of endometrial cancer and for evaluating the depth of myometrial invasion. Materials and Methods: This was a single-center retrospective study performed on patients with histopathologically proven endometrial carcinoma who underwent an MRI examination of the pelvis between October 2017 and May 2020. Results: In the present analysis, mean apparent diffusion coefficient (ADC) values for each histologic grade were 0.72 ± 0.13 × 10-3 mm2/s (G1), 0.76 ± 0.17 × 10-3 mm2/s (G2), and 0.74 ± 0.12 × 10-3 mm2/s (G3), respectively, showing no significant correlation between ADC values and tumor grade (P = 0.73). Overall, ADC minimum was significant in differentiating grades of endometrial carcinoma (P = 0.02) with the ability to differentiate Grade I and II lesions (P = 0.01). A mean tumor volume of 25.2 cc could differentiate low-grade tumors (Grade I and Grade II) from high-grade tumors (Grade III) with a sensitivity and specificity of 88% and specificity of 89%. The tumor volume/uterine volume ratio (TV/UV) differentiates high-grade tumors from low-grade tumors (P < 0.001), however, no significant difference in the ratio was observed among Grade I and II lesions (P = 0.48). The area under the curve of tumor volume was 0.875 (95% confidence interval 0.0-1.00) (P = 0.001), indicating that tumor volume was an effective tool for distinguishing high-grade and low-grade endometrioid adenocarcinomas. The corresponding sensitivity and specificity were 88.0% and 89.0%, respectively. Conclusion: Preoperative noninvasive radiological assessment for tumor volume, TV/ UV or tumor volume/uterine volume is important surrogate markers for preoperative prognostication of endometrial carcinoma.

Clinical profile and outcomes of opioid abuse gastroenteropathy: an underdiagnosed disease entity.

BACKGROUND/AIMS: Opioid-induced bowel dysfunction includes nausea, vomiting, constipation and abdominal distension. We describe patients presenting with gastrointestinal (GI) ulcers and ulcerated strictures secondary to opioid abuse, an entity not well described in literature. METHODS: This retrospective observational study included patients with opioid abuse gastroenteropathy presenting to Dayanand Medical College and Hospital, Ludhiana, India between January 2013 and December 2018. Opioid abuse gastroenteropathy was defined as gastric or small bowel ulcers and ulcerated strictures in patients abusing opioids, where all other possible etiologies of GI ulcers/strictures were excluded. Clinical, biochemical, endoscopic, radiological and histological parameters as well as response to treatment were assessed. RESULTS: During the study period, 20 patients (mean age, 38.5±14.2 years; 100% males) were diagnosed to have opioid induced GI ulcers and/or ulcerated strictures. The mean duration of opioid consumption was 6.2±3.4 years. The mean duration of symptoms at presentation was 222.1±392.3 days. Thirteen patients (65%) had gastroduodenal involvement, 6 (30%) had a jejunoileal disease and 1 (5%) had an ileocecal stricture. Two patients (10%) presented with upper GI bleeding, 11 (55%) had features of gastric outlet obstruction and 7 (35%) presented with small bowel obstruction. Abdominal pain and iron deficiency anemia were the most common presentations. Only 1 patient (5%) responded to proton pump inhibitors, 3 (15%) had a lasting response to endoscopic balloon dilatation, while all other (80%) required surgical intervention. CONCLUSIONS: Opioid abuse gastroenteropathy presents as ulcers and ulcerated strictures which respond poorly to medical management and endoscopic balloon dilatation. A majority of these cases need surgical intervention.

Clinical spectrum and outcome of pulmonary nocardiosis: 5-year experience.

