Edwin F. Williams III, M.D.*, Marcelo Hochman, M.D.**, Bret J.
Rodgers***, M.D.,
David Brockbank, B.A.****, Linda Shannon, M.S.*****,
Samuel M. Lam, M.D******
*Clinical Associate Professor, Division of Otolaryngology, Department
of Surgery,
Albany Medical College, Albany, New York
Chief of Division, Facial Plastic & Reconstructive Surgery,
Albany Medical College, Albany, New York
Medical Director, Williams Center for Facial Plastic Surgery,
Latham, New York
**The Facial Surgery Center,
Charleston, South Carolina
***Rodgers Center for Facial Plastic Surgery,
Boise, Idaho
*****Albany Medical College,
Albany, New York
*****President and Founder,
Vascular Birthmarks Foundation,
Albany, New York
******Clinical Instructor, Division of Otolaryngology, Department
of Surgery,
Albany Medical College, Albany, New York
Stratton Veteran Affairs Medical Center, Albany, New York
Abstract
Objective: To assess the psychosocial impact of hemangiomas
and of their treatment on children afflicted with the disease and
their family.
Setting: Two private, ambulatory surgery centers (Latham,
NY and Charleston, SC)
Design: 39 children who were treated for hemangiomas were
evaluated by questionnaire that addressed the emotional attitudes
of the parent and child toward the disease and the related treatment.
Results: Overall, the survey found a negative effect on
the child’s family with considerable fear caused in part by adverse
public commentary or attitudes – which was ameliorated by education
from the primary care provider and specialist. However, the family’s
perception was that the child was not deeply affected by his/her
condition and that treatment (laser, intralesional steroids, oral
steroids, surgery, or a combination) did not change the child’s
emotional response to the disease. However, most parents observed
that their child was too young to appreciate his/her malady.
Conclusions: Given earlier intervention today for children
with late-involuting hemangiomas and the advent of more effective
therapies, our survey did not seem to indicate that the children
suffered significant emotional trauma from their condition but that
nevertheless their families experienced appreciable emotional and
psychological distress.
Introduction
Hemangiomas are the most common neoplasm of infancy and childhood,
with an estimated prevalence of 1-3% of all neonates 1,2
and 10% of infants by 1 year of age.3,4 Most hemangiomas
arise in the head and neck region (60%), and 20% of patients may
suffer from more than one lesion.5 Given these facts
and that hemangiomas may be unsightly birthmarks, the psychological
stress on the developing child and family cannot be underestimated.
Hemangiomas exhibit a natural history of proliferation during the
first year of life – a fact that may only further compound familial
anxieties about their child’s condition. However, only a small
minority of hemangiomas actually requires intervention, as they
often tend to involute prior to the age when the child should enter
school. Most hemangiomas undergo involution during the second year
of life and may completely regress.
If these oftentimes disfiguring vascular lesions do not involute
early, they may have profound psychosocial effects on the child
and family and may lead at times to accusations of child abuse and
other misconceptions as this study will show. In addition, reports
of late involuting hemangiomas have found a high incidence of a
marked residual deformity. Although several studies have investigated
the impact that port-wine stains, or capillary vascular malformations,
have on the child’s tender psyche and the benefit that treatment
affords 6-12, fewer studies exist that examine the psychological
ramifications of hemangiomas on the child and family 13-15.
Technological advances in the treatment of vascular lesions have
also been remarkable and kept stride with intellectual gains. Prior
to the introduction of laser therapy, many individuals were left
only with the option of cosmetic camouflage. The earlier laser
types, the argon and ND:Yag, often led to undesirable scarring,
a side effect rarely encountered with the pulse-dye laser. Some
authors still advocate the efficacy of interstitial KTP and ND:Yag
lasers when treating the deeper component of the hemangioma not
amenable to the pulse-dye laser.16 Pharmacological intervention
with steroids (both intralesional and systemic), alpha-interferon,
and bleomycin has been investigated and implemented with varying
success.17-19 Surgery has remained a mainstay of therapy
for those lesions that are refractory to the above methods or that
are deemed more suitable to surgical debulking.
Given the recent advances in hemangioma management and the relative
paucity of literature on the psychological sequelae of this disease,
this paper is intended to address these deficiencies and hope to
provide a meaningful contribution to our understanding of the untoward
psychological effects that hemangiomas may wrought on the child
and family.
