Type Of Vascular Malformation Research Articles

Management of pediatric vascular malformations

Vascular malformations belong to the group of vascular anomalies. They are rare congenital diseases resulting from abnormal development of vessels and can arise in any location, often in the head and neck region. Clinical symptoms are variable, functional handicaps and esthetic problems may result. One differentiates between arteriovenous, lymphatic, venous, and capillary malformations and combinations thereof. Their classification, symptoms, diagnostics, and treatment options are discussed. The different types of vascular malformations as well as their clinical presentation, diagnostic workup, and therapeutic options are presented, supported by clinical examples. Interdisciplinary care of pediatric patients with vascular malformations is essential in order to generate atreatment concept after correct diagnosis and improve symptoms and quality of life.

The rare complication of vascular malformations of the limb after sclerotherapy: a report of 3 cases and brief literature review

BackgroundVascular malformations are common but complicated types of disease in infants, with unclear causes and lack of effective prevention. The symptoms usually do not disappear and tend to progress without medical intervention. It is extremely necessary to choose correct treatment options for different types of vascular malformations. A large number of studies have confirmed that sclerotherapy has a tendency to become the first-line treatment in near future, but it is also associated with mild or severe complications. Furthermore, to our knowledge, the serious adverse event of progressive limb necrosis has not been systematically analyzed and reported in the literature.Case presentationThree cases (two females and one male) were presented who were all diagnosed as vascular malformations and were treated by several sessions of interventional sclerotherapy. Their previous medical records showed the use of several sclerosants in different sessions including Polidocanol and Bleomycin. The sign of limb necrosis did not occur during the first sclerotherapy, but after the second and third sessions. Furthermore, the short-term symptomatic treatment could improve the necrosis syndrome, but could not change the outcome of amputation.ConclusionSclerotherapy undoubtedly tends to be the first-line treatment in near future, but the adverse reactions still remain major challenges. Awareness of progressive limb necrosis after sclerotherapy and timely management by experts in centers of experience of this complication can avoid amputation.

Familial CCM Genes Might Not Be Main Drivers for Pathogenesis of Sporadic CCMs-Genetic Similarity between Cancers and Vascular Malformations.

Cerebral cavernous malformations (CCMs) are abnormally dilated intracranial capillaries that form cerebrovascular lesions with a high risk of hemorrhagic stroke. Recently, several somatic "activating" gain-of-function (GOF) point mutations in PIK3CA (phosphatidylinositol-4, 5-bisphosphate 3-kinase catalytic subunit p110α) were discovered as a dominant mutation in the lesions of sporadic forms of cerebral cavernous malformation (sCCM), raising the possibility that CCMs, like other types of vascular malformations, fall in the PIK3CA-related overgrowth spectrum (PROS). However, this possibility has been challenged with different interpretations. In this review, we will continue our efforts to expound the phenomenon of the coexistence of gain-of-function (GOF) point mutations in the PIK3CA gene and loss-of-function (LOF) mutations in CCM genes in the CCM lesions of sCCM and try to delineate the relationship between mutagenic events with CCM lesions in a temporospatial manner. Since GOF PIK3CA point mutations have been well studied in reproductive cancers, especially breast cancer as a driver oncogene, we will perform a comparative meta-analysis for GOF PIK3CA point mutations in an attempt to demonstrate the genetic similarities shared by both cancers and vascular anomalies.

Sociodemographic characteristics of pediatric patients with vascular malformations: Results of a single site study.

Vascular malformations, the abnormal development of blood vessels, are a rare set of congenital anomalies. The sociodemographic factors associated with vascular malformations in pediatric patients are poorly understood. This study examined sociodemographic factors of 352 patients presenting to a single vascular anomaly center from July 2019 to September 2022. Characteristics such as race, ethnicity, sex, age at presentation, degree of urbanization, and insurance status were recorded. This data was analyzed by comparing the different types of vascular malformations, including arteriovenous malformation, capillary malformation, venous malformation (VM), lymphatic malformation (LM), lymphedema, and overgrowth syndrome. Patients were primarily white, not Hispanic or Latino, female, had private health insurance, and were from the most urban setting. No differences in sociodemographic factors were found among the different vascular malformations except patients with VM presented at a later age than patients with LM or overgrowth syndrome. This study provides novel insight into the sociodemographic factors of pediatric patients presenting with vascular malformations and indicates a need for their improved recognition for the timely initiation of treatment.

