An Update on the Management of Budd-Chiari Syndrome.

Budd-Chiari syndrome (BCS) is an uncommon condition, caused by obstruction to hepatic venous outflow. It is largely underdiagnosed, and a high index of suspicion is required for any patient with unexplained portal hypertension. The understanding of its etiology and pathology is improving with advances in diagnostic techniques. Recent studies reported an identifiable etiology in > 80% of cases. Myeloproliferative neoplasm (MPN) is the most common etiology, and genetic studies help in diagnosing latent MPN. Better cross-sectional imaging helps delineate the site of obstruction accurately. The majority of BCS patients are now treated by endovascular intervention and anticoagulation which have improved survival in this disease. Angioplasty of hepatic veins/inferior vena cava remains under-utilized at present. While surgical porto-systemic shunts are no longer done for BCS, liver transplantation is reserved for select indications. Some of the unresolved issues in the current management of BCS are also discussed in this review.

A new and simplified comprehensive ultrasound protocol of haemophilic joints: the Universal Simplified Ultrasound (US-US) protocol.

AIM: To present a new protocol to optimise ultrasound (US) assessment of haemophilic arthropathy. MATERIALS AND METHODS: Ultrasound of haemophilic arthropathy joints was performed using three different ultrasound protocols, namely, the Toronto-Vellore Comprehensive Ultrasound (TVC-US) protocol, the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US), and the newly developed Universal Simplified Ultrasound (US-US) protocol. Synovial hypertrophy, haemosiderin deposition, effusion, erosion, and cartilage loss were evaluated in 20 joints. The reliability and diagnostic efficiency of these protocols was compared using magnetic resonance imaging (MRI). RESULTS: The correlation between the TVC-US and US-US protocols for synovial hypertrophy was excellent: kappa significance (KS) was 1, but was substantial (KS=0.65) with the HEAD-US protocol. For effusion, both the TVC-US and the HEAD-US protocols had substantial correlation with the US-US protocol (KS=0.7 and 0.6 respectively). The correlation for erosion and cartilage loss was excellent between the TVC-US and the US-US with MRI (KS=1), but poor (KS=0) with the HEAD-US protocol. The US-US protocol also had good interobserver agreement (KS=1). CONCLUSION: The accuracy of the US-US protocol is comparable to the TVC-US protocol and MRI and is superior to the HEAD-US protocol in the assessment of haemophilic arthropathy.

Correlating clinical and radiological assessment of joints in haemophilia: results of a cross sectional study.

OBJECTIVES: This study was undertaken to determine the correlation between the radiological changes in haemophilic arthropathy [X-ray, Ultrasound (US) and MRI] and clinical assessment as determined by the Hemophilia Joint Health Score (HJHS); and to document the US and MRI changes in joints that appear normal on plain X-ray and clinical evaluation. MATERIALS AND METHODS: Of 55 study joints (22 knees and 33 ankles) in 51 patients with haemophilia/von Willebrand disease, with a median age of 15 years (range: 5-17) were assessed using X-rays (Pettersson score) and clinical examination (HJHS) at two centres (Toronto, Canada; Vellore, India). MRI and ultrasonographic scoring was done through a consensus assessment by imagers at both centres using the IPSG MRI and US scores. RESULTS: The HJHS had a good correlation with the Pettersson score (rs = 0.66). Though the HJHS had moderate correlation with the osteochondral component of the MRI and US scores (rs 0.51, 0.45 respectively), its correlation with the soft tissue component was poor (rs 0.19; 0.26 respectively). Of the 18 joints with a Pettersson score of zero, 88.9% had changes that were detected clinically by the HJHS. Osteochondral abnormalities were identified in 38.9% of these joints by the MRI, while US images of the same joints were deemed abnormal in 83.3% by the current criteria. US identified haemosiderin and other soft tissue changes in all of the joints, while the same changes were noted in 94.4% of these joints on MRI. There were four joints with a HJHS of zero, all of which had soft tissue changes on MRI (score 1-7) and US (score 2-7). Osteochondral changes were detected in three of these joints by US and in 2 by MRI. There were four joints with an MRI score of 0-1 that had significant US scores (3-5) and HJHS scores (0-6). CONCLUSION: US and MRI are able to identify pathological changes in joints with normal X-ray imaging and clinical examination. However, further studies are required to be able to differentiate early abnormalities from normal. Clinical (HJHS) and radiological assessment (US/MRI) provide complimentary information and should be considered conjointly in the assessment of early joint arthropathy.