BACKGROUND: Pulmonary nocardiosis is a rare but a life-threatening infection caused by Nocardia spp. The diagnosis is often missed and delayed resulting in delay in appropriate treatment and thus higher mortality. AIM: In this study, we aim to evaluate the clinical spectrum and outcome of patients with pulmonary nocardiosis. METHODS: A retrospective, 5-year (2009-2014) review of demographic profile, risk factors, clinical manifestations, imaging findings, treatment, and outcome of patients with pulmonary nocardiosis admitted to a tertiary care hospital. RESULTS: The median age of the study subjects was 54 years (range, 16-76) and majority of them (75%) were males. The risk factors for pulmonary nocardiosis identified in our study were long-term steroid use (55.6%), chronic lung disease (52.8%), diabetes (27.8%), and solid-organ transplantation (22.2%). All the patients were symptomatic, and the most common symptoms were cough (91.7%), fever (78%), and expectoration (72%). Almost two-third of the patients were initially misdiagnosed and the alternative diagnosis included pulmonary tuberculosis (n = 7), community-acquired pneumonia (n = 5), lung abscess (n = 4), invasive fungal infection (n = 3), lung cancer (n = 2), and Wegener's granulomatosis (n = 2). The most common radiographic features were consolidation (77.8%) and nodules (56%). The mortality rate for indoor patients was 33% despite treatment. Higher mortality rate was observed among those who had brain abscess (100.0%), HIV positivity (100%), need for mechanical ventilation (87.5%), solid-organ transplantation (50%), and elderly (age > 60 years) patients (43%). CONCLUSION: The diagnosis of pulmonary nocardiosis is often missed and delayed resulting in delay in appropriate treatment and thus high mortality. A lower threshold for diagnosing pulmonary nocardiosis needs to be exercised, in chest symptomatic patients with underlying chronic lung diseases or systemic immunosuppression, for the early diagnosis, and treatment of this uncommon but potentially lethal disease. Despite treatment mortality remains high, especially in those with brain abscess, HIV positivity, need for mechanical ventilation, solid-organ transplantation, and elderly.

Low Dose MDCT with Tube Current Modulation: Role in Detection of Urolithiasis and Patient Effective Dose Reduction.

INTRODUCTION: Urolithiasis is one of the major, recurring problem in young individuals and CT being the commonest diagnostic modality used. In order to reduce the radiation dose to the patient who are young and as stone formation is a recurring process; one of the simplest way would be, low dose CT along with tube current modulation. AIM: Aim of this study was to compare the sensitivity and specificity of low dose (70mAs) with standard dose (250mAs) protocol in detecting urolithiasis and to define the tube current and mean effective patient dose by these protocols. MATERIALS AND METHODS: A prospective study was conducted in 200 patients over a period of 2 years with acute flank pain presentation. CT was performed in 100 cases with standard dose and another 100 with low dose protocol using tube current modulation. Sensitivity and specificity for calculus detection, percentage reduction of dose and tube current with low dose protocol was calculated. RESULTS: Urolithiasis was detected in 138 patients, 67 were examined by high dose and 71 were by low dose protocol. Sensitivity and Specificity of low dose protocol was 97.1% and 96.4% with similar results found in high BMI patients. Tube current modulation resulted in reduction of effective tube current by 12.17%. The mean effective patient dose for standard dose was 10.33 mSv whereas 2.92 mSv for low dose with 51.13-53.8% reduction in low dose protocol. CONCLUSION: The study has reinforced that low-dose CT with tube current modulation is appropriate for diagnosis of urolithiasis with significant reduction in tube current and patient effective dose.

Paediatric brainstem: A comprehensive review of pathologies on MR imaging.

The brainstem is a midline structure formed by the midbrain, pons and medulla and is a home for various vital neurological centres of the human body. A diverse spectrum of disease entities can involve the brainstem, which includes infections, metabolic disorders, demyelination, vascular conditions, neurodegenerative disorders and tumours. Brainstem involvement can be primary or secondary, i.e., as part of systemic disorders. Due to the overlapping clinical presentation and symptomatology, imaging plays a decisive role in the detection, localisation and characterisation of brainstem pathologies. Magnetic resonance imaging (MRI) is the modality of choice and the use of advanced MR techniques such as diffusion-weighted imaging and spectroscopy can be especially helpful in providing a tenable diagnoses. This article is a compilation of the MR imaging manifestations of a spectrum of common and uncommon brainstem pathologies that can be encountered in the paediatric age group. Teaching Points • The paediatric brainstem can be afflicted by many pathologies that may overlap clinico-radiologically. • MRI is the best modality for the localisation and diagnosis of brainstem pathologies. • Diffusion-weighted imaging is useful in the diagnosis of vascular and metabolic disorders. • Occasionally, demyelination and neoplasms can be indistinguishable on imaging.

Hepatic tuberculosis: a multimodality imaging review.