A study of duplex ultrasound examination in peripheral vascular malformations

Vascular malformations belong to wide spectrum of disease involving all vascular segments- arteries, veins as well as lymphatics. Being Ultrasound as the primary diagnostic modality, all peripheral vascular malformation diagnosis can be made by clinical examination and duplex ultrasound examination.A study of 50 patients was carried over a period of 1 year from 1 May 2021 to 1 May 2022 on the patient who came with complain of slow growing, superficial swelling which is painless in nature. The machine used is Affinity Philips 70 G.With help of duplex ultrasound, various types of vascular malformations were diagnosed with incidence such as Slow Flow Venous malformation (60%- 30 patients), High flow Arterio-venous malformations (16%- 8 patients), Capillary malformations (12%- 6 patients) and Lymphatic malformations (12%- 6 patients).Vascular malformations have been classified into venous, arterial, capillary, combined and lymphatic malformations types. Besides age presentations, detailed history and clinical examination, ultra-sound imaging plays an important role in correct diagnosis and classification of vascular malformations. It assesses exact flow dynamics in order to differentiate between Slow flow v/s High flow vascular malformations. MRI is most valuable diagnostic tool for comprehensive assessment of vascular malformationsUltrasound are first imaging modality in peripheral vascular malformations. It has plays crucial role in proper treatment planning of the lesion as it describes the extent, type, flow pattern within the lesion.

Treatment of venous malformations in pediatric population – three- year experience

Venous malformations (VMs) are a type of vascular malformations that result in abnormal development of veins that become extensible over time due to an error in vascular morphogenesis. They usually appear in newborns or in early adulthood as a bluish, soft, swollen and eventually painful skin formation. Treatment includes conservative therapy, sclerotherapy and surgical excision. Aim of the paper is to evaluate the therapeutic effect of scleraotherapy in pediatric patients with venous malformations. Material and methods: In a three-year period, from 2019 to 2021, venous malformation was found in 33 patients aged 4 to 14 years (average age: 8 years). Pain as a symptom occurred in 8 patients. Two patients had lesions measuring up to 5 cm and 5 cm respectively, while in the remaining subjects the lesion was over 5 cm. Ultrasound was performed routinely in all subjects, and MRI in two patients. Conservative treatment was instituted in 13 patients with venous malformations of the extremities, surgical excision with local reconstruction was performed in 11 patients, and sclerotherapy with bleomycin under general anesthesia was performed in 8 patients. Combined treatment was used in one patient that presented with venous malformation of the upper arm that underwent partial sclerotherapy with subsequent operative excision due to a phlebolith. Follow-up examinations revealed regression of the change not only from functional but from aesthetic aspect as well. Conclusion: Sclerotherapy is the established golden standard, first-line treatment for venous malformations. Excellent results were achieved as the reduction of the lesions was below 50% of the initial size. However, the modality of treatment should be individualized to each patient as it can sometimes require a combination of more than one treatment option. Venous malformations are best treated early, but they usually recur over time. Treatment helps relieve symptoms and control the growth of vascular malformations.

Pharmacokinetics of bleomycin sclerotherapy in patients with vascular malformations.

Bleomycin, a chemotherapy agent that inhibits synthesis of DNA, has been increasingly utilized in sclerotherapy for patients with vascular malformations. A serious long-term risk of intravenous bleomycin is dose-dependent interstitial pneumonitis. Little is known about absorption and circulating levels of bleomycin when used in sclerotherapy for patients with vascular malformations. This is an Institutional Review Board (IRB)-approved prospective study on patients receiving bleomycin sclerotherapy in the management of vascular malformations. Depending on the type of vascular malformation, bleomycin was administered either in the lumen or interstitial space of the involved lesion. A bleomycin assay measured serum bleomycin plasma concentrations versus time at seven intervals following treatment. Pharmacokinetic parameters were obtained for each participant and included peak plasma concentration (Cmax ), time to reach peak plasma concentration (Tmax ), volume of distribution (Vd ), elimination half-life (t1/2 ), the volume of plasma cleared of the drug per unit time (CL), and total systemic exposure area under the curve (AUC). Fifteen patients were enrolled (5: lymphatic, 4: venous, 6: arteriovenous malformations). Bleomycin was administered interstitially (IS) in 11 patients and intraluminal (IL) in four; median age of 13years (range: 2-67). Pharmacokinetic analysis revealed terminal elimination half-life (t1/2λz ) of 88.51 (±23.09) and 111.61 (±37.75) minutes for the IS and IL groups, respectively. Vd was 4.86L (±6.74) and 1.55L (±0.54) for the IS and IL groups, respectively. AUC was 53.9 (±23.45) and 129.17 (±93.57) mgmin/L for the IS and IL groups, respectively. There were no statistically significant differences in t1/2λz , Vd , or AUC parameters between groups. Bleomycin is absorbed systemically when used as a sclerosant for vascular malformations when injected either IS or IL.

