Treatment of Pediatric Vascular

Lesions of the Eyelid and Orbit

Gabriel Garza, M.D.

Aaron Fay, M.D.
Peter A. D. Rubin, M.D., F.A.C.S

䡲 Definitions and Nomenclature

Advances in the biological characterization of vascular lesions have led

to a reformulation of their classification. The landmark work of Mulliken
and Glowacki1 in 1982 provided the basis for these changes. These au-
thors were able to distinguish vascular lesions on the basis of endothelial
behavior and demonstrated that hemangiomas show endothelial hyper-
plasia, whereas vascular malformations show normal endothelial turn-
over.1 The present classification of vascular lesions establishes clear clini-
cal, histopathological, and prognostic differences between hemangiomas
and vascular malformations. The older terms capillary and strawberry hem-
angioma should be translated into the single term hemangioma. In contrast,
cavernous hemangiomas, port-wine stains, and lymphangiomas all should
be called malformations.2
Strictly speaking, hemangiomas are hamartomatous growths composed
of proliferating capillary units. They show a characteristic early phase of
active growth during postnatal life, with a subsequent period of regression
and involution. Vascular malformations are nonproliferating clusters of ec-
tatic aberrant vessels or lymphatics. They develop during fetal life and
manifest by a progressive, passive, congestive enlargement and not by
active autonomous growth.3 This nomenclature has been incorporated
into the medical literature but is not commonly used in the ophthalmic
literature. To avoid confusion in this chapter, the ophthalmic terminology
will be used. Many of these terms, nonetheless, are inconsistent with cur-
rent scientific understanding of vascular lesions.

43
44 䡲 Garza et al.

䡲 Incidence and Clinical Features

Hemangiomas and Vascular Malformations Affecting the

Eyelids and Orbit in Children
Hemangiomas occur in approximately 2.6% of term newborns4 and in
up to 23% of premature infants having a birth weight of less than 1,000
gm.5 A 3 : 1 female-to-male predilection rate is recognized.6 Haik and
colleagues7 found in their series of periocular (orbit, eyelid, and ocular
adnexa) hemangiomas a predominance of upper eyelid involvement, with
75% showing orbital signs. In one orbital biopsy series of children younger
than 18 years, hemangiomas accounted for 4% of all orbital lesions. A pale
halo, an area of erythema, or a cluster of telangiectatic vessels character-
izes the initial presentation of a cutaneous hemangioma a few weeks after
birth.8 This may be confused with the “salmon patch” (“stork bite,” “angel
kiss”) that occurs in as many as 40% of newborns and that most often
disappears by the first year of life.9 Cavernous hemangioma of the orbit is
a lesion of adulthood, with 70% of these lesions manifesting during the
fourth and fifth decades of life.10 Cavernous hemangioma is a misnomer,
however, as these lesions, in reality, are low-flow vascular malformations11
and not true hemangiomas.
Vascular malformations affecting the eyelid and orbit in children can be
arterial, venous, lymphatic, capillary, or a combination of any of these.
The age and mode of clinical presentation vary considerably. As a general
rule, vascular malformations manifest later in life as compared to hem-
angiomas. Some cutaneous vascular malformations such as port-wine
stains are evident at birth.
Orbital lymphangioma, a lymphatic malformation, may produce pro-
ptosis at birth or, more frequently, later in the second or third decade of
life.12 The incidence of this lesion in a series of 600 consecutive orbital
biopsies was 3%. In this same series, varices or venous malformations
(excluding cavernous hemangiomas) represented 5% of all vasculogenic
orbital lesions subjected to biopsy.13
Orbital venous malformations or varices have been divided into primary
or secondary types.14 The primary type is confined to the orbit and has no
association with arteriovenous (A-V) malformation. The secondary orbital
varix occurs as a result of an intracranial A-V shunt that causes the orbital
veins to dilate. Orbital venous malformations usually become symptomatic
after years of progressive congestion and rarely manifest before the sec-
ond decade of life.
A-V malformations purely involving the orbit are extremely rare.15
Patients with congenital A-V aneurysms of the retina and midbrain—
Wyburn-Mason syndrome—may show ipsilateral orbital involve-
ment.16 A-V malformations also can involve the bony orbit. They rarely
occur during childhood and are characterized by pulsatile exophthal-
 Treatment of Vascular Lesions 䡲 45

