Medical Therapy
No pharmacologic agents have proven to be beneficial in the treatment of blepharochalasis. The ability of antihistamines, steroids, sympathomimetics, mast-cell stabilizers, and cool compresses to modify the symptoms of acute attacks has not been determined. [6]
Angioedema relief has been reported in a small case series using oral acetazolamide in conjunction with topical hydrocortisone. [22, 6]
Oral doxycycline has also been suggested as an inhibitor of matrix metalloproteinase activity, which may be increased in those affected by blepharochalasis. [12, 6]
Surgical Therapy
Surgery is performed in patients after at least 6 months of disease inactivity to correct anatomical defects resulting from previous bouts of swelling. [19]
Redundant upper eyelid skin is corrected with the usual blepharoplasty techniques, which may include reforming the eyelid crease with sutures. Excision or sculpting of prolapsed orbital fat may be considered but often in a conservative fashion. A prolapsed lacrimal gland, when present, may also be resuspended. [21]
Upper blepharoptosis is preferably corrected through repair of levator dehiscence or levator advancement. A Fasanella-Servat procedure has been described to repair blepharoptosis associated with this syndrome, but the technique does not precisely address the anatomical defect in question. [23]
Lateral canthoplasty is often effective in repairing blepharophimosis because of attenuation of the lateral canthal support structures. Repair may be accomplished with the attachment of a lateral tarsal tongue to the periosteum on the internal aspect of the lateral orbital rim with nonabsorbable sutures. A periosteal flap or lateral orbital rim drill holes may also be used. [21]
Fat atrophy and a significant superior sulcus defect may be addressed with autologous grafting techniques, including orbital fat repositioning, dermis fat grafting, fat pearl grafts, and aspirated fat transfer. Consider injectable synthetic fillers, such as hyaluronic acid or calcium hydroxylapatite. [24]
Complications
After correction of the abnormalities present in blepharochalasis, pronounced or prolonged edema may complicate the postsurgical course. The edema may be a manifestation of the underlying disease process responsible for blepharochalasis syndrome. Surgical interventions may be of limited benefit in treating recurrent postsurgical episodes of disease, and this may make postsurgical expectations less predictable. [2]
Overcorrection with levator resection surgeries has been reported to be associated with complications and so intentional undercorrection have been advocated. [19, 25]
Outcome and Prognosis
As individuals age, the frequency of attacks typically decrease; however, there is insufficient data regarding changes to the frequency of attacks following surgical correction. [6] Because surgical correction does not treat the underlying pathophysiology of the disease, post-surgical complications may recur and include blepharoptosis and fat prolapse. Reports of post-surgical attacks range from as early as a month to 6 years after surgery. [19] In order to minimize and/or delay these post-surgical recurrences, it is recommended that surgeons defer surgical correction until remission has been maintained for at least 6 months to 1 year. [6]
Future and Controversies
Blepharochalasis may have an orbital component. This has been implied in patients who exhibit proptosis with the disease, and histologic findings in orbital fat biopsies further suggest orbital involvement. Future studies may elucidate this point.
As immunologic and genetic methodologies continue to improve, the etiology of blepharochalasis syndrome will likely be further elucidated. Effective treatments of this potentially disfiguring syndrome will hopefully follow. [16]
Prevention
Future interventions will likely be towards prevention of blepharochalasis in order to avoid the need for surgical correction and therefore avoid post-surgical complications. [21]
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Bilateral lacrimal gland prolapse in the quiescent stage of blepharochalasis syndrome. Courtesy of Kathleen Duerksen, MD.
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Upper and lower eyelid edema in blepharochalasis syndrome. Notice the left pseudoepicanthal fold. Courtesy of Kathleen Duerksen, MD.
