Anophthalmia, from the Greek, meaning “absence of eye,” is uncommon as a congenital condition. Congenital anophthalmia, sometimes used interchangeably with microphthalmia, can be due to chromosomal, environmental or monogenic factors. More commonly, anophthalmia is acquired, most often due to a blinding trauma or a blind painful eye. In this article, we’ll help you understand how to evaluate and manage anophthalmic sockets to allow patients to achieve the best functional and cosmetic outcomes possible.
The Surgical History
Whether the anophthalmia was congenital or acquired, when a patient undergoes implant surgery, it’s with the following goals in mind: treat the underlying condition; replace the orbital volume; maximize motility; and provide the most comfortable and aesthetically symmetric appearance possible. Today, most implants are solid (polymethyl methacrylate or silicone), porous (hydroxyapatite and high-density polyethylene) or autogenous dermis-fat grafts. The implants come in a range of shapes and degree of integration with the overlying prosthetic. Recent surveys note porous materials as the most commonly used for implants.1,2
Figure 1. Anophthalmia, left. The left eye socket has a deep superior sulcus with lower lid retraction and lash position abnormalities, compared to the unaffected right eye.
All anophthalmic sockets undergo changes, including orbital fat atrophy and changes in the orbital circulation.3 These changes and the placement of an orbital implant can lead to a variety of complications and management difficulties.
For these reasons, it is important that the general ophthalmologist be able to adequately evaluate and treat simple problems of the anophthalmic socket.
When a patient with anophthalmia and an implant presents, there are a few basic questions the general ophthalmologist should ask after a general ophthalmic and surgical history:
• Does the patient have pain with the prosthetic in?
• Does the prosthesis fall out?
• Is there discharge or bleeding from the socket?
• How old is the current prosthetic and when was the last time it was polished?
• Does the patient have polycarbonate glasses to protect the seeing eye?
• Is the patient happy with the cosmesis and movement of the prosthetic?
When evaluating the anophthalmic socket it’s important to note the general appearance and symmetry compared to the contralateral side. With the prosthetic in place the patient should be evaluated for enophthalmos or prosthetic malposition. The lower eyelid should be evaluated for laxity. Lash position and lid margin position should be noted, as entropion can indicate socket contracture. The superior sulcus should be checked for deepening and symmetry with the opposite side (Figure 1). The upper eyelid position should be examined for ptosis, and levator function should be evaluated.
Figure 2. Anophthalmia, right. In addition to the asymmetry in primary gaze with a deep sulcus of the right upper lid, the patient has very poor motility of the prosthesis. The underlying motility should also be
Evaluate the movement of the prosthetic compared to the seeing eye. Poor movement can be due to fornix abnormalities, enophthalmos or poor prosthetic depth. Address any socket or prosthetic abnormality, and if the patient is still unhappy with the motility, there are surgical prosthetic-implant coupling methods that may improve motility for the patient (Figures 2A to 2C).
Remove the prosthetic and evaluate it. If the prosthetic is thick it may be placing pressure on the lower lid and could be camouflaging low orbital volume. Also, note whether the prosthetic is smooth and clean. Evaluate the socket for inflammation, excessive mucous, giant papillary conjunctivitis under the upper eyelid and pyogenic granulomas. Take note of the forniceal depth, and whether the superior fornix is excessively deep or the fornices aren’t well defined. Examine the tissue over the implant for thinning, fistula or defects. Lastly, on palpation of the socket, note the presence or absence of an implant and its position.
Of course the patient should wear protective glasses and have careful exams of the seeing eye, with the frequency of exams determined by the patient’s age, history and the health of the eye.
Following are the more common complaints of the anophthalmic patient, and how to manage them.
• Discharge. This is a common complaint, and there can be a range of underlying causes for it. A common etiology is giant papillary conjunctivitis. This inflammatory reaction is diagnosed by visualizing papillae of 1 mm or greater on the superior tarsal conjunctiva. The conjunctiva becomes edematous and excessive discharge can be seen. Since the pathogenesis is thought to be a combination of immunologic response to the prosthetic/deposits and mechanical trauma from the prosthetic itself, though the process isn’t fully understood, one might think the optimal treatment is removal of the prosthetic, as in contact lens-related GPC. However, since socket contraction can occur without the prosthetic or a conformer in place, this is usually the last management option. Instead, the primary treatment is prosthetic polishing and topical cromolyn sodium or topical steroids.
Other conditions that may cause discharge are poor prosthetic fit, implant extrusion, pyogenic granuloma in the socket, excessively deep fornices, conjunctival infection or nasolacrimal duct obstruction.
• Enophthalmos. This is caused by either the prosthetic’s position or a deep superior sulcus, and can be present initially or develop over time due to implant size, implant shifting and/or change or atrophy of the orbital tissues. It can be addressed by surgically augmenting the orbital volume. A variety of surgical techniques can be applied, including subperiosteal implants or secondary orbital implants. (A comprehensive discussion of implants and surgical techniques is beyond the scope of this general review.)