mos, chemosis, congested conjunctival vessels, and raised intraocular pres-

sure.17
A port-wine stain is a true capillary malformation affecting the papillary
dermis. This congenital lesion typically presents at birth as a faint, well-
demarcated pinkish spot that, with aging, becomes elevated and varie-
gated. Enjorlas and coworkers18 reported that 45% of facial port-wine
stains restrict themselves to a single trigeminal dermatome distribution.
Port-wine stains were found to have an incidence of 0.3% in a series of
newborns reported by Jacobs and Walton in 1976.4 The Sturge-Weber
syndrome includes vascular anomalies of the upper face, meninges, cho-
roid, and retina. Individuals at risk for this disorder must exhibit staining
in the ophthalmic distribution of the trigeminal nerve alone or in com-
bination with a maxillary or mandibular root involvement.19 An infant
with a port-wine stain of the eyelid should be examined by an ophthal-
mologist every 6 months until age 3 years and each year thereafter. A
critical consideration is the psychosocial impact that the facial lesion will
have on a child’s development.

Other Vascular Lesions Affecting the Eyelids and Orbit

in Children
Pyogenic granuloma is a reactive, proliferative vascular lesion. It com-
monly occurs in children, presenting as a small, circumscribed, easy-
bleeding nodule, often involving the conjunctival or dermal sides of
the eyelid. Pyogenic granuloma has been documented to arise over an
existing port-wine stain.20,21 Hemangiopericytoma is a malignant neoplasm
arising from the vascular pericyte. The tumor accounts for approxi-
mately 2% of all orbital tumors.22 This mass observes no sexual predi-
lection, presents as slowly progressive proptosis and, although most
common in adulthood, has been reported to involve the orbit of a 3-year-
old child.23 Malignant hemangioendothelioma (angiosarcoma), a malignancy
of endothelial origin, involves the orbit in approximately 3% of cases.24
Other less common lesions include the idiopathic inflammatory pro-
cess known as angiolymphoid hyperplasia with eosinophilia (Kimura’s
disease). This disorder is characterized by proliferating blood vessels
and a mixed infiltration of lymphocytes and eosinophils.25 Among
the cases reported is a 5-year-old with bilateral eyelid involvement.26
Another case of orbital involvement was reported in 1999 by Chang and
associates.27
Other vascular tumors that may affect the orbit and adnexa in
children include intravenous papillary endothelial hyperplasia, Kaposi’s
sarcoma, and the racemose hemangioma of the Wyburn-Mason syn-
drome. Their clinical presentation and treatment are not discussed
here.
46 䡲 Garza et al.

