11.4Management

11.4.1Active Nonintervention

In many uncomplicated lesions, observation without treatment is the preferred approach, but careful ocular monitoring is recommended with serial ophthalmologic assessments and repeated assessment of lid position, evaluation of ocular alignment for potential strabismus, and especially cycloplegic refraction to document potential development of anisometropia. Deciding to observe the natural evolution of the lesion instead of intervening has been referred to as “active nonintervention” by Bruckner and Frieden in opposition to benign neglect [7, 32]. This is reserved for small IHs for which the proliferative phase suggests a limited lesion. Parental support and precise explanations concerning the natural evolution of the condition and its most likely outcome will greatly help the family to cope with the transient but initially progressive lesion of their child. In periocular lesions, frequent monitoring of the visual status and early management of anisometropia or amblyopia are done even if the lesion does not need specific measures for controlling its size or growth.

To help guide that decision not to intervene, the natural history of hemangiomas of infancy is very instructive: The most important part of the growth of a specific lesion is completed at 3–6 months in most lesions. Exception to this are segmental lesions and the deeper lesions, which tend to have a late proliferative phase with a peak around 5 months and a persistence of proliferation at a slower rate for a longer period thereafter compared to the superficial lesions. Despite these di erences, almost all the lesions have reached their full size by 9 months [10]. Careful screening for amblyopia is performed. If no ocular complications are encountered, observation for eventual involution is indicated. When amblyopia is detected, a regimen of occlusion of the normal eye is started. Results are best when treatment is started early, and in some cases atropine penalization of the normal eye may be su cient when the amblyopia is mild [9]. When anisometropia is present, corrective glasses are prescribed if the induced cylinder is more than 1 diopter.

For lesions for which the amblyopia can be kept under check, the decision to treat or not should be based on the probability of complete regression without irreversible anatomic sequelae. Often, in a newborn with a freshly appeared lesion it is di cult to predict the growth pattern of a specific lesion. Repeated frequent exams will help to get a feel for the growth potential of a specific lesion. The surface involved at presentation may also be

11.4 Management 165

an indicator of the potential for growth, especially if the lesion appears to involve a large segmental facial area with its association to PHACE syndrome. These large segmental hemangiomas involve a region of skin corresponding to a derivation from the embryologic mesenchymal prominences [7].

11.4.2Indications for Treatment

Modern management of these patients is often done by teams of physicians that include a neuroradiologist or interventional radiologist, a pediatric dermatologist, a pediatric ophthalomologist, and often a pediatric plastic and oculoplastic surgeon. The extent of the lesion, its position and size, as well as the phase of development will be key elements to decide if specific treatment is necessary.

When treatment is indicated, the goal is to stop the growth of the lesion or to induce early involution. However, the clinical response of the hemangioma to treatment, even if it is spectacular, should not reassure the team of the outcome of vision. Continuous monitoring of the visual status with repeated cycloplegic refractions and assessment of the presence of strabismus or amblyopia should be performed. Occlusive therapy of the normal seeing eye is continued and depends on the response of the visual deficit independently of the involution of the hemangioma (Fig. 11.2).

Summary for the Clinician

■Knowing the risk factors for visual complications will help the clinician in management decisions.

■Active screening and treatment of amblyopia is important in all phases of an IH.