Radiation dose analysis of computed tomography coronary angiography in Children with Kawasaki disease.

BACKGROUND: There is evolving role of computed tomography coronary angiography (CTCA) in non-invasive evaluation of coronary artery abnormalities in children with Kawasaki disease (KD). Despite this, there is lack of data on radiation dose in this group of children undergoing CTCA. AIM: To audit the radiation dose of CTCA in children with KD. METHODS: Study (December 2013-February 2018) was performed on dual source CT scanner using adaptive prospective electrocardiography-triggering. The dose length product (DLP in milligray-centimeters-mGy.cm) was recorded. Effective radiation dose (millisieverts-mSv) was calculated by applying appropriate age adjusted conversion factors as per recommendations of International Commission on Radiological Protection. Radiation dose was compared across the groups (0-1, 1-5, 5-10, and > 10 years). RESULTS: Eighty-five children (71 boys, 14 girls) with KD underwent CTCA. The median age was 5 years (range, 2 mo-11 years). Median DLP and effective dose was 21 mGy.cm, interquartile ranges (IQR) = 15 (13, 28) and 0.83 mSv, IQR = 0.33 (0.68, 1.01) respectively. Mean DLP increased significantly across the age groups. Mean effective dose in infants (0.63 mSv) was significantly lower than the other age groups (1-5 years 0.85 mSv, 5-10 years 1.04 mSv, and > 10 years 1.38 mSv) (P < 0.05). There was no significant difference in the effective dose between the other groups of children. All the CTCA studies were of diagnostic quality. No child required a repeat examination. CONCLUSION: CTCA is feasible with submillisievert radiation dose in most children with KD. Thus, CTCA has the potential to be an important adjunctive imaging modality in children with KD.

Estimation of iron overload with T2*MRI in children treated for hematological malignancies.

Iron overload may contribute to long-term complications in childhood cancer survivors. There are limited reports of assessment of tissue iron overload in childhood leukemia by magnetic resonance imaging (MRI). A cross-sectional, observational study in children treated for hematological malignancy was undertaken. Patients ≥6 months from the end of therapy who had received ≥5 red-cell transfusions were included. Iron overload was estimated by serum ferritin (SF) and T2*MRI. Forty-five survivors were enrolled among 431 treated for hematological malignancies. The median age at diagnosis was 7-years. A median of 8 red-cell units was transfused. The median duration from the end of treatment was 15 months. An elevated SF (>1,000 ng/ml), elevated liver iron concentration (LIC) and myocardial iron concentration (MIC) were observed in 5 (11.1%), 20 (45.4%), and 2 (4.5%) patients, respectively. All survivors with SF >1,000 ng/ml had elevated LIC. The LIC correlated with SF (p < 0.001). MIC lacked correlation with SF or LIC. Factors including the number of red-cell units transfused and duration from the last transfusion were associated with elevated SF (p = 0.001, 0.002) and elevated LIC (p = 0.012, 0.005) in multiple linear regression. SF >595 ng/ml predicted elevated LIC with a sensitivity of 85% and specificity of 91.6% (AUC 91.2%). A cutoff >9 units of red cell transfusions had poor sensitivity and specificity of 70% and 75% (AUC 76.6%) to predict abnormal LIC. SF >600 ng/ml is a robust tool to predict iron overload, and T2*MRI should be considered in childhood cancer survivors with SF exceeding 600 ng/ml.

Efficacy of Combination Therapy of Albendazole and Praziquantel vs Albendazole Monotherapy in Children With Persistent Neurocysticercosis: A Randomized Controlled Trial.

