Abstract
Purpose
Infantile hemangiomas (IH) are the most common benign vascular tumors of infancy. Ocular complications due to orbital IH include strabismal, deprivational, or anisometropic astigmatism, and visual loss secondary to amblyopia.
Observations
We report a case of a two-month-old female infant with a severe deep orbital IH. The diagnosis was established by clinical examination and magnetic resonance imaging (MRI). The patient was treated with oral propranolol for six months. Complete tumor regression was observed clinically and by MRI following the treatment. The patient showed no adverse effects or tumor recurrence at the 14-month follow-up.
Conclusions and Importance
Despite its benign nature, periorbital IH requires a rapid diagnosis and prompt treatment to prevent vision loss, particularly in infants. Our case confirms that oral propranolol may be regarded as a safe drug for periorbital IH with an excellent outcome.
List of abbreviations
CE-MRI
Contrast-enhanced magnetic resonance imaging
IH
Infantile hemangioma
MRI
Magnetic resonance imaging
PHACE
Posterior fossa brain malformations, hemangiomas (face), arterial, cardiac, and eye abnormalities
1
Introduction
Infantile hemangiomas (IHs) are the most common benign solid tumors of the ocular adnexa in children. IHs can cause significant functional and cosmetic deformities. Although IHs tend to regress spontaneously, those located periorbitally may cause unique complications including ptosis, strabismus, and anisometropia, each of which may, in turn, result in astigmatism, amblyopia, or blindness. Therefore, early treatment of these high-risk periocular tumors is necessary to prevent permanent vision loss. Both medical and surgical modalities are suggested to manage periocular IHs, but none have been uniformly safe and effective. Until the recent introduction of beta-blockers, systemic corticosteroids (prednisolone) were considered the mainstay therapy for complicated IHs, but long-term steroid use was associated with numerous side effects.
Herein, we report a case of deep orbital proliferating phase IH in a female infant that was successfully treated with a nonselective β-blocker propranolol. We also review the literature and discuss the pathophysiology, diagnostic, and treatment considerations for this rare condition.
2
Case report
An otherwise healthy 2-month-old female infant was referred to our department for evaluation and treatment of painless unilateral left proptosis, which had been slowly progressing over the past 15 days. She was born at 37 weeks’ gestation by a cesarean section. The ‘patient’s history and family history were not contributory. Laboratory investigations, including the complete blood count and routine blood chemistry, did not reveal any abnormality. Clinical examination revealed mild left eye axial proptosis with the fullness of the eyelids ( Fig. 1 A). There was no associated pupillary abnormality, and the rest of the neurologic examination was otherwise normal. The infant had no evidence of other head and neck cutaneous IHs. A contrast-enhanced magnetic resonance imaging (CE-MRI) of the brain and orbits, at the age of 66 days, revealed the presence of large, multilobulated lesion involving extra- and intraconal space extending in the mediolateral direction, measuring 37 × 19 × 27mm (AP x LL x CC). The lesion was slightly T1w hypointense, T2w isointense with multiple serpiginous flow voids, and with somewhat homogeneous contrast enhancement and marked enhancement of intratumoral vessels ( Fig. 2 A). We opted for oral propranolol therapy. Before administering propranolol, comprehensive history taking, vital signs, physical examinations, electrocardiogram test, routine blood tests, and blood glucose test were performed and interpreted by a pediatrician. The treatment regimens and potential adverse effects of propranolol were explained to the family of the patient. Parental education related to identifying potential complications of propranolol therapy has been performed. Propranolol was initially administered on the first day at a dosage of 1 mg/kg/day and then at a dose of 2 mg/kg/day divided into three doses under careful monitoring of heart rate and blood glucose levels in the hospital, and weekly follow-up at the outpatient clinic was arranged. At follow-up clinic visits, the dosage of propranolol was doubled to a maximum of 3 mg/kg/day. A follow-up MRI of the orbits performed at the 3-month visit revealed a significant regression of the lesion that measured 17 × 15 × 14 mm (APxLLxCC). At that point, her propranolol was tapered by 0.5 mg/kg per month. A follow-up MRI of the orbits scheduled and performed at the 6-month visit revealed complete regression of the left orbital mass with no detectable left intraorbital lesion present ( Fig. 2 B). Propranolol therapy was terminated when the child reached eight months of age (treatment for six months). At the end of therapy, the child showed complete lesion regression ( Fig. 1 B). The patient showed no adverse effects or tumor recurrence at the 14-month follow-up.
3
Discussion
We present an illustrative and rare case of an infant with deep orbital IH, discovered during the evaluation of unilateral left proptosis and treated successfully with oral propranolol. We also reviewed the recent literature that reported deep IH treatment with propranolol in infants <6 months (the results are summarized in Table 1 ).
