Low-flow Vascular Malformations Research Articles

Percutaneous treatment of orofacial vascular malformations

The aim of this study was to evaluate the efficacy of fluoroscopy-guided percutaneous injection of bleomycin as the primary treatment for low-flow vascular malformations. A total of 34 patients (mean (range) age 24 (8–51) years) with orofacial vascular lesions were treated in the Department of Interventional Radiology and Maxillofacial Surgery. There were 20 low-flow venous malformations, 11 lymphatic malformations, and three of mixed type. All patients were treated by fluoroscopy-guided percutaneous injection of a mixture of bleomycin (mean (range) 15 (5–15)mg) and a radio-opaque agent (Ultravist® (iopromide), Bayer)/session. The number of sessions ranged from one to six. The clinical response was complete in 21 patients, obvious in nine, and of clinical benefit in four. Patients were reviewed within the first week, third week, and at three-month periods until 24 months. There were no serious complications such as pulmonary fibrosis. Fluoroscopy-guided intralesional injection of bleomycin should be considered as the first-line treatment for lymphatic malformations because it is effective and reliable with few complications.

Percutaneous Sclerotherapy for Low-flow Vascular Malformations in Paediatric Patients: 6-Year Experience of a Multidisciplinary Team

Percutaneous Sclerotherapy for Low-flow Vascular Malformations in Paediatric Patients: 6-Year Experience of a Multidisciplinary Team

Anomalies vasculaires bénignes agressives de l’enfant et de l’adolescent

Superficial vascular anomalies constitute a large group of malformative and tumoral conditions developed from all types of vessels. Vascular tumors are the result of cellular hyperplasia, whereas vascular malformations (VMs) are constituted of dysplastic vessels. The classification from International Society for the Study of Vascular Anomalies (ISSVA) is based on this pathogenic difference. The most common vascular tumor is infantile hemangioma, which treatment, when necessary, is propranolol. Congenital hemangiomas and tumors that might be complicated with Kasabach-Merritt phenomenon, i.e. deep thrombocytopenia, are much rarer. Management of Kasabach-Merritt phenomenon is now largely based on sirolimus. Low-flow VMs include capillary, venous and lymphatic malformations; arteriovenous malformations are high-flow malformations. These different types of VMs might be combined. Currently, there is an increasing work in delineating the different entities based on molecular findings. Treatment of VMs depends on the impairment linked to them, and is decided case by case, in pluridisciplinary consultations. Interventional treatments, especially surgery and sclerotherapy, are usually partially efficient, and management of patients with VMs increasingly involves medical drugs. First-line treatment of coagulation disorders associated with venous malformations is based on low molecular weight heparin; sirolimus seems efficient in hemorrhagic complications refractory to usual treatment. Sirolimus is about to become the standard treatment in painful inflammatory manifestations of mixed and/or complicated lymphatic malformations.

Cranial Cavernous Malformations: Natural History and Treatment.

Cavernous malformations (CMs), also known as cavernous angiomas or cavernomas, are low-flow vascular malformations of the brain and spinal cord that consist of clusters of dilated sinusoidal channels lined with endothelial cells that do not exhibit intervening tight junctions. The involved blood vessels lack muscular and elastic layers, and blood at various stages of thrombosis and organization often fills the thin-walled vascular caverns. CMs are grossly distinct from adjacent brain and have a lobulated appearance sometimes likened to a mulberry, with a characteristic dark red or purple color. Hemosiderin and gliosis often surround CMs of the brain and spinal cord; no neural tissue is present inside the lesion. Except for developmental venous anomalies (DVAs) present in ≈33% of CMs,1 no abnormal vascularity is typically seen on digital subtraction angiography, leading CMs to be termed cryptic or occult vascular malformations (Figure 1). Multiple small hemorrhage events lead to a pathognomonic popcorn appearance on magnetic resonance (MR) imaging because of hemosiderin staining from blood products of various ages. Calcifications may be present on computed tomographic imaging (Figure 2). Figure 1. A 48-year-old woman presented with headaches. A , Magnetic resonance imaging of the brain with T2-weighted imaging demonstrated a 1.1-cm lesion in the cerebellar vermis with mixed signal intensity. B – D , Susceptibility-weighted imaging demonstrated hemosiderin within the lesion’s core and a developmental venous anomaly associated with the cavernous malformation (arrowheads). Figure 2. A 28-year-old woman presented with partial seizures without secondary generalization. A , Noncontrast computed tomography of the head demonstrated a 1.4-cm right posterior cingulate hyperdensity, with evidence of mild hemorrhage and calcification. Magnetic resonance imaging of the brain demonstrated the cavernous malformation to have a core of mixed signal intensity, heterogeneous enhancement, and a rim of hemosiderin consistent with a cavernous malformation, as evidenced on ( B ) susceptibility-weighted imaging, ( C ) T2-weighted …

