Anophthalmos Treatment & Management

Updated: Aug 06, 2021
  • Author: Nick Mamalis, MD; Chief Editor: Donny W Suh, MD, MBA, FAAP, FACS  more...
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Treatment

Medical Care

Medical care in anophthalmos focuses on treating the soft tissue hypoplasia as well as asymmetric bony growth and is as follows. [26, 27, 28]

Ocular/orbital  [29]

A solid conformer may be placed in the orbit to stimulate bony orbital growth and to enlarge the orbital cavity in an attempt to attain normal proportions.

Progressively increasing the size of conformers will often help to increase the size of the orbit.

Solid conformers require multiple procedures and are generally unable to keep pace with bony growth of the contralateral normal orbit.

An ocular prosthesis may be fitted over the conformer to improve the appearance.

In patients with unilateral anophthalmos, they and their families should be aware that the target proportions of a reconstructed orbit are not planned to exactly mirror that of a healthy orbit. In all likelihood, a perfectly normal-looking orbit will not be achieved.

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Surgical Care

Surgical care in anophthalmos is as follows [30] :

Inflatable expander  [31]

If conformers are not tolerated or are unsuccessful, an inflatable expander may be placed surgically.

The expander works best if placed relatively early in life, especially within the first year.

The inflatable silicone expander is placed surgically deep into the orbit and is accessible by a tube placed in the lateral orbital rim.

The expander can be gradually filled with liquid (eg, saline) on a weekly or biweekly basis.

The advantage of an inflatable expander is that it may allow more rapid and extensive orbital tissue expansion as compared with solid conformers.

Integrated orbital tissue expanders have been shown [32] to stimulate orbital bone growth in a feline model. [33]

Recent clinical reports have shown promising results in the stimulation of anophthalmic orbital growth. [34, 35]

Self-expanding hydrophilic, osmotic expanders  [36]

A new possible treatment is the use of self-inflating expanders.

Hydrophilic expanders are placed in their dry, contracted state. The expanders then expand gradually to their full size via osmotic absorption of surrounding tissue fluid.

This method offers the benefit of controllable self-expansion, without the necessity of repeated fittings of solid conformers or surgical placement of external tubing required for inflatable expanders.

Long-term biocompatibility studies have not been completed, but early results are promising.

Dermis Fat Graft

Recently, dermis fat grafts (DFG) have been proposed as a strong alternative to other orbital implants. [37] DFG has been reported to allow for deeper fornices and improved motility while eliminating the risk for extrusion. [38] This procedure can be used for congenital anophthalmos, acquired anophthalmos, or in reconstruction of contracted sockets. Additionally, this may be a more cost-effective approach compared to alloplastic implants.

Injectables

Volume augmentation in the anophthalmic socket with injectable calcium hydroxylapatite (Radiesse) has been reported. [39]  Similarly, orbital fat injection has been used for enophthalmos. [40]

Eyelid surgery

The increase in the size of a conformer is often limited by shortening of the eyelids in the palpebral fissure, which do not permit passage of a large conformer. The horizontal length of the palpebral fissure may be increased surgically by performing a lateral canthotomy or cantholysis.

An additional method to lengthen the eyelids can be accomplished by a combination of skin, mucosal, or cartilage grafts.

Eyelid reconstruction presents a challenge. In cases of anophthalmia, skin graft is required to construct eyelids. Since the area around the eye is cosmetically important, grafts from areas of the head covered by hairline may be advantageous. [41]

Orbital surgery

If conformers and expanders are unsuccessful, the bony orbit may be expanded surgically. This method is preferred in cases of late referral or insufficient orbital volume.

The orbit can be expanded in 3 different directions, as follows: laterally, inferiorly, and superiorly.

Surgical expansion of the orbit can be accomplished by dividing the bony orbital rim into 3 parts in a stepwise fashion.

Cranial bone grafts may be used to augment deficient orbital contours.

Lastly, a bicoronal approach through the scalp may be necessary when the orbital roof has to be elevated.

A survey of oculofacial surgeons found that silicone and acrylic were the most popular orbital implant materials in both adults and children, followed by porous polyethylene. The mean implant size was 20.2 ± 1.78 mm in adults and 17.2 ± 2.36 mm in the pediatric population. [42]

3D Printing

Recently, 3D printing advances have allowed for creation of conformers, prostheses, and patient-specific implants to stabilize migrant implants. [43, 44, 45] Computed tomography (CT) has been used to evaluate the size of the socket as well as generate models for 3D-printed orbital implants. [46]

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Consultations

Anophthalmos causes serious psychological problems due to not only the absence of an eye but also the disfigurement of the orbital socket and the eyelids. [47] Psychological counseling or consultation may be warranted for these children.

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