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Objective: To explore the curative effect of surgery and selerotherapy for massive venous malformations of the tongue., Methods: From January 2005 to December 2014, subtotal resection or debulking for 15 cases of massive venous malformation in the tongue was undertaken with multiple sessions of pre- and post-operative injection therapy of pingyangmycin, lauromacrogol and absolute ethanol., Results: All signs associated with the lesions including eating, sleep and speech disorders disappeared after treatment. Complete or near complete resolution was achieved in 9 cases, and a significant reduction in size in a further 6 cases after surgical excision and peri-operative sclerotherapy., Conclusions: For massive venous malformations of the tongue, surgical excision combined with multiple sessions of sclerotherapy is a good treatment option.

Objectives: Ultra sound colored duplex sonography is the preferred method in diagnosing chronic venous disease. Data in children on incidence, indications, and results are lacking., Methods: From the total of 9180 duplex investigations performed in our hospital from 2009 to 2012, data on indication and results of the investigation as well as patient characteristics were evaluated retrospectively for the proportion of pediatric patients., Results: Duplex investigations were performed 49 times in 38 children (6-18 years), with an average of 1.3 times (1-6 times) per child. Forty percent showed abnormalities: 17 times deep venous thrombosis was suspected; deep venous thrombosis was objectified in 18%. In the 21 investigations performed for varicosis-related complaints, varicose veins or venous malformations were objectified in 57%. Edema was never a symptom of chronic venous disease., Conclusions: Duplex investigation is not often performed in children. In children with established deep venous thrombosis, a family history with deep venous thrombosis is common. In general, edema was not seen in children with varicose veins and, therefore, does not seem a reliable clinical sign at young age., (© The Author(s) 2014.)

Background: The incidence of localized intravascular coagulopathy (LIC) in venous malformations varies with lesion size and location, as well as the presence of palpable phleboliths. The development of LIC can cause pain and hemorrhage and can progress to disseminated intravascular coagulopathy (DIC) and thromboembolic disease resulting in death in some cases. Early recognition of LIC can relieve symptoms and prevent progression to life-threatening complications., Objective: The aim of this work was to identify MRI features of venous malformation associated with LIC. We hypothesized that venous malformations with larger capacitance, slower flow and less physiological compression (greater stasis) were more likely to be associated with LIC., Materials and Methods: In this HIPAA-compliant and IRB-approved study, we retrospectively reviewed clinical records and MRI for consecutive patients undergoing evaluation of venous malformations at our multidisciplinary Birthmarks and Vascular Anomalies Center between 2003 and 2013. Inclusion required consensus diagnosis of venous malformation and availability of laboratory data and MRI; patients on anticoagulation or those previously undergoing surgical or endovascular treatment were excluded. LIC was diagnosed when D-dimer exceeded 1,000 ng/mL and/or fibrinogen was less than 200 mg/dL. Two board-certified radiologists assessed the following MRI features for each lesion: morphology (spongiform vs. phlebectatic), presence of phleboliths, size, location (truncal vs. extremity), and tissue type(s) involved (subcutis, muscle, bone and viscera). Univariate logistic regression analyses were used to test associations between LIC and MRI findings, and stepwise regression was applied to assess the significance of the individual imaging predictors., Results: Seventy patients, 37 with LIC, met inclusion criteria during the 10-year study period (age: 14.5 +/- 13.6 years [mean +/- standard deviation]; 30 male, 40 female). Both elevated D-dimer and low fibrinogen were associated with the presence of phleboliths, larger lesion sizes and visceral involvement on MRI (all P < 0.05). In stepwise regressions, lesion size (P < 0.001), the presence of phleboliths (P = 0.005) and lesion morphology (P = 0.006) were all significant predictors of LIC., Conclusion: LIC is associated with larger lesion size, visualized phleboliths, truncal location and spongiform morphology on MRI in venous malformations, suggesting that lesions with larger capacitance, slower flow and less physiological compression are more likely to be associated with coagulopathy.

Purpose: To report clinical and radiographic outcomes of patients with venous malformations (VMs) treated with bleomycin foam., Materials and Methods: Twenty patients (age, 2-68 y) presented with symptoms of swelling (n = 19; 95%), pain (n = 14; 70%), and bleeding (n = 4; 20%). Lesions were located in the head and neck in 17 patients (85%), extremities in two (10%), and mediastinum in one (5%). Twenty-seven embolizations were performed, with a mean of 1.7 ± 1.0 treatments per patient (range, 1-4). An average of 0.45 ± 0.4 U/kg of bleomycin foam was used per procedure, with a range of 0.1-2.3 U/kg., Results: All procedures were technically successful with no intraprocedural complications. Mean follow-up was 66 days ± 80, with a range of 4-403 days. Postprocedure complications were minor in 6 of 27 procedures (22%) and major in 2 of 27 procedures (7%). All 20 patients (100%) reported improvement in their symptoms after a single treatment session. Postprocedural magnetic resonance (MR) imaging demonstrated volume reduction of treated lesions in 13 of 14 patients (93%), with a mean lesion volume reduction of 66% ± 21. Enhancement on MR imaging after treatment was decreased in 11 of 14 patients (79%), increased in two (14%), and stable in one (7%). T2 signal intensity on MR imaging after treatment was decreased in 12 of 14 patients (86%) and stable in two (14%)., Conclusions: The use of bleomycin foam for the percutaneous treatment of VMs is safe and effective. Foaming bleomycin may be used to address the dose limitations of the liquid., (Copyright © 2015 SIR. Published by Elsevier Inc. All rights reserved.)