Mosaic RAS/MAPK variants cause sporadic vascular malformations which respond to targeted therapy.

BACKGROUND. Sporadic vascular malformations (VMs) are complex congenital anomalies of blood vessels that lead to stroke, life-threatening bleeds, disfigurement, overgrowth, and/or pain. Therapeutic options are severely limited, and multidisciplinary management remains challenging, particularly for high-flow arteriovenous malformations (AVM).METHODS. To investigate the pathogenesis of sporadic intracranial and extracranial VMs in 160 children in which known genetic causes had been excluded, we sequenced DNA from affected tissue and optimized analysis for detection of low mutant allele frequency.RESULTS. We discovered multiple mosaic-activating variants in 4 genes of the RAS/MAPK pathway, KRAS, NRAS, BRAF, and MAP2K1, a pathway commonly activated in cancer and responsible for the germline RAS-opathies. These variants were more frequent in high-flow than low-flow VMs. In vitro characterization and 2 transgenic zebrafish AVM models that recapitulated the human phenotype validated the pathogenesis of the mutant alleles. Importantly, treatment of AVM-BRAF mutant zebrafish with the BRAF inhibitor vemurafinib restored blood flow in AVM.CONCLUSION. Our findings uncover a major cause of sporadic VMs of different clinical types and thereby offer the potential of personalized medical treatment by repurposing existing licensed cancer therapies.FUNDING. This work was funded or supported by grants from the AVM Butterfly Charity, the Wellcome Trust (UK), the Medical Research Council (UK), the UK National Institute for Health Research, the L’Oreal-Melanoma Research Alliance, the European Research Council, and the National Human Genome Research Institute (US).

4:10 PM Abstract No. 388 Ethylene vinyl alcohol copolymer (Onyx) for treatment of large venous vascular malformations: long-term results

To describe the long-term results and advantages of ethylene vinyl alcohol polymer for treatment of venous vascular malformations. Since 2005, we have used Ethylene vinyl alcohol copolymer (Onyx 18 & 34) in the treatment of 62 patients with large, low-flow venous vascular malformations. There were 34 females and 28 males with ages ranging from 7–59 years (mean 25 years). Fifty-one of the malformations involved the extremities and 11 involved the chest, abdominal wall or pelvis. All patients were evaluated with MRI/MRA pretreatment. Onyx was delivered by direct injection with ultrasound and fluoroscopic guidance under tourniquet control. In a few cases Onyx was also delivered via a transarterial micro catheter positioned in large feeding vessels. Sodium tetradecyl (3%) foam was used also in conjunction with Onyx in six patients where there were associated smaller foci of malformation. Histologic examination of tissues from patients whose embolized lesions were subsequently explanted was performed. Ethylene vinyl alcohol copolymer (Onyx) was found to be useful for occlusion of large venous channels in venous malformations. Unlike sclerosing agents, its visibility allowed direct observation of filling of the venous malformation during injection. When used alone, there was minimal post procedure discomfort. There were two complications: one extrusion of Onyx from a lesion on the sole of the foot, which healed without sequelae and one instance of embolization of a small Onyx fragment to the lung, also without sequelae. On H & E stains, the Onyx was noted to be distributed in the intraluminal spaces with associated mild foreign body type giant cell reaction. Permanency of the copolymer was demonstrated on long-term imaging follow-up. Ethylene vinyl alcohol copolymer (Onyx) is a safe, durable agent for treatment of venous vascular malformations. It is of particular value in malformations with large venous channels. Unlike standard sclerosing agents, Onyx behaves as a filler, and is not dependent upon generation of an inflammatory reaction for occlusion. Its opacity allows visualization of filled channels. It can be used alone or in conjunction with sclerosing agents.