OBJECTIVES: We aim to illustrate the multimodal imaging spectrum of hepatic involvement in tuberculosis (TB). Whilst disseminated tuberculosis on imaging typically manifests as multiple small nodular lesions scattered in the liver parenchyma, isolated hepatic tuberculosis remains a rare and intriguing entity. METHODS: Indubitably, imaging is the mainstay for detection of tubercular hepatic lesions which display a broad spectrum of imaging manifestations on different modalities. While sonography and computed tomography (CT) findings have been described in some detail, there is a paucity of literature on magnetic resonance imaging (MRI) features. Due to a significant overlap with other commoner and similar appearing hepatic lesions, hepatic tuberculosis is often either misdiagnosed or labelled as indeterminate lesions. This article is a compendium of cases highlighting the spectrum of imaging patterns that can be encountered in patients with isolated primary hepatic tuberculosis as well as disseminated (secondary) disease. Rare patterns of primary disease such as tubercular cholangitis, hypervascular liver masses, and those with vascular complications are also illustrated and discussed. CONCLUSIONS: Imaging plays a valuable role in the detection of tubercular hepatic lesions. Also, imaging can be helpful in their characterisation and for assessing associated complications. TEACHING POINTS: • Hepatic TB has myriad imaging manifestations and is often confounded with neoplastic lesions. • Imaging patterns include miliary TB, macronodular TB, serohepatic TB and tubercular cholangitis. • Concurrent splenic, nodal or pulmonary involvements are helpful pointers towards the diagnosis. • Miliary calcifications along the bile ducts are characteristic of tubercular cholangitis. • Histological/microbiological confirmation is often necessary to confirm the diagnosis.

Telltale signs of peripheral neurogenic tumors on magnetic resonance imaging.

Peripheral nerve sheath tumors are categorized into benign and malignant forms, comprising of neurofibroma and schwannoma in the benign category and malignant peripheral nerve sheath tumors in the malignant category. Magnetic resonance imaging plays an important role in the diagnosis of these lesions. The various imaging features and signs that help to identify and characterize a nerve sheath tumor are, distribution of the tumor along a major nerve, an entering or exiting nerve sign, target sign, a fascicular sign and a split-fat sign.

Acute Marchiafava-Bignami disease: clinical and serial MRI correlation.

Marchiafava-Bignami disease (MBD) is a form of toxic demyelinating disease more often seen in chronic alcoholics. The disease process typically involves the corpus callosum and clinically often presents with altered sensorium, neurocognitive defects or seizures with acute cases often deteriorating to comatose state. The death rate is high. We report a rare case of MBD with complete clinical recovery. A 50-year-old male patient presented in an unconscious state and underwent MRI of the brain which showed significant lesions involving the corpus callosum. Following treatment with thiamine and supportive therapy, he improved clinically and a follow-up MRI revealed significant resolution of the earlier lesions. Diffusion-weighted MRI showed the changes more conspicuously as compared with conventional imaging. The clinical resolution corresponded well with the MRI pattern. The case highlights that diffusion-weighted MRI is an extremely useful tool in evaluation and prognostication of MBD.

Quantitative and qualitative bowel analysis using mannitol, water and iodine-based endoluminal contrast agent on 64-row detector CT.

OBJECTIVE: To assess the performance of mannitol as a luminal contrast as compared to water and positive contrast in evaluation of bowel on multidetector computed tomography (MDCT). MATERIALS AND METHODS: Three hundred patients were randomly selected for this study and were divided equally into three groups. Each subject received 1500 ml of oral contrast. Group 1 received 3% mannitol in water, group 2 received diluted iodinated positive contrast, and group 3 received plain water without additives. Qualitative and quantitative analysis for distension, fold visibility, and overall image quality were analyzed by actual diameter measurement and point scale system at different bowel levels. One-way analysis of variance (ANOVA) followed by Tukey's HSD Post-hoc test and Pearson's Chi-square (exact test) test were applied. RESULTS: Group 1 showed better results for small bowel distension, intraluminal homogeneity, and visibility of mucosal folds on quantitative and qualitative analysis with statistically significant P value (P<0.001). The ileo-caecal junction distension and mural feature visibility was better with mannitol (P < 0.001). No significant difference in distension of stomach and duodenum was found between the three groups. CONCLUSION: Mannitol as endoluminal contrast increases the diagnostic accuracy of the investigative studies in comparison to water and iodine-based contrast by producing significantly better bowel distension and visibility of mural features with improved image quality without additional adverse effects.