Background: Venous malformations of the limbs are congenital low-flow vascular anomalies. A treatment is reasonable if they are symptomatic or if a progressive lesion may affect functional structures., Objective: The purpose of this work is the presentation of clinical results after treatment of venous malformations of the limbs according to the standard algorithm used at the University Hospital of Tuebingen, Germany., Patients and Methods: Between January 2008 and June 2015, patients with venous malformations of the limbs were subjected to either percutaneous sclerotherapy or surgical excision according to this treatment algorithm. Remaining symptoms such as pain level and disturbing appearance before and after treatment as well as overall satisfaction was assessed the earliest 3 months after last treatment., Results: Thirty-nine patients with venous malformations of the limbs were subjected to either 1 or more percutaneous sclerotherapies (n = 19) or surgical excision (n = 21). There were no serious complications that needed surgical revision. There was a statistically significant reduction in the pain level and disturbing appearance after both sclerotherapy and surgical excision (p < .05) in 30 cases in total. The overall treatment satisfaction was rated 7.9/10 and 8.8/10 after sclerotherapy and surgical excision, respectively., Conclusion: A low complication rate with a high patient satisfaction could be achieved after this treatment algorithm for venous malformations of the limbs.

Phacomatosis pigmentovascularis (PPV) is a rare genodermatosis characterized by the co-existence of an extensive vascular and a pigmentary nevus with or without extracutaneous manifestations. We report two such rare cases. The first is a 3-year-old boy exhibiting a rare association of cutis marmorata telangiectatica congenita with aberrant dermal melanocytosis along with hypospadias and melanosis oculi (traditionally classified as PPV type Vb or phacomatosis cesiomarmorata - Happle's classification). The other patient is a 5-year-old boy with Sturge-Weber syndrome, Klippel-Trenaunay syndrome, aplasia of iliac, femoral, and popliteal veins and congenital heart disease, associated with aberrant dermal melanocytosis and melanosis oculi (also classified as PPV type IIb or phacomatosis cesioflammea). These sporadic cases display a unique constellation of additional, previously unreported systemic associations, which will further expand the clinical spectrum of phacomatosis pigmentovascularis.

Objective: The purpose of this study was to determine whether phlebographic features can be used to discriminate adult from adolescent varicocele., Materials and Methods: Left and right internal spermatic venograms of 191 adolescents (< 17 years) and 224 adults (≥ 25 years) were anonymized and evaluated. Phlebographic radioanatomic features (valves, duplications, collaterals, and classifications) were compared and analyzed with univariate tests., Results: Insufficiency of the left internal spermatic vein (ISV) was confirmed in 409 of the 415 (99%) patients. Adults had no spontaneous opacification of the ISV during venography twice as frequently as adolescents (p = 0.001), a complex outflow into the renal vein 2.2 times as often (p = 0.021), and significantly more collaterals (p = 0.030). Adolescents had a significantly lower number of competent valves and significantly more instances of nutcracker phenomenon (p = 0.001). According to the Bähren classification, the distribution of the types of ISVs was significantly different between adults and adolescents (p = 0.009). Insufficiency of the right ISV was encountered 2.4 times as frequently in adults as in adolescents (p < 0.001). In adults the maximum diameter of the ISV was significantly larger (p = 0.023). Bilateral ISV insufficiency was 2.2 times as frequent in adults (p < 0.001) as in adolescents., Conclusion: Left-sided varicoceles in adults are distinct from those in adolescents. In adults, reflux is likely to be induced via collateral pathways, whereas in adolescents congenital venous abnormalities are predominantly present. The higher prevalence and the greater diameter of a right insufficient ISV in adults, combined with the absence of venous anatomic differences, supports the hypothesis that right-sided varicocele is an evolutive disease.

A case of complete angiographic suppression of the artery of Adamkiewicz and anterior spinal artery in a patient with a spinal epidural arteriovenous fistula (AVF) is reported. Slow flow AVFs typically present with progressive myelopathy secondary to spinal venous hypertension (SVH). The lack of a normal venous phase during angiography and its restoration after treatment is commonly observed with these lesions, yet a similar phenomenon seems exceptional at the arterial level. Right T11 intercostal artery angiograms obtained before and after treatment of a left L4 epidural AVF documented the initial suppression of the artery of Adamkiewicz and anterior spinal artery, and their normal appearance immediately after correction of the SVH by embolization. This report confirms that SVH can angiographically suppress prominent and functionally important spinal arteries, re-emphasizing the potential role played by secondary arterial changes in SVH induced myelopathy. This hemodynamic phenomenon also represents a potential pitfall during diagnostic and therapeutic endovascular procedures., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Background and Purpose: Developmental venous anomalies are considered variants of venous development that, in and of themselves, are of little clinical import. A possible association between intrinsic brain tumors and developmental venous anomalies has been suggested, but a rigorous investigation has not been performed. The aim of this study was to assess any association between developmental venous anomalies and intrinsic brain neoplasms., Materials and Methods: A radiology report text search of terms used to describe developmental venous anomalies was performed on a study population of 580 patients with primary intracranial neoplasms and on a control population of 580 patients without neoplasms from the same time period. All positive results were reviewed to confirm that the report was describing a developmental venous anomaly, and the imaging examination was reviewed to confirm the diagnosis., Results: Fifty-nine of the 580 subjects with brain tumors (10.17%) had a developmental venous anomaly identified by report and confirmed on review of the imaging. Thirty-one of the 580 controls (5.34%) had a developmental venous anomaly identified by report and confirmed on review of the imaging (P = .003). No statistically significant difference was noted in the prevalence of developmental venous anomalies among tumor types. No developmental venous anomaly drained the vascular territory of the tumor, and there was no correlation between the location of the developmental venous anomaly and the location of the neoplasm., Conclusions: The prevalence of developmental venous anomalies in this pediatric population with intracranial primary neoplasms is significantly greater than in those without neoplasms, suggesting an association that may be related to shared causative factors or susceptibilities to the development of these 2 separate entities., (© 2015 by American Journal of Neuroradiology.)