Abstract No. 585 Cryoablation of low-flow vascular malformations: single-center experience and how to stay out of trouble

Abstract No. 585 Cryoablation of low-flow vascular malformations: single-center experience and how to stay out of trouble

Sclerotherapy of peripheral low-flow vascular malformations: technical aspects and mid-term clinical outcome.

The therapeutical management of low-flow vascular malformations (LFVMs) is challenging because of high recurrence rate; multiple strategies have been proposed. This paper aims to report a single-center experience of direct puncture sclerotherapy of peripheral LFVMs, focusing on technical aspects and clinical outcome in mid-term follow-up. 16 patients have been treated for peripheral LFVMs (mean age 36.1years), complaining mild pain, swelling of the region of interest, and cosmetic nuisance. Preprocedural US and MR were acquired; angiography performed only in doubt vascular supply. Standard procedure consisted of direct puncture of the nidus using 20-23 gauge needles under US guidance and injection of up to 15ml foam of sodium tetradecyl sulphate under fluoroscopic guidance. Clinical and radiological follow-up were assessed at 1, 3, and 6 months. Lesions were localized: 8 in the upper and 5 the in lower limbs, 2 in the cheeks, and 1 in the vaginal labia. All procedures have been technically accomplished (100%). At 6 month follow-up, technical and clinical success were obtained in all cases, while radiological follow-up showed 81.2% (13 patients) complete vessels thrombosis after multiple sclerotherapy sessions. No major complications have been recorded; five patients (31.2%) referred minor complications. Sclerotherapy via direct puncture of LFVMs is a clinically effective procedure, well tolerated by patients, with reduced costs and mild minor complications rate; interventionalists should always clarify to the patients that multiple sessions would be performed and recurrences are expected at imaging follow-up despite clinical improvement.

MR Imaging of Atraumatic Muscle Disorders.

Atraumatic disorders of skeletal muscles include congenital variants; inherited myopathies; acquired inflammatory, infectious, or ischemic disorders; neoplastic diseases; and conditions leading to muscle atrophy. These have overlapping appearances at magnetic resonance (MR) imaging and are challenging for the radiologist to differentiate. The authors organize muscle disorders into four MR imaging patterns: (a) abnormal anatomy with normal signal intensity, (b) edema/inflammation, (c) mass, and (d) atrophy, highlighting each of their key clinical and imaging findings. Anatomic muscle variants, while common, do not produce signal intensity alterations and therefore are easily overlooked. Muscle edema is the most common pattern but is nonspecific, with a broad differential diagnosis. Autoimmune, paraneoplastic, and drug-induced myositis tend to be symmetric, whereas infection, radiation-induced injury, and myonecrosis are focal asymmetric processes. Architectural distortion in the setting of muscle edema suggests one of these latter processes. Intramuscular masses include primary neoplasms, metastases, and several benign masslike lesions that simulate malignancy. Some lesions, such as lipomas, low-flow vascular malformations, fibromatoses, and subacute hematomas, are distinctive, but many intramuscular masses ultimately require a biopsy for definitive diagnosis. Atrophy is the irreversible end result of any muscle disease of sufficient severity and is the dominant finding in disorders such as the muscular dystrophies, denervation myopathy, and sarcopenia. This imaging-based classification, in correlation with clinical and laboratory data, will aid the radiologist in interpreting MR imaging findings in patients with atraumatic muscle disorders. ©RSNA, 2018.

