CONSULTA CIUDADANA SOBRE SEGURIDAD CIUDADANA

A new treatment option has been proposed in the form of the nonselective beta-blocker propranolol [27]. Th is was discovered fortuitously, initially in corticosteroid-failure patients. Th e initial report of 11 infants with severe or dis- fi guring IH treated with propranolol at 2 mg/kg/day showed impressive results with minimal side eff ects. In all patients, 24 h aft er the initiation of treatment, a change in the color of the lesion was observed along with soft ening of the mass. In fi ve cases for which ultrasonography was done, an objective regression of the lesion was observed. Improvement leading to fl attening of the lesion was noted with continued treatment. Since this was published, many centers have started using this medication with various protocols, oft en initiating treatment in a hospital setting to monitor vital signs; in the outpatient setting, beginning medication at a lower dose (i.e., 0.5 mg/kg/day) and grad- ually increasing up to 2 mg/kg/day with close monitoring of blood pressure and pulse is another option. Th e optimal dosage and frequency of administration of propranolol for IH is still not well established, and additional studies

are necessary before this medication can be recommended on a widespread basis. At this time, this is still an off -label use. Contraindications include bronchospasm/asthma, congestive heart failure, bradycardia, or hypotension. Hypoglycemia can occur, especially in the very young infant. Th ere is concern that propranolol, because of its vasoconstrictive eff ect, could provoke complications in the setting of cerebrovascular anomalies in patients with PHACE syndrome; it is thus recommended to obtain an MRA in infants with facial segmental IHs to evaluate the cerebral arteries before considering this treatment option. Propranolol, however, appears to be an extremely promis- ing new treatment option with a better safety profi le than interferon-alfa or vincristine and potentially might become the fi rst-line treatment for complicated IHs. As with other systemic treatments for IHs, a multidisciplinary approach is optimal. Th e mechanism of action of propra- nolol in IHs is not well understood but may include vaso- constriction, explaining the very rapid color change and soft ening of the IH aft er initiating treatment; propranolol has also been shown to decrease the expression of VEGF and bFGF genes in vitro as well as to trigger apoptosis of capillary endothelial cells [28] (Figs. 11.6–11.11).

Fig. 11.6 Photograph of a toddler with continued growth of an IH despite systemic corticosteroids

Fig. 11.7 Necrosis of portion of the involved ear occurred despite initiation of steroids

Fig. 11.8 Marked regression of the lesion 6 weeks aft er initia- tion of propranolol at a dosage of 2 mg/kg/day

Fig. 11.10 Large, rapidly growing IH involving the lower lid and the cheek causing visual axis blockage

Fig. 11.11 Marked change in color, fl attening, and soft ening of the lesion 3 weeks aft er initiation of propranolol, allowing for clearing of the visual axis. No other treatment was used in this patient

Summary for the Clinician

Systemic steroids are the gold standard for large ■

IHs for which systemic treatment is necessary. Interferon-alfa is used only in severe selected cases ■

because of its potential for severe side eff ects. Intralesional injection should be used cautiously. ■

Surgery is indicated mainly in localized lesions ■

or to correct anatomical defects in the involu- tional phase.

Propranolol off ers a new and promising modal- ■

ity of treatment with potentially fewer systemic complications than the available alternatives.

Fig. 11.9 Further regression of the abnormal vessels is seen at 10 weeks aft er initiation of the treatment. Gradual tapering of steroids was initiated 4 weeks aft er the initiation of propranolol

11

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12.1 Introduction

Since the initial report by Horner of metastatic orbital disease in 1864, numerous authors have described cases and series in the medical literature [69]. Although this is an uncommon condition, its incidence is increasing as life expectancy increases with advances in medical treat- ments [41, 48, 53, 113].

Without a high index of suspicion, the variable pre- sentation of orbital metastases can easily lead to misdiag- nosis [16, 21, 41, 45, 53, 57, 76, 78, 80, 100, 126, 135]. Not infrequently, the diagnosis is unexpected, in spite of the advances in screening techniques, thereby delaying defi n- itive treatment [22, 53, 57, 132].

Ophthalmologists have a crucial role in the diagnosis of metastatic orbital cancer, off ering valuable information that can guide specifi c investigations and provide tissue for diagnosis. Furthermore, they can assist in providing specifi c therapy for palliative care and even potentially off er remission with advanced multidisciplinary thera- pies [45, 53].