Budd-Chiari syndrome computed tomographic imaging: Elucidating the devil in the details.

Hepatic venous outflow obstruction, due to hepatic venous thrombosis is the hallmark of Budd-Chiari Syndrome (BCS), a relatively uncommon disorder. The aim of this study was to explore imaging details and causes, and define invasive and non-invasive interventions for achieving successful portosystemic shunting (PSS). The variations in imaging topology and associated risk factors in BCS cases, diagnosed during the past 20 months, were studied. Of the 53 cases (32 males, 21 females; mean age: 34.4±13.5 years), 5 (9%) were Hepatitis-B virus (HBV) positive, 12 (23%) were HCV positive and 6 (11%) were co-infected, rest were negative. Imaging features included mottled cirrhotic hepatic parenchyma, thrombosed hepatic and portal-vein with or without IVC thrombosis, left lobe atrophy, caudate hypertrophy, splenomegaly, portosystemic varices and ascites. Three cases had subacute onset; two had acute while the rest were chronic BCS. The aetiology was: protein C, S and anti-thrombin deficiency in 24 (45%), JAK2 mutation in 3 (6%), lupus antibody with increased homocysteine levels in 5 (9%) and cryptogenic in 21 (40%) cases. Twelve cases were planned for liver transplantation, 20 were prepared to undergo TIPS, and the rest were optimised medically.

18F-FDG PET-CT imaging versus bone marrow biopsy in pediatric Hodgkin's lymphoma: a quantitative assessment of marrow uptake and novel insights into clinical implications of marrow involvement.

OBJECTIVE: To evaluate whether positron emission tomography/computed tomography using fluorine-18 fluoro-deoxyglucose (18F-FDG PET-CT) predicts bone marrow involvement (BMI) in pediatric Hodgkin's lymphoma (pHL) with sufficient accuracy to supplant routine staging bone marrow biopsy (BMB), and to assess the clinical importance of marrow disease by comparing the prognosis of stage IV HL with BMI versus that without BMI. METHODS: Data were retrospectively analyzed for all cases of pHL between July 2010 and June 2015 referred for staging 18F-FDG PET-CT scan and BMB. The reference standard was BMB. Stage IV patients were divided into three groups to compare their progression-free and overall survival: PET+ BMB-, PET+ BMB+, and PET- BMB-. RESULTS: Of the 784 patients, 83.3% were male and 16.7% female, with age ranging from 2 to 18 years (mean 10.3 years). Among the total cases, 104 (13.3%) had BMI; of these, 100 were detected by PET imaging and 58 by BMB. BMB and 18F-FDG PET/CT scans were concordant for BMI detection in 728 patients (93%): positive concordance in 54 and negative in 674. Of the 56 discordant cases, four had a false-negative PET scans and were upstaged by BMB, 46 with focal uptake were PET/CT-positive and BMB-negative (not obtained from active sites), and six with diffuse uptake were false-positive on PET due to paraneoplastic marrow activation. The sensitivity, specificity, PPV, and NPV of PET for identifying BMI was 93.6, 94, 53, and 99.4% respectively. On quantitative assessment, mean iBM-SUVmax of bilateral iliac crests was significantly higher in those with BMI versus those without (p < 0.05). CONCLUSIONS: 18F-FDG PET-CT imaging is more sensitive than BMB for BMI detection in pHL staging. BMB should be limited to those with normal marrow uptake in the presence of poor risk factors or those with diffusely increased uptake to exclude marrow involvement in the background of reactive marrow.

Right to Left Intrapulmonary Shunt in a Case with COVID-19-associated Pneumonia.