Results of intralesional injection of n-butyl cyanoacrylate (NBCA) and fibrin glue in hemangioma and low flow vascular malformations

Background and Objectives: Complete surgical excision of vascular anomalies is technically difficult and seldom possible, prompting interest in other treatment options, especially sclerotherapy. We decided to study the results of intralesional injection of n-butyl cyanoacrylate (NBCA) and autologous fibrin glue (FG) in hemangiomata and vascular malformations (VMs). Materials and Methods: This prospective study was conducted in fifty consecutive patients of hemangioma and VMs. Frequency of injections was decided according to size and injections were given at the interval of 4 days to complete the treatment in 30 days. Postinjection reduction in size of the lesion was assessed. Lesions which did not disappear or those having complications were taken for surgery and amount of blood loss during excision was estimated. Results: In NBCA group, the mean reduction in size of hemangioma and low flow VM was 63.85%—73.3%; in FG Group, it was 51.9%—53.80%, respectively. In FG group, the mean blood loss was 37.5 ml for a mean size of 3.6 cm2 hemangioma and 79.55 ml for low flow VM with a mean size of lesion 2.23 cm2. In NBCA group, the mean blood loss was 37.5 ml for mean size of 4.4 cm2 hemangioma and 37.5 ml with a mean size of 3.55 cm2 low flow VM. Conclusion: Repeated intralesional injections of NBCA and FG in hemangioma and VMs are associated with significant reduction in size and blood loss during excision. Small vascular lesions (

Unedited microneurosurgery of a cavernous malformation of the pineal region.

Background:Cavernous malformations are low-flow vascular malformations comprised of clusters of dilated sinusoidal channels lined with endothelial cells. The tortuous blood vessels also called vascular caverns lack muscular and elastic layers, and are filled by blood at different stages of thrombosis. Hemosiderin and gliosis often surround cavernomas. However, no neural tissue is present inside the lesion. Magnetic resonance images of cavernomas reveal a pathognomonic popcorn appearance produced by multiple small hemorrhages. Developmental venous anomalies are associated in around 30% of the cases. Cavernomas are very prevalent lesions ranging from 0.4 to 0.8% of the population. However, those located in the pineal region are very rare. Herein, we present the microsurgical treatment of a histologically confirmed cavernous malformation of the pineal region.Case Description:A 33-year-old patient with a pineal region cavernoma and progressive hydrocephalus underwent right supracerebellar infratentorial paramedian approach in a sitting praying position. The surgical planning did not require neuronavigation, but anatomical landmarks for the proper approach. Under high magnification, the pineal region was accessed over the superior cerebellar surface. After a focused lateral opening of the dorsal membrane of the quadrigeminal cistern, small vessels running in the posterior wall of the third ventricle were carefully dissected. A yellowish hemosiderin staining tissue allowed us to recognize the vicinity of the lesion. A small cottonoid delimitated the posterior border of the malformation, nonetheless, the superior limits underwent microdissection to release some cerebrospinal fluid from the third ventricle. A precise marginal dissection with bipolar forceps, microdissectors, and a thumb-regulated suction tube encircled the lesion. Gently traction of the lesion with ring microforceps associated further detachment of the cavernoma with the suction tube. Cotton dissection and water dissection technique were useful as well. A piecemeal resection, which is indicated in lesions with a deep and eloquent location, allowed us a complete removal of the cavernoma. Accurate hemostasis and continuous saline irrigation maintained a clean surgical field along the procedure. The gliotic tissue was left behind to prevent damage of the surrounding structures. Under endoscopic vision, remnants in the lower margins of the operative field were carefully evaluated. Finally, the surgical area was flushed with saline irrigation to detect any bleeding, and a small piece of tachosil was placed over the cavity. The postoperative course was uneventful. The hydrocephalus resolved after surgery and it did not require any further procedure.Conclusion:This unedited video offers all detailed aspects that a neurosurgeon as the senior author Juha Hernesniemi considers essential when performing an efficient and safe surgery for cavernous malformation of the pineal region.Videolink: http://surgicalneurologyint.com/videogallery/iii-ventricle-cavernoma/

Treatment of Low-Flow Vascular Malformations of the Extremities Using MR-Guided High Intensity Focused Ultrasound: Preliminary Experience

