Venous Malformation and Localized Intravascular Coagulopathy in Children.

Aim Localized intravascular coagulopathy (LIC) has been described in adults with venous malformation (VM) but rarely reported in children. This study aims to determine the prevalence of LIC in children with VM and associated risk factors. Methods Patients younger than 18 years with VM from 2010 to 2014 were reviewed. Diagnosis was confirmed by Doppler ultrasound and/or magnetic resonance imaging. Demographics data and VM characteristics including volume, site, extension, painful symptoms, and palpable phleboliths were studied. Plasma D-dimer level of greater than 500 ng/mL was considered as abnormal. Results Total 24 children were included, of whom 8 were boys. Median age of presentation was 9 months (range: 0-12 years). Head-and-neck VM occurred in 17 (70.8%) patients and 3 (12.5%) had multifocal lesions. Seven (29.2%) patients had VM volume greater than 10 mL. Five (20.8%) patients had painful symptoms. Palpable phleboliths were found in two patients. Plasma D-dimer was raised in eight cases (33.3%). One patient with Klippel-Trenaunay syndrome (KTS) had D-dimer level of 5,000 ng/mL. Raised D-dimer was found in 23.5% of small VM (volume < 10 mL) and 57.1% of large VM (p = 0.167). D-dimer was significantly raised in multifocal VM (p = 0.028) and showed increasing trend in lesions with palpable phleboliths (p = 0.101). All patients had sclerotherapy performed with indications (cosmesis 41.7%, enlarging lesion 29.2%, pain 20.8%, bleeding 8.3%). Perioperatively, bolus intravenous fluid and mannitol were given to selected patients. All patients had VM volume reduction after sclerotherapy. There were no major thromboembolic complications. Conclusion LIC with raised D-dimer level occurred in one-third of pediatric VM. It was more common in large, multifocal VM and in those with palpable phleboliths or KTS.

Angiographic factors associated with haemorrhagic presentation of brain arteriovenous malformation in a Chinese paediatric population.

OBJECTIVE: To identify specific angiographic factors associated with haemorrhagic presentation of brain arteriovenous malformation in Chinese paediatric patients. DESIGN: Retrospective cross-sectional observational study. SETTING: Four locoregional tertiary neurosurgical centres in Hong Kong: Queen Elizabeth Hospital, Tuen Mun Hospital, Kwong Wah Hospital, and Pamela Youde Nethersole Eastern Hospital. PATIENTS: Patients aged 18 years or younger who underwent pretreatment digital subtraction angiography for brain arteriovenous malformation between 1 January 2005 and 31 July 2013 were included. Patients were divided into haemorrhagic and non-haemorrhagic groups based on the initial presentation. Pretreatment digital subtraction angiographies were independently reviewed by two experienced neuroradiologists. MAIN OUTCOME MEASURES: The following parameters were evaluated for their association with haemorrhagic presentation by univariate and multivariate analyses: nidus location, nidus size, nidus morphology (diffuse or compact); origin and number of arterial feeders; venous drainage; number of draining veins; presence of aneurysms, venous varices, and venous stenosis. RESULTS: A total of 67 children and adolescents (28 male, 39 female) with a mean age of 12 years were included. Of them, 52 (78%) presented with haemorrhage. Arteriovenous malformation size (P=0.004) and morphology (P=0.05) were found to be associated with haemorrhagic presentation by univariate analysis. Small arteriovenous malformation nidus size and diffuse nidal morphology were identified as independent risk factors for haemorrhage by multivariate analysis. CONCLUSION: Smaller arteriovenous malformation size and diffuse nidal morphology are angiographic factors independently associated with haemorrhagic presentation. Bleeding risk is important in determining the therapeutic approach (aggressive vs conservative) and timeframe, particularly in paediatric patients.

Percutaneous cementoplasty of osteolytic metastases induces immediate and long-lasting pain relief in oncological patients.

OBJECTIVE: To evaluate the clinical efficacy of percutaneous cementoplasty with respect to pain relief in patients with refractory painful bone metastases. DESIGN: Case series. SETTING: Regional hospital, Hong Kong. PATIENTS: All oncological patients with painful bone metastases despite conventional treatment seen between October 2006 and May 2010 were recruited. INTERVENTIONS: Cementoplasty with or without radiofrequency ablation. MAIN OUTCOME MEASURES: Pain score before and after the procedure. RESULTS: In all, 12 patients with 13 lesions received cementoplasty. Two patients were excluded from the analysis because of inadequate documentation of pain score due to rapid disease progression. For the remaining 10 patients with 11 metastases, the primary sites were the lung (n=3), renal cell carcinoma (n=2), rectum (n=2), pancreas (n=1), multiple myeloma (n=1), and soft tissue sarcoma (n=1). The locations of the metastatic lesions were scapula (n=1), thoracic vertebrae (n=1), lumbar vertebrae (n=3), and pelvic bones (n=6). Eight lesions were treated by cementoplasty alone, whereas the other three associated with large soft tissue components had radiofrequency ablation followed by cementoplasty in a single setting. Immediate or near-immediate pain relief after treatment was achieved in 10 out of 11 lesions; the median pain score was 5 before treatment and decreased to 2 a week after treatment (P=0.039). In all lesions for which the pain was successfully controlled in the first week, the palliation effect persisted at subsequent follow-ups. The median follow-up period for these patients was 16 weeks, and the longest pain-relieving effect was at least 9 months. CONCLUSION: In our experience, cementoplasty with or without radiofrequency ablation achieves satisfactory and long-lasting pain control in oncological patients with bone metastases. This is the first local study to describe the effect of cementoplasty for pain relief. Patients with painful bone metastases that are refractory to conventional treatments can benefit from cementoplasty, which should therefore be considered when conservative treatments fail.

