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Interventional Neuroradiology logoLink to Interventional Neuroradiology
. 2016 May 12;22(4):489–494. doi: 10.1177/1591019916647194

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

Stephanie A Kenny 1, Nevin Majeed 1, Naista Zhand 2, Rafael Glikstein 1,3,4, Ronit Agid 5, Marlise P dos Santos 1,3,4,
PMCID: PMC4984393  PMID: 27177874

Abstract

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.

Keywords: Cutaneous vascular malformation, congenital vascular malformation, proliferating vascular tumor, hemangioma, psychological comorbidity, disfigurement, compliance, interventional radiology, interdisciplinary healthcare team, psychological support services

Introduction

Cutaneous vascular malformations and hemangiomas (CVM-H) encompass a diverse group of vascular abnormalities. The treatment approaches for these often complex lesions combine several surgical and medical specialties,1 and interventional radiology plays an increasingly important role in their management.

Treatment of CVM-H has historically been complicated by confusing terminology, leading to improper management and sub-standard treatment outcomes.2 In 1996, the International Society for the Study of Vascular Anomalies (ISSVA) developed a classification system dividing vascular anomalies into two main categories: proliferating vascular tumors (hemangiomas) and vascular malformations. Vascular malformations are subdivided into low and high blood-flow groups and complex combined groups.35 Although usually present at birth, vascular malformations often grow exponentially as the child grows older. Some lesions such as infantile hemangiomas have a tendency to involute spontaneously, whereas arteriovenous vascular malformations do not.6 If these do not regress or if they become symptomatic, they may require treatment.7

Many cases of CVM-H are referred to interventional radiology for confirmation of diagnosis and for assessment of the deformities of the soft tissues involved. Interventional procedures for CVM-H management include embolic therapy, sclerotherapy, and laser coagulation.2 With the proliferation of treatment options, communication between members of the interdisciplinary care team becomes very important. These include professionals from various medical specialties such as radiology, plastic surgery, dermatology, ENT, physiotherapy, and psychology.

The psychosocial aspects of CVM-H treatment are commonly under-recognized. Several online support groups exist for patients and legal guardians dealing with CVM-H such as the hemangioma newsline (http://www.hnnewsline.org), maintained by the National Organization for Vascular Anomalies. However, there is unfortunately a paucity of data in the literature to support a role for fully integrated, multidisciplinary psychological support services.8 This is in spite of the presence of several published studies demonstrating the social stigmatization of cutaneous vascular malformations and the benefit of intervention to the psychological well-being of the patient.9,10 Psychological stressors for people suffering from CVM-H range from being the target of staring11 to having a significant disadvantage in interpersonal relationships.12 Patients with CVM-H face daily psychosocial difficulties as a direct consequence of their physical disfigured appearance, with substantial implications for their quality of life.13,14

Over the course of several years, we have treated pediatric and adults populations with CVM-H in an interventional neuroradiology setting and identified a high prevalence of psychological comorbidities in these patients as a result of these psychosocial stressors. We have noted a high prevalence of incomplete treatments or “non-compliance” to the management plan during the assessments. The primary goal of our study was therefore to assess qualitatively the prevalence of psychological comorbidities seen in our patient cohort, with the secondary aim of identifying potential associations between the psychological comorbidities and lack of compliance to treatment. Understanding such associations could allow us to identify risk factors for non-compliance and help improve patient care and outcomes.

Methods

We performed a retrospective chart review of all patients treated for CVM-H in the neuro-interventional clinic from July 2009 to July 2014. Local Research Ethics Board approval was obtained.

Criteria for inclusion were one or more cutaneous venous malformations or hemangiomas with visible disfigurement appreciable on physical examination. Patients with diagnosed mental illness at the time of referral were excluded from the study. Patients with arteriovenous malformations were also excluded. All patients in the study group had received some form of medical or surgical intervention for their CVM-H.

