Clinical characteristics associated with pain in patients with peripheral vascular malformations.

OBJECTIVE: Vascular malformations (VM) can negatively impact the patient's quality of life (QoL). Pain is a common problem in these patients. The aim of this study was to investigate risk factors associated with pain and to assess how pain affects QoL. METHODS: This prospective cross-sectional study was conducted in a tertiary vascular anomaly expertise center. Between June and December 2020, all patients from our local database (334 adults and 189 children) with peripheral VMs were invited to complete the Outcome Measures for VAscular MAlformations questionnaire to evaluate the presence, frequency, and intensity of pain. Additionally, patients were asked to complete several Patient-Reported Outcome Measurement Information System scales to evaluate their QoL. Risk factors associated with pain were identified in bivariate analysis and multivariable logistic regression. QoL domains were compared between patients who experienced pain and patients who did not. RESULTS: A total of 164 patients completed the questionnaire about pain and 133 patients completed all QoL questionnaires. Approximately one-half of the patients (52%) reported pain in the past four weeks and 57% of these patients reported pain daily or several times a week. Female sex (P = .009), lesions located in the upper extremity (P < .001) or lower extremity (P < .001), and intramuscular/intraosseous lesions (P = .004) were independently associated with the presence of pain. The following QoL domains were diminished in patients who experienced pain in comparison with patients who did not: pain interference (P < .001), physical functioning (P < .001), and social participation (P < .001) in adults, and pain interference (P = .001), mobility (P = .001), and anxiety (P = .024) in children. CONCLUSIONS: Pain is a frequently reported complaint in patients with VMs and is present in approximately one-half of the patients. Patients with lesions located in the upper or lower extremity, intramuscular/intraosseous lesions, and female patients are more likely to experience pain. The presence of pain negatively impacted patients' QoL. Although VM are a benign condition and expectative management is frequently applied, our study shows that pain is a serious concern and needs to be actively assessed. Pain is a sign of various etiologies and should be examined to properly treat the pain.

Quality of life and mental health of patients with vascular malformations in a single specialist center in the United Kingdom.

OBJECTIVE: Patients with vascular malformations suffer from chronic debilitating symptoms that have been shown to contribute negatively to their quality of life (QoL) and mental health. Despite this, the current literature evaluating the QoL and mental health of patients with vascular malformations remains scarce. Our aim was to evaluate the QoL and mental health of patients with vascular malformations. METHODS: We prospectively analyzed the validated health-related QoL (HRQoL) questionnaires: the RAND Health Care 36-Item Short Form Survey (SF-36), Hospital Anxiety and Depression Scale (HADS), and visual analogue score for pain reported by 253 patients with vascular malformations in a specialist center of vascular anomalies in the UK over 2 years. RESULTS: Patients with vascular malformations reported significantly poorer SF-36 scores in all domains compared with the UK general population. Patients with low-flow vascular malformations and arteriovenous malformations reported little variations in SF-36, HADS, and visual analogue score for pain scores. No significant association was found between age and any of the health-related QoL scores, other than the physical functioning in SF-36. Female patients reported significantly lower physical and social functioning of SF-36 and worse HADS-Depression than their male counterparts. Patients with syndromic vascular malformations reported significantly lower SF-36 scores in role-physical, role-emotional and bodily pain than nonsyndromic vascular malformations. CONCLUSIONS: This study concluded that patients with vascular malformations reported worse QoL than the UK general population. Therefore, the assessment and management of QoL and mental health should be incorporated into the overall treatment strategies of patients with vascular malformations.

Diffuse Venous Malformations of the Upper Extremity (Bockenheimer Disease): Diagnosis and Management.

BACKGROUND: Diffuse venous malformations that involve all tissues in the upper limb and ipsilateral chest wall are known as "phlebectasia of Bockenheimer." The authors describe their experience with management of this uncommon vascular anomaly. METHODS: The authors' Vascular Anomalies Center registry comprised 18,766 patients over a 40-year period. This review identified 2036 patients with venous malformations of the extremities (10.8 percent), of whom only 80 (0.43 percent) had Bockenheimer disease. The authors retrospectively analyzed patient characteristics, diagnostics, treatments, and complications. RESULTS: The venous malformation was first noted at birth or within the first few years of life with slow and gradual progression. Pain was related to engorgement of the limb. Thromboses and phleboliths were common, but diffuse intravascular coagulopathy occurred in only 12 patients (15 percent). Skeletal involvement was demonstrated as lytic lesions, cortical scalloping, osteopenia, and pathologic fractures. Management included compression garments (100 percent), sclerotherapy (27.5 percent), and resection of symptomatic areas in 35 percent of patients. Adjunctive pharmacologic medication was given in 7.5 percent. Following resection, 17 patients (60 percent) had one or more complications: hematoma, wound dehiscence, flap loss, contracture, and psychosis. There were no deaths. Symptoms improved in all patients with useful functional outcomes. CONCLUSIONS: The decision to pursue compression, sclerotherapy, pharmacologic treatment, or resection alone or in combination was made by an interdisciplinary team. Although extensive venous malformations cannot be completely ablated, debulking of symptomatic regions, resection of neuromas, and noninvasive treatments improve the quality of life. Despite the bulk and weight of the arm, forearm, and hand, and the ominous appearance on magnetic resonance imaging, these patients remain functional. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Quality of life in patients with vascular malformations outside the central nervous system: Comparison with the general Norwegian population.