Transcatheter embolisation of intrahepatic arteriovenous shunts in patients with hepatocellular carcinoma.

This paper assesses the feasibility of transcatheter embolisation of arteriovenous shunts in patients with hepatocellular carcinoma, and reviews available embolic agents, based on our experience and a literature review. From 2001 to 2007, 11 patients with unresectable hepatocellular carcinoma and significant arteriovenous shunts underwent transcatheter embolisation of liver arteriovenous shunts. The age range was 36 to 80 years. A total of 17 embolisations were performed using different embolic agents including absolute ethanol (n=11), histoacryl (n=1), coils (n=2), and polyvinyl alcohol particles (n=1). We reviewed the degree of shunt occlusion and the clinical outcomes. There were 15 arteriovenous shunts. Nine (60%) were arterioportal venous shunts and six were arteriohepatic venous shunts. Two were classified as 'simple' types, according to our protocol, and 13 were 'complex' types. More than 80% occlusion was achieved in 80% of the shunts. In the simple shunts, coil embolisation achieved complete occlusion. In complex shunts with multiple feeders and draining veins, liquid or particulate agents were required to achieve satisfactory occlusion. Managing arteriovenous shunts with embolisation was feasible. The choice of embolic agent should be based on good understanding of the underlying mechanism of the shunts and their angio-architecture.

Metabolic syndrome, endothelial injury, and subclinical atherosclerosis in patients with systemic lupus erythematosus.

OBJECTIVES: To study the link between metabolic syndrome (MetS), endothelial injury, and atherosclerosis in patients with systemic lupus erythematosus (SLE). METHODS: Consecutive SLE patients without a history of arterial thrombosis were screened for atherosclerosis at the carotid and coronary arteries by B-mode ultrasound [intima-media thickness (IMT)] and multidetector computed tomography (MDCT) scan (Agatston calcium scores), respectively. Plasma levels of homocysteine, high-sensitivity C-reactive protein (hsCRP), soluble vascular cell adhesion molecule (sVCAM)-1, P-selectin, and soluble thrombomodulin (sTM) were assayed. Patients were stratified according to the National Cholesterol Education Program (NCEP) Adult Treatment Panel III (ATP III) criteria for MetS, using the Asian criteria for abdominal obesity. Risk factors for atherosclerosis were studied. RESULTS: Of the 123 SLE patients (93% women; age 47.9+/-11 years; SLE duration 10.9+/-7.0 years) studied, 20 (16.3%) had MetS. The prevalence of MetS in the SLE patients was significantly higher than in 492 age- and sex-matched healthy controls (9.6%; p=0.03). Coronary calcification and abnormal carotid IMT were detected in 38 (31%) and 72 (59%) of SLE patients, respectively. Patients with MetS had a significantly higher Agatston score (69.5+/-95 vs. 16.4+/-57; p=0.03) and a numerically higher carotid IMT (p=0.43) than those without. In a logistic regression model, the MetS [odds ratio (OR) 3.11, 95% confidence interval (CI) 1.01-9.59, p=0.049] was associated with coronary atherosclerosis after adjustment for age and other risk factors. In addition, patients with MetS had significantly higher levels of hsCRP (p=0.002), homocysteine (p=0.03), and sTM (p=0.01). CONCLUSIONS: The MetS is more prevalent in SLE patients than the general population and is associated with endothelial injury and coronary atherosclerosis. More aggressive control of risk factors is justified in these patients.

Radiation-induced peripheral nerve neurofibromata in a patient receiving hypofractionated radiation therapy.

Radiation-induced peripheral nerve tumor, in particular a benign entity such as a neurofibroma, is rare, with only a few cases being reported so far. We demonstrate a case of radiation-induced neurofibromata along the left cervical nerve roots in a man with a background of localized targeted hypofractionated radiation therapy as adjuvant treatment for left cervical nodal metastasis complicating nasopharyngeal carcinoma. The toxicity of high-dose radiation in a hypofractionated regime is also stressed.