Ureteropelvic junction obstruction due to intrinsic causes is often diagnosed antenatally during routine ultrasonography. Cases of extrinsic obstruction often present later and symptomatically, during childhood. We describe the rare case of an 8-year-old boy with a 2-day history of severe left flank pain, no fevers, and Society of Fetal Urology grade 3 hydronephrosis on ultrasonography. Laparoscopic dismembered pyeloplasty revealed a left ureteropelvic junction obstruction secondary to a large fibroepithelial polyp in the proximal ureter with a concomitant anterior crossing vein. We also provide a focused review of the pertinent published literature., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Purpose: The molecular pathophysiology of venous malformations (VMs), which are a type of vascular malformation, is poorly understood. Until now, it is known that VM lesions are related to the process of angiogenesis. Because angiogenesis is induced under hypoxic conditions, hypoxia is thought to be important in VM lesion formation. Therefore, we examined the implications of hypoxia on the biological behavior of VM vascular smooth muscle cells (VSMCs). In doing so, we investigated the expression patterns of hypoxia-inducible factor-1α (HIF-1α), which plays a key role in hypoxia-induced angiogenesis, to provide a further understanding of the molecular mechanisms involved in VM., Methods: Vascular smooth muscle cells from 5 normal veins and 5 VM lesions were cultured under moderate hypoxic conditions (3% O2, 5% CO2). The effects of hypoxia on HIF-1α expression were measured by immunocytochemical staining, reverse transcription-polymerase chain reaction, and real-time reverse transcription-polymerase chain reaction., Results: Overall, the expression of HIF-1α in cells was high after exposure to hypoxia for 6 or 12 hours, but decreased after 24 hours of hypoxia. HIF-1α expression in VM VSMCs was 2 times higher than that in normal VSMCs. Immunocytochemically, HIF-1α was mainly located in the nucleus and the intensity in VM VSMCs was stronger after 6 and 12 hours of hypoxia when compared to the expression pattern of HIF-1α in VSMCs from normal tissue. This suggested that VM tissue is more susceptible to the effects of hypoxia than normal tissue., Conclusions: These results indicate that the high expression of HIF-1α in VM VSMCs under hypoxic conditions could be an important factor for stimulating downstream angiogenesis in VM. Furthermore, the results of this investigation could provide the basis for future studies of VM pathophysiology, and ultimately lead to the development of new therapeutic approaches.

Objective: To investigate the safe and effective treatment for painful venous malformation (VM) in limbs., Method: (1) 97 cases with painful VM underwent MRI to detect the location of VM, as well as its size and structure, its relationship with the surrounding tissue. Statistical analysis was also performed. (2) The embolic agent (ethanol) was first injected to embolize the draining vessels of VM, then the Polidocanol plus Methotrexate (MTX) was followed to keep the embolization effect on VM. The therapeutic effect was observed and analyzed., Results: From January 2010 to January 2012, 97 patients with painful VM were treated. A Spearman correlation analysis showed no significant correlation between symptoms of pain and lesion growth, volume, or MRI grades (P > 0.05). The lesions in the muscle space are more likely to have the symptoms of pain (P < 0.01), followed by the lesions in the muscle, then the lesions in the joint and subcutaneous tissue. The pain relieve percentage was 95.9% (93/97) after one time embolic sclerotherapy. No severe complication, such as distant embolization, nerve damage, or muscle atrophy happened. No pain reoccurrence happened after 0.5-1.5 years of follow-up period., Conclusions: The treatment of embolic scleratherapy is minimal invasive, safe and effective for painful VM with stable results.

Management of patients with complex vascular anomalies (VAs) is often associated with significant morbidity and mortality because of the lack of effective treatment modalities that may lead to significant improvement of the disease and/or healing. Recently, reports of treatment of patients with complex VAs with sirolimus revealed encouraging results. Sirolimus inhibits the mammalian target of rapamycin, which acts as a master switch of numerous cellular processes. We report a successful use of sirolimus for the treatment of a patient with a complex CLVM of the trunk and the right lower extremity believed to be untreatable. Our patient had 44 hospitalizations during the 10-year period, with various unsuccessful treatments and continuous deterioration of his clinical condition, ending up in a wheelchair. His condition reversed to normal everyday activities 9 months after initiation of sirolimus therapy. We conclude that sirolimus is a very promising therapeutic option for children with complex VAs of capillary-lymphatico-venous type., (Copyright © 2015 by the American Academy of Pediatrics.)

Objective: To explore the therapeutic effect of radiofrequency ablation for tongue venous malformation( VM)., Methods: From July 2013 to July 2014, 30 cases with tongue VM (local or diffuse) were retrospectively analyzed. 23 cases underwent radiofrequency ablation treatment. The radiofrequency electrode tip(0. 5 mm in diameter) was inserted into the lesion 1 mm below the bottom with 25 W in power and 15-30 s of pulse. The treatment was repeated when the electrode tip was removed back every 1 mm. Multi-point treatment was performed., Results: 15 cases with unilateral VM were completely healed after one-stage radiofrequency ablation. 8 cases with bilateral VM received two-stage radiofrequency ablation with a 3-6 months of interval. Among the 8 cases, completely healing was achieved in 5 cases, partial VM residue happened in 3 cases due to its diffuse lesion and reservation of tongue function. 23 cases were followed up for 3 month to 1.5 years. Good cosmetic and functional results was achieved in 20 cases with no relapse. Partial VM residue was left in 3 cases., Conclusions: Radiofrequency ablation can effectively treat tongue VM with minimal morbidity and good cosmetic appearance. It also avoids the disadvantages of surgery.