PURPOSE: The aim of this study was to assess Health-Related Quality of Life (HRQoL) in a population of patients with vascular malformations outside the central nervous system (CNS) and to compare the results with data from a national reference population. METHODS: In total, 111 consecutive patients above 14 years of age and referred for the first time to the national vascular malformation center from September 2011 to December 2012 were included. HRQoL was assessed using the Short-Form 36-item questionnaire (SF-36), which is a validated questionnaire with eight domains, covering both physical and mental aspects of HRQoL. The results were compared with national reference values. Possible association between HRQoL and selected demographic and clinical variables was analyzed using linear regression analysis. RESULTS: The sample consisted of 47 men (42.3%) and 64 women (57.7%). The median age of patients was 27 years (range 14-63 years). Ninety-six patients (86.5%) were diagnosed with venous malformations and nine patients (8.1%) with arteriovenous malformations. Six patients had other types of malformations (9%). The patients had significantly lower SF-36 scores in all domains, except for General health, than the general population. There was a significant association between muscular involvement and lower SF-36 scores in the physical domains Bodily pain and Role limitation due to physical problems. CONCLUSIONS: Our data suggest that patients with vascular malformations outside the CNS have impaired quality of life when compared with the general population. Muscular involvement seems to be associated with worse HRQoL in the physical aspects.

Vascular Malformations and Health-Related Quality of Life: A Systematic Review and Meta-analysis.

Importance: Patients with vascular malformations (VAMs) and vascular overgrowth syndromes have lower health-related quality of life (HRQoL) attributable to social stigmatization, poor mental health, severity, and pain. However, the factors that contribute to this decreased HRQoL are not clear. Objective: To perform a systematic review and meta-analysis of studies that used validated HRQoL instruments to compare the HRQoL of persons with VAMs with the US general population. Data Sources: A comprehensive search was performed in MEDLINE, Embase, PsycINFO, CINAHL, and Scopus from 1946 to March 31, 2017, with the consultation of an experienced librarian. Study Selection: All VAM studies with validated HRQoL instruments published in the English language were included. Case reports, review articles, non-English-language publications, and studies about the development of new HRQoL instruments were not included. Data Extraction and Synthesis: Two reviewers assessed studies' eligibility and the risk of bias and performed data extraction. The meta-analysis was performed using the random-effects model. Comparisons of means were performed using the unpaired, 2-sample t test. Main Outcomes and Measures: The outcome was HRQoL. Results: Eleven studies met the inclusion criteria for a total of 692 patients with VAMs. Six studies (320 patients) were included in the meta-analysis, whereas 5 studies were included in the qualitative analysis (372 patients). Those with VAMs had lower 36-Item Short-Form Health Survey scores in bodily pain (mean difference, -11.87; 95% CI, -21.45 to -2.29; I2 = 92%; P = .02) and mental health (mean difference, -6.04; 95% CI, -11.55 to -0.52; I2 = 83%; P = .03) compared with the US general population. Conclusions and Relevance: Patients with VAMs had increased pain and psychosocial distress compared with the US general population. Pain and psychological morbidity are associated with poorer HRQoL and may serve as indicators for quality of life.

Psychological comorbidities and compliance to interventional treatment of patients with cutaneous vascular malformations.

PURPOSE: The aim of this study was to assess qualitatively the psychological stressors affecting patients with cutaneous vascular malformations and hemangiomas (CVM-H) and their impact on compliance to interventional treatment. METHODS: A retrospective chart review was conducted of all patients with CVM-H treated by interventional neuroradiology at a single academic institution during a five-year period (2009-2014). Psychological complaints were documented during each clinic visit by a neuroradiologist. Compliance to interventional treatment was defined by adherence to the scheduled treatment sessions. Fisher's exact test was used to assess for associations between psychological complaints and compliance. RESULTS: Seventy-five patients were assessed, of whom 49 (65.3%) were female, with an age range of 2-78 years (mean age 30.2 years). All except one patient older than seven years of age (n = 71; 94.6%) had a psychological complaint, including fear of negative appearance (n = 53; 70.6%), dissatisfaction with appearance (n = 46; 61.3%), low self-esteem (n = 35; 46.6%), anxiety (n = 16; 21.3%), stress (n = 13; 17.3%), bullying (n = 5; 6.6%), and low mood (n = 4; 5.3%). Twenty-three (31%) patients were non-compliant. Low self-esteem was significantly associated with non-compliance (p = 0.0381). CONCLUSION: There is a high prevalence of psychological comorbidities among patients treated for CVM-H. This has potential implications for interventional treatment, as it was found that low self-esteem is significantly associated with non-compliance. These results suggest the need for early psychological support in these patients in order to maximize compliance to interventional treatment.