AIM: To compare the efficacy of combined albendazole and praziquantel therapy vs albendazole monotherapy in a placebo-controlled, double-blinded, randomized trial in children with persisting neurocysticercosis. METHODS: Children with persistent neurocysticercosis were randomized into 3 groups-albendazole (n = 19), albendazole and praziquantel (n = 21), and placebo (n = 20)-for 30 days and followed up at 3 and 6 months for resolution and recurrence of seizures. RESULTS: Mean age of children was 9.3 ± 2.9 years (range 3-14). At baseline, the majority of lesions were ring-enhancing (70%), colloidal (97%), with scolex (68%) and perilesional-edema (45%), and located in the parietal (58%) lobe. One case each in albendazole and placebo groups had a recurrence of seizure in the first month of treatment. The majority (62%) of children in the combination therapy group showed complete resolution of the persisting lesion at the end of 6 months compared to the albendazole alone group (26.3%, P = .02). Percentage reduction in the lesion's mean area at 6 months was highest in the combination group compared with other groups (P = .006). Rate of calcification was identical in all 3 groups (10%). None of the patients required interruption of therapy. CONCLUSION: Our study demonstrates the safety and efficacy of albendazole and praziquantel in combination for complete radiologic resolution in children with persistent neurocysticercosis when compared with albendazole monotherapy or placebo. The combination therapy did not result in increased seizure recurrence or adverse drug reaction compared with albendazole monotherapy.

Comparison of Long-Term Outcomes Between 7 Days and 28 Days Albendazole Monotherapy in the Treatment of Single-Lesion Neurocysticercosis in Children.

OBJECTIVES: The objective was to compare the long-term clinical, radiological, and cognitive outcomes in children with single-lesion neurocysticercosis who received 7 or 28 days of albendazole therapy. METHODOLOGY: This observational study conducted over 1 year included (1) consecutive children with single-lesion neurocysticercosis who received 7 or 28 days of albendazole therapy in the acute state and (2) completed follow-up for at least 5 years. Seizure recurrence, resolution of lesions, cognition (Malin's Intelligence Scale for Indian Children), behavior, and school performance (National Initiative for Children Healthcare Quality Vanderbilt Assessment Scale) were assessed. RESULTS: Group A (albendazole for 7 days) comprised 55 children, and group B (albendazole for 28 days) included 48 children. The mean age at the time of diagnosis of neurocysticercosis was 6.6 ± 1.8 years; the mean age at the time of assessment for the study was 13.2 ± 1.2 years. Focal-onset seizures were the most common clinical presentation (58.3%). The majority of lesions were ring-shaped (92.3%) or colloidal (58.2%), with perilesional edema (89.3%). In the long-term follow-up, radiological resolution of the lesions was comparable in both groups. Complete resolution was seen in 52.7% receiving 7 days and 54.2% receiving 28 days albendazole. Seizures recurred in 20% receiving 7 days and 20.8% receiving 28 days albendazole. Overall, a low intelligence quotient (IQ < 70) was seen in 55.3% cases, "somewhat problematic" school performance in 12%, and behavioral abnormalities were present in 20% of the cases. The results were comparable between the 2 groups. CONCLUSION: Seizure control, radiological resolution of lesion, school performance, cognitive and behavioral outcomes in the long term are comparable in children with single-lesion neurocysticercosis who have received albendazole cysticidal therapy for 7 days and 28 days. Recurrence of seizure is seen with both regimens in the long term, necessitating regular follow-up and discussion regarding the risk of recurrence before a withdrawal of anticonvulsant therapy.

Multiparametric MRI Evaluation of Complex Ovarian Masses.

OBJECTIVE: To assess the role of diffusion-weighted imaging and dynamic contrast-enhanced magnetic resonance imaging in the categorization of complex ovarian masses into benign and malignant. MATERIALS AND METHODS: This prospective study was done on 33 complex ovarian masses. T1 and T2-weighted sequences, diffusion-weighted imaging, apparent diffusion coefficient, and dynamic contrast-enhanced magnetic resonance imaging were performed on 1.5 T MRI. Time-intensity curves, tissue signal intensity on unenhanced T1 images (SI0), maximum absolute contrast enhancement (SImax), time to reach SImax (Tmax), maximum relative SI (SIrel = [SImax - SI0]/SI0 ×100), maximum Slope (Slopemax = SIrel/Tmax ×100), and wash in rate (WIR = [SImax - SI0]/Tmax) were calculated. Histopathological diagnosis was taken as gold standard. RESULTS: A total of 20/33 masses were benign, 2/33 were borderline tumors, and 11/33 were malignant. Diffusion restriction was seen in all malignant masses and 13/20 benign masses. The mean apparent diffusion coefficient values showed a significant difference between malignant and benign, with 81.8% sensitivity and 63.6% specificity. Type III curve showed 100% specificity for malignant lesions. Tmax and Slopemax were useful in differentiating benign and malignant masses; with Tmax cut-off at 73.5 seconds having a high specificity (81.8%) and Slopemax cut-off at 0.83%/s having high sensitivity (91%) and negative predictive value (94.4%). CONCLUSION: Multiparametric MRI confers high diagnostic accuracy in stratifying complex ovarian masses.

Comparison of virtual computed tomography enteroscopy using carbon dioxide with small-bowel enteroclysis and capsule endoscopy in patients with small-bowel tuberculosis.

OBJECTIVE: Comparison of virtual CT enteroscopy (VCTE) using carbon dioxide with small-bowel enteroclysis (SBE) and capsule endoscopy (CE) in small-bowel tuberculosis (SBTB). METHODS: This prospective study comprised consecutive patients suspected to have SBTB. VCTE and SBE were performed on the same day and evaluated by independent radiologists. CE was performed within 2 weeks. VCTE was performed following insufflation of carbon dioxide via catheters in the jejunum and anorectum. A contrast-enhanced CT was followed by a delayed non-contrast CT. Image processing was done using virtual colonoscopy software. Findings on VCTE, SBE, and CE were compared. The final diagnosis of SBTB was based on either histopathological or cytological findings, response to antitubercular treatment, or a combination of these. RESULTS: Of the 55 patients in whom VCTE was performed, complete data was available in 52 patients. A final diagnosis of SBTB was established in 37 patients. All patients had VCTE and SBE. CE was performed in 34 patients. Adequate luminal distension was achieved in all patients with SBE and 35 patients with VCTE. SBE showed more strictures in jejunum (10.8%) and ileum (75.7%) compared with VCTE (jejunum, 8.1%, and ileum, 64.9%) and CE (jejunum, 5.9%, and ileum, 61.8%). However, difference was not statistically significant. VCTE revealed a greater length of strictures in both the jejunum and ileum compared with SBE and CE. CONCLUSION: VCTE allows adequate evaluation of the bowel in most patients with SBTB. It allows detection of greater length of abnormality in jejunum and ileum compared with SBE and CE. KEY POINTS: • The use of VCTE using CO2 bowel insufflation in patients with SBTB should be considered. • VCTE allows detection of a greater length of abnormality in the jejunum and ileum.

A prospective cohort study to assess the frequency and risk factors for calcification in single lesion parenchymal neurocysticercosis.

BACKGROUND: Calcified neurocysticercosis (NCC) predisposes patients to an enduring state of epilepsy. The predictors for calcification in parenchymal neurocysticercosis are not well defined. METHOD: In this prospective cohort study, consecutive children with single-lesion parenchymal NCC were enrolled and followed up for one year. All patients were investigated with brain 3 T-MRI and electroimmunotransfer blot (EITB). Clinical follow-ups were performed every 3 months. Radiology was repeated at the 6-month and one-year follow-ups. The proportion of calcified lesions at one year and the predictors of calcification were studied. RESULT: During the study period from June 2013 to December 2015, 93 children with single lesion parenchymal NCC were enrolled. At presentation, 90 % of the lesions were in the colloidal stage, and 71 % of the lesions had moderate to severe perilesional oedema. All children had 6 months of follow-up, and 86 (92.5 %) had one year of follow-up. Seizure recurrence was present in 13 (14 %) children. Follow-up radiology at one year showed lesion resolution in 51 (59 %) lesions and calcification in 28 (32.5 %) lesions. Children with calcified lesions during follow-up had a higher odds of seizure recurrences {OR, 95 % CI 3.6(2.3-5.6)}. The presence at baseline of diffusion restriction {OR, 95% CI 2.9 (1.01-8.8)}, scolex or wall calcification in the T2 Star weighted angiography MRI images {OR, 95% CI 3.7 (1.7-8.2)} and >10 mm size of the lesion {OR, 95 % CI 2.4 (1.2-5.01)} predicted lesion calcification. CONCLUSION: Children with calcification of the parenchymal NCC lesions have a higher risk for seizure recurrence during follow-up. The presence of diffusion restriction, calcified nidus in the colloidal nodular stage, and >10 mm size of the lesion at baseline predicted calcification of the lesion during follow-up.

Impact of volume of irradiation on survival and quality of life in glioblastoma: a prospective, phase 2, randomized comparison of RTOG and MDACC protocols.

BACKGROUND: Though conformal partial-brain irradiation is the standard adjuvant treatment for glioblastoma, there is no consensus regarding the optimal volume that needs to be irradiated. European Organisation for Research and Treatment of Cancer (EORTC) and The University of Texas MD Anderson Cancer Center (MDACC) guidelines differ from the Radiation Therapy Oncology Group (RTOG) in their approach toward peritumoral edema, whereas RTOG and MDACC guidelines differ from EORTC in the concept of boost phase. A scarcity of randomized comparisons has resulted in remarkable variance in practice among institutions. METHODS: Fifty glioblastoma patients were randomized to receive adjuvant radiotherapy using RTOG or MDACC protocols. Apart from dosimetric and volumetric analysis, acute toxicities, recurrence patterns, progression-free survival (PFS), overall survival (OS), and quality of life (QoL) were compared using appropriate statistical tests. RESULTS: Both groups were comparable with respect to demographic characteristics. Dosimetric analysis revealed significantly lower boost-phase planning treatment volumes and V60 Gy in the MDACC arm (chi-squared, P = .001 and .013, respectively). No significant differences were observed in doses with respect to organs at risk, acute toxicity, or recurrence patterns (chi-squared, P > .05). On the log-rank test, median PFS (8.8 months vs 6.1 months, P = .043) and OS (17 months vs 12 months, P = .015) were statistically superior in the MDACC group.Age, extent of resection, and proportion of whole brain receiving prescription dose were associated with improved PFS and OS on regression analysis. QoL of patients was significantly better in the MDACC group in all domains except cognitive, as assessed with the EORTC Quality of Life Questionnaire (QLQ-C30) and Brain Cancer Module (QLQ-BN20) (general linear model, P < .05). CONCLUSIONS: Use of limited-margin MDACC protocol can potentially improve survival outcomes apart from QoL of glioblastoma patients, as compared with the RTOG protocol.

Does Shear Wave Elastography Score Over Strain Elastography in Breast Masses or Vice Versa?

OBJECTIVE: This study was done to compare parameters of strain elastography (SE) and shear-wave elastography (SWE) for differentiation of benign and malignant breast masses and determine their best cut-offs. MATERIAL AND METHODS: B mode ultrasound (USG), SE, and SWE were performed in 199 breast masses. During SE, 5-point visual elastography score (SEvisual score) and strain ratio (SEstrain ratio) were obtained. During SWE, mean and maximum elasticity values in kilopascals, and shear wave ratio were obtained in two orthogonal planes. The shear wave mean average (SWEmean avg), shear wave maximum average (SWEmax avg) and shear wave ratio average (SWEratio avg) were calculated by averaging the respective values in the two planes. The SE and SWE parameters of every mass were correlated with its histopathology. RESULTS: The areas under the receiver operating characteristic curve of SEvisual score, SEstrain ratio, SWEmean avg, SWEmax avg, and SWEratio avg were 0.815, 0.814, 0.846, 0.846, and 0.799, respectively. CONCLUSIONS: The best cut-off values that achieved the highest sensitivity and specificity for SEstrain ratio, SWEmean avg, SWEmax avg, and SWEratio avg were 3.91, 113 kPa, 123.5 kPa, and 7.32, respectively. Quantitative parameter of SE showed comparable diagnostic performance with quantitative parameters of SWE.

Percutaneous glue embolization as a primary treatment for visceral pseudoaneurysms.

Aim: To evaluate the feasibility and outcome of percutaneous glue embolisation as primary treatment for pseudoaneurysms in selective patients.Material and methods: A review of the pseudoaneurysm cases managed with percutaneous glue at our institute was analyzed in this study. A total of 21 patients over a period of last one year who were treated for pseudoaneurysm at varied sites primarily with percutaneous n-butyl cynoacrylate were retrospectively evaluated. Site of pseudoaneurysm was Pulmonary (seven cases), renal (four cases), pelvic (4four cases) and miscellaneous abdomen (six cases). Combined ultrasound and fluoroscopy approach was used in 17 cases, CT guidance in three cases and combined ultrasound and CT guidance in one case. Patients were evaluated for therapeutic response (radiological and clinical) and procedure related complications.Results: Complete occlusion of the pseudoaneurysm was seen in all patients. Two patients showed mild pneumothorax and one patient showed reflux into the intercostal artery. All the patients showed symptomatic improvement and discharged in stable condition.Conclusions: Percutaneous glue embolisation of the pseudoaneurysms as a primary treatment is feasible and can be safely carried out in select group of patients with good therapeutic response and minimal complications.