Congenital anomalies of the thoracic veins are rare yet important developmental abnormalities, usually classified into systemic and pulmonary. They may be encountered incidentally; as such the radiologist must be aware of their imaging presentation and clinical relevance. Furthermore, to understand these anomalies, knowledge of the embryological development and of the normal anatomy of the thoracic veins is required. In the age of non-invasive imaging modalities, magnetic resonance is paramount for the characterization of these developmental abnormalities., (Copyright © 2015 Mosby, Inc. All rights reserved.)

Background: Lehmann et al., [2003, 2006] have documented two different substitutions at position 486 of the BMPR1B gene which resulted in a phenotype of brachydactyly A2 [MIM 112600] or brachydactyly C with symphalangism [MIM 113100]., Methods: In this article we report a family of Polish extraction with a novel mutation: c.1457G>T (R486L) which segregated with a complex brachydactyly. Clinical and radiological data are presented and details of previously reported patients with a pathogenic change of an amino acid at position 486 of the BMPR1B gene are summarized., Conclusion: Our data extends the previously known mutational and radiological spectrum associated with mutations in the BMPR1B gene and confirms the existence of a universal hotspot in the BMPR1B gene in this distinctive autosomal dominant brachydactyly disorder. It is of interest that an affected female in the Polish family had a severe congenital malformation of the venous system in addition to her digital anomalies. This observation raises the possibility of disturbance of embryonic angiogenesis by specific mutations in BMPR1B., (© 2015 Wiley Periodicals, Inc.)

Background: Sclerosing foam is recommended to treat venous malformations (VMs). However, determining the precise dosage for satisfactory efficacy and few side effects are still challenging., Objective: To develop a new method of preparing sclerosing foam by adding contrast agent to treat patients with VMs visibly and precisely under radiologic guidance., Materials and Methods: Sclerosant (polidocanol) and contrast agent (iopromide) were mixed to prepare a new sclerosing foam according to Tessari method, and different ratios of the 2 materials were used to study the stability (half-life) of the produced foams. X-ray absorption of the radiopaque foams was observed during patient treatment with digital subtraction angiography (DSA)., Results: The foams generated with the new method demonstrated a high x-ray absorption under DSA. The foam half-life among groups varied. The ratio of iopromide/polidocanol at 1:2 had a significantly longer half-life than the 1:1 or polidocanol-only group. The 1:2 group had a relative high x-ray absorption and was radiopaque to allow visualization of the sclerosing foam and lesion with DSA., Conclusion: Mixing contrast agent with sclerosant to prepare radiopaque foams is a new method for treating complex VMs with DSA, and a ratio of 1:2 is recommend for polidocanol and iopromide.

Coronary venous anomaly is a less studied and rarely encountered subject in contrast to the coronary arterial system. In this article, we present 2 cases of anomalous great cardiac veins that drain into the left atrium individually., (Copyright © 2015 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.)

Objective: To assess and compare the effectiveness of the two sclerosing agents (95% alcohol and Bleomycin) for the treatment of head and neck venous malformation (VM)., Material and Method: The authors retrospectively reviewed our experience in treating VM of the head and neck region using two sclerosing agents, 95% alcohol (November 2001 to June 2008) and bleomycin (July 2008 to July 2010). Patients' demography (age, sex), lesion number location, type (focal/extensive), and characteristic features (cystic/tubular/mixed) were recorded. The treatment outcome was determined by decrease in size of VM in photographs taken at one month and at final clinical follow-up. These were analyzed by two radiologists using visual rating scale (worst or not improved, <50%, 50-75%, >75% of size reduction). One-way Anova test (p < 0.1) was used to show the differences in treatment effectiveness of the two sclerosing agents at short- and long-term intervals., Results: Thirty-three patients, age ranged from 11 to 62 years (mean 25.1 years), with 27 female and six male patients were included in this study. The majority of patients were less than 16 years (17 patients, 51%). The 43 lesions were categorized as 28 VMs were focal (65.1%), 15 (34.9%) diffuse, and30 (69.7%) were of the mixed type. Sixteen lesions were treated with 95% alcohol, 23 lesions with bleomycin, and four lesions with a combination of the two sclerosants. The range of number of procedures was 1 to 16 (mean 3.76 procedures per patient) for alcohol, and 1 to 5 (mean 2.27 procedures per patient) for bleomycin. The cumulative dose ofsclerosant used was 101 ml for alcohol and 32.11 mg for bleomycin. Total follow-up at 1-month and at final was 43/43.(100%) and 35/43 (81.4%) respectively. Mean follow-up interval was 14.7 months. Differences in size reduction after treatment by different sclerosing agents were found. At more than 1-year follow-up, those treated with bleomycin gained graded 3 (> 75%) size reduction more than treated by 95% alcohol. No VM treated with 95% alcohol obtained grade 3 of size reduction at 1-year follow-up. Multiple regression analysis showed VM's favorable character for bleomycin treatment by decreasing mixed, cystic, and tubular Pediatrics had relatively more benefit with bleomycin treatment., Conclusion: Sclerotherapy using either alcohol or bleomycin is an effective treatment for VMs. Different treatment outcomes were significant at long-term with group of VM those treated with bleomycin but not at short-term (p < 0.1).

