Correlation of diaphragmatic mobility and thickening assessed by lung ultrasound with severity of interstitial lung disease.

BACKGROUND: Studies conducted in interstitial lung disease (ILD) patients to assess diaphragmatic excursion and thickening fraction suggest a weak to strong correlation with pulmonary function parameters. However, diaphragmatic excursion velocity, a novel imaging marker, has not been correlated with pulmonary function and high-resolution computed tomography (HRCT) fibrosis score in ILD patients previously. METHODS: We conducted a cross-sectional analytical study in 40 ILD patients during quiet (QB) and deep breathing (DB) to measure diaphragmatic thickening, excursion and excursion velocity using transthoracic ultrasound and correlated them with pulmonary function parameters and HRCT fibrosis score. RESULTS: Most diaphragm parameters in DB correlated more strongly with lung function parameters compared to quiet breathing. Right diaphragmatic excursion, during QB and DB, showed positive correlations with forced vital capacity (FVC) z-score (r = 0.591, 0.676) and diffusion capacity of the lung for carbon monoxide (DLCO) z-score (r = 0.437, 0.438), and negative correlations with HRCT fibrosis score (r = -0.439, -0.425), respectively. In addition, right diaphragmatic velocity exhibited positive correlations with FVC z-score (r = 0.388, 0.667) and DLCOz-score (r = 0.139, 0.412), and negative correlations with HRCT fibrosis score (r = -0.454, -0.445). Right diaphragm thickening fraction showed positive correlations with FVC z-score (r = 0.330, 0.460) and DLCOz-score (r = 0.400, 0.426), and negative correlations with HRCT fibrosis score (r = -0.199, -0.237). Similarly, right diaphragmatic thickness indicated positive correlations with FVC z-score (r = 0.526, 0.614) and DLCOz-score (r = 0.298, 0.298), and negative correlations with HRCT fibrosis score (r = -0.398, -0.401). CONCLUSION: Diaphragmatic excursion velocity during DB showed a weak to moderate correlation with pulmonary function parameters and HRCT fibrosis score and may be utilized as a surrogate marker in ILD patients unable to perform pulmonary function tests or undergo sequential HRCT thorax in follow-up.

Association Between Clinical Severity, Neuroimaging, and Electroencephalographic Findings in Children with Subacute Sclerosing Panencephalitis.

BACKGROUND: Children diagnosed with subacute sclerosing panencephalitis (SSPE) display a range of neuroimaging abnormalities during different stages of the disease, but their exact clinical significance remains unclear. METHODS: In this retrospective cohort study, our objective was to examine magnetic resonance imaging (MRI) abnormalities in the brains of patients aged 18 years or younger with subacute sclerosing panencephalitis. We aimed to correlate these MRI abnormalities with clinical severity, sociodemographic variables, electroencephalographic (EEG) abnormalities, and cerebrospinal anti-measles antibody titers. RESULTS: The study included 112 cases of subacute sclerosing panencephalitis (mean age at onset: 8.9 ± 2.6 years). MRI analysis at the time of presentation revealed the following abnormalities: subcortical white matter signal changes (n = 95), periventricular white matter signal changes (n = 76), splenium of corpus callosum involvement (n = 39), diffuse corpus callosum involvement (n = 27), cerebral atrophy (n = 35), basal ganglia involvement (n = 10), and brain stem involvement (n = 2). Notably, subcortical white matter involvement, periventricular white matter involvement, diffuse corpus callosum involvement, and basal ganglia involvement were more prevalent in patients with stage III and IV subacute sclerosing panencephalitis (P < .05 for all). Cerebral atrophy was also significantly more common in patients with stage III compared to those with stage IV subacute sclerosing panencephalitis (P < .0001). However, no substantial positive or negative associations were found between MRI findings and EEG abnormalities, other sociodemographic/clinical variables, and cerebrospinal fluid measles-specific antibody titers (P > .05). CONCLUSION: Early in the disease progression of subacute sclerosing panencephalitis, the temporoparietal and parietooccipital regions of the subcortical white matter are affected. Neuroimaging abnormalities exhibit a stronger association with Jabbour's clinical staging, but do not show significant associations with other clinical, sociodemographic, and EEG features.