The clinical manifestations of coronavirus disease 2019 (COVID-19)-associated pneumonia show a wide range of variations. It ranges from mild hypoxemia without significant signs of respiratory distress, to rapid clinically deteriorating course with severe hypoxemia. Unexplained severe hypoxemia, associated with platypnea, triggers the possibility of ventilation-perfusion (V/Q) mismatch, ranging from intrapulmonary shunts (IPS) to alveolar dead space ventilation. In the literature, very few cases with COVID-19-pneumonia have been reported with IPS. Herein, we report a COVID-19 confirmed 45-year male patient, who developed IPS without apparent pulmonary perfusion defect on lung perfusion scintigraphy. The patient had no cardiovascular disease except chronic pulmonary hypertension secondary to interstitial lung disease. The clinical manifestations combined with nuclear imaging features enabled in making the ultimate diagnosis. The patient's clinical condition improved on appropriate clinical management, using high flow oxygen combined with intravenous steroids and anticoagulants. Key Words: COVID-19, Adult respiratory distress syndrome, Right to left shunt, Lung perfusion scintigraphy, Platypnea.

Right to Left Intrapulmonary Shunt in a Case with COVID-19-associated Pneumonia.

ABSTRACT       The clinical manifestations of coronavirus disease 2019 (COVID-19)-associated pneumonia show a wide range of variations. It ranges from mild hypoxemia without significant signs of respiratory distress, to rapid clinically deteriorating course with severe hypoxemia. Unexplained severe hypoxemia, associated with platypnea, triggers the possibility of ventilation-perfusion (V/Q) mismatch, ranging from intrapulmonary shunts (IPS) to alveolar dead space ventilation. In the literature, very few cases with COVID-19-pneumonia have been reported with IPS. Herein, we report a COVID-19 confirmed 45-year male patient, who developed IPS without apparent pulmonary perfusion defect on lung perfusion scintigraphy. The patient had no cardiovascular disease except chronic pulmonary hypertension secondary to interstitial lung disease. The clinical manifestations combined with nuclear imaging features enabled in making the ultimate diagnosis. The patient's clinical condition improved on appropriate clinical management, using high flow oxygen combined with intravenous steroids and anticoagulants. Key Words: COVID-19, Adult respiratory distress syndrome, Right to left shunt, Lung perfusion scintigraphy, Platypnea.

Comparison between sentinel lymph node hybrid scintigraphy and blue dye technique in breast cancer patients: An institutional experience.

To compare the detection efficacy of radionuclide lymphoscintigraphy (LS) versus patent blue dye (PBD) technique for hidden sentinel lymph node (SLN) in breast cancer patients and to determine which modality is better for SLN detection. One hundred and thirty-four early stage breast cancer female patients with clinically negative axilla who underwent post technetium-99m nanocolloid injection single photon emission computed tomography (SPECT-CT) for negative SLN on planar imaging were studied prospectively between 2015 and 2017. Following SPECT-CT, patients underwent peroperative gamma probe count detection GP-CD and PBD technique. Visually, blue stained ± hot nodes were surgically removed and subjected to histopathological analysis. The detection rate by individual method was calculated. Kappa statistics were applied to calculate overall agreement between radioisotope and PBD techniques for diagnostic value assessment. One hundred and thirty-four patients underwent SPECT-CT LS and PBD injection. Mean age: 47 ± 7.6 years (range: 26-82 years). Forty-nine (36.6%) had T1 and 85 (63.4%) T2. SPECT-CT LS detected SLN in 105/134 cases (success rate: 78.4%), later GP-CD localized "hot nodes" in additional 20 cases (success rate: 93.3%). The PBD successfully localized SLN in 131/134 (97.8%) cases. Three cases remained negative on both radioisotope and PBD localization, which on subsequent nodal dissection had metastatic disease. All SLNs detected on SPECT-CT showed blue dye uptake. In 112 cases, more than one SLN was surgically removed. Frozen section analysis of excised SLNs showed metastasis in 31%. Overall moderate agreement (k = 0.56) was calculated. No statistically significant difference was seen between isotope detection and PBD. Radionuclide sentinel mapping has good detection rate particularly combined with peroperative GP-CD. The PBD has added value to reduce false-negative rate of SLN mapping and can substitute radionuclide imaging with negative results.

99mTc Sodium Pertechnetate Uptake in Ectopic Mediastinal Thyroid Tissue on Hybrid Thyroid Scintigraphy.