䡲 Indications for Treatment

Ophthalmic complications such as astigmatism result from direct

globe deformity by an adjacent expanding mass. Treatment in this in-
stance is controversial, because the induced refractive error has been
observed to persist beyond the resolution of the occlusive-compressive
mass.28 Occlusion of the visual axis is a consequence of a rapidly growing
mass that causes mechanical ptosis. Strabismus is due to mechanical effect
over an extraocular muscle or its motor nerve. Corneal exposure occurs
secondary to axial globe displacement by an orbital mass. Other ophthal-
mic indications include compressive optic neuropathy and orbital-
palpebral bony asymmetry.7
Dermatologic complications manifest as hypertrophy of the epidermis
and subcutaneous tissues that results in ulceration, an outcome that is
seen in 5% of hemangiomas.29 The final result may be secondary infection
and permanent scarring.
Systemic complications include cardiovascular disorders such as high-
output congestive heart failure. Hematological complications take the
form of thrombocytopenia and bleeding (Kasabach-Merritt syndrome).
Finally, nasopharyngeal, oral, or supraglottic obstructions may occur.
The psychosocial implications of massive facial deformities in an in-
fant are great. Facial deformities in an infant may impede a caregiver’s
ability to provide genuine human affection to that child. Even before 1
year of age, infants may be denied the necessary stimuli for healthy emo-
tional development. Self-awareness of body image usually occurs between
18 and 24 months of age.30 Therefore, initial treatment often is planned
to precede this developmental milestone. Chronic irritation, rapid en-
largement, or hemorrhage resulting from chronic trauma such as inten-
tional or unintentional rubbing may necessitate earlier intervention. By
age 5 years, children in the United States are introduced into formal peer
groups such as school or day care. These nonfamilial social contacts may
be adversely affected by deforming lesions and should be carefully con-
sidered when interventions are being planned.
The cosmetic indications demand distinguishing reconstructive from
cosmetic facial surgery, which may be very difficult, especially in consid-
ering vascular lesions of childhood.31,32 During the early developmental
period, every effort must be made to optimize the child’s self-image.

䡲 Treatment Options

Hemangiomas
The historical trend has been to observe all periocular hemangiomas
and to intervene only when complications are foreseen. Recently, some
have advocated a more aggressive approach. Warner and Suen,30 in re-
 Treatment of Vascular Lesions 䡲 47

gard to what they believe is still an empirical approach to the treatment of

vascular lesions in childhood, stated, “In the best interest of our patients,
it is imperative that we establish protocols of management based on what
is best for the patient rather than what we are most familiar with.”

Observation Only The “biological approach” advocated by Mul-

liken33 remains a solid proposition in the case of periocular hemangiomas
as long as the growing lesion does not compromise visual development. It
should be recognized that untreated hemangiomas uniformly fail to re-
gress entirely, often leaving a fibrofatty subcutaneous mass and dusky
cutaneous surface to one degree or another.

Pharmacological Therapy Corticosteroids Zarem and Edgerton’s dis-

covery of the effect of oral corticosteroid on hemangiomas in 1967 was
fortuitous. These researchers observed resolution of a facial hemangioma
after administering systemic corticosteroid for the treatment of hemangi-
oma-associated thrombocytopenia.34 Corticosteroids remain the form of
treatment most familiar to the ophthalmologist, and systemic corticoste-
roids are considered by some to be the standard of treatment for vision-
threatening hemangioma. All routes of administration have been imple-
mented. The definitive mechanism of action is not clear, but proposed
explanations include sensitization of the hemangiomatous vasculature to
circulating endogenous vasoconstrictors.35 Sasaki and colleagues36 de-
scribed the influence of corticosteroids over hormone receptors on hem-
angiomas. Folkman and coworkers37 reported that cortisone inhibited
angiogenesis in the presence of heparin.
In an effort to overcome the systemic side effects or the risk of in-
tralesional injections, clobetasol cream, a potent topical steroid, was used
in 8 children in two separate studies.38,39 The results revealed a measur-
able decrease in the size of the lesions that was, nonetheless, insufficient
to prevent amblyogenic astigmatism. Furthermore, topical clobetasol
cream has been known to cause adrenal suppression. Kushner40 first re-
ported the use of intralesional steroids for periocular hemangiomas in
1979. He recommended a combination of betamethasone and triamcino-
lone, to provide both rapid action (betamethasone) and long effect (tri-
amcinolone). Currently, the most widely used combination is 3 to 5 mg
triamcinolone per kilogram of body weight and 0.5 to 1.0 mg betameth-
asone per kilogram of body weight. The injections should be given
separately via different syringes into the bulk of the lesion, to pre-
vent the formation of solid precipitates.41 The effects are usually dra-
matic, although additional injections sometimes are required. Zadok and
associates42 reported regression of a distant hemangioma after local
steroid injection into a separate hemangioma. Current consensus sug-
gests that the steroid effect is purely systemic, with no specific local effect
based on regional injection. Reported complications vary from growth
48 䡲 Garza et al.

retardation with adrenal suppression43 to bilateral retinal emboliza-

tion.44 Other less dramatic but significant complications are subcutaneous
linear fat atrophy at the site of injection,45 eyelid depigmentation,46 and
necrosis.47
Reports of successful treatment vary from 30%48 to 93%.30 It is be-
lieved that steroids are effective only in the early, active proliferating
phase that occurs in the first 6 to 8 months of a child’s life. Waner and
Suen30 have recommended the following regimen: prednisone or pred-
nisolone, 5 mg/kg body wt, as one morning dose. Appropriate gastritis
prophylaxis is recommended. If no response is seen after the first week on
this protocol, alternative management should be implemented. If the
lesion responds by complete cessation of growth or even diminution in
size, the full dose should be continued for 2 or 3 weeks. Potential com-
plications include immunosuppression with increased frequency of child-
hood infections.49 Cushingoid facies, failure to thrive, increased appetite,
and irritability all were reported by Sadan and Wolach50 in a series of 60
infants treated. These authors concluded that all side effects were revers-
ible.50

Interferon-alpha2a Interferon alpha2a was originally developed as an

antiviral agent. During treatment trials in patients with the acquired im-
munodeficiency syndrome, unexpected improvement of Kaposi’s sarcoma
(a vascular tumor) led to the discovery that this agent also acted as an
antiangiogenic drug. Further studies revealed that interferon inhibits the
advancement of capillary endothelium in vitro and angiogenesis in mice.
Subsequent to these discoveries, the role of interferon in treating life-
threatening hemangiomas has become more accepted.57
The effect of interferon on vision-threatening hemangiomas was stud-
ied in 15 children by Hastings and colleagues52 in 1997. Dramatic reduc-
tion in hemangioma volume, allowing eyelid opening, was observed after
6 weeks of treatment. However, 5 of the 15 children enrolled were re-
ported to have residual amblyopia. From this observation it appears that
interferon is highly effective but too slow in clearing the visual axis of
amblyogenic hemangiomas. Given the reported side effects (elevation of
liver enzymes, low-grade fever, neurotoxicity),53 this agent should be re-
served for cases that have failed to respond to other mainstream treat-
ments such as steroids.
More recently, empirical experience has demonstrated these unac-
ceptably high incidence (approximately 20–25%) of severe spastic diple-
gia (bilateral leg spasticity) presenting sometimes months after treatment
with interferon. Therefore, with the exception of severe, bilateral sight-
threatening, intracranial, or life-threatening lesions that fail to respond to
steroid treatment, the present recommendation appears to be to avoid
interferon altogether.
Vincristine has been shown to be effective and now is recommended
 Treatment of Vascular Lesions 䡲 49

for refractory cases of hemangioma in place of interferon (Dr. Milton

Waner, personal communication, March 2000).