Baseline characteristics of the patients including age, sex, date of consult, and type, size, location, and number of the lesions were collected. Patients were divided into age group clusters of ≤ 5, 6–18, 19–50 and > 50 years of age. Lesions were classified based on ISSVA type (proliferating vascular tumors [hemangiomas], arteriovenous, venous, lymphatic, capillary, or combined malformations), size (focal [≤15 cm diameter] vs. diffuse [>15 cm diameter]), and location (head and face vs. trunk and extremities).

Psychological complaints were routinely questioned in a conversational format during clinic consultation by a single interventional neuroradiologist with background in clinical neurology and internal medicine (M.P.d.S.) and documented in the medical chart for each initial and follow-up visit. All patients or their legal guardians were asked to describe the reasons for consultation, the reasons for wishing to have the lesion(s) assessed or treated, and whether there was any positive or negative change in their self-perception, in their own behavior or the behavior of others because of the presence of the lesion. No formal psychological assessment questionnaire was used. Psychological complaints were then retrospectively classified by a psychiatrist (N.Z.) into categories, including anxiety, stress, low mood, fear of negative appearance, dissatisfaction with appearance, past or present experience of bullying in school, or low self-esteem based on a precedent of similar classification schemes in the literature.1518

Patients were also assessed for compliance to treatment. Compliance was defined as attendance at all or all but one of the scheduled treatment sessions and/or consults. Non-compliance was defined as absence at two or more scheduled treatment sessions and/or consults despite telephone reminders and rescheduling options.

Descriptive and statistical analysis was subsequently performed. Descriptive data were presented as percentages, means ± standard deviations (SD) for normally distributed variables, and medians ± interquartile ranges (IQR) for non-normally distributed variables. Association between type of CVM-H and sex, age, and location were assessed. Association between type of psychological complaint with age and location were also assessed. Finally, association between psychological complaints and compliance was determined. The chi-square test or Fisher’s exact test were used for all analyses. All statistical tests were two-sided. The results were considered significant at α < 0.05.

Results

A total of 75 patients met inclusion criteria. The types of CVM-H encompassed in the study group included venolymphatic malformations, capillary or cavernous hemangiomas, arteriovenous malformations, congenital lymphedema/Klipel–Trenaunay, mixed (lymphedema, port-wine stain, venolymphatic malformations), cirsoid aneurysms, angiokeratomas, port-wine vascular stains, Morel–Lavalee, and sinus pericranii. Venolymphatic malformations (n = 46; 61.3%) were by far the most frequent lesions followed by capillary or cavernous hemangiomas (n = 8; 10.6%) and arteriovenous malformations (n = 7; 9.3%).

Forty-nine (65.3%) patients were females. Forty-seven (62.6%) lesions were located on the face, and 28 (37.3%) on the trunk or extremities. Facial distribution was more common than trunk or extremity distribution (n = 47; 62.6%). Fifty-seven (76%) patients had focal lesions, whereas 18 (24%) patients had diffuse lesions. We found no statistically significant relation between type of cutaneous lesion and sex (Table 1), age (Table 2), or location (Table 3).

Table 1.

Distribution of types of CVM-H according to sex.

Type of lesion (N = 75) Sex-M (n = 26; 34.6%) Sex-F (n = 49; 65.3%)
Venolymphatic malformation/46 (61.3%) 15 (32.6%) 31 (67.3%)
Capillary hemangioma/cavernous hemangioma/8 (10.6%) 2 (25%) 6 (75%)
Arteriovenous malformation/7 (9.3%) 2 (28.5%) 5 (71.4%)
Congenital lymphedema/Klipel– Trenaunay/4 (5.3%) 2 (50%) 2 (50%)
Mixed (lymphedema, port-wine stain, venolymphatic)/3 (4%) 1 (33.3%) 2 (66.6%)
Cirsoid aneurysm/2 (2.6%) 2 (100%) 0
Angiokeratoma/2 (2.6%) 0 2 (100%)
Port-wine stain/1 (1.3%) 1 (100%) 0
Morel–Lavalee/1 (1.3%) 1 (100%) 0
Sinus pericranii/1 (1.3%) 0 1 (100%)

There was no statistically significant association between any type of cutaneous lesion and sex.

