ISSVA classification
Abstract
Mulliken and Glowacki, in 1982 created a classification system of vascular anomalies which divided vascular anomalies into tumors and malformations which provided the framework for great advances in the management of these patients. This classification system was recently expanded at the 2014 ISSVA workshop in Melbourne. This revision again provides much greater detail including newly named anomalies and identified genes to account for recent advances in knowledge and clinical associations.
References (3)
- J.B. Mulliken et al.
Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics
Plast Reconstr Surg
(1982)
Cited by (206)
Vascular anomalies
2024, Surgery (United Kingdom)Vascular anomalies are vascular lesions that are present from childhood. They are classified into tumours or malformations based on clinical and histological features. Benign infantile haemangiomas are the most common vascular tumour and have a predictable self-limiting course. Rarer vascular tumours exist, and some may cause platelet consumption. Management is usually conservative, with active treatment reserved for functional or cosmetic complications (ulceration, or obstruction and distortion of vital structures). Oral propranolol is useful for troublesome lesions. Vascular malformations are structural anomalies of vascular morphogenesis present at birth without cellular proliferation that, in general, grow with the patient. They are sub-classified by vessel type as ‘low flow’ (capillary, lymphatic and venous) and ‘high flow’ (arteriovenous) or lesions with a combination of vessel type. They become problematic under certain circumstances, usually puberty and also pregnancy. The most troublesome are extensive lesions, especially venous and arteriovenous. Their effects may be cosmetic, those of a space-occupying lesion, infection, bleeding, pain or coagulopathy. Venous lesions cause consumptive coagulopathy, sometimes with life-threatening risks. Treatment options include medication and symptom control with antibiotics, analgesia, control of menses, compression garments and intervention with laser (capillary type), sclerotherapy, embolization and/or surgical excision. Patients with complex lesions are best managed by a multidisciplinary team and all surgical sub-specialties may be involved.
Pediatric hepatic vascular tumors: clinicopathologic characteristics of 33 cases and proposed updates to current classification schemes
2023, Human PathologyPediatric hepatic vascular tumors (HVTs) are rare neoplasms with features distinct from their cutaneous counterparts. Their behavior ranges from benign to malignant, with each subtype having therapeutic differences. Histopathologic descriptions of large cohorts are scarce in the literature. Thirty-three putative HVTs diagnosed from 1970 to 2021 were retrieved. All available clinical and pathologic materials were reviewed. Lesions were reclassified according to the World Health Organization (WHO) classification of pediatric tumors [1] as hepatic congenital hemangioma (HCH; n = 13), hepatic infantile hemangioma (HIH; n = 10), hepatic angiosarcoma (HA; n = 3), and hepatic epithelioid hemangioendothelioma (HEH; n = 1). Vascular malformations (n = 5) or vascular-dominant mesenchymal hamartoma (n = 1) were excluded. HCH frequently showed involutional changes, whereas HIH often had anastomosing channels and pseudopapillae formation. HA had solid areas with epithelioid and/or spindled endothelial morphology, significant atypia, increased mitoses, high proliferation index, and occasionally necrosis. On morphology analysis, a subset of HIH showed features worrisome for progression to HA including solid glomeruloid proliferation, increased mitoses, and epithelioid morphology. The widely metastatic and fatal HEH was observed in a 5-year-old male with multiple liver lesions. Immunohistochemically, HIHs and HA were Glucose transporter isoform 1 (GLUT-1) positive. One HIH patient died from postoperative complications, whereas 3 are alive without disease. Five HCH patients are alive and well. Two of three HA patients died of disease, and 1 is alive without recurrence. To our knowledge, this is the largest series of pediatric HVTs reviewing clinicopathologic features based on current Pediatric WHO nomenclature [1]. We highlight diagnostic challenges and propose inclusion of an intermediate category between HIH and HA which warrants closer follow-up.
Clinical phenotype of adolescent and adult patients with extracranial vascular malformation
2023, Journal of Vascular Surgery: Venous and Lymphatic DisordersIn recent years, genotypic characterization of congenital vascular malformations (CVMs) has gained attention; however, the spectrum of clinical phenotype remains difficult to attribute to a genetic cause and is rarely described in the adult population. The aim of this study is to describe a consecutive series of adolescent and adult patients in a tertiary center, where a multimodal phenotypic approach was used for diagnosis.
We analyzed clinical findings, imaging, and laboratory results at initial presentation, and set a diagnosis according to the International Society for the Study of Vascular Anomalies (ISSVA) classification for all consecutively registered patients older than 14 years of age who were referred to the Center for Vascular Malformations at the University Hospital of Bern between 2008 and 2021.