The importance of Nuclear Magnetic Resonance (MRI) in the diagnosis of cerebral cavernomas

Introduction: Cavernomas are one of the four types of vascular malformations which develop in the central nervous system. From an imaging point of view, they are low flow small vessel malformations that cannot be seen on conventional angiography and are rarely visible on CT. They can be seen on MRI because of the para-magnetic haemoglobin break-down products within them.
 Case presentation: A 35 years old male presented at the emergency room following a tonic-clonic seizure, without urine emission. After a clinical exam, it was decided to perform a cerebral MRI with contrast substance and angiographic sequence. Clinical and imagistic results showed a superficial left temporal cavernoma, which showed signs of recent bleeding. After the surgery, the patient has no neurological deficits and is discharged afebrile and without any other symptoms. At 6 weeks after the surgery, the patients returned for neurosurgical reevaluation in perfect neurological condition, without motor deficit or intracranial hypertension.
 Conclusions: Cavernomas are angiographic cryptic vascular lesions that need an MRI investigation in order to appreciate them correctly.

ORTHOPEDIC MANIFESTATIONS OF CONGENITAL VASCULAR MALFORMATIONS OF THE LOWER EXTREMITIES

The spectrum of orthopedic manifestations of congenital vascular malformations of lower limbs remains insufficiently studied. Objective. To analyze the results of examination of patients with orthopedic manifestations of congenital vascular malformations. Methods. 24 patients with various forms of congenital vascular malformations of the lower extremities were examined. Distribution of patients according to the Hamburg classification of vascular malformations: arteriovenous type — 14 patients; venous — 6; capillary — 2; capillaryvenous — 1; lymphatic — 1 patient. The diagnosis of orthopedicpathology was performed on the basis of clinical examination, X-ray, ultrasound, X-ray densitometry. Results. All patients with congenital vascular malformations were diagnosed with orthopedic pathology: leg length discrepancy (75 %), secondary scoliotic deformity (16 %), knee flexion contracture 40° (8 %), flatfoot (12 %), equinus deformity of the foot (8 %), osteoporosis of the affected limb (16 %), areas of pathological reconstruction of the affected bone (lytic areas) (4 %), hip osteoarthritis of the affected limb (grade 2) (4 %). Leg length discrepancy was observed due to the elongation of the affectedlimb — in 16 patients (67 %), shortening — in 2 (8 %). Leg length discrepancy was in the range of 0.5–6 cm. Elongation of the affected limb < 2 cm was noted in 5 patients (21 %), > 2 cm — in 11 patients (46 %); shortening of the affected limb < 2 cm was observed in one patient (4 %), > 2 cm — in one patient (4 %). Among the patients with leg length discrepancy, 61 % had combinations of several orthopedic manifestations of the disease. Conclusions. Patients with congenital vascular malformations have a wide range of orthopedic pathology, among them the leading is leg length discrepancy (75 %) due to elongation(67 %) or shortening of the affected limb (8 %). Leg length discrepancy is most often observed in patients with arteriovenous type of vascular malformation (67 %), elongation of the lower limb > 2 cm (75 %) significantly predominating in this category. Among patients with a difference in the length of the lower limbs in 61 % of cases, there is a combination with other orthopedic manifestations of the disease. Systematization of orthopedic pathology and development lesions’ classification of the musculoskeletal system taking into account the forms of vascular malformation will improve the diagnosisof the disease and improve the tactics of its orthopedic treatment.