Five patients with painful vascular malformations of the extremities that were refractory to standard treatment and were confirmed as low-flow malformations on dynamic contrast-enhanced magnetic resonance (MR) imaging were treated with MR imaging–guided high intensity focused ultrasound. Daily maximum numeric rating scale scores for pain improved from 8.4 ± 1.5 to 1.6 ± 2.2 (P = .004) at a median follow-up of 9 months (range, 4–36 mo). The size of the vascular malformations decreased on follow-up MR imaging (median enhancing volume, 8.2 mL [0.7–10.1 mL] before treatment; 0 mL [0–2.3 mL] after treatment; P = .018) at a median follow-up of 5 months (range, 3–36 mo). No complications occurred.

Videodermoscopy and doppler-ultrasound in spider naevi: towards a new classification?

Spider naevi (SN) are considered a subtype of telangiectasias, currently classified as low-flow vascular malformations. To describe the videodermoscopy and Doppler-ultrasound (US) features of a large group of SN. A retrospective study of cases of SN collected at our Dermatology department during the period between June 2015 and June 2017 was performed. Clinical images, dermoscopic, videodermoscopic and Doppler-US files were reviewed. For each case, the age of the patient, time since onset, size and dermoscopic pattern of the lesions were recorded. The presence of pulsatility was also evaluated visually on the videodermoscopy. Two hundred and thirty-three SN in 189 patients were included. The mean age was 39.5years (range: 10-76years). Mean size of the lesions was 4.1±2.0mm. We described three dermoscopic patterns: network, star and looping. Older age, longer time since onset and larger size were found associated with higher frequency of the looping and star patterns compared to that of network pattern (P<0.01). Pulsatility during videodermoscopy was found in 88 patients (37%). This pulsatility phenomenon was more commonly associated with the looping pattern (64.7%) than star- (40.3%) or network-like patterns (29.9%) (P<0.001). In Doppler-US studies, a high-flow with arterial biphasic waveform was found. In the light of the results, we support that SN could be reconsidered in upcoming classifications as lesions closer to the group of high-flow arteriovenous malformations.

Application of sclerotherapy in small vessel diseases

Sclerotherapy, an effective technique for the treatment of vasodilatation, exerts its therapeutic and cosmetic effects by injecting sclerosing agents into blood vessels to make them into fibrous stripes followed by absorption by surrounding tissues. Sclerotherapy is the first choice for the treatment of dilated intradermal venules, including reticular varicosities and telangiectasia, and also has good therapeutic effects on low-flow vascular malformations. Many factors can affect clinical effects of sclerotherapy, such as types of sclerosing agents, stability of foamed sclerosants and injection methods. With extensive application of sclerosing agents in clinic and improvement of the technology of manufacturing sclerosing foam, the working concentration and dosage of some sclerosing agents have reached a consensus. With development in sclerotherapy and application of newly-emerging technology such as imaging techniques, some progress in sclerotherapy has been made, providing deeper insights into the effectiveness and safety of sclerotherapy. Key words: Blood vessels; Telangiectasis; Vascular malformations; Sclerotherapy; Therapeutic uses

Diode laser to treat small oral vascular malformations: A prospective case series study.

The current work examined a consecutive series of patients presenting vascular malformations (VMs) and venous lakes (VLs) of the lip and oral mucosa who were treated with transmucosal diode laser applications and assessed over a 1 year period. Fifty-nine patients (31 males and 28 females) presenting low-flow VMs or VLs of the oral cavity were treated transmucosally using a diode laser (with an 830 nm operating wavelength and 1.6 W output power) with a 320 µm diameter flexible fiber. All the lesions were assessed 7 days, 30 days, and 1 year after the laser treatment, and the lesion reduction percentage was scored on a one to five scale. The patients were also asked to assess their pain perception daily during the 7 days following the treatment using a visual analog scale (VAS). There were no procedure-related intra- or post-operative complications; only modest pain intensity was reported. Thirty days after the treatment, lesion reduction was described as excellent or good in 52 cases; it was fair or poor in 7. Six patients (F:M ratio 2:4) required a second diode laser application. At the 1 year follow-up, volume reduction was complete in 48 out of 59 patients; there were five recurrences (F:M ratio 3:2). No relevant gender-related differences were noted. The use of diode laser application to treat small oral VMs and VLs was associated to shorter operating times and fewer postoperative complications with respect to the scapel surgery approach. More than one session may nevertheless be required if the anomaly is larger than 10 mm. Lasers Surg. Med. 50:111-116, 2018. © 2017 Wiley Periodicals, Inc.

