Treatment of Anophthalmia/Microphthalmia
All children with microphthalmia have hypoplastic orbits. Because most microphthalmic eyes have no potential for vision, treatment focuses on achieving a cosmetically acceptable appearance that is reasonably symmetrical. Treatment begins shortly after birth and consists of socket expansion with progressively larger conformers, which are used until the patient can be fitted with a prosthesis. Enucleation is usually not necessary for the fitting of a conformer or an ocular prosthesis and is ordinarily avoided because it may worsen the bony hypoplasia. Orbital volume may be augmented with autogenous materials, such as dermis-fat grafts (Fig 3-2), or with synthetic implants. When placed at an early age, dermisfat grafts may grow with the child, resulting in progressive socket expansion. In cases of severe bony asymmetry, intraorbital tissue expanders can be progressively inflated to enlarge the hypoplastic orbit.
Figure 3-2 Dermis-fat graft in the left anophthalmic socket.
(Courtesy of Cat N. Burkat, MD.)
Craniofacial techniques have been used to reposition and resize the orbit in patients with severe microphthalmia or anophthalmia or in older children with previously untreated microphthalmia. Such repairs are complex, as noted in the following discussion of craniofacial clefting.
Microphthalmia with orbital cyst results from failure of the choroidal fissure to close in the embryo (Fig 3-3). This condition is usually unilateral but may be bilateral. The presence of an orbital cyst may be beneficial for stimulating normal growth of the involved orbital bone and eyelids. In some cases, the orbital cyst may be removed to allow the fitting of an ocular prosthesis. When these conditions are bilateral, significant visual impairment can result. Prompt referral for low vision rehabilitation and early intervention should be initiated in infancy, as soon as vision impairment is suspected.
Figure 3-3 Microphthalmia with orbital cyst. A, Young child with left micropthalmia with orbital cyst. T2-weighted axial magnetic resonance imaging (MRI) (B) and coronal MRI (C) show the orbital cyst component.
(Courtesy of Don O. Kikkawa, MD.)
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.