Laser Therapy Almost all forms of medical lasers available have

been used to treat hemangiomas. Mulliken33 advocates the use of lasers
for only superficial telangiectasis of the regressing hemangioma and re-
marks that laser treatment that causes any cutaneous scarring is unwar-
ranted.
A study was conducted that compared the results of argon and neo-
dymium–yttrium aluminum garnet (Nd:YAG) lasers in 55 children with
infantile hemangiomas. The Nd:YAG laser was associated with a more
dramatic result but a much higher rate of complications, including de-
layed healing and permanent scarring.54 The use of carbon dioxide laser
was reported by Korn and colleagues,55 who demonstrated the efficacy of
this modality as an incisional and coagulating tool as opposed to an ab-
lative instrument. Waner and Suen30 reported promising results using
carbon dioxide laser for resurfacing of atrophic changes of involutional
hemangiomas. However, the flashlamp–pumped dye laser (also called
pulsed-dye laser, tunable dye laser) has been enthusiastically applied to the
treatment of hemangiomas and now is regarded as the laser of choice.56
The flashlamp–pumped dye laser is tunable—that is, the wavelength
can be modified. To best treat hemangiomas, the wavelength should be
set between 585 and 600 nm (yellow light). A longer wavelength will result
in deeper penetration, but this carries with it an increased risk of hypopig-
mentation. Pulse duration is another critical factor in the use of this type
of laser. Longer exposure time will address larger vessels but at the same
time increase thermal damage to surrounding tissue.
Geronemus and Ashinoff56 treated 10 children with facial hemangio-
mas, and while there were no cases of ulceration, hypopigmentation, or
scarring, there also was only a modest reduction in the size of all heman-
giomas treated. The researchers concluded that the flashlamp–pumped
dye laser is a safe treatment modality and proposed that earlier treatment,
when the lesion is smaller and thinner, would result in a superior effect.
Another series of 30 patients was treated with the flashlamp–pumped dye
laser. Similar low complication rates and comparable poor mass regres-
sion were reported by these authors, who concluded that deeper beam
penetration produces a better response.57
The following treatment plan has been recommended by Waner and
Suen30: A flashlight–pumped dye laser tuned to a wavelength of 585 nm
and a pulse width of 450 to 500 msec should be employed. The fluence
should be adjusted to between 6.5 and 7.5 J/cm2. Then, using a 5-mm spot
size, the entire surface of the lesion should be treated to an end point of
purpura.
Overall, it appears that laser treatment for infantile hemangiomas is
associated with few complications but lacks the efficacy of steroids. In the
50 䡲 Garza et al.

near future, certain technical modifications of laser devices could allow

them to become the standard of treatment for infantile periocular hem-
angiomas.

Surgical Therapy Excision of a periocular hemangioma is a poten-

tially legitimate approach, particularly when the lesion has not responded
to other forms of therapy. The limited window of opportunity in which the
clinician may effectively address the amblyogenic influences of an enlarg-
ing hemangioma demands rapid, effective action.
In 1992, Deans and coworkers58 presented a meticulous technique for
the surgical removal of hemangioma. Their results suggested an alterna-
tive to intralesional steroid injection in selected well-circumscribed le-
sions. Another report discussed the surgical outcome of 12 children: Total
mass excision was achieved in all but 2 cases, and no recurrences were
reported in a 5-year follow-up period.59 Additional studies have recorded
the resolution of astigmatism and good postoperative cosmesis after sur-
gical debulking of hemangiomas.60 A review of these reports suggests that
surgical intervention for periocular hemangiomas represents a feasible
option in confined, well-localized lesions. Contact laser delivery systems
have been used successfully to minimize intraoperative bleeding in hem-
angioma excision. The carbon dioxide and contact Nd:YAG lasers can be
effectively employed for sealing bleeding vessels. In other cases, surgery
may be used as a reconstructive tool years after medical treatment.

Vascular Malformations
As a rule, superficial vascular malformations such as the port-wine
stain are best approached by laser. Conversely, vascular malformations
affecting the orbit and adnexa, such as lymphangiomas and A-V commu-
nications, are better suited for surgery, sclerotherapy, embolization, or a
combination of these methods.
High-grade orbital A-V malformations may manifest early in life and
tend to worsen over time. Furthermore, the ocular effects and the possi-
bility of intracranial involvement may be devastating. Therefore, prompt
intervention is mandatory. A-V malformations may be treated by emboli-
zation, surgical resection, or both. Embolization is performed by an in-
terventional radiologist under fluoroscopic guidance.61 Materials success-
fully used in combination with surgery include Gelfoam, polyvinyl alcohol,
and isobutyl-1-2-cyanoacrylate. A combination of surgery with emboliza-
tion must be completed within a 48-hour span to avoid collateral blood
flow establishment. A new technique involving intraoperative direct ve-
nography with control of venous outflow by pressure at the superior or
inferior orbital fissure and embolization with cyanoacrylate glue has re-
cently been described. An orbitotomy then is performed and the vascular
anomaly removed as a cast.62
 Treatment of Vascular Lesions 䡲 51

Orbital varix treatment is reserved for highly symptomatic lesions.