Endovascular Transjugular Occlusion of Congenital Intrahepatic Portosystemic Venous Shunt Using Simultaneous Fluoroscopy and Transabdominal Ultrasound Guidance: Report of 2 Cases.

Congenital intrahepatic portosystemic venous shunts (CIPVS) are rare anomalies that can be detected before birth or in early infancy or later in life. Symptomatic shunts are treated as they carry high risk of complications like hepatic encephalopathy. Various treatment options include surgery, endovascular embolization, and percutaneous closure devices. We treated 2 infants with CIPVS successfully by endovascular embolization of the shunt using vascular plug through transjugular route. Transabdominal ultrasound guidance in addition to fluoroscopy was used at the time of vascular plug placement. We emphasize that the use of transabdominal ultrasound during endovascular occlusion enhances the safety and technical success rate.

Acquired neuro-secretory defect in growth hormone secretion due to Imatinib mesylate and the efficacy of growth hormone therapy in children with chronic myeloid leukemia.

Imatinib results in growth retardation in children with chronic myeloid leukemia (CML). The study was planned to assess the GHRH-GH-IGF1 axis in children with CML, receiving Imatinib and to evaluate the efficacy of human growth hormone (hGH) therapy. Twenty children with CML, receiving Imatinib for a period exceeding 6 months, with resultant growth retardation were included. The GHRH-GH-IGF1 axis was assessed using growth hormone stimulation tests. IGF-1 generation test was performed for the evaluation of GH insensitivity. The mean age at inclusion was 15.2 years. The mean duration of treatment with Imatinib was 5.7 years. The mean decrease in height SDS since the start of Imatinib was -0.95 (p = 0.008). IGF-1 SDS was <-2 in all the patients. 71.4% of patients had a suboptimal GH response following stimulation with GHRH-Arginine. All patients had stimulable, although a delayed GH response with glucagon stimulation. 20% of patients had GH insensitivity. Four patients were treated with hGH for a mean duration of 5.75 months, achieved normalization of IGF-1 levels and improvement in growth velocity improved from 0.21 to 0.86 cm/month. Imatinib results in an acquired neurosecretory defect in GH secretion. Treatment with growth hormone leads to an improvement in growth velocity and normalization of IGF-1.

Fusion Image-Guided and Ultrasound-Guided Fine Needle Aspiration in Patients With Suspected Hepatic Metastases.

AIM: The aim of this study was to compare the diagnostic adequacy of computed tomography (CT)-ultrasound (US) fusion image-guided fine needle aspiration (FNA) and US-guided FNA in patients with suspected hepatic metastases. METHODS: Thirty consecutive patients of either sex with known or unknown primary malignancy suspected of having liver metastases on both US and CT, whose multiphasic contrast-enhanced computed tomography was performed using a 64-slice or a higher slice CT scanner, and who were referred for percutaneous FNA were included in this prospective study approved by the institutional review board of the study institute. CT-ultrasound fusion image-guided FNA of the largest lesion using electromagnetic tracking and with freehand ultrasound-guided FNA were performed in the same sitting. Value of fitness, which is a rough estimate of how well the fusion has been achieved, was recorded. Diagnostic adequacy of smears was assessed by a scoring system based on cellular material, background blood/clot, degree of cellular degeneration or trauma, and retention of architecture. RESULTS: The size of the lesions ranged from 1 to 10 cm, and the depth of location of the lesions ranged from 1.4 to 9.3 cm. The fusion fitness values ranged from 1.2 to 10 mm. The scores of the smears did not correlate with lesion size, depth of location, and fusion fitness value. Diagnostic adequacy was seen in 90% and 93.3% of lesions sampled by fusion image guidance and ultrasound guidance, respectively (p = 0.655). All the lesions that yielded inadequate smears by fusion guidance were deep-seated lesions (>5 cm). All the lesions that yielded inadequate smears by ultrasound guidance were small lesions (<3 cm). No complications were encountered in any of the patients. CONCLUSION: Fusion image-guided FNA is a safe procedure with a high diagnostic adequacy rate. Fusion image-guided FNA is not better than US-guided FNA for conspicuous hepatic lesions; however, it may be useful in inconspicuous lesions.