Methods
39 families were interviewed by phone about their child’s hemangioma
using a 38-point questionnaire that covered the child’s birth history,
the natural history of the hemangioma, physician encounters, treatment
interventions and the family and child’s emotional attitudes toward
the hemangioma and related treatment. Initially 112 charts were
evaluated for this study, but the majority was excluded from inclusion
due to the presence of a vascular malformation rather than a true
hemangioma, the lack of any therapy administered, or the inability
to contact the family. Of the 39 patients, 17 children were patients
of Dr. Williams (Latham, NY), and 19 children were patients of Dr.
Hochman (Charleston, SC). 29 children were female, and 10 were
male, which correlates well with the sex distribution reported in
the literature.
Results
Birth History
The birth history of the child reveals a high incidence of complications
(35.9%), which included 4 cases of preeclampsia, 1 case of prematurity,
2 traumatic births, 2 cases of hyperemesis, 2 cases of gestational
diabetes, 2 twin-twin transfusions, and 1 failure to thrive. Only
one case of prematurity (28-weeks gestation) occurred, which is
much lower than the reported figure of 25%. A third of the patients
(33.3%) reported a family history of hemangiomas, which is higher
than one study which found a 10% rate of familial association.20
35.9% of mothers took oral contraceptives prior to pregnancy,
but all stopped their prophylactic medications one month prior to
conception. None, however, took any fertility medications.
History of the Hemangioma
Almost half of the hemangiomas presented at birth (43.6%), and
all were evident by 2 months of age (Figure 5). The majority of
the treated hemangiomas occurred in the head and neck region (75%),
most frequently on the cheek (n=10) and forehead (n = 8) (Figure
6). Eleven children (25.6%) had multiple hemangiomas, ranging from
2 to 4 with an average of 2.5 lesions. However, only one of the
children had more than one hemangioma (specifically 2 lesions) treated.
Physician Encounters
Parents attested to the accuracy with which their primary care
physicians diagnosed the vascular lesion (94.9%) and remarked that
only 23.1% of the time did they recommend any treatment. (As stated,
all patients in this study were selected who eventually underwent
treatment.) Nevertheless, these families sought more expert opinion
from the Vascular Birthmark Clinic at various stages in the evolution
of the hemangioma, with the children ranging from 2 weeks to 12
years of age (Figure 7). All parents perceived that their visit
to the Vascular Birthmark Clinic was informative and that a treatment
plan was clearly formulated. 97.4% of the parents professed that
they could make a rational decision on the management of their child’s
hemangioma based on the information supplied by the Vascular Birthmark
Clinic.
Treatment History
As detailed in the introductory remarks of this paper, the treatment
algorithm was determined based on the guidelines enumerated in Williams
et al.’s study.21 Hemangiomas that were deemed rapidly
proliferating in a cosmetically sensitive area, i.e., the face and
neck; that risked impending ulceration or had ulcerated; that were
categorized as late involuters; or those lesions that remained stable
into the school years were candidates for therapy. Only patients
who underwent therapy for their hemangioma were included in this
study in order to assess the psychological effects of treatment
intervention.
When treatment was recommended, the modalities used were pulse-dye
laser, intralesional steroid injections, oral steroids, and surgery,
or a combination of the above. 79.5% of patients underwent pulse-dye
laser therapy, with a range of 1 to 10 treatments and an average
of 3. Most patients (77.4%) were 1 year old or younger and were
administered laser therapy in order to retard the proliferative
nature of the hemangioma. 22.5% of patients treated with the laser
were significantly older (at least 2 years old), and laser treatment
was primarily aimed at eliminating dermal ectasias and/or reduce
the residuum and often was combined with surgery. Steroids are
only effective during the proliferative phase of the hemangioma,
and treatment was confined only to this period. Intralesional steroid
injection was employed in 7 patients (17.9%), with a range of 1
to 6 treatments and an average of 1.9. All steroid injections were
given as an adjunct to laser therapy during the proliferative phase
of the hemangioma, as all patients were less than 1 year of age.
Oral steroids were administered in 6 patients (15.4%) during the
proliferative phase of the hemangioma, 4 of whom had therapy initiated
prior to presentation at the Vascular Birthmark Clinic and 2 of
whom were started on systemic therapy to treat an obstructing lesion
(1 near the eye and the other in the subglottic airway). Finally,
surgery was performed, either once or twice, in 22 patients (56.4%),
ranging in age from 2 months to 7 years.