Venous malformations (VMs) are the most common vascular developmental anomalies (birth defects) . These defects are caused by developmental arrest of the venous system during various stages of embryogenesis. VMs remain a difficult diagnostic and therapeutic challenge due to the wide range of clinical presentations, unpredictable clinical course, erratic response to the treatment with high recurrence/ persistence rates, high morbidity following non-specific conventional treatment, and confusing terminology. The Consensus Panel reviewed the recent scientific literature up to the year 2013 to update a previous IUP Consensus (2009) on the same subject. ISSVA Classification with special merits for the differentiation between the congenital vascular malformation (CVM) and vascular tumors was reinforced with an additional review on syndrome-based classification. A "modified" Hamburg classification was adopted to emphasize the importance of extratruncular vs. truncular sub-types of VMs. This incorporated the embryological ongm, morphological differences, unique characteristics, prognosis and recurrence rates of VMs based on this embryological classification. The definition and classification of VMs were strengthened with the addition of angiographic data that determines the hemodynamic characteristics, the anatomical pattern of draining veins and hence the risk of complication following sclerotherapy. The hemolymphatic malformations, a combined condition incorporating LMs and other CVMs, were illustrated as a separate topic to differentiate from isolated VMs and to rectify the existing confusion with name-based eponyms such as Klippei-Trenaunay syndrome. Contemporary concepts on VMs were updated with new data including genetic findings linked to the etiology of CVMs and chronic cerebrospinal venous insufficiency. Besides, newly established information on coagulopathy including the role of D-Dimer was thoroughly reviewed to provide guidelines on investigations and anticoagulation therapy in the management of VMs. Congenital vascular bone syndrome resulting in angio-osteo-hyper/hypotrophy and (lateral) marginal vein was separately reviewed. Background data on arterio-venous malformations was included to differentiate this anomaly from syndromebased VMs. For the treatment, a new section on laser therapy and also a practical guideline for follow up assessment were added to strengthen the management principle of the multidisciplinary approach. All other therapeutic modalities were thoroughly updated to accommodate a changing concept through the years.

We present a group of patients with regional capillary malformations of the upper limbs and few additional findings other than prominent veins. We believe that this entity is the upper extremity equivalent of capillary-venous malformation of the lower limb and, likewise, belongs at the minor end of the spectrum of vascular disorders with overgrowth., (© 2015 Wiley Periodicals, Inc.)

We present imaging findings of aberrant left gastric vein (ALGV) and associated pseudolesions and mimickers including metastases and focal nodular hyperplasia. ALGV is formed due to interrupted involution of anastomotic omental veins, and it can drain into left portal vein or segments II and III of the liver as third inflow. Focal fat, focal fat sparing, and perfusion changes can be seen due to the presence of ALGV, which can mimic metastasis in cancer patients. ALGV may also serve as a pathway for direct tumor spread into the liver in patients with gastric cancer. Magnetic resonance imaging can be used as a problem solving tool in the presence of equivocal findings on ultrasonography and computed tomography.

Purpose: To estimate the risk of nerve injuries and assess outcomes after sodium tetradecyl sulfate (STS) sclerotherapy of venous malformations (VMs) in children., Materials and Methods: Sclerotherapy is the treatment of choice for most VMs, but all sclerotherapy agents are associated with the risk of complications. Neuropathy is considered a rare but potentially serious complication of venous sclerotherapy. The institutional review board waived ethical approval for this retrospective review, in which 647 sclerotherapy procedures were performed in 204 patients (104 female and 100 male patients; mean age, 9 years 6 months [range, 6 months to 17 years 11 months]) as treatment for symptomatic VMs. Technical and clinical success of the treatment was evaluated. Complications were reviewed with a particular focus on nerve injury. Informed consent, specifying the risk of neuropathy, as well as pain, swelling, infection, risks of anesthesia, skin injury, nonresolution or worsening of symptoms, and possible need for further or multiple procedures, was obtained for all patients. Standard sclerotherapy techniques were used. Technical details of all procedures were recorded prospectively. Follow-up included immediate postprocedural assessment and outpatient clinic review. All nerve injuries were recorded. Patients were monitored and treated according to clinical need. Confidence intervals were calculated by using the Wilson method, without correction for continuity., Results: Treatment was technically successful in 197 of 204 patients (96.6%), and clinical success was achieved in 174 of 204 (85.3%). Thirty-seven of the 647 procedures (5.7%) resulted in a complication, including 11 cases of excessive swelling, nine cases of skin injury, two patients with infection, and two with pain. Motor and/or sensory nerve injuries occurred after seven procedures (1.1%). Five of the seven children had undergone at least one previous sclerotherapy procedure. Neuropathy resolved spontaneously in four patients and partially recovered in three, of whom two underwent surgery. Surgery included debridement of necrotic tissue, carpal tunnel decompression, and external neurolysis., Conclusion: Nerve injury is an unusual but not rare complication of STS sclerotherapy. A degree of recovery, which may be complete, can be expected in most patients.

Background and Purpose: Developmental venous anomalies are the most common intracranial vascular malformation and are typically regarded as inconsequential, especially when small. While there are data regarding the prevalence of MR imaging findings associated with developmental venous anomalies, FDG-PET findings have not been well-characterized., Materials and Methods: Clinical information systems were used to retrospectively identify patients with developmental venous anomalies depicted on MR imaging examinations who had also undergone FDG-PET. Both the MR imaging and FDG-PET scans were analyzed to characterize the developmental venous anomalies and associated findings on the structural and functional scans. Qualitative and quantitative assessments were performed, including evaluation of the size of the developmental venous anomaly, associated MR imaging findings, and characterization of the FDG uptake in the region of the developmental venous anomaly., Results: Twenty-five developmental venous anomalies in 22 patients were identified that had been characterized with both MR imaging and FDG-PET, of which 76% (19/25) were associated with significant metabolic abnormality in the adjacent brain parenchyma, most commonly hypometabolism. Patients with moderate and severe hypometabolism were significantly older (moderate: mean age, 65 ± 7.4 years, P = .001; severe: mean age, 61 ± 8.9 years, P = .008) than patients with developmental venous aberrancies that did not have abnormal metabolic activity (none: mean age, 29 ± 14 years)., Conclusions: Most (more than three-quarters) developmental venous anomalies in our series of 25 cases were associated with metabolic abnormality in the adjacent brain parenchyma, often in the absence of any other structural abnormality. Consequently, we suggest that developmental venous anomalies may be better regarded as developmental venous aberrancies., (© 2015 by American Journal of Neuroradiology.)