Donor Site Changes in Bone Thickness, Volume, and Density Following Split Cranial Bone Graft Harvest.

The calvarium can provide large amount of good quality corticocancellous autogenous bone graft. Although many studies have highlighted the advantages of the split cranial bone graft, there is no published work available in the literature about the fate of donor site of the split cranial bone graft. The present study was undertaken to assess the donor site as regards to the thickness, volume, and the density of the residual bone over a period of 12 months in the postoperative period. A total of 30 patients in the age group of 15 to 43 years were studied from January 2015 to January 2016. Postoperative computer tomography scans were taken at 2 weeks, 6 months, and 1 year postoperative to measure the bone thickness, volume, and density at the donor site of the split cranial bone graft harvest. The bone thickness at the donor site showed progressive increase in the thickness over the period of study and the average increase in thickness was about 12.4% at the end of 1 year. The average increase in volume at the donor site was of 2.65% after 12 months. Similarly, the average bone density increased by 3.7% at the end of 1 year. This prospective study conclusively proves that the residual bone at the donor site of the split cranial bone graft harvest site continues to grow in thickness and density over a period of 1 year.

A Screening CAD Tool for the Detection of Microcalcification Clusters in Mammograms.

Breast cancer is the most common cancer diagnosed in women worldwide. Up to 50% of non-palpable breast cancers are detected solely through microcalcification clusters in mammograms. This article presents a novel and completely automated algorithm for the detection of microcalcification clusters in a mammogram. A multiscale 2D non-linear energy operator is proposed for enhancing the contrast between the microcalcifications and the background. Several texture, shape, intensity, and histogram of oriented gradients (HOG)-based features are used to distinguish microcalcifications from other brighter mammogram regions. A new majority class data reduction technique based on data distribution is proposed to counter data imbalance problem. The algorithm is able to achieve 100% sensitivity with 2.59, 1.78, and 0.68 average false positives per image on Digital Database for Screening Mammography (scanned film), INbreast (direct radiography) database, and PGIMER-IITKGP mammogram (direct radiography) database, respectively. Thus, it might be used as a second reader as well as a screening tool to reduce the burden on radiologists.

An Ambispective Cohort Study to Assess Seizure Recurrences in Children with Calcified Parenchymal Neurocysticercosis.

Calcified neurocysticercosis (NCC), previously thought to be a dead end, is an important risk factor for seizure recurrences. We studied the pattern of seizure recurrences and associated radiological findings in children with calcified NCC. In this ambispective cohort study, we enrolled children (age 1-15 years) with calcified NCC attending the pediatric NCC clinic between January 2017 and December 2017. Retrospective data were collected from the hospital records, and all enrolled children were prospectively followed up till June 2018. The study group divided into two groups: 1) children first presenting with calcified granuloma and 2) children presented with ring-enhancing lesion (REL) and transformed into the calcified lesion during follow-up imaging. During the study period (January 2017-December 2017), 520 children with NCC were screened and 128 with calcified NCC were enrolled. The mean age was 10.8 ± 3.2 years, and 63% were boys. Among 128 children, 40 (31%) had calcified granuloma and 88 (69%) had REL transformed to calcified granuloma. Sixty-one (49%) children had seizure recurrence: 22 (58%) within calcified granuloma group and 39 (45%) within REL transformed to calcified granuloma group (P = 0.18). Seizure recurrence was associated with the presence of perilesional edema (PE) in 35 (57.4%) children on computed tomography scan. The median interval between two seizure recurrences was 30 (17-56) months, and the median antiepileptic drug-free interval was 17 (12-22) months. The total duration of continued seizures was 42 (26-58) months, slightly longer in children with REL transformed to calcified granuloma group (42, 95% CI: 18-66 months) in comparison to calcified granuloma group (35, 95% CI: 10-60 months, P = 0.32). To conclude, children with calcified NCC have seizure recurrences over a prolonged period. Seizure recurrences are intermittent and may be interspersed with a prolonged period of quiescence in between. The presence of PE and contrast enhancement around the lesion during seizure recurrence suggests lesion reactivation.