Surgical excision can be complicated, although cases of successful exci-
sion have been documented.63 Lacey and associates62 believe that the
development of a biodegradable intravascular sclerosant or thrombosant
will lead to permanent regression of such varices without the need for
surgery.
Lymphangioma of the orbit is best managed conservatively. Exacer-
bations tend to occur during upper respiratory infections and may be
managed with short-course systemic corticosteroids. Rapid expansion may
also be seen in cases of intralesional hemorrhage with the formation of
the so-called chocolate cyst. In these cases, partial resection and drainage
may be required to manage acute orbital symptoms and compressive optic
neuropathy. Because of the infiltrative character of this malformation,
complete removal is considered impossible. However, we have, on occa-
sion, advocated bony orbital decompression to treat progressive proptosis
secondary to lymphangioma of the orbit.
Eyelid surgery such as permanent tarsorrhaphy and upper eyelid re-
cession may also be used in cases of corneal exposure. Surgery should be
restricted to cases of severe proptosis, corneal exposure, severe orbital
congestion, pain, or optic neuropathy. Aesthetic reconstruction is a rela-
tive indication and requires that one consider the degree of deformity as
well as the degree of remaining ocular function.
According to a recent report, intralesional injection of a sclerosing
agent (Sotradecol) shows promise but requires further investigation be-
fore it can be recommended for general use.64 OK-432, a streptococcus
derived sclerosing agent, has been used successfully for the treatment of
macrocystic lymphangiomas. Orbital lymphangiomas, in contrast, are typi-
cally microcystic, and reports on the use of OK-432 have shown disap-
pointing results.65
Port-wine stains have been treated unsuccessfully with various meth-
ods such as dermabrasion, surgical resection, radiotherapy, tattooing,
cryosurgery, and sclerotherapy. Over the last decade, the development of
new lasers has provided additional treatment options for this problematic
lesion that carries tremendous psychosocial implications for a child and
his or her family. The laser selection is defined by the stage of the lesion.
Early in childhood, these lesions are light, flat, and pink, and currently the
most accepted laser for their treatment is the flashlamp–pumped dye
laser. Darker raised spots in young adults can be better treated with a
copper bromide laser or a potassium titanyl phosphate laser. The
flashlamp–pumped dye laser adjusted to a wavelength of 585 nm, a pulse
duration of 450 µsec, a spot size of 5 to 7 mm, and a fluence of 5.0 to 7.5
J/cm2 is recommended for a facial port-wine lesion in a child. The end
point of treatment is a uniform bluish black discoloration of the entire
lesion. The treated area should heal in 2 to 3 weeks.
Postoperative care includes antibiotic ointment and avoidance of sun-
52 䡲 Garza et al.

light for at least 6 to 8 weeks. Complications of the laser treatment of

port-wine stains include hyperpigmentation, hypopigmentation, atrophic
scarring, and hypertrophic scaring. Hyperpigmentation has been associ-
ated with use of the flashlight–pumped dye laser in children. This side
effect is seen in 10% to 20% of cases.66 Although the hyperpigmentation
has been recognized as temporary, it may take up to 6 months to resolve.
Hypopigmentation is another potential complication that develops 6 to 9
months after treatment. The true incidence is not known, but this out-
come is thought to be underreported.

䡲 Conclusion

Vascular lesions of the ocular adnexa should be understood by oph-

thalmologists according to current, biological and pathological classifica-
tion as they are by other physicians. Childhood lesions affecting the orbit
and eyelids may be amenable to a variety of treatment protocols including
observation, medications, laser intervention, or surgical excision. Indi-
vidual cases should be approached with an open view to the numerous,
relatively new alternatives offered by modern medical technology.

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