Psychosocial Questionnaire
A 15-point questionnaire was then administered to assess the emotional
and psychological effects that the hemangioma had on both the family
and child (Table 1, 2). Parents were asked to respond to questions
with one of the following opinions: strongly agree, agree, no change,
disagree, or strongly disagree. The first part of the questionnaire
pertained to the attitudes that the family and child had toward
the hemangioma, and the second part concerned the emotional response
to treatment. Parents expressed fear and anxiety towards the presence
of the lesion (43.6% strongly agree and 43.6% agree). This anxiety
was only partially alleviated by the primary care physician’s advice
(15.4% strongly agree and 43.6% agree) regarding the hemangioma,
but a greater percentage of parents professed that the advice delivered
by the Vascular Birthmark Clinic mitigated their concern (51.3%
strongly agree and 41.0% agree).
Most parents testified to the negative commentary or stares they
received from others (66.7% strongly agree and 23.1% agree), leading
them to seek professional advice from a specialty clinic (51.3%
strongly agree and 23.1% agree). 25.6% of parents professed that
they were actually accused of child abuse because of their child’s
vascular lesion. Although the hemangioma provoked anxiety in parents,
a mixed response was given regarding the negative emotional effect
on the family (17.9% strongly agree, 35.9% agree, 7.7% believe no
change, 28.2% disagree, and 10.3% strongly disagree) and even less
of an emotional burden on the afflicted child according to parents’
perceptions (10.3% strongly agree, 7.7% agree, 17.9% believe no
change, 43.6% disagree, and 20.5% strongly disagree.) Similarly,
the parents thought that the hemangioma did not adversely interfere
with the child’s social activities (43.6% disagreeing that it interfered
and 28.2% strongly disagreeing). However, parents acknowledged
that their children were too young to appreciate their own condition.
The second half of the questionnaire addressed the emotional response
of the parent and child to the treatment of the lesion (Table 2).
Three questions that pertained to the child’s attitude toward his
condition after treatment received similar responses from parents,
namely, that the child was not affected positively or negatively
by the treatment. 51.2% reported no change in self-esteem; 53.8%
witnessed no change in the degree of embarrassment; and 46.1% claimed
that no change occurred in the child’s willingness to participate
in social gatherings. Most respondents, however, qualified their
opinions by declaring that their child was too immature to appreciate
his/her condition. However, the few, older children (n=8) that
ranged in age from 3 to 8 years old revealed proportionally greater
benefit from treatment compared to their younger confreres. The
majority of parents of these older children thought that treatment
effected significant improvement in the child’s self-esteem with
50% strongly agreeing, 25% agreeing, and 25% believing no change
was evident. Also they agreed that their children were less embarrassed
with 37.5% strongly agreeing, 50% agreeing and 12.5% believing that
no change existed. Overall, most parents (66.7%) confirmed that
no change occurred in the relationship they maintained with their
child after treatment. Nevertheless, 94.9% (66.7% strongly agree
and 28.2% agree) of parents believed that the emotional removal
was commensurate to the physical removal of the hemangioma, which
testifies to the intangible benefit that treatment may afford.
Comment
Treatment of vascular lesions has undergone a revolution in thought
and practice in the past ten years. Earlier intervention and advanced
therapeutic modalities, such as laser therapy, have permitted the
patient and family the opportunity to remove the hemangioma earlier
and more effectively and thereby mitigate the psychological impact
that the hemangioma may otherwise have. The aforementioned psychological
profiles on hemangioma patients and family members were conducted,
for the most part, prior to 1993 and may be considered outdated
in some respects considering the new treatment algorithms and methods.
In contrast, the psychological studies on port-wine stains have
been published principally at the turn of this millennium (1997-2000)
and may reflect more current treatment designs.
The understanding of the nature and evolution of hemangiomas has
been further refined since the seminal work of Mulliken and Glowacki
22, who distinguished hemangiomas from vascular malformations
based on the former’s endothelial proliferative characteristics.
Older terminology, such as capillary and cavernous hemangiomas and
strawberry nevi, has fallen into disfavor and has been replaced
with a more standardized nomenclature of superficial, deep, and
compound hemangioma. More recently, hemangiomas have been further
subdivided clinically into early and late involuters, with the former
resolving at 1 to 2 years and the latter, after 2 years. Based
on these characteristics, an algorithm for early intervention has
been proposed for the late involuters in order to avoid the attendant
social stigma that would occur after entering school and further
to address the substantial residuum often seen in these children.21
Similarly, rapidly proliferative hemangiomas in a cosmetically sensitive
area or that risk ulceration are also treated early. The patients
who were evaluated in this study were managed following the guidelines
of this new paradigm.