Background: Formation or pre-existence of collateral gonadal veins in varicocele patients has been reported as the main cause of surgical treatment failure., Purpose: To describe venographic findings in patients with postsurgical recurrent varicoceles and to assess the efficacy of the following minimally invasive endovascular treatment., Material and Methods: Thirty-three men with failed surgical treatment of left-sided varicocele were examined between 2006 and 2013, using retrograde venography to assess the anatomy of varicocele draining veins before the attempted transcatheter embolization. Anatomic variants of gonadal veins were categorized according to the classification modified for the purpose of the present study. 3% polidocanol was used as an embolic agent together with pushable fibered coils., Results: In 31 (93%) out of 33 patients venography demonstrated incompetence of the gonadal vein or veins draining varicoceles after failed surgical treatment. The most frequent venographic finding was gonadal vein duplication - 66% of cases (39% in its mid-portion). Technical success of embolization was achieved in all 31 patients. No major complications were observed., Conclusion: Retrograde varicocele embolization may be superior to surgery because of its ability to detect gonadal vein variants. In our study group, transcatheter embolization with 3% polidocanol and fibered coils allowed successful, minimally invasive treatment of postsurgical varicoceles., (© The Foundation Acta Radiologica 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Purpose: We performed intraoperative antegrade venography to assess the prevalence of internal spermatic venous malformations in adolescents with varicocele., Materials and Methods: During a 2-year period 58 adolescent males with visible or palpable varicocele underwent antegrade venography before varicocele surgery. Antegrade venography was performed through a scrotal incision. A vein within the pampiniform plexus was cannulated and up to 1.75 mg/kg iohexol 300 mg/ml was injected to outline the entire length of the internal spermatic vein. The radiographs were reviewed and classified according to Bähren and Murray criteria., Results: Of the patients 43 (74.1%) demonstrated parallel duplications (Murray classification type P) of the internal spermatic vein. This rate is higher than the 2% reported based on retrograde venography. Of the patients with parallel duplications 21 (48.8%) showed duplications arising superior to the iliac crest (subtype A) and 22 (51.2%) had a combination of proximal duplications (subtypes B and C). Ten patients (17.2%) had a single internal spermatic vein, 2 (3.4%) had lumbar collaterals and 3 (5.2%) had renal collaterals., Conclusions: Parallel duplication of the internal spermatic vein is a common finding on antegrade venography. The various levels of duplication need to be identified before treatment of varicocele to maximize the success of the procedure., (Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

We present a rare case of a 47-year-old woman admitted to our maxillofacial surgery department for a large cervicofacial venous malformation. The patient underwent fluoroscopy-guided intralesional foam sclerotherapy with sodium tetradecyl sulfate and air under general anaesthesia. On awakening, after 48 h of endotracheal intubation, she displayed dysarthria and dysmetria. Her brain CT scan showed no haemorrhagic lesions. A chest CT scan ruled out a potential pulmonary embolism. Suspicion for a paradoxical embolism was high and echocardiography confirmed a patent foramen ovale, which acted as a passageway for the embolus. Transcranial ultrasound showed mild right-to-left heart shunting. The dysarthria and dysmetria disappeared gradually over 48 h, thus confirming a reversible ischaemic neurological deficit. A brain MRI performed 1 week later showed no ischaemic or haemorrhagic lesions. The patient recovered completely. She was advised cardiosurgical follow-up and discharged., (2014 BMJ Publishing Group Ltd.)

A 49-year-old man was admitted to our hospital complaining of severe epigastric pain. Contrast-enhanced abdominal computed tomography revealed an arteriovenous malformation of the pancreatic uncus. Because of unbearable epigastric pain, transcatheter arterial embolization was performed with n-butyl-2-cyanoacrylate. Subsequently, his symptoms fully resolved; no recurrence of the arteriovenous malformation was seen more than 1 year after the embolization.

Intraarticular venous malformation (IAVM) of the knee is a rare vascular disease that manifests with pain, swelling, and hemarthrosis. A young man with left knee pain and swelling was admitted to our institution for the treatment of the IAVM of the left knee which was diagnosed by a local orthopedic doctor via arthroscopy. A total dose of 40 Gy of radiotherapy was delivered with a daily dose of 2.0 Gy using 6 MV X-ray beams and a linear accelerator through anteroposterior portals. Fifteen months after radiotherapy, follow-up examination using radiologic imaging showed distinct shrinkage of the venous malformations. Swelling and pain of the left knee had decreased, and range of motion of the left knee was maintained. This report describes a case involving a 38-year-old man with IAVM of the left knee in whom favorable outcomes were obtained in response to radiotherapy., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Venous malformations (VMs) are often painful and may enlarge over time. Chronic coagulopathy is common in VMs and may contribute to phleboliths and potentially to disease progression. Few studies have examined the effects of anticoagulation on VMs and to our knowledge none have examined the use of aspirin therapy. A survey was administered to patients and parents of patients with VMs who attended the University of California at San Francisco Vascular Anomalies Center over a 4-year period (2008-2012) to whom aspirin had been recommended. They were surveyed regarding whether they were taking aspirin and, if yes, whether aspirin had resulted in any appreciable benefit. Sixty-five letters were sent to potential subjects: 38 participated and 27 declined to participate or could not be contacted. Twenty-eight of the 38 had begun aspirin and 22 reported current use. Seventeen reported some benefit, including less aching (n = 2), less shooting pain (n = 15), less fullness and swelling (n = 13), and shrinking of the VM (n = 1). Discontinuation of aspirin was associated with worsening VM symptoms in five of six patients. Side effects were reported in 6 of 28 patients, including five episodes of minor bleeding or excessive bruising and one of nausea and vomiting. This study suggests that aspirin may be a beneficial treatment for VM, with a reduction in pain and soft tissue swelling and an acceptable side-effect profile, but the retrospective nature of the study and the small size of the cohort limited our conclusions. Larger prospective studies of aspirin for VM using clinical and laboratory outcome measures are needed to confirm these observations., (© 2014 Wiley Periodicals, Inc.)