Can a warning leak in a patient with unruptured aneurysm mask an underlying gastrointestinal pathology? A case report.

Aggravating headache accompanied by nausea and epigastric discomfort suggesting a warning leak in a 39-year-old woman with a giant thrombosed intracranial aneurysm prompted us to undertake coiling of the aneurysm. After uneventful coil embolization of the aneurysm, collapse of the lung related to bronchospam developed, and was found to have a gastrointestinal pathology which had gone undetected before the procedure. Despite its rarity, gastrointestinal pathology mimicking warning leak should have been considered in a patient with a warning leak sign.

An in vivo study of common carp (Cyprinus carpio L.) liver during prolonged hypoxia.

Hypoxia induced apoptosis has been studied extensively in many mammalian cell lines but there are only a few studies using whole animal models. We investigated the response of the intact liver to hypoxia in a hypoxia tolerant fish, the carp (Cyprinus carpio, L). We exposed carp to hypoxia for up to 42 days, using oxygen level (0.5 mgO(2)/L) that were slightly higher than the critical oxygen level of carp. There was extensive DNA damage in liver cells, especially during the first week of exposure, indicated by a massive TUNEL signal. However there was no change in cell proliferation, cell number or size, no increase in caspase-3 activity, no increase in single stranded DNA and this, combined with a number of other observations, led us to conclude there was no increase in apoptosis in the liver during hypoxia. There was up-regulation of some anti-apoptotic genes and proteins (Bcl-2, HSP70, p27) and down-regulation of some pro-apoptotic genes (Tetraspanin 5 and Cell death activator). The cells appeared to enter cell cycle arrest, presumably to allow repair of damaged DNA. As there was no change in cell proliferation and cell number, the damaged cells were not entering apoptosis and must have recovered during prolonged hypoxia.

3D time of flight MR angiography: acquisition with small field of view and low phase encodes.

The aim of the study was to evaluate and compare the image quality of the 3D TOF MRA acquired with a small FOV and low phase encodes with those MR angiographic images acquired with standard pulse sequence parameters. Twenty patients who were referred to our institution for MR imaging of the brain and strictly satisfied the selection criteria were included in this study. Apart from the routine protocol for MR imaging of the brain, 3D TOF MRA of the circle of Willis with a small FOV and a standard FOV were performed. The image quality of all MRA was evaluated by two independent observers who were blind to the pulse sequence parameters. From the standard FOV MRA, 22.5, 12.5, and 5% of the patients were graded as mild, moderate, and severe stenosis of the internal carotid artery, respectively. On the contrary, no apparent stenosis was observed from the small FOV MRA with low phase encodes. Regarding the reduction in MR artifacts and acquisition time achieved with the small FOV 3D TOF MRA with low phase encodes, this might be a useful MR angiographic technique to be used in routine clinical practice.

Management of a direct carotid cavernous fistula caused by rupture of a cavernous aneurysm previously embolized with coils.

SUMMARY: The development of a high-flow carotid-cavernous fistula from the rupture of a large cavernous aneurysm successfully embolized by coils is rare. A 50-year-old male patient developed a high-flow carotid-cavernous fistula 48 hours after successful coiling of a large left cavernous aneurysm, presumably due to rupture of a focal dissection at or close to the neck of the aneurysm. He initially responded to daily selfcompression of the left common carotid artery, but the fistula recurred. After failing to approach the fistula site via transvenous route, balloon trapping of the internal carotid artery was planned. Prior to its placement for functional occlusion test, the detachable balloon slipped into the fistula site and occluded it. It was thereafter detached in this position. The sequence of events, a large cavernous aneurysm spontaneous ruptured after coiling, suggested dissecting process or disease.We address in the report the complexity of the endovascular management of this rare association.

Secondary spontaneous thrombosis of a giant aneurysm located distally on a feeding artery after embolization of an associated arteriovenous malformation.

SUMMARY: Giant aneurysm located in the distal part of the feeding artery associated with a cerebral arteriovenous malformation is rare and the treatment is clinically challenging. We report the spontaneous and complete thrombosis of a flow-related giant aneurysm immediate up-stream to a cerebral arteriovenous malformation by embolization of that malformation alone in a patient presenting with complex partial seizure and no history of intracranial haemorrhage. We obviated the need to directly intervene on the giant aneurysm, thus reducing unnecessary procedure related risks to the patient. Follow up one year later confirms the thrombosis and show shrinkage of the mass. The patient is asymptomatic.

Mechanical thrombectomy in inferior vena cava thrombosis after caval filter placement: a report of three cases.

Inferior vena caval (IVC) filter thrombosis in patients with contraindications to anticoagulant therapy is a difficult and challenging clinical problem. We report our experience in treating three such patients using a mechanical thrombectomy device, which resulted in rapid symptomatic relief until anticoagulant therapy could be safely introduced.