After surgery, if any superior sulcus deformity remains, there are surgical and filler options to improve the appearance. However, for the patient interested in a non-surgical fix, placing a +2 D sphere or higher over the affected side will magnify the eye socket and make the enophthalmos less noticeable. Caution should be used in increasing the thickness of the prosthetic to compensate for this volume loss, as over time the lower lid will become more lax and the prosthetic will sink inferiorly.
• Lower eyelid laxity. Lower lid laxity, with or without lid margin malposition, can be partially due to the weight of the prosthetic and can lead to other changes, including ptosis of the upper lid. This is addressed by having the prosthetic remade, followed by surgery with horizontal lid shortening. If there’s any forniceal shortening or other findings, they would also need to be addressed.
• Socket contracture. This can accompany lid malposition and has myriad causes. There’s no single surgical technique to address socket contracture and management should be individualized and address lid position abnormalities at the same time.
• Orbital implant exposure or extrusion. Thankfully, this is an uncommon patient presentation, with average incidences of 5.6 percent (exposure) and 1.3 percent (extrusion) with porous implants (Figures 3A and 3B).4 When this occurs, the time since surgery and size of the defect can impact the management. Surgery is often required, and involves a simple closure or a patch graft.
Figure 3. Anophthalmia with implant exposures. Figure A shows exposure of a smooth, acrylic implant. Figure B shows an exposed, porous implant leading to mucoid discharge.
• Upper eyelid ptosis. This is common in the anophthalmic socket. This can be a true ptosis or a pseudoptosis due to poor support from the prosthetic or poor orbital volume and implant location. As is well known, any orbital surgery, such as volume augmentation, should precede eyelid correction. Once any socket problems have been corrected, the ptosis can be addressed.
If the ptosis is mild the ocularist can build up the prosthetic superiorly to support the upper eyelid. However, since this increases the weight of the prosthetic it can begin a cycle of problems in the future by inducing lower eyelid laxity, which then leads to a deeper superior sulcus and the need for a larger prosthetic in a perpetuating cycle. If the ptosis is to be addressed surgically, the ophthalmologist should keep in mind that levator strength may be underestimated.5
Orbital pain in the anophthalmic socket can be difficult to diagnose, since the etiology can range from prosthetic irritation or migration/extrusion of the implant to neurologic causes. One should also rule out lacrimal insufficiency, inflammation (scleritis, sympathetic ophthalmia and GPC), trochlear irritation and possible recurrent tumors.6 Amputation neuromas, an exuberant overgrowth of neurons and connective tissue at the transected nerve, are uncommon but can result in pain, particularly with eye movement. Periorbital pathologies, such as sinus diseases or central nervous system tumors can also cause referred socket pain and should be considered. If the etiology isn’t found on examination or improved with medical therapy, imaging may be warranted.
Psychogenic factors, such as drug-seeking behavior, can also lead to pain, but these are diagnoses of exclusion. Chronic pain syndromes, which can overlap with psychogenic factors, can also complicate the evaluation and are diagnosed in conjunction with other specialists after ruling out orbital/prosthetic etiologies. In these cases, patients nearly always need referral to an ocularist for assessment and prosthetic modification and/or polishing. If the etiology isn’t clear and persists after prosthetic polishing and lubrication, the patient may need a CT scan to aid in diagnosis.
The History of Anophthalmos Management
The first enucleations were possibly performed by the Chinese as early as 2,600 BC. However, the first report by Johannes Lange7 was not until 1555, and the first description of the procedure appeared in 1583, by George Bartisch.8 In the 1800’s the procedure made dramatic advances, including extraocular muscle disinsertion, as well as the introduction of controlled anesthesia, leading to great improvements and increased use of the procedure.9 Evisceration seems to have first been performed unintentionally by James Beer in 1817 after an expulsive choroidal hemorrhage; in 1874 J.F. Noyes completed the first planned evisceration. There were several modifications of the procedure, later discarded, until Alfred Graefe and P.H. Mules’ more modern version in 1884.2
The first ocular prosthetics, of painted metal, were described in 1579, and glass prosthetics from Vienna were also starting to be made. Since then, many other materials and shapes have been tried, such as ivory, rubber, wool and cartilage. In recent history, the advent of inert plastics has given us the implants we use today. —AM and CB
It’s worth mentioning that pediatric anophthalmia can be even more difficult to manage, especially because the orbit of a child is not fully developed until after age 5. As the soft tissue and orbit can require expansion, these often challenging cases may require multiple surgeries and sometimes subspecialty management to avoid orbital and facial asymmetry.
The anophthalmic socket has a unique set of problems and requires a different clinical and surgical approach than a socket with a globe. The management of these patients should be carried out with close communication between the ophthalmologist and ocularist to achieve optimal comfort and cosmesis for patients. REVIEW
Dr. Murchison is the director of the Wills Eye Emergency Department and a member of the Oculoplastic and Orbital Surgery Service at Wills Eye Hospital. Dr. Bernardino is an oculoplastic surgeon at Vantage Eye Center in Salinas. The authors report no financial interest in any product discussed.
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