Spondylocostal Dysostosis with Anal Atresia and Urogenital Anomalies in a Young Infant: First Case Entity of Casamassima-Morton-Nance Syndrome from Asia.

Casamassima-Morton-Nance syndrome (CMNS) includes a heterogeneous group of spondylocostal dysostosis along with anal atresia and genitourinary abnormalities. In 1981, Casamassima et al first described the syndrome in a fetus, and since then, only seven such cases have been reported so far. CMNS phenotype shows a significant clinical variability as documented in the reported cases. Etiology remains unknown yet, and it carries a poor prognosis. Here, we reported on a young female infant born out of nonconsanguineous marriage with normal karyotype and spondylocostal dysostosis, anal and genitourinary malformations suggesting CMNS. Ours is the eighth, and first case entity of CMNS reported from Asia as per the literature search. In our case, the additional feature of bilateral clubfoot has not been documented earlier in the literature. It extends the clinical spectrum of the syndrome and prompts us to consider it a close differential diagnosis to VACTERL (vertebral defects, anal atresia, cardiac malformations, tracheoesophageal fistula/esophageal atresia, renal anomalies, limb abnormalities) syndrome, which is commonly known and diagnosed. It also raises the question of whether cases of CMNS are being misdiagnosed as VACTERL syndrome due to its rarity.

Assessment of Carotid Plaque Enhancement on Contrast-Enhanced Ultrasound as a Predictor for Severe Coronary Artery Disease.

Background and Aim Contrast-enhanced ultrasound (CEUS) can reliably identify vulnerable plaques. As atherosclerosis is a systemic disease, we evaluated whether contrast enhancement of carotid plaque (CECP) can predict severe coronary artery disease (CAD) by comparing CECP in patients who have had acute coronary syndromes (ACS) recently with asymptomatic individuals. Settings and Design This case-control study was done at a tertiary care center during 2022. Materials and Methods Fourteen participants were recruited in each group, after screening in-patients for carotid plaques and inclusion and exclusion criteria. Those who had history of ACS were enrolled as cases, while those who did not were enrolled as controls. All these patients underwent grayscale, Doppler, and CEUS examination for characterization of the carotid plaque. For cases, findings on CEUS were also compared with the severity of CAD on catheter coronary angiography. Statistical Analysis Diagnostic parameters including sensitivity, specificity, and diagnostic accuracy were calculated and proportions were compared by using Fisher's exact test. Results Eight out of 28 patients showed CECP. CECP and CAD were positively associated with p -Value of 0.033. Eighty-three percent patients with triple vessel disease and 50% patients with double vessel disease on coronary angiography showed CECP. Sensitivity and specificity of CECP for prediction of CAD were 50 and 92.9%, respectively. Conclusion CECP on CEUS can predict CAD and is a more reliable indicator of severe CAD than plaque characteristics on grayscale and Doppler imaging; making it useful for screening of patients at risk of having CAD.

Benign paediatric liver tumours: The radiological maze demystified.

The trajectory from the clinical identification of a benign liver mass to establishing a precise diagnosis is perplexing and arduous. Related contributory factors are that such lesions are encountered infrequently and that there is a nonavailability of dedicated paediatric radiologists in the developing world. The objective of this study was to review the spectrum of benign liver lesions in children and their typical imaging features. Cross-sectional imaging of all paediatric patients (< 18 years) with liver lesions (single and multiple) performed in the institute from 01 January 2018 to 01 January 2019 as well as those acquired at outside institutions and referred to the institute for management was included. Ultrasound was done as the first line of investigation in all the cases with suspicious liver masses and retrospectively performed in referral cases in whom CT or MRI was already done. Images were analysed by two senior radiologists. Most of the cases were diagnosed based on clinical, biochemical and imaging findings, and biopsy was only performed in equivocal cases. Most of the benign liver lesions in the paediatric age group were hepatic haemangioma and mesenchymal hamartomas. A simplified clinical-radiologic paradigm should be established for benign liver lesions in children to assist in reaching the correct diagnosis. Contribution: The article demonstrates the salient radiological findings of various benign liver lesions in the paediatric age group and the role of demographic, clinical and biochemical findings, which plays a substantial role in the diagnosis and avoids unnecessary biopsies.

Revisiting Rosai Dorfman disease: A rare histiocytic disorder with nodal and extranodal involvement.

Rosai Dorfman disease is a rare but benign and self-limiting disorder. A case of Rosai Dorfman disease in a 16-year-old female with classic radiological and pathological findings is presented. T2W hypointense signal of the soft tissue masses was a distinguishing MRI feature. Contribution: Rosai Dorfman disease is often overlooked in the differentials of lymphadenopathy and soft tissue masses on account of its rarity. This case report comprehensively discusses the imaging approach to this rare disorder.

Development and validation of a predictive model assessing the risk of seizure recurrence in children with neurocysticercosis.

INTRODUCTION: Neurocysticercosis (NCC) is a significant factor contributing to the incidence of seizures in developing countries. While numerous studies have investigated the recurrence of seizures in NCC, their reliability is often limited. METHODS: We assessed the socio-demographic, clinical, and neuroimaging details of 161 children with seizures caused by NCC. We monitored them for seizure recurrence over a 6-month follow-up period. We divided the children into two groups: those with seizure recurrence and those without. Subsequently, we identified predictive factors associated with seizure recurrence through univariate analysis, followed by multivariate binary logistic regression. We evaluated the prognostic model for discrimination and calibration and then internally validated it using the bootstrap method. RESULTS: A total of 23 children experienced breakthrough seizures. In multivariate analysis, the presence of epileptiform abnormalities in electroencephalogram (EEG), more than 5 NCC lesions, the presence of perilesional edema greater than 2 cm in maximum dimension, and a cluster of seizures before presentation were significantly associated with seizure recurrence (p < 0.05). These factors were included in the final NEPC (Number of NCC lesions, Epileptiform EEG abnormality, Perilesional edema, and Cluster of seizures) score. The final model exhibited good discrimination (AUC = 89.1 %; 95 % CI=80.5-95.3 %, p < 0.001) and calibration (p = 0.54). A score of 4 appeared to be the optimal threshold for discriminating between individuals with and without seizure recurrence, with sensitivity and specificity values of 85 % and 87 %, respectively. Interrater reliability was very strong between two pediatric neurologists and strong between a pediatric neurologist and a pediatric neurology trainee resident (k = 0.92 and 0.86, respectively). CONCLUSION: The NEPC score demonstrates good sensitivity and specificity in predicting seizure recurrence in pediatric children with NCC.

Utility of MR lymphangiography in chylous ascites: A report of two cases.

Dynamic contrast-enhanced (DCE) lymphangiography is a novel imaging technique with a potential role in suspected cases of lymphatic leaks. A 15-year-old male with a post operative chylous leak and an 8-year-old male who developed chylous ascites secondary to disseminated tuberculosis are presented. Both children underwent MR lymphangiography. Contribution: The role of DCE-MR lymphangiography in cases of chylous ascites to help guide appropriate management.

Role of T2 relaxometry in localization of mesial temporal sclerosis and the degree of hippocampal atrophy in patients with intractable temporal lobe epilepsy: A cross sectional study.

Mesial temporal lobe epilepsy is one of the most common causes of refractory epilepsy worldwide. A good percentage of patients do not have detectable hippocampal atrophy on magnetic resonance imaging (MRI). The objective of this study is to evaluate whether T2 relaxometry can identify hippocampal pathology and lateralize the epileptic focus in patients with intractable temporal lobe epilepsy (TLE). T2 relaxometry can also be used to correlate the clinical severity of the disease with the relaxometry readings in those who have hippocampal atrophy as well as those who do not. Thirty two patients having clinical and electrophysiological features of TLE were enrolled and a MRI brain with T2 relaxometry was done. Hippocampal T2 relaxometry values were calculated in the head, body, and tail of the hippocampus and average T2 relaxometry values were calculated, and a comparison was done with the controls. For patients with unilateral involvement, the contralateral side was taken as control and in cases of bilateral involvement, controls were identified from normal subjects. T2 relaxometry is found to be superior to MR visual analysis in the early detection of cases of hippocampal sclerosis where there is no atrophy on visual analysis. Nine out of 32 patients (28%) were normal on MR visual analysis; however, showed increased values on T2 relaxometry, correlating with clinical and electrophysiological diagnosis. The rest of the patients with hippocampal atrophy showed a correlation of T2 relaxometry values with the degree of atrophy. The hippocampal T2 measurement is thus more sensitive and specific. The study was clinically significant (p < .0001). There was a mild female predilection of the disease and there was no significant correlation with comorbidities. There was a strong positive correlation with patients having a history of febrile seizures in childhood. T2 relaxometry may accurately lateralize the majority of patients with persistent TLE and offers evidence of hippocampus injury in those patients who do not show evidence of atrophy on MRI and also the T2 relaxometry values correlated with the degree of atrophy. Early identification of hippocampal sclerosis is crucial for prompt management which offers better outcomes.

Imaging in Van Wyk Grumbach syndrome: An uncommon presentation of hypothyroidism.

Isosexual precocious puberty with ovarian masses in long-standing juvenile hypothyroidism is well described in the literature as Van Wyk Grumbach syndrome (VWGS). The present case reports this rare entity in a 4-year-old girl who was referred for imaging to evaluate the cause of non-traumatic bleeding per vagina. Antecedent history, clinical features and thyroid function tests were consistent with long-standing juvenile hypothyroidism with documented clinical response to thyroxine replacement therapy. Contribution: Typical clinical and radiological features of the syndrome are reported, which helps in the early diagnosis and management, henceforth avoiding the associated complications.

A prospective study on the role of foot evertor muscle activity in recurrence of clubfoot.

BACKGROUND: Even after corrective casting and bracing, clubfoot recurrence is challenging throughout childhood, with around 14-40 % recurrence rates. Most of the literature on recurrence was attributed to various factors, and minimal research was conducted to know the role of foot evertor muscle activity and its stimulation benefits. This study aimed to assess the role of foot evertor muscle activity in idiopathic congenital clubfoot recurrence by using clinical, sonographic, and electromyographic parameters. METHODS: A prospective cohort study was conducted at our tertiary care hospital from 2020 to 2022. The patient's demographic data, Pirani, Dimeglio, Clinical Evertor Muscle Activity scores, sonographic cross-sectional areas of leg muscle, and evertor motor activity using surface electromyography were recorded in adherence to the pre-defined intervals. RESULTS: In total, 51 patients (51 feet) were included in the study, and the overall recurrence rate was 27.5 % (14/51). In this study, around 47 % (24/51) of children had mild or poor clinical evertor activity; among them, 58 % (14/24) children had a recurrence, and the insufficient clinical evertor activity and recurrence were strongly correlated (p = 0.01). Evertor muscle cross-sectional area ratio, motor unit potentials, and recruitment were comparatively less in the recurred group; however, only the cross-sectional area ratio was statistically significant (p = 0.02). CONCLUSION: Early detection of evertor muscle weakness can help to individualize the treatment plans by predicting recurrence. Therefore, it should be included in routine clinical evaluations. Further research is required to determine the advantages of evertor muscle-strengthening activities in preventing idiopathic clubfoot deformity. LEVEL OF CLINICAL EVIDENCE: A prospective cohort study, Level of evidence-II.

Diagnostic dilemma in left flank mass in an infant: A rare case of intrarenal neuroblastoma.

Neuroblastoma (NBL) and nephroblastoma present with abdominal mass and various imaging features help in differentiating the two; however, localisation is difficult in large masses and at times imaging features can be confusing. Here, we describe the case of large left-sided NBL arising from the adrenal and involving the left kidney with moderate hydronephrosis.

Development and Validation of a Prognostic Model and Bedside Score for the Neurological Outcome in Children with Tuberculous Meningitis.

Only a few studies have explored prognostic factors for tuberculous meningitis (TBM) in children, and an easily applicable bedside prognostic score for TBM has not been developed yet. We compared the sociodemographic, clinical, radiological, and cerebrospinal fluid parameters in the cohort of 94 TBM cases aged 1 to 18 years, with at least 6 months of completed follow-up and determined the prognostic factors associated with poor functional outcome. We assessed our proposed prognostic model using both discrimination and calibration and subsequently used the bootstrap method to validate the model internally. We finally derived an easily applicable bedside prognostic score by rounding off the regression coefficients to the nearest integers. A total of 39 (41%) and 55 (59%) patients had poor and good functional outcomes, respectively, at the end of 6 months (12 died, 13%). In multivariate analysis, a high baseline Pediatric Cerebral Performance Category (PCPC) score, brain infarction in neuroimaging, tonic motor posturing, younger age, and stage III TBM were independent predictors of poor functional outcomes. The final model showed good discrimination (area under the curve = 88.2%, P < 0.001) and good calibration (Hosmer-Lemeshow test, P = 0.53). Bootstrapping also confirmed the internal validity of this model. The PITAS (PCPC score [P], brain infarction in neuroimaging [I], tonic motor posturing [T], age [A], and stage of TBM [S]) score developed from this model has a score ranging from 0 to 12, with a higher score predicting a higher risk of poor functional outcome. The PITAS score performed better than medical research council staging alone in predicting poor functional outcomes (area under the curve = 87.1% versus 82.3%). Our study's PITAS score, developed and internally validated, has good sensitivity and specificity in predicting poor functional outcomes in pediatric TBM cases at 6 months.

Comparison of Seizure Characteristics and Recurrence Rates in Children with Viable Parenchymal Neurocysticercosis and Tuberculoma in India.

Inflammatory granulomas (neurocysticercosis [NCC] and tuberculomas) are important causes of seizures in children and adults in the developing world. Although several studies have explored seizure characteristics individually in patients with either NCC or tuberculoma, none has compared the seizure recurrence rate between them. This study included patients aged 5 to 18 years with viable parenchymal NCC or tuberculomas who had completed regular follow-up of at least 12 months at a tertiary institute in India. Their baseline seizure and electroencephalographic characteristics, antiseizure medications (ASMs), and breakthrough seizure rates at 12, 24, and 52 weeks were noted. A total of 103 and 54 patients with active NCC and tuberculomas respectively were included. The number of patients who had at least one breakthrough seizure at 12, 24, and 52 weeks in both groups was comparable (P = 0.32, 0.27, and 0.13), and the vast majority were controlled on monotherapy (98% cases in each group). The proportion of patients who required an increase in the dose or change of ASMs or polytherapy, the proportion of children who had status epilepticus at or before 12 months, abnormal electroencephalogram at 12 months, and calcified and active granuloma in neuroimaging at 12 months were also comparable between the two groups (P > 0.05 for all). The number of patients who had ASM-related adverse events and discontinued ASM due to serious adverse events was comparable between both groups, except for hepatotoxicity in the tuberculoma group. The predictors for breakthrough seizures that were found to be statistically significant in the NCC group were the presence of perilesional edema in the baseline magnetic resonance imaging (P = 0.02) and more than five active granulomas (P = 0.01); predictors in the tuberculoma group were the presence of severe perilesional edema causing midline shift in the baseline magnetic resonance imaging (P = 0.01) and more than five active granulomas (P = 0.04). The recurrence rates of breakthrough seizures over the next 12 months in newly detected cases of active NCC and tuberculomas were comparable.

Enterolith Causing Small Bowel Obstruction: Report of a Case and Review of Literature.

Enterolithiasis, also known as gastro-intestinal concretions, is an uncommon medical disorder that arises from intestinal stasis. Enteroliths are commonly caused by tuberculosis-related constriction and arise from intestinal diverticula. Small bowel obstruction caused by an enterolith is extremely uncommon and might be difficult to diagnose. The mortality rate of uncomplicated enterolithiasis is relatively low, but it rises to 3% in patients who have background comorbid illness, have significant bowel obstruction, and are diagnosed late. We present a rare case of an uncomplicated partial intestinal obstruction caused by an enterolith in an elderly male patient with small bowel diverticular disease who was treated nonoperatively and did not develop further symptoms in the six-month follow-up and discuss the difficulty in diagnosis and its management.