The Anophthalmic Syndrome

 Updated 3/2017-- photos and all links removed (except to my own posts) as many no longer active.

See my previous post. It is the reason I went looking for more information. Until now I didn't know about the anophthalmic syndrome. As you can see from the articles below, it doesn't seem to be an area that garners much interest these days. Most of the articles (that I had easy access to) are pre-1990. The intro from the 4th referenced article puts the issue in perspective:

The patient who has had the misfortune of losing an eye, be it secondary to disease or trauma, often has difficulty in getting the help he needs from the medical profession. Many ophthalmologists, being "eye surgeons," lose interest in a patient when the globe is gone. Many reconstructive surgeons hesitate to venture into this area which seems to be surrounded by a certain amount of mystique. Both may escape by referring the patient to an ocularist for continued care related to his appearance. The ocularist, who is not a medical doctor, often gains more practical knowledge of the problems of the anophthalmic state than does either the ophthalmologist or the reconstructive surgeon but, because he is unfamiliar with all possible reconstructive surgical techniques, he often fails to recommend reconstructive surgery when it is indicated. Lars M Vistnes, MD

Within this post, all the issues will be discussed with the stipulation that there is a normal bony orbit. Only the soft tissue elements will be discussed. You may wish to read this description of the enucleation procedure.

The anophthalmic syndrome consists of:

• enophthalmos (eye appears sunken)
• superior sulcus depression (upper eyelid crease is too deep compared to normal eye)
• lower lid ptosis
• upper lid ptosis

Enophthalmos

Deficits in the orbital volume contents and superior sulcus depression are recognized to be the cause of enophthalmos. Several caused have ben postulated (ref 1):

• levator disinsertion
• atrophy of orbital fat
• loss of volume when the globe is removed
• depression in the floor of the orbit due to an unrecognized fracture in the floor (rare)
• malposition in the superior rectus muscle

Replacement of lost volume can be done using a variety of materials -- autogenous bone, cartilage, dermis, glass beads, and silicone are some of the materials used. First, see what an experienced ocularist can do

Superior Sulcus Depression

As with, enophthalmos surgery should only be done if an experienced ocularist is unable to correct the problem. Correction of the two problems go hand-in-hand.

Lower Lid Ptosis

The pathomechanics is believed to be secondary to gravity acting with altered vectors of force on a prosthesis that is heavier than a normal eyeball. This appears to be independent of periorbital trauma.

 

Correction of this deformity may be best done by use of a fascial sling (ref 2). There are two key features that need to be remembered in correcting lower eyelid ptosis.

• The normal lower eyelid, with the eyeball in a horizontal gaze, has its upper border at the level of the lower margin of the limbus. The curvature of this border is not uniform. In its lateral third it assumes a more superior direction. To recreate this normal curvature the lower eyelid requires a higher positioning of the orbital rim burr hole than once thought.
• A second consideration is the normal motor function of the inferior rectus. Through its connection into the capsulopalpebral ligament, the lower eyelid upon downward gaze is simultaneously pulled in a inferior direction. A static sling suspended between the medial canthal ligament and the orbital rim restricts this motion. Therefore, a static sling is most applicable in the case of an anophthalmic orbit in which a static lower eyelid does not interfere with vision.

The fascia lata sling is described in the second reference article in great detail. I would like to relay the technical tips that Dr. Vistnes makes.

1. The optimal surgical correction must begin with an ideal prosthesis. Such a prosthesis is made to fit the socket and does not attempt to compensate for enophthalmos or lower or upper eyelid ptosis.

2. The width of the fascial strip is 2 mm. By pulling on a smaller area of the lower lid, directly below the lash margin, the lid can be positioned more precisely.

3. The use of the Wright's needle allows the fascia to be passed under the space anterior to the tarsal plate. The needle can be positioned immediately beneath the lash margin, and the fascial strip will seat itself in this track without displacing itself inferiorly on the tarsal plate. Low placement of the sling can result in eversion of the lash border ("tumbling" of the lid into ectropion).

4. Positioning the orbital burr hole at approximately 5-6 mm above the level of the lateral canthal tendon will not recreate the normal anatomy. The proper site can be chosen by following the curvature of the normal lower eyelid and marking the point where the curve intersects the orbital rim. An identical point on the anophthalmic orbital rim is then marked. This is the appropriated site for the orbital burr hole.

