Capillary Hemangioma: 4 month-old female with occlusive vascular lesion of right upper eyelid

Jordan M. Graff, MD, Adam G. de la Garza, MD, and Keith D. Carter, MD

January 31, 2006

Chief Complaint: 4 month-old female presents with an occlusive vascular lesion involving the right upper eyelid resulting in ptosis.

History of Present Illness: A strawberry-like, compressible area was first noted on the right upper eyelid within weeks of a normal delivery. The lesion has increased in size over the past three months. An intralesional injection of steroid was done without any resultant decrease in size of the mass. Topical and systemic steroid therapies were attempted therafter. On oral prednisone for almost a month, the child became increasingly irritable and there was no noted decrease in size of the lesion. Oral steroids were discontinued. The patient was continued on topical steroid cream only and referred to the University of Iowa Department of Ophthalmology for further evaluation and care. Throughout this time, the local ophthalmologist directed the parents to use part-time occlusion therapy of the child's left eye. At the time of referral, the parents were patching the left eye for 4 hours each day.

Past Ocular History: As noted above.

Medical History: Recent otitis media was treated with amoxicillin. The infant has two small hemangiomas on the toes.

Medications: Topical clobetasol cream (0.05%) applied generously to the right upper lid twice a day.

Family History: Noncontributory.

Social History: Noncontributory.

Ocular Exam:

  • Visual Acuity, without correction: Right eye (OD)--difficult to open lid well; unable to assess reliably; Left eye (OS)--will fix and follow
  • Motility: OD--unable to assess; OS--full up, down, left, and right gaze (at least)
  • Intraocular pressure(IOP): Unable to assess reliably
  • Pupils: OD--unable to assess reliably; OS--briskly reactive
  • External and anterior segment examination: OD--unable to assess; OS--normal to portable slit-lamp exam
  • Dilated fundus exam (DFE): OD--Unable to assess; OS--No pallor or edema of the optic disc. Normal macula, vessels, and periphery
  • Lids:
    • Palpebral fissures: R: 1-3mm; L: 11mm
    • MRD1: R: -2mm; L: -5mm
  • Ultrasound: no orbital extension
Figure 1
Capillary hemangioma is seen involving the entire right upper lid of the sleeping patient. Even when awake, elevation of the right upper eyelid is severely restricted.

Figure 2
A: Side view of the upper lid hemangioma demonstrates the elevated nature of the lesion.

B: Stretching the skin of the upper lid clearly shows two distinct portions of the lesion -- an elevated, red area where there is superficial hemangioma and a smooth, bluish-purple area representing a deeper component.

Course: The examination and history are consistent with a capillary hemangioma of the right upper eyelid resulting in nearly total occlusion. Orbital echography demonstrated no intraorbital extension of the lesion. Due to the severity of the ptosis, amblyopia is a serious concern in this patient. Strict part-time occlusion of the left eye was continued and repeated intralesional steroid injections were offered. If the lesion continues to severely restrict vision in spite of repeated steroid injections, additional oral steroids or surgical excision should be considered.

Discussion: The history and presenting appearance of this patient are classic for capillary hemangioma. As the name implies, the lesions are made up of masses of very small-caliber vessels, and are created by abnormal growth of vascular endothelial cells. Therefore, capillary hemangiomas are hamartomas -- abnormal proliferations of tissue in a normal location. The lesion has a low rate of blood flow and is non-pulsatile.

In the vast majority of cases, the history and presentation of a periorbital capillary hemangioma is so typical as to be diagnositic. Caregivers will usually describe a growing red or purple spot that was not present at birth but becomes larger and thicker over the first few months of life. The lesion will blanch with pressure, which is helpful in distinguishing it from the non-blanching port-wine stain of Sturge-Weber syndrome. While most hemangiomas are not present at birth, more than half of them will manifest by 1-2months of age and 90-100% of the lesions will be present by 6-8months of age. The peak size of the lesion is reached by 6-12 months, at which point the hemangioma - left untreated - will remain stable for a time before beginning to involute. In fact, spontaneous involution is the rule for untreated capillary hemangiomas, with progressive involution continuing until age 8. The classic teaching is that 40% of hemangiomas completely regress by age 4 and 80% completely regress by age 8. It is important to educate concerned parents about the natural course of involution.