Cellular heterogeneity and immune microenvironment revealed by single-cell transcriptome in venous malformation and cavernous venous malformation

Venous malformation (VM) and cavernous venous malformation (CVM) are two types of vascular malformations. Even if the two diseases are similar in appearance and imaging, the distinct cellular components and signaling pathways between them might help distinguish the two from a molecular perspective. Here, we performed single-cell profiling of 35,245 cells from two VM samples and three CVM samples, with a focus on endothelial cells (ECs), smooth muscle cells (SMCs) and immune microenvironment (IME). Clustering analysis based on differential gene expression unveiled 11 specific cell types, and determined CVM had more SMCs. Re-clustering of ECs and SMCs indicated CVM was dominated by arterial components, while VM is dominated by venous components. Gene set variation analysis suggested the activation of inflammation-related pathways in VM ECs, and upregulation of myogenesis pathway in CVM SMCs. In IME analysis, immune cells were identified to accounted for nearly 30% of the total cell number, including macrophages, monocytes, NK cells, T cells and B cells. Notably, more macrophages and monocytes were discovered in VM, indicating innate immune responses might be more closely related to VM pathogenesis. In addition, angiogenesis pathway was highlighted among the significant pathways of macrophages & monocytes between CVM and VM. In VM, VEGFA was highly expressed in macrophages & monocytes, while its receptors were all abundantly present in ECs. The close interaction of VEGFA on macrophages with its receptors on ECs was also predicted by CellPhoneDB analysis. Our results document cellular composition, significant pathways, and critical IME in CVM and VM development.

Pilot investigation of circulating angiogenic and inflammatory biomarkers associated with vascular malformations

BackgroundVascular malformations in the central nervous system are difficult to monitor and treat due to their inaccessible location. Angiogenic and inflammatory proteins are secreted into the bloodstream and may serve as useful biomarkers for identifying patients at risk for complications or with certain disease phenotypes.MethodsA validated multiplex protein array consisting of 26 angiogenic and inflammatory biomarkers (Angiome) was assessed in plasma isolated from healthy controls and patients with either sporadic brain arteriovenous malformation (BAVM), familial cerebral cavernous malformation (CCM), or hereditary hemorrhagic telangiectasia (HHT). These samples were obtained from archives of ongoing research studies at the University of California San Francisco and through prospective collection at the Toronto HHT Centre at St. Michael’s Hospital.ResultsWe compared circulating biomarker levels from each patient group to healthy controls and analyzed each pairwise combination of patient groups for differences in biomarker levels. Additionally, we analyzed the HHT samples to determine the association between biomarker levels and the following HHT-specific phenotypes, BAVM, pulmonary arteriovenous malformation (PAVM), liver vascular malformation (LVM), and gastrointestinal (GI) bleeding. Compared to controls, levels of SDF1 were significantly elevated in HHT patients (Proportional Increase [PI] = 1.87, p < 0.001, q = 0.011). Levels of sENG were significantly reduced in HHT patients compared to controls (PI = 0.56, p < 0.001, q < 0.001), reflecting the prevalence of HHT1 patients in this cohort. Levels of IL6 (PI = 3.22, p < 0.001, q < 0.001) and sTGFβR3 (PI = 0.70, p = 0.001, q < 0.029) differed significantly in CCM patients compared to controls. Compared to controls, ten of the biomarkers were significantly different in sporadic BAVM patients (q-values < 0.05). Among the pairwise combinations of patient groups, a significant elevation was observed in TGFβ1 in CCM patients compared to sporadic BAVM patients (PI = 2.30, p < 0.001, q = 0.034). When examining the association of circulating biomarker levels with HHT-specific phenotypes, four markers were significantly lower in HHT patients with BAVM (q-values < 0.05), and four markers were significantly higher in patients with LVM (q-values < 0.05).ConclusionsThis pilot study suggests that the profile of circulating angiogenic and inflammatory biomarkers may be unique to each type of vascular malformation. Furthermore, this study indicates that circulating biomarkers may be useful for assessing phenotypic traits of vascular malformations.

The Versatility of the Free Vastus Lateralis Muscle Flap: Orbital Reconstruction After Removal of Complex Vascular Malformation in a Pediatric Patient.