By virtue of their scientific nature, physicians are prone to measure
the success of their treatment in terms of objective criteria, such
as removal of disease or avoidance of morbidity. At times, physicians
look toward their patients for approbation and confirmation that
their patients are satisfied with the care they have received. Rarely,
do health-care providers weigh the psychological burden that a disease
process carries or, even less frequently, what steps should be taken
to avoid the development of such emotional trauma. The psychological
condition of the patient may be considered inaccessible or too elusive
to ascertain reasonably in an objective fashion. Therefore, few
studies have investigated the psychological import of a disease
or how treatment may favorably alter the patient’s outlook.
Management of hemangiomas has remained shrouded in uncertainty
for many years given the potential for these lesions to regress
spontaneously. Many physicians have advocated a policy of benign
neglect in which the child is permitted to mature into early childhood
without intervention. Newer studies have documented that a substantial
proportion of hemangiomas do not involute: one study established
that only 50% regressed by age 6 and of that group, 38% retained
a marked cosmetic deformity.23 Based on these findings,
hemangiomas that exhibit signs of late involution should be subjected
to earlier management to avoid the potential psychological sequelae
that this protracted waiting period may engender in the child.
The few psychosocial studies that have examined the effects of
late intervention have documented the very real trauma that children
and their families sustained from the presence of the hemangioma
at such a late age.13-15 The child’s body image is poorly
developed prior to age 3, but by age 7 he/she usually has a mature
self-identity and is able to distinguish aesthetic concepts that
may render the child feeling different from his peers. During the
intervening years between 3 and 7 years of age, the child has already
slowly acquired his/her perception of body identity; and it becomes
imperative that the surgeon/physician intervene prior to this period
to abort any negative social effects. The advantage of early intervention
should be apparent for parent and child alike to avoid the negative
social perceptions toward the parent and the ostracism that may
ensue for the child at school.
This paper underscores the importance of evaluating the psychological
role that hemangiomas may have on the entire family unit and that
treatment should be tailored in this respect to curtail the damaging
effects. Our findings overwhelmingly indicate that the parents
believe the emotional burden matches the physical nature of the
disease, and this opinion should help to guide physicians as they
counsel their patients about treatment. However, a caveat must
be offered at this point: parental anxiety should never dictate
the timing of treatment because early involuting hemangiomas have
a high likelihood of complete regression and should be given the
chance to do so. Premature intervention in stable, regressing,
or non-obstructive lesions does a disservice both to the child and
family. We must also consider the cost constraints dictated by
insurance providers yet maintain the need for treatment when appropriate.
We believe that a judicious policy should be advocated of early
intervention in hemangiomas that are rapidly proliferating or that
fail to involute early in order to preclude the negative psychological
impact on the developing child. In fact, the children in this study
were not significantly affected by their disease process likely
because they were too immature, an opinion that their parents repeatedly
offered without provocation from the interviewer. The few, older
children who were enrolled in this study showed proportionally greater
psychological suffering from their disease than their younger counterparts.
All children who were included in this study underwent therapy
for their hemangioma – a fact that may predispose this study toward
some bias. Only treated patients were studied in order to assess
both the disposition of the family and child toward the disease
and to determine whether any beneficial change should arise from
treatment. Clearly, both younger and older children who never underwent
treatment or who remained only under the care of their primary physicians
would be a subject worthy of further analysis. A multi-armed study
in which treated and untreated children were evaluated in a prospective
fashion would hold greater scientific merit. However, the authors
strongly feel that the proposed treatment algorithm represents a
standard of care and that patients intentionally left untreated
for the purposes of a scientific study would be, in our opinion,
inappropriate. Despite the limitations of this retrospective approach,
we hope that this study should still encourage physicians to weigh
the psychological dimensions with the more tangible physical attributes
of the hemangioma when counseling the family and when deciding a
course of therapy.
Conclusions
Treatment of hemangiomas has undergone a remarkable transformation
in the past decade owing in part to better understanding of the
disease and a more effective therapeutic arsenal. Few studies have
investigated the psychological ramifications of these particular
vascular lesions on the child and family. Our results indicate
that the parents bear the burden of psychological distress concerning
their child’s disease and that the young child remains relatively
unaware of his/her condition according to parents’ perceptions.
Earlier treatment protocols may account for the immature child’s
immunity from psychological repercussions. Further clinical studies
are needed to confirm these preliminary findings.
References
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4. Jacobs AH. Strawberry hemangiomas: the natural
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5. Margileth AM, Museles M. Cutaneous hemangiomas
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6. Augustin M, Zschocke I, Wiek K, et al. Coping
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LG. An interdisciplinary group for parents of children with hemangiomas.