A total of 457 patients were included for analysis (mean age, 35 years; females, 56%). Simple CVMs were the most common (n = 361; 79%), followed by CVMs associated with other anomalies (n = 70; 15%), and combined CVMs (n = 26; 6%). Venous malformations (n = 238) were the most common CVMs overall (52%), and the most common simple CVMs (66%). Pain was the most frequently reported symptom in all patients (simple, combined, and vascular malformation with other anomalies). Pain intensity was more pronounced in simple venous and arteriovenous malformations. Clinical problems were related to the type of CVM diagnosed, with bleeding and skin ulceration in arteriovenous malformations, localized intravascular coagulopathy in venous malformations, and infectious complications in lymphatic malformations. Limb length difference occurred more often in patients with CVMs associated with other anomalies as compared with simple or combined CVM (22.9 vs 2.3%; P < .001). Soft tissue overgrowth was seen in one-quarter of all patients independent of the ISSVA group.
In our adult and adolescent population with peripheral vascular malformations, simple venous malformations predominated, with pain as the most common clinical symptom. In one-quarter of cases, patients with vascular malformations presented with associated anomalies on tissue growth. The differentiation of clinical presentation with or without accompanying growth abnormalities need to be added to the ISSVA classification. Phenotypic characterization considering vascular and non-vascular features remains the cornerstone of diagnosis in adult as well as pediatric patients.
Ultrasound-guided microwave ablation of soft tissue venous malformations
2023, Journal of Vascular Surgery: Venous and Lymphatic DisordersTo assess the feasibility, safety, and effectiveness of microwave ablation (MWA) for soft tissue venous malformations (VMs).
We retrospectively analyzed 20 patients with symptomatic VMs in the lower extremities and trunk. MWA was performed in all 20 patients. Contrast-enhanced ultrasound (CEUS) was performed immediately and 1 month after ablation. The data on the complete ablation rate, symptom improvement, and patient satisfaction were collected. Follow-up examinations began 1 month after ablation and continued for 2 to 19 months.
MWA was performed on 20 patients. The overall complete ablation rate was 75% (15/20). The complete ablation rate for the 11 patients with lesions <5 cm and the 9 patients with lesions >5 cm were 100% (11/11) and 44% (4/9), respectively. The complete ablation rate for the 14 patients with localized and 6 patients with extensive lesions was 93% (13/14) and 33% (2/6), respectively. Five patients had experienced incomplete ablation; 1 had a lesion >5 cm with ectopic drainage vessels, one had multiple lesions adherent to blood vessels and nerves, and three had large and extensive lesions. Thirteen patients had experienced significant improvements in clinical symptoms; 6 showed slight improvement; and 1 showed no change in symptoms. No complications or recurrences were observed. All 20 patients were satisfied with the therapeutic effect (100%). Twelve patients were significantly satisfied and 8 were slightly satisfied.
MWA with ultrasound guidance is a safe, effective, and minimally invasive therapy for symptomatic soft tissue VMs and may be a promising approach in the future.
Long-term changes of health-related quality of life in patients with peripheral vascular malformations – a prospective observational study
2023, Journal of Plastic, Reconstructive and Aesthetic SurgeryThe aim of this observational study was to assess health-related quality of life (HRQOL) changes in patients with vascular malformations, over a period of almost eight years, and to assess clinical and demographic characteristics possibly associated with HRQOL changes.
Eighty out of 111 patients who were included in a previously published comparative HRQOL study accepted inclusion in this follow-up study. HRQOL at baseline and follow-up was assessed with the Short-Form 36-item questionnaire (SF-36). Median observation time was 7.9 years. Linear mixed models and linear regression models were applied to assess HRQOL change and possible associations with demographic and clinical variables.
The median age of the patient cohort at baseline evaluation (n = 111) was 27.0 years. Ninety-six out of 111 (86.5%) patients were diagnosed with venous malformations. Significantly higher SF-36 scores at follow-up were found for the physical domains Role limitations due to (RLDT) physical problems (difference=13.5; 95% CI [1.6, 25.3]) and Bodily pain (difference=11.3; 95% CI [3.8, 18.8]). No deterioration of HRQOL was found in any domain. In multivariate analyses, female gender, muscle/bone involvement, and higher age were associated with a positive relative change in SF-36 in the domains Physical functioning, RLDT physical problems, and RLDT emotional problems, respectively. Invasive treatment was not associated with long-term HRQOL change.
Over a period of almost eight years, significant improvement of SF-36 scores was observed in the physical domains RLDT physical problems and bodily pain. Female gender, muscle/bone involvement, and higher age were associated with HRQOL improvement in certain domains.
Vascular Malformations
2023, Problem Solving in Pediatric ImagingVascular malformations arise from disorganized angiogenesis and manifest as congenital abnormalities that may be isolated events or associated with syndromes. The International Society for the Study of Vascular Anomalies (ISSVA) recognizes vascular malformations as a separate entity from vascular tumors and classifies vascular malformations into four major groups: simple, combined, anomalies of major vessels, and those associated with other vascular anomalies. This chapter will discuss the clinical and imaging findings of vascular malformations across ultrasound and magnetic resonance imaging modalities. Vascular tumors is discussed in chapter 16.