Introduction: Vascular orbital lesions in pediatric population represent a demanding therapeutic challenge which requires a multidisciplinary team. In severe cases, orbital enucleation can be considered. Surgical management of enucleated orbital region in children, differently from the adults, represents a challenging procedure owing to the intrinsic relation between volume replacement and normal orbital growth. Many reconstructive options have been proposed, and many donor sites have been utilized for this purpose but each one have demonstrated potential disadvantages. Despite its well-known versatility, no report of the vastus lateralis free flap in children requiring orbital reconstruction exists in literature. Herein, we propose this surgical strategy as a valid option for the reconstruction of an extended orbital defect in a pediatric patient suffering from a mixed type of vascular malformation.Material and Methods: A patient was referred from a foreign country with an unclear medical history, presenting exorbitism and exophthalmos, proptosis of the eyeball, visus 4/10, and limited ocular motility. We made clinical-instrumental investigations with a diagnosis of complex vascular malformation. It expanded in intraorbital and retrorbital space with bulb anterior dislocation and optic nerve involvement. We performed an emptying of the orbital content via transconjunctival and via coronal incision with eyelid preservation. A free vastus lateralis muscle flap was used for reconstruction, filling the orbital cavity. We anastomosed the flap on the superficial temporal artery. An ocular conformator was then positioned.Results: We report the result at 12 months, showing a good orbital rehabilitation with an adequate prosthetic cavity, a good recovery of volume and facial symmetry, guaranteeing balanced orbital and periorbital growth. There were no major or minor complications associated with the procedure.Discussion: The reconstruction of the orbit remains a “surgical challenge” both in adults, whose goal is the restoration of volume, adequate symmetry and facial esthetics, and children, in which correcting the asymmetry has the additional objective to balance orbital growth. Many reconstructive techniques have been proposed, including the use of free flaps. The versatility of the free vastus lateralis muscle flap is well-known. It offers adequate amount of tissue with minimal morbidity to the donor site, provides a long pedicle, gives the possibility of simultaneous work in a double team, and has a constant anatomy and a safe and rapid dissection. There are no descriptions of its use for pediatric orbital reconstructions.Conclusions: In our opinion, the free vastus lateralis flap should be included as one of the best option for orbital pediatric reconstruction after enucleation.

Conventional and advanced MRI evaluation of brain vascular malformations.

Vascular malformations (VMs) of the central nervous system (CNS) include a wide range of pathological conditions related to intra and extracranial vessel abnormalities. Although some VMs show typical neuroimaging features, other VMs share and overlap pathological and neuroimaging features that hinder an accurate differentiation between them. Hence, it is not uncommon to misclassify different types of VMs under the general heading of arteriovenous malformations. Thorough knowledge of the imaging findings of each type of VM is mandatory to avoid these inaccuracies. Conventional MRI sequences, including MR angiography, have allowed the evaluation of CNS VMs without using ionizing radiation. Newer MRI techniques, such as susceptibility-weighted imaging, black blood sequences, arterial spin labeling, and 4D flow imaging, have an added value of providing physiopathological data in real time regarding the hemodynamics of VMs. Beyond MR images, new insights using 3D printed models are being incorporated as part of the armamentarium for a noninvasive evaluation of VMs. In this paper, we briefly review the pathophysiology of CNS VMs, focusing on the MRI findings that may be helpful to differentiate them. We discuss the role of each conventional and advanced MRI sequence for VMs assessment and provide some insights about the value of structured reports of 3D printing to evaluate VMs.

Patient radiation exposure from embolo-sclerotherapy of peripheral vascular malformations

ObjectiveEmbolo-sclerotherapy (EST) is the mainstay therapy for peripheral vascular malformations that involves the exposure of patients to ionizing radiation. We analyzed the radiation exposure to patients from EST of peripheral vascular malformations during a 5-year period in a single specialist center. MethodsAll patients who had undergone EST at a single specialist center for peripheral vascular malformations from January 1, 2013 to January 8, 2018 were identified from a prospectively collected database. Data collection included basic demographics, procedure date, anatomic site, type of vascular malformations, and procedural details. Radiation exposure, measured as the dose–area product (DAP) and fluoroscopy time, of all patients who had undergone EST during the study period were retrospectively reviewed. Statistical analysis was performed using the Mann-Whitney U and Kruskal-Wallis tests for comparison between subgroups. P < .05 was considered statistically significant. ResultsA total of 237 patients (median age, 30 years; range, 1-73 years) had undergone 419 EST sessions during the study period. Of the 237 patients, 61 (25.7%) had had arteriovenous malformations (AVMs) and had undergone 140 EST sessions (33.4%) and 176 (74.3%) had had venous and lymphatic malformations and had undergone 279 EST sessions (66.6%). Patients with AVMs had undergone a median of 2 procedures (range, 1-13) compared with a median of 1 (range, 1-6) for venous and lymphatic malformations within the study period. The median DAP for the single and cumulative EST for peripheral vascular malformations was 1.26 Gycm2 (range, 0.00-698.36 Gycm2) and 1.91 Gycm2 (range, 0.00-1300.24 Gycm2), respectively. The median fluoroscopy time for single and cumulative EST was 19 seconds (range, 1-3846 seconds) and 30 seconds (range, 1-5843 seconds), respectively. Significantly greater patient radiation exposure, in DAP and fluoroscopy time, was measured for single and cumulative EST for AVMs compared with venous and lymphatic malformations (P < .01 for both; Mann-Whitney U test). A significant difference in DAP but not fluoroscopy time was found when the anatomic areas of vascular malformations were compared. ConclusionsPatient radiation exposure for EST for peripheral vascular malformations, measured in DAP and fluoroscopy time, appeared to be generally less than that reported for endovascular arterial and deep venous interventions. However, some patients with peripheral vascular malformations received relatively high radiation doses. Further studies to investigate the risk factors and long-term side effects of radiation exposure in these patients and strategies to reduce these are required.