Upper Lid Ptosis
The cause will fall into one or more of three main categories:

• the trauma which necessitated the enucleation
• the surgeon (ie iatrogenic)
• the surgery (ie the creation of an anatomical or pathomechanical situation that produces malfunction of a delicately balanced mechanism)

There is a change in the size of the orbital support for the levator mechanism that occurs with enucleation. This, in most instances, is responsible for the upper lid ptosis. The "pivot" point of the levator muscle is lowered and more posterior than in a normal eye. Often this is corrected by experienced ocularist who add a superior sulcus "bridge" on the prosthesis thereby pushing the picot point of the muscle higher. (ref 4)

Vistnes feels that the upper lid ptosis is a function of several factors:

• the levator muscle tone and its adaptability
• the tightness or laxness of the check ligaments
• the size of the implants
• the size of the prosthesis

MANAGEMENT (according to Vistnes):

When the implant is small and the prosthesis is large, and the degree of ptosis is moderate to severe -- correction may be obtained by a traditional levator shortening (Berke method).

When the levator action is good over a prosthesis of average size and the ptosis is minimal, then a lid-shortening "ptosis correction" procedure may be used (Fasanella and Servat).

The order in which the various operations are done in patients with the anophthalmic orbit syndrome (per Lars Vistnes MD)

1) The volume deficit should be corrected first. In the cases of mild ptosis where an added mass (RTV silicone) is placed along the orbital floor, the pushing upward of the implant is often all that is required. This will also correct the enophthalmos and the superior sulcus depression.

2) If lower lid ptosis is present, it should be corrected next (as a separate procedure). This correction will tend to push the prosthesis upward and may also correct the upper lid ptosis.

3) Any ptosis of the upper lid should be corrected last -- and only after an experienced ocularist has been unable to correct it with a new prosthesis that is not out of proportion in appearance to the normal eye.

I realize I have just begun to learn about the anophthalmic patient needs. I have not actually cared for any either in training or since. I hope this post will be of use to others who may be in a position to care for these patients. So if I made any mistakes, major or minor, please let me know so that I can correct this post. Thank you.

Jarling Ocular Prosthetics, Inc --nice source of information on the actual prosthetics.
The Artificial Eye Clinic -- another good source of information on the actual prosthetics.
REFERENCES

1. Correction of Enophthalmos and Superior Sulcus Depression in the Anophthalmic Orbit: A Long-Term Follow-Up; Plastic & Reconstructive Surgery. 79(3):331-338, March 1987; Sergott, Thomas J. M.D.; Vistnes, Lars M. M.D.

2. Correction of Lower Eyelid Ptosis in the Anophthalmic Orbit: A Long-Term Follow-Up; Plastic & Reconstructive Surgery. 72(3):289-292, September 1983; Nolan, William B. III M.D.; Vistnes, Lars M. M.D.

3. Blepharoplasty in Patients with an Anophthalmic Orbit; Plastic & Reconstructive Surgery. 59(5):670-674, May 1977; Horton, Charles E. M.D.; Graham, John K. M.D.

4. Mechanism of Upper Lid Ptosis in the Anophthalmic Orbit; Plastic & Reconstructive Surgery. 58(5):539-545, November 1976; Vistnes, Lars M. M.D.

5. Correction of Enophthalmos in the Anophthalmic Orbit; Plastic & Reconstructive Surgery. 51(5):545-554, May 1973; Iverson, Ronald E. M.D.; Vistnes, Lars M. M.D.; Siegel, Richard J. M.D

6. Blepharoplasty, Upper Lid Ptosis Surgery; eMedicine Article, Jan 30, 2008; Jorge I de la Torre MD

7. Correction of Superior Sulcus Deformity and Enophthalmos with Porous High-density Polyethylene Sheet in anophthalmic Patients; Korean Journal of Ophthalmology, 19(3):168-173, 2005; Byeung-hun Choi, MD; Sang-hyeok Lee, MD; Wha-sun Chung, MD (PDF file)

8. Evaluation of the Anophthalmic Socket: It's Important to Understand the Management of Anophthalmic Patients and Recognize Complications; Review of Ophthalmology, Vol 13:09, Sept 5, 2006; Ann P Murchison MD and C Robert Bernardino MD