Ultrasound and magnetic resonance imaging of healthy paediatric ankles and knees: a baseline for comparison with haemophilic joints.

The study was undertaken to document cartilage and soft tissue changes/findings in ankles and knees of normal children of different age groups to be used for comparison in the assessment of children with haemophilia. Cartilage thickness and soft tissue changes were recorded at predetermined sites of ankles/knees on both US and MRI in healthy boys in three age groups: 7-9; 10-14; and 15-18 years. To assess the validity of the ultrasound and MRI measurements, an ex vivo study was done using agar phantoms with techniques and scanners similar to those applied in vivo. Twenty (48%) knees and 22 (52%) ankles of 42 boys, were evaluated. There was a reduction in the thickness of joint cartilage with age. A difference in cartilage measurements was noted in most sites between the age groups on both US and MRI (P < 0.05 each), but such difference was not noted for joint fluid in ankles or knees (P = 0.20, P = 0.68 or P = 0.75, P = 0.63 for US, MRI, respectively). Although cartilage measurements were smaller on US than on MRI for both ankles and knees (P < 0.05 each), this observation was not recorded for fluid in knees (P = 0.02). For diminutive measurements (2 mm) mean US measurements were smaller than corresponding phantom's measurements, P = 0.02. Age-related measurements were noted for cartilage thickness on US and MRI in ankles and knees. US measurements were smaller than corresponding MRI measurements at most joint sites, which were supported by results on small-diameter phantoms.

Aortic coarctation associated with an absent segment of the proximal right subclavian artery.

Coarctation of the aorta is a congenital anomaly of the thoracic aorta with many known associations. We describe the case of a young man referred for management of subarachnoid hemorrhage, in whom subsequent work-up revealed the previously undescribed combination of severe postductal aortic coarctation and an absent segment of the proximal right subclavian artery.

Evaluation of normal abdominal aortic diameters in the Indian population using computed tomography.

OBJECTIVES: The aim of this study was to establish normal diameters for the suprarenal and infrarenal abdominal aorta measured at T12 and L3 vertebral levels in the Indian population and to study the variation in aortic diameters with age, sex, height, weight, body mass index (BMI), and body surface area (BSA). MATERIALS AND METHODS: One hundred and forty-two patients who underwent helical contrast-enhanced computed tomography (CT) scans of the abdomen for non-cardiovascular reasons were recruited.. The mean internal diameters of the suprarenal and infrarenal abdominal aorta (maximum anteroposterior and transverse diameter) were measured at T12 and L3 vertebral levels and tabulated according to various age groups for both men and women. Pearson correlation coefficient was used to evaluate the correlation between aortic diameters, height, weight, BSA, and BMI. RESULTS: The mean diameters of the suprarenal and infrarenal abdominal aorta measured at T12 and L3 vertebral levels, in men were 19.0 ± 2.3 and 13.8 ± 1.9 mm and in women 17.1 ± 2.3 and 12.0 ± 1.6 mm, respectively. The aortic diameter progressively increased in caliber with increasing age of the patients and was smaller in women than men. A significant positive correlation was found in men between the suprarenal and infrarenal aortic diameters and weight, BSA, and BMI. In women, this correlation was significant in the infrarenal aorta but not in the suprarenal aorta. CONCLUSION: We obtained a set of normal values for the abdominal aorta in the Indian population. The aortic diameters correlated with age, gender, and body size of the patients as seen with previously published data in the Western population. A brief comparison of data between Indian and Western population showed that the values obtained were less than published elsewhere and hence, this should be considered while formulating intervention protocols.

MRI in patients with tuberculous spondylitis presenting as vertebra plana: a retrospective analysis and review of literature.

AIM: To present the magnetic resonance imaging (MRI) findings of 10 patients with histopathologically proven tuberculous spondylitis (TS) presenting as vertebra plana (VP) on radiographs. MATERIAL AND METHODS: Radiographs of 451 adult TS patients were reviewed. In this consecutive series, there were 11 patients who presented as VP. MRI of 10 of these patients was available for review. RESULTS: VP-like collapse of a single vertebral body of the dorsal spine with preserved endplates and disc was seen in all cases. Epidural, pre- and para-vertebral soft tissue was found in all patients. Epidural soft tissue presenting on sagittal images as a convexity of the posterior longitudinal ligament was also found in all the signal of which was different from the involved vertebra on axial images. All patients showed posterior element involvement, which was characterized by preserved cortical outline without expansion. CONCLUSION: TS presenting with VP-like collapse of the bone is rare, accounting for 2.4% of the cases in the present series. MRI may show a collapsed vertebra with preserved endplates and disc. MRI findings that are suggestive of TS include: (1) signal intensity of the epidural soft-tissue mass on axial images, which is different from the vertebral body; (2) presence of a thin, T2-weighted hypointense capsule of the para-vertebral soft tissue; (3) posterior element involvement characterized by intact hypointense cortical outline without expansion; and (4) involvement of the costovertebral joint.