CMV-H: cutaneous vascular malformations and hemangiomas.

Table 2.

Distribution of types of CVM-H according to age group.

Type of lesion (N = 75) ≤5 years (n = 3; 4%) 6–18 years (n = 20; 26.6%) 19–50 years (n = 40; 53.3%) >50 years (n = 12; 16%)
Venolymphatic malformation/46 (61.3%) 1 (2.1%) 11 (23.9%) 25 (54.3%) 9 (19.5%)
Capillary hemangioma/cavernous hemangioma/8 (10.6%) 1 (12.5%) 3 (37.5%) 3 (37.5%) 1 (12.5%)
Arteriovenous malformation/7 (9.3%) 0 1 (14.2%) 6 (85.7%) 0
Congenital lymphedema/Klipel–Trenaunay/4 (5.3%) 1 (25%) 2 (50%) 1 (25%) 0
Mixed (lymphedema, port-wine stain, venolymphatic)/3 (4%) 0 2 (66.6%) 1 (33.3%) 0
Cirsoid aneurysm/2 (2.6%) 0 1 (50%) 1 (50%) 0
Angiokeratoma/2 (2.6%) 0 0 2 (100%) 0
Port-wine stain/1 (1.3%) 0 0 0 1 (100%)
Morel–Lavalee/1 (1.3%) 0 0 0 1 (100%)
Sinus pericranii/1 (1.3%) 0 0 1 (100%) 0

There was no statistically significant association between any type of cutaneous lesion and age group.

Table 3.

Distribution of types of CVM-H according to location.

Type of lesion (N = 75) Head/face (n = 47; 62.6%) Trunk/extremities (n = 28; 37.3%)
Venolymphatic malformation/46 (61.3%) 30 (65.2%) 16 (34.7%)
Capillary hemangioma/cavernous hemangioma/8 (10.6%) 7 (87.5%) 1 (12.5%)
Arteriovenous malformation/7 (9.3%) 6 (85.7%) 1 (14.5%)
Congenital lymphedema/ Klipel–Trenaunay/4 (5.3%) 0 4 (100%)
Mixed (lymphedema, port-wine stain, venolymphatic)/3 (4%) 1 (33.3%) 2 (66.6%)
Cirsoid aneurysm/2 (2.6%) 2 (100%) 0
Angiokeratoma/2 (2.6%) 0 2 (100%)
Port-wine stain/1 (1.3%) 0 1 (100%)
Morel–Lavalee/1 (1.3%) 0 1 (100%)
Sinus pericranii/1 (1.3%) 1 (100%) 0

There was no statistically significant association between any type of cutaneous lesion and location.

All but one patient older than seven years of age suffered from some type of psychological complaint. Fear of negative appearance (n = 53; 70.66%), dissatisfaction with appearance (n = 46; 61.33%), and low self-esteem (n = 35; 46.66%) were the most common symptoms. Depressed mood was less common, found in four patients (5.33%). Five (6.66%) patients also discussed present or past experience of bullying in school. There was considerable overlap in symptomatology with patients describing varying degrees of each symptom. Asymptomatic patients (n = 4; 5.33%) significantly belonged to both ends of our age groups, with three (75%) patients younger than four years of age and one (25%) patient older than 50 years of age having no symptoms (p < 0.001). Also significant was fear of negative appearance (n = 53; 70.66%), which was more common in the 6–18 (n = 15; 28.3%) and 19–50 (n = 32; 60.3%) year age groups (p = 0.009; Table 4). There was no significant relationship between the location of lesion and the symptomatology (Table 5).

Table 4.

Distribution of symptoms in relation to age.