Complex giant arteriovenous and lymphatic malformation of the abdominal wall

Vascular malformations can be congenital or traumatic, the former being the most common and resulting from the unregulated vascular development during the embryogenic period. They can be classified as fast or slow-flow congenital malformations, with arteriovenous malformations being in the first group. It is rare when it happens in childhood and are associated with the lymphatic component. Girl, 6 years old, with nodulation of growth in the region of the anterior chest-abdominal wall until close to the spine. Angiotomography showed a mass volume of parts, next to the hemithorax wall and left flanks, suggestive of a mixed (lymphatic) component. After undergoing surgical resection of the lesion, she had good evolution, with satisfactory follow-up. Vascular malformations are most commonly diagnosed in childhood. Lymphatic malformations are a type of vascular malformation that can cause rare anomalies and, despite their benign nature, can infiltrate different planes of adjacent tissues. Surgical resection is not always possible due to the nature of the lesion; however, when it is performed, it can be curative. The arteriovenous malformations of mixed components are rare. Also, they should be discussed in a multidisciplinary manner among the surgical specialties and can have good results when properly diagnosed and treated. • Vascular malformations can be congenital or traumatic. • It can be classified as fast-flow, slow-flow, and lymphatic malformations. • Vascular malformations of mixed component are rare and may have repercussions. • Surgical resection is not always possible due to the nature of the lesion. • One of the curative treatments is surgical resection. • Vascular malformations can be congenital or traumatic. Besides, they can be classified as fast-flow, slow-flow, and lymphatic malformations. • Vascular malformations of mixed component are rare and may have important repercussions for pediatric patients. • Surgical resection is not always possible due to the nature of the lesion; however, when it is performed, it can be curative.

Spontaneous intracerebral hematoma: Which etiologies? And what predictions prognosis? A study about 253 patients

Intra-cerebral hematoma (ICH) is a serious pathology, destructive for the parenchyma, whose mortality is very hight and had a reserved prognosis. The factors influencing the vital and functional prognosis are however poorly known and the decision to resuscitate or evacuate a hematoma is sometimes very delicate in the acute phase, We conducted a retrospective, descriptive and analytical study of 253 patients who presented spontaneous ICH collected at the Military Hospital of Tunis, over a period of 10 years. The first cause of HIC was hypertension (54.1%). The mean age of the patients was 62 years and the clinical manifestations were dominated by the motor deficit. Vascular malformations were the main secondary causes of ICH. Radiologically, the lobar seat of the hematomas was the most recurrent with varying volumes depending on the type of vascular malformation In terms of prognosis, the leading cause of death from all causes was septic shock (17.39%). The ICH score has also been applied in our population and it has been observed that the mortality rate increases in parallel with the ICH score. Complete functional recovery (Rankin <= 2) was seen in 29,2% of our patients whereas 70.8% had moderate to severe disability (Rankin> = 3). Contemporary with the aging of the population, the frequency of HIC is constantly increasing. Prognosis is generally less favorable than ischemic stroke with a high mortality rate. Thus acting on these factors associated with poor life and functional prognosis for better management.

