Vascular Anomalies In Children Research Articles

Genitourinary and perineal vascular anomalies in children: A Seattle children's experience

We sought to evaluate the initial presentation and diagnosis, to compare presenting symptoms of these anomalies with respect to location, and describe the management course and review the treatment outcomes of vascular anomalies (VA) in genitourinary and perineal area. We described changes in the evaluation and management approach over time. After IRB approval, we reviewed the Vascular Anomalies Program 3,780 patient database from 1998-2013, identifying 85 patients with GUP VA. Demographics, presentation, anatomic location, imaging modalities and treatments were reviewed. For the 76 patients that were managed at our institution, we analyzed data for management approach change over time, duration of follow-up, complications, and treatment outcomes. Using Chi-squared test, we analyzed the treatment eras for any difference in presenting symptoms, or management approach. From the VAP database, we identified 85 patients with GUP VA. Vascular tumors (VT) were more common than vascular malformations (VM) (62/85, 72.9% vs. 23/85, 27.1%). The 62 patients with VT were subdivided into infantile hemangioma (IH) (49, 79%) and congenital hemangioma (CH) (13, 20.9%). The 23 patients with VM were subdivided into simple (18, 78.2%) and complex (2, 8.7%), and one patient had Klippel-Trenaunay syndrome. Overall, genital VA were more common than perineal (43/85, 54.2% vs. 24/85, 28.2%). None of our patients had urinary involvement. Ultrasound was used in 34% (29/85) of VA patients, the majority were VM (18/23, 78%). Majority of patients were symptomatic with ulceration, pain and bleeding (54/85, 63.5%). Malformations were more complex, presenting as extensive lesions (6/23, 26.1%), which included pelvic extension in 5 (21.7%). Seventy-six patients were managed at our institution. Treatment was utilized more frequently than observation overall (46/76, 60% vs. 30/76, 40%), and more frequently for VM than VT (12/18, 63% vs. 34/58, 57%). After introduction of oral propranolol for treatment of IH in 2010, fewer patients presented with ulceration (p=0.213), and more were treated with propranolol over procedural therapy (p=0.157). Overall, complete treatment of these lesions was achieved in 82.9% (63/76), with low complication rate (6/76, 7.9%). Evaluation and management of VA is best performed with expert multidisciplinary team at tertiary care center. Imaging studies are mostly used in evaluation of VM. Symptomatic VA involving the perineum and genitalia require therapeutic management. With the introduction of propranolol for treatment of IH, oral therapies were used more often with excellent outcomes. A multidisciplinary approach is essential to treatment innovation, patient-centered therapies and improving therapeutic outcomes.

Versatility of Tissue Expansion in Abdominal Wall Reconstruction After Removal of Vascular Malformations in Children

Children who have had extensive abdominal wall vascular anomalies (VAs) face 3 common reconstructive options: either tolerating the complications of these lesions, exposure to repeated laser therapy session with its sequelae, or excision followed by a color-matched skin graft. The use of serial tissue expansion and excision of the affected skin offers a potentially better option under the right circumstances. Tissue expanders (TEs) have broad applicability but are not without complications. To study the versatility of use of TEs in the management of children who had extensive VAs in their abdominal wall with special emphasis to the complications and children and parental satisfaction. Retrospective data collection of 12 patients' charts, operative data of 84 operative procedures, and follow-up visits. Statistical analysis done using Student t test significance and P<0.05 was considered statistically significant. Using 35 different sizes of TEs and 84 operative procedures in 9 boys and 3 girls aged from 2 to 12 years with different types of abdominal wall VAs revealed an overall complications rate of 20%. Child and parental satisfaction was found to be good overall. Tissue expander is a useful and feasible tool for reconstruction of the abdominal wall with extensive VAs in children; parents and children were satisfied and body image is acceptable.

Extremity amputations for vascular anomalies in children

Extremity amputations for vascular anomalies in children

Multimodality imaging of vascular anomalies

Vascular malformations and hemangiomas are common in children but remain a source of confusion during diagnosis, in part because of the lack of a uniform terminology. With the existing treatments for hemangiomas and vascular malformations, it is important to make the correct diagnosis initially to prevent adverse physical and emotional sequelae in not only the child but also the family. The diagnosis of vascular malformations is made primarily by the clinician and based on the physical exam. Imaging is carried out using predominantly ultrasound (US) and magnetic resonance imaging (MRI), which are complementary modalities. In most cases of vascular anomalies, US is the first line of imaging as it is readily available, less expensive, lacks ionizing radiation and does not require sedation. MRI is also of great help for further characterizing the lesions. Conventional arteriography is reserved for cases that require therapeutic intervention, more commonly for arteriovenous malformations. Radiographs usually play no role in diagnosing vascular anomalies in children. In this article, the author describes the terminology and types of hemangiomas and vascular malformations and their clinical, histological features, as well as the imaging approach and appearance.