Venous malformations are slow-flow congenital malformations. They consist of abnormal venous channels that do not involute. Venous malformations can either be superficial or involve deeper structures. Patients with venous malformations are often symptomatic. The most common complaint is pain from congestion, mass effect, or compression of neural structures. Swelling, compromise of function, and disfigurement are other common symptoms. Large lesions cause coagulopathy. Therapeutic options for the treatment of these patients include sclerotherapy, compression garments, and surgical resection. These complex patients are best treated in a multi-disciplinary clinic environment, as they require long-term follow-up throughout childhood into adulthood., (Copyright © 2014 Elsevier Inc. All rights reserved.)

In recent years the advent of high-resolution and color Doppler ultrasound has enabled a more comprehensive examination of the veins at the cardiac level. These veins include both the superior and inferior vena cava, the pulmonary veins, the azygos vein, the coronary sinus, and the brachiocephalic (or innominate) vein. This article gives a review of the normal and abnormal conditions of the cardiac venous system. Normal anatomy and abnormal findings of these veins are demonstrated by grayscale and color Doppler. Three groups of anomalies are presented: 1) the interrupted inferior vena cava with azygos continuity, 2) the left persisting superior vena cava and 3) the total and partial anomalous pulmonary venous connections. Many of these abnormal findings can be detected by a dedicated examination of the veins during fetal echocardiography, but some anomalies are detectable by focusing on indirect signs such as the compensatory dilation of other veins., (© Georg Thieme Verlag KG Stuttgart · New York.)

Mulliken and Glowacki, in 1982 created a classification system of vascular anomalies which divided vascular anomalies into tumors and malformations which provided the framework for great advances in the management of these patients. This classification system was recently expanded at the 2014 ISSVA workshop in Melbourne. This revision again provides much greater detail including newly named anomalies and identified genes to account for recent advances in knowledge and clinical associations., (Copyright © 2014. Published by Elsevier Inc.)

Background and Purpose: Abnormal signal in the drainage territory of developmental venous anomalies has been well described in adults but has been incompletely investigated in children. This study was performed to evaluate the prevalence of brain parenchymal abnormalities subjacent to developmental venous anomalies in children and young adults, correlating with subject age and developmental venous anomaly morphology and location., Materials and Methods: Two hundred eighty-five patients with developmental venous anomalies identified on brain MR imaging with contrast, performed from November 2008 through November 2012, composed the study group. Data were collected for the following explanatory variables: subject demographics, developmental venous anomaly location, morphology, and associated parenchymal abnormalities. Associations between these variables and the presence of parenchymal signal abnormalities (response variable) were then determined., Results: Of the 285 subjects identified, 172 met inclusion criteria, and among these subjects, 193 developmental venous anomalies were identified. Twenty-six (13.5%) of the 193 developmental venous anomalies had associated signal-intensity abnormalities in their drainage territory. After excluding developmental venous anomalies with coexisting cavernous malformations, we obtained an adjusted prevalence of 21/181 (11.6%) for associated signal-intensity abnormalities in developmental venous anomalies. Signal-intensity abnormalities were independently associated with younger subject age, cavernous malformations, parenchymal atrophy, and deep venous drainage of developmental venous anomalies., Conclusions: Signal-intensity abnormalities detectable by standard clinical MR images were identified in 11.6% of consecutively identified developmental venous anomalies. Signal abnormalities are more common in developmental venous anomalies with deep venous drainage, associated cavernous malformation and parenchymal atrophy, and younger subject age. The pathophysiology of these signal-intensity abnormalities remains unclear but may represent effects of delayed myelination and/or alterations in venous flow within the developmental venous anomaly drainage territory., (© 2014 by American Journal of Neuroradiology.)

Vascular malformations affect the viscera less commonly than the head and neck, extremities, and extra-cavitary soft tissues. They present with a wide spectrum of symptoms and findings including pain, respiratory compromise, hemoptysis, chylothorax, ascites, gastrointestinal bleeding, and obstruction. Management options depend upon the subtype of malformation and anatomic extent and may include sclerotherapy, embolization, surgical extirpation, coloanal pull-through, and occasionally more innovative individualized surgical approaches., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Importance: Low-flow orbital or periorbital venous malformation (OVM) is the most common periorbital vascular lesion that may produce an appearance defect, visual dysfunction, internal hemorrhage, and thrombosis. Intralesional injection of pingyangmycin as a minimally invasive, gentle intervention may have better outcomes in treating low-flow OVMs compared with other currently used methods., Objective: To investigate the efficacy and safety of intralesional injection of pingyangmycin for treatment of low-flow OVM., Design, Setting, and Participants: A retrospective, noncomparative, interventional case series was conducted in a single medical center. Thirty-three consecutive patients with low-flow OVMs undergoing intralesional injection of pingyangmycin were included in the study., Interventions: Injections of 1 to 5 mL of a pingyangmycin 1.5-mg/mL mixture with lidocaine hydrochloride, 2%, were given. Each patient received 1 to 4 injections at an interval of 6 to 8 weeks between February 2002 and January 2013. Mixture volume was determined on a basis of 0.5 mL of solution per cubic centimeter of the lesion. The maximum dose for 1 injection was 8 mg. Clinical observations were well documented before and after treatment., Main Outcomes and Measures: Reduction of lesion volume based on ultrasound-measured volume; overall appearance, including blue color and thickness of lesions before and after treatment; and adverse events were evaluated., Results: Patients received a median of 2 (range, 1-4) intralesional injections of pingyangmycin. The mean pretreatment volume was 4.4 cm3 and posttreatment volume was 1.0 cm3 (t = 4.63; P < .001), with a mean decrease of 84% (range, 28%-100%). Marked to moderate improvement in the volume of the lesions was noticed in 31 eyes (94%; 25 of 33 [76%] with marked improvement and 6 of 33 [18%] with moderate improvement). Improvement occurred in 95% (18 of 19) of superficial lesions, 100% (3 of 3) of deep lesions, and 91% (10 of 11) of combined lesions. We noticed significant improvements in blue color and thickness on the basis of investigator scores from clinical photographs taken before and after treatment. None of the patients had recurrence noted at their final follow-up. Adverse events were limited to swelling of the conjunctiva and localized subcutaneous atrophy., Conclusions and Relevance: The results of intralesional pingyangmycin injection for treatment of low-flow OVM are encouraging and associated with a low risk of adverse events.