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care collaberation. Child Health Care. 1992;21:84-9.
15. Dietrich-Miller CA, Cohen BA, Liggett J. Behavioral
adjustment and self-concept of young children with hemangiomas.
Pediatr Dermatol. 1992;9:241-5.
16. Burstein FD, Simms C, Cohen SR, Williams JK,
Paschal M. Intralesional laser therapy of extensive hemangiomas
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2000;44:188-94.
17. Bennett ML, Fleischer AB Jr, Chamlin SL, Frieden
IJ. Oral corticosteroid use is effective for cutaneous hemangiomas:
an evidence-based evaluation. Arch Dermatol. 2001;137:1208-13.
18. Soumekh B, Adams GL, Shapiro RS. Treatment
of head and neck hemangiomas with recombinant interferon alpha 2b.
Ann Otol Rhinol Laryngol. 1996;105:201-6.
19. Sarihan H, Mocan H, Yildiz K, Abes M, Akyazici
R. A new treatment with bleomycin for complicated cutaneous hemangioma
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Table 1: Psychological Profile of Hemangioma Patients
and Their Family Involving the Emotional Aspects of the Disease
| |
SA* |
A |
NC |
D |
SD |
| The presence of the lesion caused
fears and anxieties in you and/or your family. |
17
(43.6%) |
17 (43.6%) |
0
(0%) |
4
(10.3%) |
1
(2.6%) |
| The information you received from
your primary care physician helped alleviate these fears and anxieties. |
6
(15.4%) |
17
(43.6%) |
3
(7.7%) |
8
(20.5%) |
5
(12.8%) |
| The fears and/or anxieties decreased
after you visited the Vascular Birthmark Clinic. |
20
(51.3%) |
16
(41.0%) |
1
(2.6%) |
2
(5.1%) |
0
(0%) |
| I encountered critical comments,
negative stares and/or opinions from others regarding my child’s
birthmark. |
26
(66.7%) |
9
(23.1%) |
1
(2.6%) |
2
(5.1%) |
1
(2.6%) |
| This experience motivated me to find
a specialty clinic. |
20
(51.3%) |
9
(23.1%) |
3
(7.7%) |
2
(5.1%) |
5
(12.8%) |
| Fears and anxieties eliminated or
decreased after your visit to the Vascular Birthmark Clinic. |
17
(43.6%) |
20
(51.3%) |
1
(2.6%) |
1
(2.6%) |
0
(0%) |
| The birthmark had a negative emotional
effect on you and your family. |
7
(17.9%) |
14
(35.9%) |
3
(7.7%) |
11
(28.2%) |
4
(10.3%) |
| The birthmark had a negative emotional
effect on your child who is affected by the birthmark. |
4
(10.3%) |
3
(7.7%) |
7
(17.9%) |
17
(43.6%) |
8
(20.5%) |
| The birthmark adversely interfered
with normal childhood activities such as attending parties, play-time
sessions, day care, etc. |
3
(7.7%) |
4
(10.3%) |
5
(12.8%) |
17
(43.6%) |
11
(28.2%) |
| Were you ever accused of child abuse
because of the birthmark? |
10(YES)
(25.6%) |
29(NO)
(74.4%) |
|
|
|
*SA = Strongly Agree, A = Agree, NC = No Change, D = Disagree,
SD = Strongly Disagree
Table 2: Psychological Profile of Hemangioma Patients and Their
Family After Treatment
| |
SA* |
A |
NC |
D |
SD |
| A change was noted in my child’s
self-esteem following treatment of the lesion. |
5
(12.8%) |
4
(10.2%) |
20
(51.2%) |
7
(17.9%) |
3
(7.7%) |
| Less embarrassed: |
4
(10.2%) |
4
(10.2%) |
21
(53.8%) |
8
(20.5%) |
2
(5.1%) |
| Less avoidance of social
gatherings: |
5
(12.8%) |
4
(10.2%) |
18
(46.1%) |
9
(23.1%) |
3
(7.7%) |
| You had an improved relationship
with the child. |
3
(7.7%) |
4
(10.2%) |
26
(66.7%) |
4
(10.2%) |
2
(5.1%) |
| Overall, the emotional removal is
as important as the physical removal of the birthmark. |
26
(66.7%) |
11
(28.2%) |
1
(2.6%) |
1
(2.6%) |
0
(0%) |
*SA = Strongly Agree, A = Agree, NC = No Change, D = Disagree,
SD = Strongly Disagree.
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