Clinical experience using neodymium-doped yttrium aluminum garnet laser in cutaneous vascular malformations among Indian patients

Introduction: Cutaneous vascular anomalies are relatively common in clinical practice with varied clinical features and deformity. Various treatment modalities are used to manage this condition. Aims and Objectives: In this study, our aims were to evaluate the outcome of neodymium-doped yttrium aluminum garnet (Nd:YAG) laser in this type of vascular malformations. Materials and Methods: This was a prospective study conducted in the Department of Plastic Surgery in our institute from January 2014 to December 2015 among the patients having cutaneous vascular malformations. Results: There were total 34 patients; 20 patients were females and 14 were males. Sixteen were adults (>18 years) and 11 patients were below 1 year of age. Among different types of lesions, 23 (67.7%) were vascular malformations and 11 (32.3%) were hemangiomas. Most of the lesions (58.7%) showed positive effect of laser treatment as it becomes flat. All (100%) hemangiomas, i.e., infantile hemangioma and pyogenic granuloma become flat. However, 41.3% lesions showed no significant change in height. Among them, 58.3% were venous malformations, 25% capillary malformations, and 16.7% capillary venous malformations. Skin color was not changed in color among 58.9% cases. About 58.9% cases showed no skin texture changes (atrophic/hypertrophic). Excellent-to-fair outcome was observed among 64.8% cases. Conclusion: Nd:YAG laser therapy on cutaneous hemangiomas and vascular malformations is a good therapeutic option among Indian patients.

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.

Short-term side effects and patient-reported outcomes of bleomycin sclerotherapy in vascular malformations.

Vascular malformations (VM) are congenital lesions that can be debilitating and cause significant aesthetic and functional limitations. The chemotherapeutic agent bleomycin has been utilized as a sclerosant, directly injected percutaneously into the VM. Unfortunately, little is known about the benefits and short-term side effects of bleomycin with intralesional injections. An IRB approved, retrospective chart review was performed on patients with VM who had been treated with intralesional bleomycin. Data included type of VM, number of treatments, total bleomycin dose per m², and adverse effects. A questionnaire was administered to available patients to assess subjective outcomes and side effects. Forty-six patients were treated with 141 procedures of bleomycin sclerotherapy for VM. Patient ages ranged from 1 to 20years (median age 10 years). The median cumulative bleomycin dose was 16.3units/m²/person (range of 1.7-97.0units/m²/person). Sixty-three percent of patients were reached for a questionnaire to assess short-term side effects. Ninety percent of patients surveyed were satisfied to very satisfied with the results from the procedure. About 24% of patients experienced transient nausea, vomiting and/or local hyperpigmentation. Bleomycin sclerotherapy can be an effective treatment of VM with repeat exposure with minor risk of short-term side effects, however, long-term risks are of great concern. Further studies are required to assess systemic absorption and long-term risks.

Combined capillary-venous-lymphatic malformations without overgrowth in patients with Klippel-Trénaunay syndrome.

Klippel-Trénaunay syndrome (KTS) is described in the literature as a complex syndrome characterized by various combinations of capillary, venous, and lymphatic malformations associated with limb overgrowth. In the first description by Maurice Klippel and Paul Trénaunay, tridimensional bone hypertrophy was believed to be the cause of limb enlargement. The purpose of this study was primarily to assess the presence of real bone hypertrophy as a cause of enlargement of the limb and to underline the rare presence of undergrowth of the affected limb in patients with KTS. A two-center retrospective review including 17 KTS patients with various combinations of capillary, venous, and lymphatic malformation affecting the lower limb was performed. Differences in limb dimension were evaluated clinically. Width and length discrepancy of the affected limb was measured with radiologic imaging. We found an increase of length in the affected limb in 80% of the patients. The leg length discrepancy varied from 0.2 to 2.6cm. The median leg length discrepancy was found to be 1.4cm. Three patients had a reduced length of the affected limb. Girth enlargement of the affected extremity was noticed in 60% of the patients, and 2 of 17 patients had hypotrophy of the involved limb. Hypertrophy (an increase in both length and width) of the bone was found in none of our cases, and the circumferential enlargement of the affected extremity was related only to soft tissue enlargement. In the literature, KTS is considered the prototype of overgrowth syndromes associated with complex vascular malformations. The majority of our patients showed limb length increase associated with soft tissue enlargement without an increase of bone width; there were also two patients with limb undergrowth. A real bone overgrowth (an increase in both length and width) was not present in our patients. Therefore, we could consider the absence of real bone hypertrophy as probably a new aspect of such confusing and controversial definitions of KTS. In addition, it would be more accurate to classify KTS patients on the basis of their phenotypic features (type of vascular malformation, types of overgrown tissue) rather than by use of an outdated eponym.