Treatment is indicated in cases of amblyopia, astigmatism or bony orbital asymmetry. Initiation of treatment for cosmetic deformity alone may be desired by the family, but this must be approached with caution given the natural course of involution and the potential risks of treatment. Commonly, first line therapy is intralesional steroid injection, with a mix of long and short acting steroid (i.e. 40mg/ml of triamcinolone and 6mg/ml of betamethasone mixed in a syringe). The injection should be through a medium-bore needle (i.e. 23-25 gauge) because smaller needles may clog with steroid particles. Though most hemangiomas will be reduced considerably with a single injection (see Figure 3), a repeat injection may be indicated at the 6th week of follow-up. Intralesional steroid treatment carries a risk of soft-tissue atrophy, hypopigmentation, glaucoma, and possible systemic absorption. Additionally, there have been exceedingly rare reported cases of central retinal artery occlusion secondary to embolization of depot material from steroid injections (Egbert, 1996). The second line of therapy is usually oral steroids (prednisone 1-2mg/kg/day). Due to the increased risks associated with oral steroid therapy (aggitation, changes in sleep pattern, increased risk of infection, adrenal crisis, etc.), the assistance of a pediatrician, pediatric endocrinologist, or family physician is strongly encouraged. Oral steroids will likely be continued for months and must be tapered with caution due to the risk of adrenal crisis. If steroid treatment fails, surgical excision is an option. Surgery carries with it the risks of general anesthesia and recurrence of the lesion, but may help to significantly debulk large hemangiomas. Because of the complicated reconstruction that may be needed, referral to an oculoplastic surgeon is usually best. Throughout the treatment for vision-threatening complications of the hemangioma itself, the ophthalmologist needs to be concurrently minimizing the development of amblyopia. The consultation of a pediatric ophthalmologist may be helpful.

Figure 3
A: Example of a similar patient with capillary hemangioma of the left upper lid. B: Significant reduction in the lesion is seen after a single intralesional steroid injection.

Less commonly used (and controversial) treatments include interferon alfa-2a, external beam irradiation, and various laser treatments (including CO2, argon, and Nd:YAG laser). Pulsed dye laser has also been used with some success, but only in very superficial lesions. The use of topical steroid creams (such as clobetasol) has also been reported, but the response has been both slower and less impressive (without avoiding the associated risk of elevated intraocular pressure and glaucoma that is associated with steroid use). Certainly, topical steroid should not be the only treatment for lesions where vision is affected by occlusion, amblyopia or mass effect.

The ophthalmologist should be aware that many children with periorbital hemangioma may also have intraorbital extension of the lesion and/or systemic angiogenic lesions (including pulmonary, soft-tissue and/or skin lesions). B-scan ultrasonography of the orbit is helpful in determining the posterior extent of suspicious lesions while chest or abdominal imaging may uncover other hemangiomas. Some patients with large visceral capillary hemangiomas may develop thrombocytopenia, a condition known as Kasabach-Merritt syndrome. If the child bruises or bleeds easily or angiomatous lesions are suspected, a pediatrician should be involved in the work-up and treatment of the patient. As mentioned above, the pediatrician should also be involved if oral steroids are required for treatment of the lesion.

Diagnosis: Capillary Hemangioma

EPIDEMIOLOGY
  • Capillary hemangiomas are present in 1-2% of neonates (one of the most common benign periorbital tumors of childhood)
  • Half of all capillary hemangiomas occur in the head and face
  • 3:1 ratio of females to males
SIGNS

Red or purple elevated, subcutaneous soft mass that will blanch with pressure

Hemangiomas are usually unilateral and located on the eyelid or brow

  • A mass effect of the lesion may result in significant ptosis of an involved eyelid

Reduced visual acuity may be noted (either due to amblyopia or uncorrected astigmatism from mass effect of a hemangioma on the cornea)

May have both cutaneous and subcutaneous portions of the lesion

  • Subcutaneous portion may extend into the orbit causing painless, non-pulsating proptosis of the involved eye
SYMPTOMS

Care-givers usually notice a red or purple lesion

  • >50% of lesions evident by 1-2 months
  • 90-100% of lesions evident by 6-8 months

Amblyopia is seen in approximately 50% of patients with eyelid hemangioma

  • Symptoms of reduced vision may be noticed

In rare cases of associated thrombocytopenia, a propencity for easy bruising or bleeding may be noted

  • Kasabach-Merritt syndrome is a consumption coagulopathy with mortality rates that may approach 40-50%
TREATMENT

Spontaneous involution is the rule - observation may be appropriate

  • 40% completely involute by age 4
  • 80% completely involute by age 8

In cases of occlusion amblyopia or significant astigmatism, prompt treatment should be initiated:

  1. Intralesional steroid injection with a mix of long and short acting steroid (i.e. 40mg/ml of triamcinolone and 6mg/ml of betamethasone mixed in a syringe and injected through a medium-bore needle)
  2. Oral prednisone (1-2mg/kg/day given with the involvement of the child's pediatrician - treatment is usually for months with slow taper)
  3. Surgical excision (for debulking - lesion is not encapsulated and there is a risk of recurrence. Anticipate bleeding and control with cautery)

Other treatments have been described (topical steroid, radiation, and a variety of lasers) but they are considered somewhat controversial and not used routinely

Differential Diagnoses:

  • Vascular malformation (i.e. lymphangioma or arteriovenous malformations)
    • These, however, are present at birth and persist into adult life
  • Nevus Flammeus (port-wine stain)
    • seen in Sturge-Weber syndrome and Klippel-Trenaunay-Weber syndrome
    • Port-Wine stain will not typically blanch with pressure - - a capillary hemangioma will usually blanch
  • Lymphangioma
  • Dermoid cyst
  • Neuroblastoma, metastatic
    • with painless proptosis and spontaneous hemorrhage (red or ecchymotic lids), neuroblastoma can be mistaken for either trauma or deep hemangioma