The comparison of efficacy of different imaging techniques (conventional radiography, ultrasonography, magnetic resonance) in assessment of wrist joints and metacarpophalangeal joints in patients with psoriatic arthritis

SummaryBackground:Vascular anomalies are usually diagnosed through their clinical picture and history. The purpose of this study was to assess the role of MR imaging in initial assessment of cervicofacial vascular anomalies in children.Material/Methods:Twenty pediatric patients with vascular anomalies located in the cervicofacial region underwent MRI examination in our department. Images were evaluated for lesion detectability and its signal characteristics (on T1w, T2w images with fat suppression and contrast enhanced T1w sequences); the extent of the lesions and surrounding tissue involvement were also assessed.Results:In the studied group MR images revealed all anomalies and provided information of their anatomic extent and invasion of surrounding anatomic structures. Nine hemangiomas and six venous malformations were found among studied patients. Two children had multiloculated lesions corresponding to lymphatic malformations. One examination visualized a lesion consisting mainly of dilated vascular channels with an apparent feeding artery, which was consistent with arteriovenous malformation. Two remaining lesions were mixed malformations. Nine patients had lesions limited to subcutaneous tissue. Two masses infiltrated bone structures. There was muscle involvement found in nine cases.Conclusions:MR imaging is a well-established method for detection and monitoring of vascular anomalies in children. With ultrasound used mostly for initial diagnosis and additional flow assessment, angiography viewed as an invasive therapeutic method and computed tomography used only in specific situations due to its high irradiation dose, magnetic resonance is the best imaging method used in differential diagnosis and topographical characterization of vascular malformations and tumors of cervicofacial area in pediatric patients. Noninvasively and without irradiation, it enables evaluation of the extent and characteristics of lesions and planning proper therapeutic strategy.

MR imaging characteristics of soft tissue vascular anomalies in children

Accurate classification of soft tissue vascular anomalies is critical since treatment options and morbidity differ significantly for the various groups of vascular anomalies (VA). A classification system introduced by Mulliken and Glowacki in 1982 explained the biology of VA, thus resulting in improved management and communication between different disciplines taking care of children with soft tissue vascular anomalies. This classification has been updated by the International Society for the Study of Vascular Anomalies in 1996 and forms the basis for the current nomenclature. Although the majority of vascular anomalies can be accurately classified by their clinical history and a physical exam, imaging is necessary to determine the full anatomical extent of the anomaly, to follow up treatment response, to confirm diagnosis, and to provide correct classification in challenging cases when necessary. This article will review the key magnetic resonance imaging (MRI) features of most common soft tissue vascular anomalies in children. MRI and dynamic contrast-enhanced magnetic resonance angiography are crucial for the evaluation of vascular anomalies in children because of lack of radiation, high soft tissue resolution, and the capability of dynamic contrast-enhanced images to reflect the hemodynamics of the anomalies.

A Case Series of Genital Vascular Anomalies in Children and Their Management: Lessons Learned

To review our experience with genital vascular anomalies and discuss the management considerations for patients with associated genitourinary defects. We reviewed the presentation, course, management considerations, surgical treatment, and follow-up of all cases of genital vascular anomalies treated at a single institution from January 2008 to October 2011. The lesions were classified according to the International Society for the Study of Vascular Anomalies. All patients were boys <18 years old. We identified 3 patients with genital vascular anomalies. Of these 3 patients, 2 had an infantile hemangioma and 1 had a venous malformation. All lesions were identifiable on physical examination, and 2 of the patients presented within a few months of birth. One patient had associated genitourinary abnormalities that complicated his treatment. Scrotal ultrasonography and pelvic magnetic resonance imaging consistently showed the vascular anomalies to be highly vascular and distinct from the underlying testes. Both intrascrotal lesions were excised, and the cutaneous lesion was excised as a part of a larger genitourinary reconstruction. At a mean follow-up of 33 months (range 23-42), the intrascrotal infantile hemangioma had recurred, requiring repeat intervention, but the cutaneous hemangioma had not. Vascular anomalies of the male genitalia are rare. Pelvic magnetic resonance imaging is useful for characterizing the internal extent of vascular anomalies and ultrasonography is useful in monitoring these lesions over time. The timing of surgery and the high recurrence rate are important considerations when planning surgical resection of genital vascular anomalies, especially when associated with concomitant genitourinary defects.

Surgical management of head and neck vascular anomalies in children: a retrospective analysis of 42 patients

This article aimed to present a series of surgically treated head and neck vascular anomalies during a 12-year period, highlighting the epidemiology, diagnostic approach, indications for surgery, and final clinical outcome. The medical records of all patients with head and neck vascular anomalies, surgically treated at our department from 1998 to 2010, were reviewed retrospectively. A total of 42 patients with 46 vascular anomalies were identified. Patients' diagnoses included vascular tumors, hemangiomas mainly (18 cases), and various vascular malformations (26 cases). All patients were submitted to surgical treatment (excision-resection) to resolve main clinical symptoms (ulceration, bleeding, impaired mastication and feeding, airway obstruction) and/or esthetic issues. Recurrence was noted in 3 patients. Accurate differential diagnosis based on history, physical examination, and imaging, is the key to optimal treatment. Surgical intervention is warranted for small to moderately extended lesions to avoid complications and/or esthetic concerns and psychosocial distress.

Vascular and Perivascular Lesions of Skin and Soft Tissues in Children and Adolescents

Vascular anomalies in children and adolescents are the most common soft tissue lesions and include reactive, malformative, and neoplastic tumefactions, with a full spectrum of benign, intermediate, and malignant neoplasms. These lesions are diagnostically challenging because of morphologic complexity and recent changes in classification systems, some of which are based on clinical features and others on pathologic findings. In recent decades, there have been significant advances in clinical diagnosis, development of new therapies, and a better understanding of the genetic aspects of vascular biology and syndromes that include unusual vascular proliferations. Most vascular lesions in children and adolescents are benign, although the intermediate locally aggressive and intermediate rarely metastasizing neoplasms are important to distinguish from benign and malignant mimics. Morphologic recognition of a vasoproliferative lesion is straightforward in most instances, and conventional morphology remains the cornerstone for a specific diagnosis. However, pathologic examination is enhanced by adjunctive techniques, especially immunohistochemistry to characterize the type of vessels involved. Multifocality may cause some uncertainty regarding the assignment of "benign" or "malignant." However, increased interest in vascular anomalies, clinical expertise, and imaging technology have contributed greatly to our understanding of these disorders to the extent that in most vascular malformations and in many tumors, a diagnosis is made clinically and biopsy is not required for diagnosis. The importance of close collaboration between the clinical team and the pathologist cannot be overemphasized. For some lesions, a diagnosis is not possible from evaluation of histopathology alone, and in a subset of these, a specific diagnosis may not be possible even after all assembled data have been reviewed. In such instances, a consensus diagnosis in conjunction with clinical colleagues guides therapy. The purpose of this review is to delineate the clinicopathologic features of vascular lesions in children and adolescents with an emphasis on their unique aspects, use of diagnostic adjuncts, and differential diagnosis.

Vascular anomalies in children

Vascular anomalies are divided in two major categories: tumours (such as infantile hemangiomas) and malformations. Hemangiomas are common benign neoplasms that undergo a proliferative phase followed by stabilization and eventual spontaneous involution, whereas vascular malformations are rare structural anomalies representing morphogenetic errors of developing blood vessels and lymphatics. It is important to properly diagnose vascular anomalies early in childhood because of their distinct differences in morbidity, prognosis and need for a multidisciplinary management. We discuss a number of characteristic clinical features as clues for early diagnosis and identification of associated syndromes.

Pediatric Small Vessel AVMs Mistaken for Hemangioma

Objective: A majority of vascular anomalies in children are infantile hemangiomas (IH). However, some children with an atypical growth pattern can be suggestive of an AVM. This case series discusses the clinical presentation, diagnosis, radiology, pathology, and treatment of small vessel AVMs at a multidisciplinary vascular anomalies center. Method: Retrospective case series of three patients. Chart review was obtained with IRB approval. Results: Three patients presented with increasing head and neck masses at 12 months to 2.5 years. All patients had magnetic resonance imaging indicating likely infantile hemangioma. All patients underwent surgical excision, one requiring preoperative embolization. All pathology specimens were GLUT 1 negative and were diagnosed as small vessel arteriovenous malformations. Serial clinical photographs, imaging studies, and pathology will be presented. Conclusion: Many pediatric vascular anomalies appear to be an IH, but atypical growth patterns should raise clinical suspicion of an AVM. Diagnostic error leads to inadequate treatment, disease progression, and increased morbidity. This series highlights the importance of clinical suspicion and a multidisciplinary team approach toward pediatric vascular anomalies.

Sirolimus for the treatment of complicated vascular anomalies in children

Vascular anomalies comprise a diverse group of diagnoses. While infantile hemangiomas are common, the majority of these conditions are quite rare and have not been widely studied. Some of these lesions, though benign, can impair vital structures, be deforming, or even become life-threatening. Vascular tumors such as kaposiform hemangioendotheliomas (KHE) and complicated vascular malformations have proven particularly difficult to treat. Here we retrospectively evaluate a series of six patients with complicated, life-threatening vascular anomalies who were treated with the mTOR inhibitor sirolimus for compassionate use at two centers after failing multiple other therapies. These patients showed significant improvement in clinical status with tolerable side effects. Sirolimus appears to be effective and safe in patients with life-threatening vascular anomalies and represents an important tool in treating these diseases. These findings are currently being further evaluated in a Phase II safety and efficacy trial.

Head and neck vascular anomalies in children

Vascular lesions are the most common congenital and neonatal abnormalities. The aim of this work is to point out differences between various vascular anomalies, in order to define accurate diagnosis, and to present different therapeutic options now used for the treatment of the vascular lesions in children. According to biological classification described by the work of Mulliken and Glowacki there are two major types of vascular abnormality: haemangioma and vascular malformation. Haemangioma is a distinct biologic tumour entity characterised by rapid endothelial proliferation shortly after birth. The lesion is absent at birth and growth in early infancy, followed by a spontaneous resolution in childhood. Vascular malformations are structural anomalies that have a normal growth rate and endothelial turnover. According to the morphology of the vessels and the flow rate we distinguish: slow-flow and fast-flow vascular malformation. The authors document their personal experience in diagnosis, clinical evaluation, treatment and follow-up of the vascular lesions.

Extremity Vascular Anomalies in Children: Introduction, Classification, and Imaging

Vascular anomalies are vascular lesions noted at birth or that arise during the first months of life. The purpose of this article is to illustrate how vascular anomalies involve the extremities of infants and children. Cases illustrate the range of vascular anomalies as defined by the International Society for the Study of Vascular Anomalies. Both clinical and imaging features of extremity vascular anomalies are discussed. A comprehensive classification that uses a simple, universal, and common terminology is presented. Ultrasound and magnetic resonance imaging are noninvasive diagnostic tools that are widely used in pediatric practice, in particular to categorize many of these lesions, provide a diagnosis if there is clinical doubt, evaluate these lesions fully, and guide management and therapy.

O.498 Oral vascular anomalies in children: our 8 years experience

O.498 Oral vascular anomalies in children: our 8 years experience

Complex pancreatic vascular anomalies in children

Background Vascular anomalies are vascular tumors and congenital malformations that rarely involve the pancreas. Diagnosis and management of these lesions is complex. Methods An institutional review board–approved retrospective database and record review from 1994 through 2004 at a quaternary referral center for vascular anomalies was conducted. Results Of 5051 patients with a vascular anomaly, 6 had a lesion involving the pancreas. All patients were younger than 3 years. There were three tumors (two infantile hemangiomas and one Kaposiform hemangioendothelioma) and three malformations (two lymphatic and one venous). The referring diagnoses were correct for 4 patients. All anomalies were diagnosed with a combination of cross-sectional imaging and angiography. Five patients received medical therapy including two malformation patients who were initially treated with antiangiogenic drugs. Two patients with lymphatic malformations underwent operative intervention. One infantile hemangioma was treated with percutaneous biliary drainage for obstructive jaundice. Conclusion Complex pancreatic vascular anomalies are rare. Vascular tumors are generally amenable to treatment with antiangiogenic medications. Operative resection is reserved for refractory cases or complications. Symptomatic vascular malformations are treated exclusively by surgery. Evaluation and management of these lesions is complex and may benefit from coordinated interdisciplinary care.

Increased Expression of Urinary Matrix Metalloproteinases Parallels the Extent and Activity of Vascular Anomalies

Matrix metalloproteinases (MMPs) and the angiogenic proteins basic fibroblast growth factor (bFGF) and vascular endothelial growth factor (VEGF) have been implicated in mechanisms of human cancer and metastasis. Assays were conducted on the urine of patients with vascular anomalies (tumors and malformations), relatively common and occasionally life-threatening disorders for which few therapies exist. We sought to determine whether these angiogenesis modulators are present in the urine and whether their expression is associated with the extent and clinical course of the vascular lesion. A total of 217 patients with vascular anomalies and 74 age-matched control subjects participated. Urinary MMP expression was determined by substrate gel electrophoresis. Urinary bFGF and VEGF levels were measured by enzyme-linked immunosorbent assay. Each patient was assigned to 1 of 2 categories (tumor or malformation) and 1 of 9 specific groups. Extent of the vascular lesion and activity were scored by a blinded clinician. Urinary high molecular weight (hMW) MMPs and bFGF were significantly increased in patients with vascular tumors (53%) and vascular malformations (41%), compared with control subjects (22%). These percentages increased as a function of extent of the lesion and disease activity. hMW MMPs were increased in 4 groups: infantile hemangioma, other vascular neoplasms, lymphatic malformation and capillary-lymphaticovenous malformations, and extensive and unremitting capillary malformation and arteriovenous malformation. No significant differences among the groups were detected for low molecular weight MMPs or VEGF. Expression patterns of hMW MMPs and bFGF in the urine of patients with tumors and malformations are consistent with their different clinical behavior. These data represent the first evidence that MMPs are elevated in the urine of children with vascular anomalies. These data also suggest that the increased expression of urinary MMPs parallels the extent and activity of vascular anomalies in children. In addition to tumors, vascular malformations are angiogenesis dependent, suggesting that progression of a vascular malformation might be suppressed by angiogenic inhibitors, which would target bFGF and MMPs.

Practical Aspect of Intervention in Vascular Anomalies in Children

Practical Aspect of Intervention in Vascular Anomalies in Children

Intestinal vascular anomalies in children

Vascular anomalies are an uncommon cause of gastroin-testinal bleeding in childhood. Confusing nomenclature has made objective comparisons of published cases difficult and has interfered with an established consensus regarding diagnosis and therapeutic modalities. The purpose of this study was to clarify the situation by reviewing the records of all children who had intestinal vascular anomalies who were referred to our institution from 1975 to 1995. Thirteen lesions were identified in nine children (five boys and four girls). The median age at clinical onset was 8 years. Only two patients presented with a complex syndrome (Klippel-Trenaunay, 1; Osler-Rendu-Weber, 1). Diagnosis, location, and extension of these anomalies was only possible by angiography, which indicated that seven patients had isolated venous malformations and two had arteriovenous malformations. Because the lesions did not involve the serosa, intraoperative localization was a major problem. The main findings were a few slightly dilated mesenteric veins. Treatment was conservative in four children and surgical in five. Pathological findings on resected bowel demonstrated dilated and abnormal veins in the mucosa and submucosa. Selective angiography should not be delayed in patients with gastrointestinal bleeding if results of all other investigations are negative. Because these lesions are rarely recognizable on operative inspection, precise preoperative angiographic localization of intestinal vascular anomalies is essential to allow for a safe and limited resection of the involved bowel segment. Based on a better understanding of the natural history of these lesions, a classification of vascular anomalies of intestines in children is proposed.

Upper airway obstruction caused by vascular anomalies in children.

Vascular ring or sling is one of the important etiologies for congenital upper airway obstruction in children. Between July 1988 and July 1995, a total of 92 children with stridor were initially evaluated by an esophagogram and two-dimensional echocardiogram. Seven children (7.7%) with variable types of vascular anomalies were diagnosed including pulmonary artery sling (2 cases), double aortic arch (1 case), right arch with left aberrant subclavian artery and ligamentum ductus (1 case), double aortic arch with one atretic arch (2 cases), and innominate artery compression of the trachea (1 case). The age at diagnosis ranged from 18 days to 3 years old. Only one patient was associated with an intracardiac anomaly. Five of the 7 patients were diagnosed as having laryngomalacia previously. Six patients had esophageal indentation on their esophagograms. Besides external airway compression, three patients also had intrinsic tracheal stenosis. Six patients received surgery because of severe respiratory distress. The patients' symptoms improved after surgery, but a premature baby died after a second operation for intrinsic tracheal stenosis at the age of 8 months.