An MRI scale for assessment of haemophilic arthropathy from the International Prophylaxis Study Group.

Evaluation of prophylactic treatment of haemophilia requires sensitive methods. To design and test a new magnetic resonance imaging (MRI) scale for haemophilic arthropathy, two scales of a combined MRI scoring scheme were merged into a single scale which includes soft tissue and osteochondral subscores. Sixty-one joint MRI's of 46 patients with haemophilia were evaluated by four radiologists using the new and older scales. Forty-six of the joints were evaluated using two X-ray scales. For all MRI scores, interreader agreement and correlations with X-ray scores and lifetime number of haemarthroses were analysed. The interreader agreement intraclass correlation coefficient was 0.82, 0.89 and 0.88 for the soft tissue and osteochondral subscores and the total score, as evaluated according to the new MRI scale, compared to 0.80 and 0.89 as for the older scales. The total score and osteochondral subscore according to the new scale, as well as scores according to the older scales were correlated (P < 0.01) with number of haemarthroses (Spearman correlation 0.35-0.68) and with the X-ray scores (Spearman correlation 0.40-0.76), but no correlation (P > 0.05) was found between the soft tissue subscore of the new MRI scale and the X-ray scores. The new MRI scale is simpler to apply than the older and has similar reader reliability and correlation with lifetime number of haemarthroses, and by separating soft tissue and osteochondral changes it gives additional information. The new scale is useful for analyses of early and moderate stages of arthropathy, and may help to evaluate prophylactic haemophilia treatment.

Cystic artery pseudoaneurysm after laparoscopic cholecystectomy presenting as haemobilia: a case report.

Cystic artery pseudoaneurysm which developed following a cholecystectomy and resulting in upper gastrointestinal bleeding is a rare entity, with only three cases described in the literature. We report the case of a 26-year old man who presented with upper gastrointestinal bleeding approximately three months after laparoscopic cholecystectomy. Emergency abdominal angiogram revealed a cystic artery stump pseudoaneurysm, with no evidence of active contrast extravasation. The pseudoaneurysm was coil embolised and the patient had no further bleeding episodes. In this situation an angiogram and embolisation rather than surgery is the preferred mode of management both in terms of diagnosis and treatment. The presence of a dilated cystic artery stump on angiogram following cholecystectomy is an "ominous sign", even in the absence of active extravasation of contrast.

Trans-caval trans-jugular liver biopsy--a technical modification of trans-jugular liver biopsy.

OBJECTIVE: To (a) describe the technical modification of trans-caval TJLB and (b) review our series of nine cases. METHODS: We performed a retrospective review of all trans-caval TJLBs performed; we assessed indications for the procedure, technical success, complications, adequacy of specimen and histological positivity. RESULTS: The technical success rate of the procedure was 9/9 (100%); the minor complication rate was 1/9 (11%), adequate specimen was obtained in all cases and a histological diagnosis was achieved in 8/9 (89%) cases. CONCLUSION: This preliminary report suggests that trans-caval modification of TJLB is a relatively safe procedure that may be useful in cases where conventional TJLB is infeasible. ADVANCES IN KNOWLEDGE: (a) We describe the technique of trans-caval TJLBs and report our findings in the largest series of published cases. (b) Trans-caval TJLB is relatively safe and can be used to increase the success rates of conventional TJLB.

Dual arterial supply to normal lung: within the sequestration spectrum.

We report a rare case of dual arterial supply to an otherwise normal left lower lobe. In addition to normal pulmonary arterial supply, the lower lobe of the left lung also received systemic arterial supply from the coeliac axis. The relevant anatomy and literature are reviewed. To the best of our knowledge, there are no other reported cases of anomalous systemic arterial supply from the coeliac axis to the basal segments of the left lower lobe with normal bronchial branching and pulmonary arterial supply.

Skull base hydatid cyst with intracranial extension presenting as vocal cord palsy: a case report.

Hydatid disease of the skull base is extremely rare, and intracranial extension of hydatid cysts through the skull base is even rarer. We report an interesting case of a 42-year-old man who presented with features of right vocal cord palsy. The diagnosis of hydatid cyst was made based on his history and on pre-operative MRI and was confirmed by surgery and histopathological examination.