Impact of Cosmetic Camouflage on the Quality of Life of Children With Skin Disease and Their Families.

BACKGROUND/OBJECTIVES: Cosmetic camouflage is known to improve quality of life in adults. Few data are available regarding cosmetic camouflage in children, and thus it is not often selected as a mode of treatment. We sought to determine whether cosmetic camouflage leads to improved quality of life of pediatric patients with visible dermatoses and their parent or primary caregiver. METHODS: Patients aged 5 to 17 years with visible skin disease and their parent were assessed with the Children's Dermatology Life Quality Index (CDLQI) and the Family Dermatology Life Quality Index (FDLQI) before and after consultation regarding cosmetic camouflage. RESULTS: Twenty-two children with skin conditions were included in the study. The mean CDLQI decreased from 6.82 (SD = 1.28) to 3.05 (SD = 0.65; P = .0014), while the mean FDLQI decreased from 7.68 (SD = 1.15) to 4.68 (SD = 0.92; P = .0012). CONCLUSIONS: Our study highlighted improvement in quality of life in patients with skin disorders who were managed with cosmetic camouflage.

Microvascular and Macrovascular Abnormalities and Cognitive and Physical Function in Older Adults: Cardiovascular Health Study.

OBJECTIVES: To evaluate and compare the associations between microvascular and macrovascular abnormalities and cognitive and physical function DESIGN: Cross-sectional analysis of the Cardiovascular Health Study (1998-1999). SETTING: Community. PARTICIPANTS: Individuals with available data on three or more of five microvascular abnormalities (brain, retina, kidney) and three or more of six macrovascular abnormalities (brain, carotid artery, heart, peripheral artery) (N = 2,452; mean age 79.5). MEASUREMENTS: Standardized composite scores derived from three cognitive tests (Modified Mini-Mental State Examination, Digit-Symbol Substitution Test, Trail-Making Test (TMT)) and three physical tests (gait speed, grip strength, 5-time sit to stand) RESULTS: Participants with high microvascular and macrovascular burden had worse cognitive (mean score difference = -0.30, 95% confidence interval (CI) = -0.37 to -0.24) and physical (mean score difference = -0.32, 95% CI = -0.38 to -0.26) function than those with low microvascular and macrovascular burden. Individuals with high microvascular burden alone had similarly lower scores than those with high macrovascular burden alone (cognitive function: -0.16, 95% CI = -0.24 to -0.08 vs -0.13, 95% CI = -0.20 to -0.06; physical function: -0.15, 95% CI = -0.22 to -0.08 vs -0.12, 95% CI = -0.18 to -0.06). Psychomotor speed and working memory, assessed using the TMT, were only impaired in the presence of high microvascular burden. Of the 11 vascular abnormalities considered, white matter hyperintensity, cystatin C-based glomerular filtration rate, large brain infarct, and ankle-arm index were independently associated with cognitive and physical function. CONCLUSION: Microvascular and macrovascular abnormalities assessed using noninvasive tests of the brain, kidney, and peripheral artery were independently associated with poor cognitive and physical function in older adults. Future research should evaluate the usefulness of these tests in prognostication.

[Head and neck superficial venous malformations]. / Malformations veineuses superficielles cervico-faciales. Mise au point.

Management of vascular malformations and vascular tumors has recently been maximized by the establishment of an accurate clinical and histological classification and by the development of multidisciplinary expert consultations. Head and neck localizations of venous malformations are common, thus maxillo-facial surgeons should be aware of the characteristics of this pathology and the principles of its management. Diagnosis is mainly clinical and must be certified by Doppler-ultrasonography and contrast enhanced magnetic resonance imaging with T2 fat-saturation sequence. Therapeutic decision depends on the volume of the lesion, and on functional, cosmetic and psychological complaints. Sclerotherapy is now the preferred treatment of head and neck venous malformations. It is performed in specialized interventional radiology units by intralesional injections of sclerosing solution under fluoroscopic guidance. Surgery is useful in some cases, either on its own or following sclerotherapy. In case of bulky lesion, it is necessary to search for and prevent a severe coagulopathy before planning any intervention.