Psychological complaint (N = 75) ≤5 years (n = 3; 4%) 6–18 years (n = 20; 26.6%) 19–50 years (n = 40; 53.3%) >50 years (n = 12; 16%)
None/4 (5.3%) 3 (75%), p ≤ 0.0001 0 0 1 (25%)
Low mood/4 (5.3%) 0 2 (50%) 1 (25%) 1 (25%)
Anxiety/16 (21.3%) 0 3 (18.7%) 10 (62.5%) 3 (18.7%)
Low self-esteem/35 (46.6%) 0 11 (31.4%) 19 (54.2%) 5 (14.2%)
Bullying/5 (6.6%) 0 4 (80%) 1 (20%) 0
Stress/13 (17.3%) 0 1 (7.6%) 10 (76.9%) 2 (15.3%)
Fear of negative appearance/53 (70.6%) 0, p = 0.009 15 (28.3%) 32 (60.3%) 6 (11.3%)
Dissatisfaction with appearance/46 (61.3%) 0 14 (30.4%) 25 (54.3%) 7 (15.2%)

Statistically significant associations are shown with bold p-values.

Table 5.

Distribution of psychological complaints according to lesion location.

Psychological complaint (N = 75) Head/face (n = 47; 62.6%) Trunk/extremities (n = 28; 37.3%)
None/4 (5.3%) 2 (50%) 2 (50%)
Low mood/4 (5.3%) 4 (100%) 0
Anxiety/16 (21.3%) 13 (81.2%) 3 (18.7%)
Low self-esteem/35 (46.6%) 25 (71.4%) 10 (28.5%)
Bullying/5 (6.6%) 4 (80%) 1 (20%)
Stress/13 (17.3%) 11 (84.6%) 2 (15.3%)
Fear of negative appearance/53 (70.6%) 33 (62.2%) 20 (37.7%)
Dissatisfaction with appearance/ 46 (61.3%) 29 (63%) 17 (37%)

There was no statistically significant relationship between any psychological complaint and location of the lesion.

One (1.3%) patient was not suitable for treatment or follow-up and was excluded from the analysis for compliance. Fifty-one (68.9%) patients completed the recommended treatment. Twenty-three (31%) patients did not complete the recommended treatment due to lack of compliance. Patients with low self-esteem had a significantly higher rate of non-compliance (p = 0.0381). We found no significant relationship between compliance and any of the other psychological symptoms (Table 6).

Table 6.

Distribution of compliance according to psychological complaints.

Psychological complaint (N = 74) Compliance (n = 51; 68.9%) Non-compliance (n = 23; 31%)
None/4 (5.4%) 3 (75%) 1 (25%)
Low mood/4 (5.4%) 2 (50%) 2 (50%)
Anxiety/16 (21.6%) 8 (50%) 8 (50%)
Low self-esteem/35 (47.2%) 20 (57.1%) 15 (42.8%), p = 0.0381
Bullying/5 (6.7%) 3 (60%) 2 (40%)
Stress/13 (17.5%) 7 (53.8%) 6 (47.1%)
Fear of negative appearance/53 (71.6%) 36 (67.9%) 17 (32.1%)
Dissatisfaction with appearance/45 (61.3%) 28 (62.2%) 17 (37.7%)

Statistically significant associations are shown with bold p-values.

Discussion

Patients with CVM-H are confronted with many psychosocial stressors on a daily basis as a result of their physical disfigurement.14 Our experience at a single academic center suggests a high rate of psychological comorbidities among an interventional radiology–treated group encompassing a wide spectrum of CVM-H. These results are potentially important for interventional treatment providers, given a possible association with treatment compliance.