Background: There is no easy road map for venous malformations (VMs) of the head and neck according to which treatment modality can be chosen. The purpose of this study was to identify different types of VMs of the head and neck based on clinical, histopathology, MRI, and venography findings that help in specification of different treatment modalities., Methods: Sixty-nine patients with VMs of the head and neck were included in this study., Results and Conclusion: Our results proposed a diagnostic approach for VMs of the head and neck. MRI, venography, and clinical examination had important impact in decision-making, whereas histopathology had no impact. A management approach has been suggested for each type and its subtypes., (Copyright © 2013 Wiley Periodicals, Inc.)

The paired vitelline veins selectively involute and form a part of the portal vein during embryonic development. The presence of a persistent vitelline vein segment after birth is very rare and can be confused with anomalies of the portal and umbilical veins. We present sonographic, CT and MRI findings of aneurysmal dilatation of a persistent vitelline vein with thrombus in a neonate; this case was first misdiagnosed as an umbilical vein varix by prenatal US. MRI was used to identify the persistent vitelline vein segment and the remnant umbilical vein segment.

Purpose: To characterize intraosseous vascular malformations and describe the most appropriate approach for treatment according to clinical experience and a review of the published data., Materials and Methods: We performed a retrospective review of 11 vascular malformations (7 venous and 4 arteriovenous) of the facial bones treated during a 10-year period using en bloc resection or intraoral aggressive curettage alone or preceded by endovascular embolization. Corrective surgery was planned to address any residual bone deformities. The cases were reviewed at a mean follow-up point of 6 years., Results: Facial symmetry was restored in the cases requiring reconstruction. Tooth sparing was possible in the case of jaw and/or maxillary localization. Recanalization occurred in 14% of the venous and 33% of the arteriovenous malformations., Conclusions: Facial intraosseous venous malformations can be successfully treated using surgery alone. Facial intraosseous arteriovenous malformations will be better addressed using combined approaches. Aggressive curettage will obviate the need for extensive surgical resection in selected cases., (Copyright © 2014 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Venous malformations (VMs) are among the most common slow-flow vascular malformations characterized by irregular venous channels, luminal thrombi, and phleboliths. To systematically manifest the disorganized vascular structures in sporadic VMs, we initially evaluated histopathological characteristics, perivascular cell coverage, adhesion molecules expression and vascular ultrastructures. Then, the expression of Tie2 and TGF-β in VMs was detected. Meanwhile, the in vitro studies were performed for mechanism investigation. Our data showed that the perivascular α-SMA(+) cell coverage and expression of adhesion molecules in VMs were significantly decreased compared with those in the normal skin tissues. We also found that the expression and phosphorylation levels of Tie2 were upregulated, whereas TGF-β was downregulated in VMs, and they were negatively correlated. Moreover, the in vitro results also revealed a possible balancing effect between Tie2 and TGF-β, as demonstrated by the findings that Ang-1 (agonist of Tie2) treatment significantly downregulated TGF-β expression, and treatment with recombinant TGF-β could also suppress Tie2 expression and phosphorylation. This study provided strong evidence supporting the disorganized vascular structures and dysregulation of related molecules in sporadic VMs, and demonstrated a possible balancing effect between Tie2 and TGF-β, which might help to develop novel therapeutics for vascular disorganization-related disorders.

Objectives: To evaluate the feasibility and effectiveness of an isolation technique during ethanol injection sclerotherapy for venous malformations (VMs) in the head and neck region., Methods: The subjects were 23 patients with 35 VM lesions in the head and neck, treated between 1999 and 2012. The mean lesion area was 3.75 ± 3.09 cm(2) (±standard deviation). We confirmed the contour of the lesions to be treated on a fully filled image on direct injection cisternography, and observed patterns of communicating drainage to systemic veins. The cisterns were evacuated by squeezing and were isolated by manual compression of the communicators. Ethanol (94.5%) with a contrast agent was then injected into both isolable and unisolable lesions, up to a total volume of 1 mL/cm(2), avoiding complications. We investigated the relationship between lesion size and injected ethanol dose, and also dose per unit area., Results: Both manual evacuation by compression and isolation were performed in 20 (57.1%) isolable lesions, but not in 15 unisolable lesions. The mean injected ethanol dose was 0.65 ± 0.31 mL/cm(2) overall, 0.70 ± 0.32 in isolable and 0.59 ± 0.30 in unisolable lesions (NS). However, the injected ethanol dose was significantly lower for lesions sized >6 cm. Complete to near-complete shrinkage was observed in all isolable lesions, and in 60% of unisolable lesions (P < 0.05). Clinical outcome seemed unrelated to the injected ethanol dose or the dose per unit area. There was one case of recurrence and one complication in the unisolable lesions. No further relapses or complications were observed during the follow-up period of 38.6 ± 12.3 months., Conclusions: Clinical outcome was related to the isolability not to the injected dose. The isolation appears useful for improving the safety and effectiveness of ethanol sclerotherapy for VM., (© The Authors(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)