Multiple Low Flow Vascular Malformation with Phleboliths – A Case Report

: Objective: Venous malformation involving head and neck region is a rare entity. Hence, application of precise and leading diagnostic techniques, gives the accurate localization of the source vessel allowing for making treatment plan more satisfactory. Methods: We present a case of 30-year-old male patient with venous malformation involving head and neck region. We update the review of literature and also discuss the leading diagnostic aids for this rare entity disease so that iatrogenical accidents can be lessened. Case description: Our patient presented with solitary swelling in the left lower cheek region, left side of lateral border of tongue and 2 – 3 small nodular swellings at the lateral border of the neck, lined obliquely over the sternocleidomastoid muscle. All the swellings were soft in consistency and non-tender, with no rise in temperature except for the swelling on the tongue. The lesion was confirmed by a radiograph which showed phleboliths. The ultrasound shows heterogeneously hypoechoic with multiple anechoic sinusoidal spaces (longitudinal plane) with mild vascularization and spectral analysis shows monophasic venous flow. This was later confirmed by direct puncture venography of the left cheek. Conclusion: The spectrum of vascular malformations is too vast; hence it becomes challenging to diagnose the lesion and fit it in the spectrum. These lesions are termed as intramuscular venous malformations based on clinical imaging and pathologic criteria and hence dentists must be aware of the clinical expression of these lesions in order to prevent iatrogenically related accidents and to minimize potential spontaneous crises for the patient. Having said that and keeping in mind the broad spectrum of vascular malformation, we present a clinically well diagnosed case of venous malformation, which illustrates phleboliths.Key words: Venous malformations (VMs), Arteriovenous malformation (AVM), Phleboliths, sclerotherapy, Venography and Hemangioma.

Percutaneous ultrasound-guided sclerotherapy with polidocanol microfoam for lymphatic malformations.

Lymphatic malformations (LMs) are low-flow congenital lesions that consist of cysts of varying size. Sclerotherapy with intralesional bleomycin and OK-432 has been reported to yield dramatically beneficial results for this disorder. However, inflammation-related symptoms are often seen after treatment with these sclerosing agents. On the other hand, polidocanol (POL) is reportedly associated with fewer allergic and inflammatory reactions. Up to now, however, very few reports have documented the use of POL microfoam for treatment of LMs. This study was performed to assess the efficacy and safety of POL microfoam sclerotherapy for LMs. Between 2003 and 2016, cases were identified from a prospectively compiled database on low-flow congenital vascular malformations before undertaking a retrospective electronic chart review. Patients were included if they had LMs that had been treated by POL microfoam sclerotherapy. The location, size, and type of LMs were assessed using ultrasound and magnetic resonance imaging. Twenty-gauge venous catheters were inserted into the lymphatic space under ultrasound visualization. The LMs were then fully aspirated if they were macrocystic in form. Microfoam composed of 3% POL was then injected under ultrasound guidance. Microcystic LMs were treated by direct injection with POL microfoam under ultrasound guidance. The outcome was assessed by clinical examination combined with findings of postsclerotherapy imaging using ultrasound and magnetic resonance imaging. During a 13-year period, 32 patients met the inclusion criteria. These were 11 (34%) male patients and 21 (66%) female patients with a mean age of 18years. The LMs were localized to the head and neck (47%), the trunk (38%), and the extremities (15%). The lesions were subdivided into macrocystic (56%), mixed macrocystic and microcystic (31%), and microcystic (13%) LMs. The average lesion size was 6.6× 4.6× 3.0cm. The mean number of treatment sessions was 2.8 (range, 1-15), with a mean foam volume of 4.6 (range, 1-10) mL. Excellent (47%) and moderate (41%) responses were seen in 88% of the patients. Notably, half of the patients achieved excellent or moderate resolution with a single treatment session. Intralesional hemorrhage occurred in four patients (13%) but resolved spontaneously. Only one patient with mixed macrocystic and microcystic LMs developed post-therapy infection. However, the other patients did not develop any post-therapy inflammation-related symptoms, including fever, pain, and marked swelling. Percutaneous sclerotherapy using POL microfoam appears to be safe and effective for treatment of LMs. Ultrasound-guided POL microfoam sclerotherapy should be considered for such lesions, particularly those that are exclusively macrocystic.