Our study group was largely reflective of the prevalence of the various types of CVM-H present in the general population. Venolymphatic malformations were the most common malformation in our group, followed by hemangiomas and arteriovenous malformations. In the literature, venolymphatic malformations represent 10–49% of vascular malformations, while arteriovenous malformations comprise 36% of cutaneous vascular malformations.19 Although infantile hemangiomas are the most common congenital vascular malformation in infancy, with an incidence in up to 10% of Caucasian infants,19,20 these lesions often spontaneously involute and are therefore underrepresented in our cohort of patients referred for specialist interventional treatment. Prior literature has demonstrated varying predilections by sex and location for certain types of CVM-H.19,20 However, we did not find any statistically significant relation between the type of cutaneous lesion and either variable.

All but one of our patients older than the age of seven suffered from some form of psychological comorbidity. Our rates are higher than what is described in the literature.21,22 We hypothesize the relatively higher rates of psychological comorbidities in our study might be due to the fact that the patients were encouraged to describe their psychological complaints on each visit, and due to the lack of dedicated specialized psychological support services at our institution for this patient population. The most common complaint we found was fear of negative appearance, followed by dissatisfaction with appearance and low self-esteem. The fear of negative appearance was significant in the 6–18 and 19–50 year age groups. Although one might assume a linear relationship between the extent of physical deformity and the psychosocial impact, we found this is rarely so. Our results confirm those from multiple previous studies showing that neither the extent nor the duration of the deformity is a sensitive predictor of its psychological impact.23,24 The same cannot be said for location, with prior studies showing that facial deformities have a stronger association with psychological stress than nonvisible lesions do.23,25 Our study did not support this result, instead demonstrating no statistically significant relationship between any psychological complaint and location of the lesion. We hypothesize that this may be due to an underpowered sample size and the lack of a standardized scaling questionnaire.

Compliance to interventional treatment within our study group was 68.9%. To the best of our knowledge, there is no prior literature on the rates of compliance to treatment or risk factors for non-compliance for CVM-H. We newly identified low self-esteem to be significantly associated with non-compliance in this complex patient group. Based on the experience at our single academic center, we propose that integrated psychological support services within the multidisciplinary healthcare team could potentially improve interventional treatment compliance, and is a worthwhile question for future research.

Bradbury23 suggests a three-pronged approach starting with appraising the patient during each visit. A simple 10-minute questionnaire could aid greatly in this regard, which might be filled out while waiting to be seen. A trained liaison nurse could give support and counseling services. Finally, there should be a simple referral route to a specialist such as a psychologist or psychiatrist who could work toward empowering the patient to accept living with the deformity. Cognitive behavioral therapy has been exceptionally successful in this regard, particularly in addressing social anxiety and avoidance behavior.13,23

The greatest limitation of our study is the lack of contemporary expert psychologist evaluation or a standardized psychological questionnaire. The lack of a graded standardized questionnaire makes the study non-quantitative and limits the understanding of the extent of psychological impact of CVM-H. Our study group was largely reflective of the prevalence of the various types of CVM-H present in the population.19,20 However, given that we examined a selected group referred for specialist interventional procedures, it is possible that the rates of psychological comorbidity are higher than the distribution of the problem in the general pool of CVM-H. Finally, retrospective analysis with small sample size is a significant limitation with inherently low statistical power.

Nevertheless, our single-center experience with psychological comorbidities and compliance of patients with CVM-H to interventional treatment highlights the need for provider sensitivity to the many psychosocial stressors faced by this patient group and their families. In her article ‘Can’t they like me as I am?’ Hearst argues that it is society that ultimately needs to change.26 Until then, there may be benefit to early psychological support within the interdisciplinary team in order to maximize interventional treatment compliance and improve outcomes.

Acknowledgments

Statistical analysis was provided by Liying Zhang, PhD, Senior Biostatistician at MacroStat Inc., Toronto, ON, Canada. The authors wish to thank Betty Anne Schwarz and Christina Tsoukanas for research assistance, and Dr. Sharyn Laughlin, MD, FRCPC, for contribution to clinical cases.

Declaration of conflicting interests

The authors declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article.

Funding

The authors received no financial support for the research, authorship, and/or publication of this article.

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