Jejunal access loop cholangiogram and intervention using image guided access.

Jejunal access loop is fashioned in patients who undergo Roux en Y hepaticojejunostomy and biliary intervention is anticipated on follow up. Post-operative study of the biliary tree through the access loop is usually done under fluoroscopic guidance. We present a series of 20 access loop cholangiograms performed in our institution between August 2004 and November 2008. We aimed to evaluate the safety and efficacy of the procedure and to highlight the role of CT guidance in procuring access. Access loop was accessed using CT (n = 13), ultrasound (n = 3) or fluoroscopic guidance (n = 4). Fluoroscopy was used for performing cholangiograms and interventions. Twelve studies had balloon plasty of the stricture at anastomotic site or high up in the hepatic ducts. Seven studies showed normal cholangiogram. Plasty was unsuccessful in one study. Technical success in accessing the jejunal access loop was 100%; in cannulation of anastomotic site and balloon plasty it was 95%. One case required two attempts. Procedure-related complications were not seen. All patients who underwent balloon plasty of the stricture were doing well for variable lengths of time. Access loop cholangiogram and interventions are safe and effective. CT guidance in locating/procuring the access loop is a good technique.

Transjugular intrahepatic portocaval shunt placed through the strut of an inferior vena cava stent in a patient with Budd-Chiari syndrome: a technical modification.

Transjugular intrahepatic portocaval shunt (TIPS) is performed in patients with symptomatic Budd-Chiari syndrome (BCS) who do not have repairable hepatic veins. We report the case of a patient who had an inferior vena cava (IVC) stent placed previously as part of the management for BCS, and who subsequently required TIPS. The TIPS tract was created through the strut of the previously placed IVC stent; the TIPS stent was placed after dilatation of the liver parenchyma as well as the strut of the IVC stent. This novel technique of "strutplasty" of a previously placed stent as part of TIPS has not been reported in the literature.

Psychometric analysis of the Functional Independence Score in Haemophilia (FISH).

Joint morbidity in haemophilia has traditionally been measured using clinical and radiological scores. There have been no reliable, validated tools for the assessment of functional independence in persons with haemophilia till recently. The Functional Independence Score in Haemophilia (FISH) has been developed as a performance based assessment tool to address this need. The FISH is designed to measure the patient's independence in performing activities of daily living (grooming and eating, bathing and dressing), transfers (chair and floor), and mobility (walking, step climbing and running). On assessment of its psychometric properties in 63 patients with haemophilia (mean age 14 years), FISH was found to have good internal consistency (Cronbach's alpha of 0.85). It had moderate correlation with the World Federation of Hemophilia clinical score (r = -0.61), and a correlation with the Pettersson score of -0.38. It had good correlation with other self-rated functional scores, such as the Stanford Health Assessment Questionnaire (r = -0.75); the Western Ontario and McMaster Universities Osteoarthritis Index (r = -0.66) and the Haemophilia Activities List (HAL) (r = -0.66). It had good reliability with a pooled intra class correlation of 0.98. On assessing responsiveness following treatment of flexion deformities of the knee in 12 patients, the FISH showed significant changes in the score with a standardized responsiveness mean of -1.93. In conclusion, the FISH was found to be a reliable and valid tool with good internal consistency and responsiveness to therapy, for the assessment of functional independence in persons with haemophilia.

Anatomical variations of the internal jugular veins and their relationship to the carotid arteries: a CT evaluation.

The right internal jugular vein (IJV) is a common vessel to obtain venous access. It is important to have a clear understanding of the anatomy of the IJV and its relationship to the common carotid arteries (CCA) to avoid inadvertent arterial puncture. This study aims to objectively evaluate the variations in the anatomy of IJV and its relation to the CCA. A total of 176 right and left IJV were retrospectively evaluated using CT imaging with the CentraRad Diagnostic Viewer Version 4.09.0190 (CDN Telemedicine Solutions, Wollongong, Australia). The data were recorded and analysed. The right IJV (80.5%) was more often larger than the left IJV. With reference to the CCA, 85.2% of the IJV were found in the lateral position, 12.5% anteriorly, 1.1% medially and 1.1% posteriorly. Seven IJV were found to be hypoplastic, and in one case this was seen bilaterally in both the right and left IJV. The maximum depth of IJV from the skin was 27.9 mm. More than half (69.5%) of the IJV were less than 1 mm from the carotids. Computed tomography is an excellent method to delineate the anatomy of IJV. Variations in the anatomy of the IJV and their correlation to the CCA are common.