Prolonged antibiotic treatment for infected low flow vascular malformations.

Infection in low flow malformations is difficult to diagnose and treat. Initial presentation can be followed by cycles of recurrent infection lasting several years. The optimal duration of antibiotic therapy to prevent recurrence of infection has not been established. All cases of infection in low flow malformations at the Royal Children's Hospital over a ten-year period were reviewed. Clinical markers of infection and duration of initial antibiotic treatment were correlated with the development of recurrent episodes of infection. Twenty-one patients met criteria for inclusion. Nineteen were diagnosed as lymphatic malformations and two as venous malformations. The majority of patients (13 or 62%) received a prolonged course of six weeks or more of antibiotics. Eleven (52%) patients went on to have recurrent infections, but these were significantly less likely to be in those treated with a long course of antibiotics (Fisher's exact test, p=0.026). In only 12 of 21 cases could a bacterium be grown. Elevated CRP was the most consistent abnormal laboratory finding in infection. Longer courses of antibiotics reduce the risk of recurrent infection in low-flow vascular malformations. We recommend an antibiotic course of three months or more at the initial presentation of infection in a low flow malformation. Elevated CRP is the most sensitive test for diagnosis of infection in low-flow malformations. Treatment study. III.

Malformación venolinfática con erosión ósea. Una afectación rara en estas lesiones

A 30 year old male presented with a cutaneous lesion on the distal area of the 5th toe with involvement of the outer edges. Macroscopically, it was a 1cm violaceous and keratotic lesion. Radiography showed an increase in soft tissue, possibly due to a vascular lesion. MRI showed a hyper-intense signal with erosion of the distal phalanx compatible with a low-flow vascular malformation. The distal phalanx was amputated. Histopathology revealed a lesion formed by venous and D2-40 positive lymphatic vessels. This case highlights the fact that even minimal skin involvement in vascular malformations may conceal an important deeper lesion, such as erosion of the cortical bone.

The Klippel-Trenaunay Syndrome

The Klippel-Trenaunay Syndrome (KTS) includes congenital, low flow vascular malformation(s) that leads to structural and functional anomalies of the vascular system characterized by variability of presenting symptoms and often unpredictable clinical course. Traditionally described pathognomonic triad of findings in KTS patients are capillary malformation, venous malformation, and bone and/or soft tissue hypertrophy. KTS frequently includes the presence of lymphatic malformation(s), as well. Historically, the management of KTS was hampered by the lack of understanding of underlying hemodynamics and pathophysiology responsible for development and progression of KTS symptoms, confusing nomenclature which characterized majority of the early literature discussing KTS, as well as by the absence of established guidelines for KTS management. A relatively recent advancement in the diagnostic and treatment modalities have resulted in better understanding of the pathophysiology and natural history of KTS and represents a fertile environment for improved management of these patients. Multidisciplinary approach and diagnostic algorithm utilized to distinguish vascular malformations from vascular tumors, arterio-venous lesions from low flow vascular malformations have been validated as clinically applicable for making an accurate anatomical and hemodynamic assessment of the lesions found in KTS and serve as a basis for proper treatment selection. It has to be emphasized that treatment of majority KTS patients is palliative and goal oriented. In this manuscript we describe diagnostic protocols and therapeutic algorithms which results in favorable outcomes with an acceptable complication rates in the management of this frequently challenging patient population.