Propranolol therapy in a case of capillary hemangioma.

Capillary hemangioma is one the most common tumors of eyelid and orbit reported in pediatric age group. Oral propranolol therapy is latest addition to the armamentarium of treatment options available to ophthalmologists in treating capillary hemangiomas. We report the successful response to propranolol therapy to a 5-year-old child with capillary hemangioma involving lids, orbit, and the paranasal sinuses. A long-term follow-up is necessary for the prognostic efficacy of the therapy.

Introduction

Capillary hemangiomas are the most common tumors of the lids and orbit in the pediatric age group with an incidence of about 10%. Preponderance is seen age wise in infants (premature infants are at a higher risk), gender-wise in females while racially in Caucasians. Capillary hemangiomas are characterized by rapid proliferation during the 1st year followed by spontaneous complete involution in 72–76% cases, usually by 7 years of age.[1]

Functional consequence like amblyopia secondary to induced astigmatism or mechanical ptosis or cosmetic sequelae may occur and require early intervention. Various treatment modalities encompass-intra lesional steroid injections, interferon injections, resection, etc. In the following study, the treatment response to oral propranolol therapy has been reviewed in a case of rapidly progressive periorbital hemangioma extending up to the palate associated with ametropic amblyopia.[12]

Case Report

A 5-year-old male child presented to the out-patient department with chief complaints of redness and swelling around the right eye. These deformities were present since birth and had exacerbated in the past 1-month. The patient's mother also complained of child spitting out fresh blood clots for the past 1-week. There was no preceding history of any recurrent episodes of upper respiratory tract infection or increase in the size of the swelling with straining or on cough. The child had not been provided any treatment for the mentioned symptoms in the past.

On examination, a diffuse bluish compressible swelling was seen in right eye involving both upper and lower eyelids. Surface of the swelling was nodular near the eyelid margins. An erythematous plaque extending from medial part of the right eyebrow to medical canthus was observed which showed blanching on pressure. The plaque appeared to be in continuity with the swelling of lids. The swelling was nonpulsatile with no bruit on auscultation. Patient had anisometropic amblyopia in the affected eye which may be attributed to astigmatism induced by pressure affect of the lesion on the globe. Examination of the oral cavity revealed an ill-defined reddish lesion involving posterior part of the palate. The clinical examination gave an impression of sight threatening, rapidly progressive, bleeding capillary hemangioma of the periorbital region [Figure 1].

Figure 1

Clinical pictures of the patient

Imaging was done by gadolinium-enhanced magnetic resonance imaging (MRI), which depicted a lesion in the frontal region of scalp on the right side with extensions into retro-orbital, infero-temporal, and oropharyngeal regions on the right side. Encasement of the optic nerve was also seen in the intraconal part of the lesion. The lesion was hypointense on T1-weighted images and hyperintense on T2-weighted and fluid-attenuated inversion recovery imaging postcontrast enhancement [Figures 2 and 3]. A complete systemic evaluation of the patient was performed by the pediatrician in accordance with a pediatric cardiologist and pulmonologist before instituting treatment, to rule out any known or unknown systemic association and congenital anomalies.

Figure 2

Magnetic resonance imaging-transverse and sagittal sections

Figure 3

Magnetic resonance imaging-coronal sections

Treatment was planned as described. A dose of 1 mg/kg/day intravenous (I.V.) propranolol solution was initiated on the 1st day. Patient was kept under observation for the 1st 24 h, during which blood pressure and heart rate were measured at hourly intervals. Blood glucose was measured if clinically indicated based on symptoms of clamminess, distress, or irritability. The dose was doubled on the following day (administered orally) as the vitals and blood glucose levels were normal. The maintenance dose was 2 mg/kg/day divided in two oral doses. Patient was followed up on a weekly basis for the 1st month, then every 2 weeks in the 2nd month, and finally at 4 weekly intervals for a period of 2 months.

Regression in the size of swelling was noted within the 1st 48 h of initiating treatment. Regression started with opening of the palpebral aperture and palpable softening of the lesion. After this initial response, hemangioma continued to decrease in size. Flattening of the lesion and more evident blanching of the color were noted. There was seen a significant decrease in size of the tarsal conjunctival chemosis at the end of 2 weeks. Furthermore, a complete regression was seen in the size of hemangioma involving the palate region at the end of 3-month therapy. Oral propranolol 2 mg/kg body weight was continued for 1-month postevidence of regression of lesion on MRI at the end of 4 months and then tapered gradually to 1 mg/kg body weight in the 6th month following which it was stopped.

Repeat follow-up MRI of the patient at 4 months showed complete regression of infero-temporal and oropharyngeal extension of the lesion and mild postcontrast enhancement in retro-orbital region [Figures 4 and 5]. The guardians were instructed to bring the patient for follow-up immediately in case of recurrence of any previous signs of the lesion, otherwise every 2 months for 1-year.

Figure 4

Magnetic resonance imaging-posttreatment

Figure 5

Clinical pictures-posttreatment

Discussion

The main indications for intervention in capillary hemangioma include sight-threatening, rapidly progressive or recurrently bleeding lesions. Sight-threatening hemangioma are defined as lesions causing obstruction of the visual axis or inducing astigmatism, strabismus, amblyopia, or anisometropia. There are multiple modalities for management of periocular capillary hemangioma described in the literature. The most commonly used modality has been intra-lesional or systemic corticosteroids. But these are accompanied by the high rate of grave systemic and local adverse effects. Other systemic treatment options include vincristine, interferon alpha, laser therapy, cyclophosphamide, and bleomycin.[3456]

Recent studies have shown nonselective beta blockers to project encouraging results in the management of capillary hemangioma. Their exact mechanism of action is not defined yet, but it has been proposed that beneficial effects of propranolol are probably due to reduction of the expression of genes for vascular endothelial growth factor, basic fibroblast growth factor, and matrix metalloproteinase-9, in addition to induction of apoptosis in capillary endothelial cells. Except I.V. other routes of administration for propranolol that have been tried are topical and timolol gel formulations for infantile hemangiomas.[78]

Though, relatively harmless propranolol therapy is not devoid of adverse effects. Bradycardia and hypotension are the most common side effects. Propranolol-induced hypoglycemia or the masking of its symptoms are particularly important in children. Wheezing, hypokalemia lethargy, and hypothermia have been reported and require termination of therapy. (1) Prematurity and asthma cause exacerbation of side effects induced by therapy. (2) Rebound growth has been seen in 25% of cases after cessation of therapy, especially in patients <5 months of age.[910]

Notwithstanding propranolol therapy is an efficacious and relatively safer modality for treatment of periocular hemangiomas though a thorough pretreatment work-up and a posttreatment follow-up is mandatory to extract best results.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed