A. Edward Maumenee

Birdshot Retinochoroidopathy Associated with Hla-A29 Antigen and Immune Responsiveness to Retinal S-Antigen

Antigen HLA-A29 was present in 16 of 20 patients (80%) with birdshot retinochoroidopathy, but only in 31 of 418 controls (7.4%) (P less than .0001). The relative risk for this disease in persons possessing HLA-A29 antigen was 49.9, one of the highest reported. Of the 20 patients, 13 were also tested for evidence of an in vitro mitotic immune response to purified retinal S-antigen. Of these, 12 (92.3%) responded to the purified preparation, whereas one responded only to a crude retinal homogenate. The histopathologic and clinical findings in an eye from one of the responders to the S-antigen were similar to those in the disease induced in monkeys with the S-antigen. Our findings suggested that birdshot retinochoroidopathy has a genetic predisposition and that retinal autoimmunity, resulting from the S-antigen or other retinal antigens, plays a role in the manifestation of this disease.

Histologic Studies of the Vasculature of the Anterior Optic Nerve

Thirty-one normal human anterior optic nerves were studied in thin serial sections and their vessels stained by standard histologic stains and a new modification of the silver reticulin stain. The retrolaminar cribrosa was profusely supplied by centripetal and longitudinal vessels of pial origin; small branches of the central retinal artery were also seen. Longitudinal continuity of small vessels extended from the retrolaminar nerve to the retinal surface. This system freely anastomosed with three transverse systems; a significant anteriolar input from short posterior ciliary arteries in the sclera to the lamina cribrosa; smaller short posterior ciliary branches and occasional choroidal capillaries to the prelaminar portion; and branches derived from the central retinal artery in and around the disk. Vessels entering the prelaminar region at the level of the choroid were mainly derived from scleral branches of short posterior ciliary vessels entering through the border tissue of Elschnig, rather than from choroidal branches of ciliary arteries. Venous drainage for the anterior optic nerve was primarily through the central retinal vein, although alternate smaller paths to surrounding tissues were also identified.

Possible Mechanisms for the Loss of Goblet Cells in Mucin-deficient Disorders

By studying the pathological changes in human conjunctival flaps and the conjunctival transdifferentiation in rabbits, we have identified and correlated two pathological processes with the loss of goblet cells: (1) loss of vascularization, and (2) intense inflammation. Loss of vascularization may be correlated with the loss of goblet cells in the chronic cicatricial stage of various mucin-deficient disorders, whereas inflammation may be responsible for their absence in the acute inflammatory stage. The exact interrelationship between these two processes remains unknown. The loss of goblet cells appears to be an early sign of a spectrum of squamous metaplasia, an abnormality of epithelial differentiation. We therefore speculate that loss of vascularization and intense inflammation may have an important effect on epithelial differentiation.

Topical retinoid treatment for various dry-eye disorders

We evaluated the clinical efficacy of treating various dry-eye disorders using 0.01% and 0.1% (weight/weight) topical all-trans retinoic acid ointment. Twenty-two patients were selected and classified into four major groups: keratoconjunctivitis sicca (6 patients; 11 eyes), Stevens-Johnson syndrome (9 patients; 17 eyes), ocular pemphigoid or drug-induced pseudopemphigoid (3 patients; 6 eyes), and surgery or radiation-induced dry eye (4 patients; 4 eyes), based on the criterion that they remained symptomatic even under maximum tolerable conventional medical and/or surgical therapies. The results indicated that squamous metaplasia with mucin deficiency secondary to goblet cell loss and keratinization may be the basis for the development of clinical symptoms and morbidities, as these epithelial abnormalities were invariably present before treatment. After treatment, all patients demonstrated clinical improvements in symptoms, visual acuity, rose Bengal staining, or Schirmer test. Most importantly, this topical vitamin A treatment caused the reversal of squamous metaplasia as evidenced by impression cytology. Therefore, this treatment may represent the first nonsurgical attempt to treat these disorders by reversing diseased ocular surface epithelium.

The Efficacy and Safety of Combined Trabeculectomy Cataract Extraction, and Intraocular Lens Implantation

The safety and efficacy of combined trabeculectomy, extracapsular cataract extraction (ECCE), and posterior chamber intraocular lens (PC IOL) implantation were evaluated by retrospectively analyzing 108 consecutive operations. Postoperatively, 89% of eyes achieved 20/40 or better visual acuity when preoperative macular and optic nerve diseases were excluded. Mean follow-up was 16.8 months. Intraocular pressure (IOP) control (less than or equal to 21 mmHg) was achieved in 92% of eyes; 57% required no medications. Capsulotomy (20%) and transient hyphema (15%) occurred significantly more often (P less than 0.001) than in a comparison group. These results suggest that the combined procedure gives excellent visual rehabilitation and IOP control in the majority of patients included in this analysis.

Retinal Detachment after Extracapsular Cataract Extraction with Posterior Chamber Intraocular Lens

The authors reviewed 3065 consecutive cases of extracapsular cataract extraction with posterior chamber lens implant (ECCE-PC IOL) and found the incidence of retinal detachment to be 1.4% overall (44/3065) and 1.7% in a group of eyes followed for at least 1 year (40/2330). Retrospective analysis of the retinal detachment (RD) group (n = 44) showed the patients to be significantly younger than the overall group (n = 3065) (P less than 0.0001). Comparison with an age-matched group of 302 eyes without RD showed higher rates of RD in males (P = 0.0013) and in eyes with axial eye length (AEL) greater than 25 mm (P less than 0.0001). No significant correlation was found between RD and PC IOL manufacturer, phacoemulsification (PKE), or primary discission. Sixty-four percent of RDs occurred within 1 year of cataract operation. Only 10% occurred more than 2 years later. Visual results in eyes with ultimate anatomic success of RD repair (43 of 44 eyes, averaging 15 months follow-up) are substantially better than previously reported in series with other types of IOLs (96% greater than or equal to 20/40 if the macula was not involved, 75% greater than or equal to 20/40 if the macula was involved).

Cystoid Macular Edema in Pseudophakia

Cystoid macular edema after cataract surgery, with or without intraocular lens implantation, has been reported to develop in more than 50% of patients as detected by fluorescein angiography. It is associated with reduced visual acuity in up to 8% of cases. Analysis of ongoing clinical trials at the Wilmer Institute indicates that clinically significant cystoid macular edema develops in a lower percentage of cases (2% total incidence and 0.3% persistent cystoid macular edema) if the intraocular lens implantation was uncomplicated. The factors associated with cystoid macular edema and the importance of considering clinically significant rather than just fluorescein-proven cystoid macular edema are discussed. Results of intraocular lens studies reported to the Federal Drug Administration are updated, and results of an ongoing study of prostaglandin inhibitors at the Wilmer Institute are reported.

Acute Posterior Multifocal Placoid Pigment Epitheliopathy

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare inflammatory eye disease affecting the outer retina, retinal pigment epithelium (RPE), and choriocapillaris in mostly otherwise healthy adults between the ages of 20 and 50 years. It shows no clear gender preference. APMPPE was first described by Gass in 1968 in a series of three young female patients who presented with multiple large plaque-like lesions at the level of the RPE associated with temporary bilateral visual loss (Gass 1968). APMPPE is considered one of the “placoid diseases” together with serpiginous choroiditis (SC), relentless placoid chorioretinitis, and persistent placoid maculopathy due to the “placoid” nature of the lesions and similarities on fluorescein angiography (FA) and indocyanine green angiography (ICGA) findings (Mirza and Jampol 2012).

Quantitative Corneal Endothelial Evaluation in Intraocular Lens Implantation and Cataract Surgery

We analyzed pre- and postoperative corneal endothelial photographs of 33 intraocular lens patients and found an average endothelial cell density loss of 14%. Twenty-six cataract patients, similarly examined, showed a 13% mean endothelial cell loss. In 14 of the intraocular lens patients, a second postoperative evaluation of the central endothelium, at a later date, revealed a trend toward continuing cell loss. Our results indicate fewer endothelial cells were lost after intraocular lens implantation than has been previously reported. This may be accounted for by our considering only patients with normal corneas for lens implantation, and by our constant maintenance of the anterior chamber with an air bubble during lens implant surgery.

Surgical Management of Epithelial Ingrowth

In ten consecutive cases of epithelial ingrowth operated on during the past 33 months, therapy included photocoagulation of the iris followed by excision of involved iris tissue and vitreous gel by means of instruments designed for vitreous surgery. Epithelium remaining on the posterior surface of the cornea, ciliary body, and in the anterior chamber was destroyed by controlled transcorneal and transscleral cryotherapy. An intraocular air bubble was used to provide an insulating effect and a more effective, controllable freeze. All patients except two had improved vision postoperatively, and four of the ten patients had postoperative visual acuity of 6/12 (20/40) or better.