Ectopic thyroid is a rare/incidental imaging finding. When discovered, 90% of ectopic thyroid is found typically along the pathway of embryologic migration of thyroid tissue, whereas around 10% have been discovered in other anatomical locations including the mediastinum and the heart. Thyroid scintigraphy with Tc sodium pertechnetate (TcO4) is peculiar for thyroid tissue uptake. The current case, clinically euthyroid, had heterogeneous uptake in multinodular goiter with uptake in the ectopic thyroid tissue in right paratracheal location on functional imaging with TcO4. Subsequent single photon emission computed tomography/computed tomography (SPECT/CT) imaging confirmed the ectopic thyroid tissue.

The Usefulness of SPECT/CT in Sentinel Node Mapping of Early Stage Breast Cancer Patients Showing Negative or Equivocal Findings on Planar Scintigraphy.

OBJECTIVES: This study sought to determine the diagnostic yield of SPECT/CT in localizing axillary sentinel lymph nodes (SLNs) in early breast cancer patients where planar scintigraphy (PS) was equivocal or negative. METHODS: Prospective analysis of early stage breast cancer patients with non-palpable axillary nodes undergoing SLN localization prior to nodal sampling for axillary staging. PS findings were categorized as: Category A: non-visualization of SLN; Category B: unusual uptake location; Category C: equivocal uptake / difficult interpretation. The K-coefficient of Cohen was used to evaluate the correlation between PS and SPECT/CT results. PS and SPECT/CT images were interpreted separately, and SLN identification on each of the modalities was correlated to BMI (Body mass index) and peroperative radio guided results. RESULTS: Between April 2015 and January 2017, 1028 early breast cancer cases underwent sentinel lymphoscintigraphy. Of total, 134 (13%) patients underwent SPECT/CT in addition to PS. All were females with mean age of 48.15 years (range: 26-82 years). Right sided in 68, left in 64 and 2 with bilateral carcinoma. By TNM classification: 49 (37%) T1, 78 (58%) T2 and 7 (5%) had DCIS/Paget's disease.Overall SLNs were detected on both PS and SPECT/CT in 60% cases. Of category A (n=54); 35/54 (64%) SLN localized on SPECT/CT; 32 were level-I; 2 Level-II; 1 Level-III nodes. In 19, SLN was not localized. Of category B (n=18), 5 had prior lumpectomy, SPECT/CT localized tracer uptake to 17 level-I sentinel nodes, 3 level-II and level III / IMC in 9. Of category C (n=62), 29 had prior lumpectomy. SPECT/CT confirmed SLN in all the cases. Radio-guided surgery confirmed SPECT/CT results. The correlation between the two techniques was low (K=0.34). Where PS was negative; SPECT/CT localized nodes in statistically significant number of cases (=0.01). PS identified SLN uptakes in 80/134 (60%) cases with a mean BMI of 21.6±4.8 kg/m2 while SPECT/CT detected ''hot'' nodes in 115/134 (86%) cases with a mean BMI of 29.6±5.6 kg/m2. For overweight/obese patients (n=59) (BMI>25 kg/m2), PS failed to identify SLNs in 49 and SPECT/CT failed to do so in 18 (<0.001). CONCLUSION: SPECT/CT has diagnostic yield and helps in precise SLN localization where planar imaging is negative or shows unusual site of uptake.

Frequency of Ischemic Stroke Subtypes Based on Toast Classification at a Tertiary Care Center in Pakistan.

BACKGROUND: The purpose of this study was to determine the frequency of ischemic stroke subtypes based on Trial of Org 10172 in Acute Stroke Treatment (TOAST) classification at a tertiary care center in Pakistan. MATERIALS AND METHODS: A cross-sectional study was conducted in Stroke Unit of Shifa International Hospital, Islamabad. We included 145 patients who presented to us from November 2015 to February 2016 with radiological confirmed neurological deficits consistent with ischemic stroke. The causes of ischemic stroke were classified according to TOAST criteria. Regression analysis and Chi-square test were used to compute P value. RESULTS: Among the 145 patients diagnosed with ischemic stroke, there were 54.1% males and 45.9% females with a mean age of 65 ± 14 years. Nearly 62.7% patients had hypertension (HTN) as the most common risk factor, followed by 38.6% diabetes mellitus (DM), 27.5% heart failure, 19.3% valvular disease, 18.6% previous stroke, 16.4% smoking, 15.1% dyslipidemia, 13.7% ischemic heart disease, and 13.1% atrial fibrillation. HTN was significantly associated with large vessel disease (P = 0.028). DM was significantly associated with small vessel disease (P = 0.001). Smoking and atrial fibrillation both were associated with unknown etiology of stroke (P = 0.001 and P = 0.039, respectively). Most common etiology of stroke was cardioembolism (40%), and atrial fibrillation is found to be the most common cause of cardioembolic stroke with 30.6% incidence. CONCLUSION: Our study concludes that cardioembolic stroke is the most common cause of acute ischemic stroke in our stroke unit. Atrial fibrillation is found to be the most common cause of cardioembolic stroke.