Cross-sectional Imaging of Gallbladder Carcinoma: An Update.

Gallbladder Carcinoma (GBCA) is the most common biliary tract malignancy. As the disease is often diagnosed clinically in an advanced stage, the survival rates are dismal. Imaging studies allow for an early diagnosis of malignancy, though the findings may be indistinguishable from non-malignant disease processes affecting the gallbladder. Attempts have been made to make a specific diagnosis of GBCA at an early stage on imaging studies. Ultrasonography (US) is the most commonly employed technique for gallbladder evaluation. Gallbladder wall thickening is the most common finding of early GBCA and in this context, US is non-specific. Recently, contrast enhanced ultrasound has been shown to be effective in differentiating benign from malignant disease. Multidetector computed tomography represents the most robust imaging technique in evaluation of GBCA. It provides relatively sensitive evaluation of mural thickening, though it is not entirely specific and issues in differentiating GBCA from xanthogranulomatous cholecystitis do arise. Due to its superior soft tissue resolution, Magnetic Resonance Imaging (MRI) provides excellent delineation of gallbladder and biliary tree involvement. When coupled with functional MRI techniques, such as diffusion-weighted and perfusion imaging, it provides a useful problem solving tool for interrogating the malignant potential of nonspecific gallbladder lesions and detection of metastases. Positron emission tomography has a role in detection of distant metastases and following patients following treatment for malignancy. We review the current role of various imaging modalities in evaluating patients with GBCA.

Phenotypic heterogeneity of kyphoscoliosis with vertebral and rib defects: a case series.

Disorders associated with multiple vertebral segmentation defects may have additional rib anomalies in form of absence or hypoplastic ribs, fanning of ribs, etc. Spondylocostal dysostosis is genetic disorder with abnormal vertebral segmentation and rib anomalies. Diagnosis is often delayed because of non-familiarity with the characteristic features. There are six genes identified for spondylocostal dysostosis, of which SCDO5 is responsible for autosomal dominant form of the disorder. Retrospective study was conducted in Genetic and Metabolic unit of a tertiary hospital in north India over a period of 9 years. Twenty patients with a clinical diagnosis of congenital scoliosis were identified, and reviewed. Three patients were discussed in an earlier report and 11 subsequent patients, are described in this case series here. The median age at presentation was 34 months. The patients showed hemivertebrae, vertebral fusion, fusion of ribs, fanning of ribs. Hydrocephalus/ventriculomegaly was found in three cases and diastematomyelia was identified in one case. Other associated anomalies included corpus callosal agenesis, club foot and capillary malformation. One parent showed rib/spinal defects in two cases. Further studies are needed to characterise the phenotype and genetic basis of scoliosis in Indian patients.

Irreversible Electroporation for Unresectable Hepatocellular Carcinoma: Initial Experience.

PURPOSE: To evaluate the efficacy and safety of irreversible electroporation (IRE) in the treatment of unresectable hepatocellular carcinoma (HCC). MATERIALS AND METHODS: A retrospective study was conducted from September 2014 to June 2017. A total of 21 HCCs in 21 patients with cirrhosis were treated with IRE. There were eight subcapsular or exophytic, ten perivascular and three peribiliary tumors. The median tumor size was 26 mm (range 14-40 mm). The technical success of the procedure was recorded. Median follow-up, median time to local recurrence, median local tumor progression-free survival (PFS) and complications were recorded. RESULTS: Technical success was achieved in all the patients. The median follow-up was 10 months (range 2-30 months). The median time to local recurrence and local tumor PFS were 4 months (range 3-4 months) and 7 months (range 3-30 months), respectively. The tumor-related factor that was significantly associated with local PFS was the size. Maximum tumor diameter < 25 mm was significantly associated with local tumor PFS (p = 0.045). Other parameters including tumor location, segmental portal vein thrombosis, baseline alpha-fetoprotein level and underlying etiology did not affect local tumor PFS. Complications were noted in nine patients and were classified as grades 1 and 2. No procedure-related mortality was encountered. CONCLUSION: IRE is an effective treatment for ablation of small HCCs. Larger prospective studies with strict selection criteria will establish the safety and efficacy of IRE in the treatment of unresectable HCC in patients who cannot undergo thermal ablation.