Medullary Thyroid Carcinoma: Prognostic Variables And Tumour Markers Affecting Survival.

BACKGROUND: Medullary thyroid carcinoma (MTC) is a relatively rare thyroid malignancy and its clinical course varies among patients due to its familial association. A number of prognostic factors have been studied, but the significance of these factors remains controversial. We evaluated the progression free survival (PFS) and overall survival (OS) of MTC and its association with tumour marker rising velocity and serum calcitonin (Ct) doubling time (DT). METHODS: Analysis of 83 (8.7%) consecutive MTC patients registered at a single centre between 1995 and 2015. The impact of tumour respectability, TNM stage, multiple endocrine neoplasia (MEN) syndrome, local recurrence, Ct DT and Ct rising velocity on PFS and OS was analysed. Median follow-up was 4.3 years (range: 1-18 years). RESULTS: Eighty-three (8.7%) of all thyroid cancers registered at our centre were MTC. Fifty-five males, 28 females. Mean age 39 years [range: 17-72 years]. Twenty-two were unresectable and 61 resectable. Five-year and 10-year OS was 84% and 77% respectively. Of 68 with follow up greater than a year; 20 (29.4%) were cured, 15 (22.1%) had biochemical evidence of disease, three (4.4%) had stable macroscopic disease and 30 (44.1%) had recurrent/progressive disease. Sixteen (23.5%) died. On multivariate analysis, T4 tumour, male gender, nodal and distant metastases, tumour resectibility, Ct DT less than two years and tumour marker rising velocity of greater than 0.05pg/ml/month were poor prognostic factors (pvalue <0.05). Age and association with MEN syndrome had no statistically significant survival impact. Radiotherapy reduced local relapse in patients with nodal disease. Total thyroidectomy with nodal clearance lessened relapses. CONCLUSION: Clinical stage and pathological aspects are predictors of disease progression. Persistent biochemical evidence of MTC does not affect OS, however, Ct DT < 2 years and rapid rate of tumour marker rise predict disease progression.

A complex microcephaly syndrome in a Pakistani family associated with a novel missense mutation in RBBP8 and a heterozygous deletion in NRXN1.

We report on a consanguineous Pakistani family with a severe congenital microcephaly syndrome resembling the Seckel syndrome and Jawad syndrome. The affected individuals in this family were born to consanguineous parents of whom the mother presented with mild intellectual disability (ID), epilepsy and diabetes mellitus. The two living affected brothers presented with microcephaly, white matter disease of the brain, hyponychia, dysmorphic facial features with synophrys, epilepsy, diabetes mellitus and ID. Genotyping with a 250K SNP array in both affected brothers revealed an 18 MB homozygous region on chromosome 18 p11.21-q12.1 encompassing the SCKL2 locus of the Seckel and Jawad syndromes. Sequencing of the RBBP8 gene, underlying the Seckel and Jawad syndromes, identified the novel mutation c.919A>G, p.Arg307Gly, segregating in a recessive manner in the family. In addition, in the two affected brothers and their mother we have also found a heterozygous 607kb deletion, encompassing exons 13-19 of NRXN1. Bidirectional sequencing of the coding exons of NRXN1 did not reveal any other mutation on the other allele. It thus appears that the phenotype of the mildly affected mother can be explained by the NRXN1 deletion, whereas the more severe and complex microcephalic phenotype of the two affected brothers is due to the simultaneous deletion in NRXN1 and the homozygous missense mutation affecting RBBP8.