Evaluation for Cystoid Maculopathy after Pars Plicata Lensectomy­Vitrectomy for Congenital Cataracts STEVEN M. GILBARD, MD, GHOLAM A. PEYMAN, MD, MORTON F. GOLDBERG, MD

Abstract: Seventeen patients (25 eyes) who underwent a pars plicata len­sectomy and vitrectomy for congenital cataract were evaluated with fluorescein angiography and angioscopy for the presence of cystoid macular edema. Studies were done from 1 month to 7 years after surgery (mean interval, 22.5 months; median interval, 12 months). Except in one eye with questionable grade 1 cystoid macular edema, the authors did not find this complication in any of their patients. [Key words: congenital cataracts, cystoid macular edema, fluorescein angiography, pars plicata lensectomy.] Ophthalmology 90: 1201-1206, 1983

Cystoid macular edema is a common postoperative finding after cataract surgery in the adult population. Studies have indicated that it may occur in as many as 60 to 77% of cases after intracapsular surgery using flu­orescein angiography. 1,2 It has been suggested that planned extracapsular extraction may result in a lower incidence of cystoid macular edema, while intraocular lens im­plantation after intracapsular cataract extraction may in­crease its frequency. 3-5

A recent subject of interest and concern has been the risk of cystoid maculopathy after varied surgical proce­dures for congenital cataracts. Poer and colleagues re-

From the University of Illinois Eye and Ear Infirmary, Chicago, Illinois.

Presented in part before the joint meeting of the International Society for Genetic Eye Disease and the International Society of Pediatric Ophthal· mology, San Francisco, California, October 29, 1982.

Supported in part by Training Grant No. 7038 and Core Grant No. 1792 from the National Eye Institute, Bethesda, Maryland, and by an unrestricted grant from Research to Prevent Blindness, Inc., New York, New York.

Reprint requests to Steven M. Gilbard, MD, 1855 West Taylor Street, Chicago, IL 60612.

0161-6420/83/1000/1201/$1.10 © American Academy of Ophthalmology

ported a series of25 juvenile cataracts in which fluorescein angiography failed to verify cystoid macular edema in any of the operated eyes.6 In only three of these cases was a limballensectomy and anterior vitrectomy the initial surgical procedure. The remaining patients all underwent an initial discission and aspiration. In these patients the posterior capsule was left intact in 28%; otherwise, it was broken or removed.

Hoyt and Nickel evaluated 27 children who underwent surgery for bilateral infantile cataracts.7 Each infant had the lens in one eye removed by discission and aspiration and the other by limbal lensectomy and anterior vitrec­tomy. Ten of the 27 eyes undergoing lensectomy and vitrectomy developed aphakic cystoid macular edema; only one eye that underwent discission and aspiration developed macular edema. Six of the eyes in the first group had persistent macular edema, which affected visual acuity in four of these eyes.

We have been performing pars plicata lensectomy and vitrectomy for congenital cataracts for over 7 years with clinically acceptable results.8- lo We evaluated the inci­dence of cystoid macular edema in our cases after this procedure and compared it to those studies cited above.

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Fig 1. Performing lensectomy through the pars plicata.

SUBJECfS AND METHODS

All patients with congenital cataract who had under­gone pars plicata lensectomy since 1975 were contacted for evaluation. These included patients from both the clinic and private services of the University of Illinois

Fig 2. Pars plicata lensectomy-vitrectomy being completed with large core vitrectomy to retinal surface.

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Eye and Ear Infirmary who were operated on by either residents, fellows, or attending staff. The pars plicata len­sectomy and vitrectomy procedures performed on these patients were described previously by Peyman and co­workers.9 In brief, a sclerotomy for the vitrophage was made about 2.5 mm posterior to the limbus, and the entire lens was removed (Fig 1). Then, a primary vitrec­tomy was performed posteriorly to the surface of the fundus (Fig 2).

Evaluations consisted of visual acuity (when possible), slit-lamp examination, and ophthalmoscopy. Pupils were dilated with 2.5% phenylephrine hydrochloride (Neo-Sy­nephrine) and 1 % tropicamide. Fundus photographs were taken of both eyes in all patients when possible. One milliliter of a 10% sodium fluorescein solution per 14 kg of body weight was injected intravenously. When nec­essary, chloral hydrate was administered for sedation. Angiography was attempted in all cases; if this was not possible, angioscopy was performed.

All angiograms were read independently by two retinal specialists (GAP and MFG) in a masked fashion; neither knew the name of the patient nor the type of surgery, if any, that had been performed. The severity of the cystoid macular edema was graded with the system suggested by Miyake2

: grade 0, no leakage; grade I, trace of fluorescein leakage recognized; grade II, a typical stellate pattern of fluorescein almost established with its diameter equal to or less than 2.0 mm (4/3 optic disc diameter; and grade III, a dramatic stellate pattern of fluorescein with a di­ameter greater than 2.0 mm.

RESULTS

We attempted to contact for evaluation 34 patients with congenital cataract (48 eyes) who had undergone surgery since 1975. Of these, 17 patients (25 eyes) could be located and examined (Table 1). The group of patients consisted of seven girls and ten boys. The mean age at the time of surgery was 8.09 years, with a range of 1 month to 29 years (Fig 3). There was no known etiology for 16 congenital cataracts. One patient (no. 1) had bi­lateral juvenile familial cataracts. Two patients (nos. 11 and 14) (four eyes) had Marfan's syndrome and sublux­ated cataractous lenses. Patients 5, 7, and 16 had rubella, Down's syndrome, and Mittendorfs dot, respectively.

Postoperative intervals at the time evaluations were performed (Fig 4) ranged from 1 month to 7 years (mean interval, 22.5 months; median, 12 months). The clarity of media appeared equivalent to 6/6 (20/20) acuity in all eyes; The actual visual acuities (Fig 5) represent the most recent and best corrected postoperative determinations. Only five eyes had a visual acuity of 6/12 (20170) or worse. These poor acuities were seen in three patients; one had pendular nystagmus, one had amblyopia, and one had a postoperative retinal detachment that was suc­cessfully reattached.

Fluorescein evaluation was performed in all eyes except the one with the retinal detachment. Angiograms were

GILBARD, et aI • EVALUATION FOR CYSTOID MACULOPATHY

Table 1. Data on Patients with Pars Plicata Lensectomy and Vitrectomy

Postoperative Interval Visual Acuity before Angiographic/ (Clarity of Media) CME Grade

Patient Age at Angioscopic Evaluation Angiographic/ No. Eye Surgery Etiology (Mos.) Preoperative Postoperative Angioscopic' Comment

00 5 mo. Autosomal dominant 84 6/60 (20/200) 6/6 (20/20) 0 Nystagmus OS 5 mo. 6/30 (20/100) 6/6 (20/20) 0 Nystagmus

2 00 4 yr. Idiopathic 65 CF 2 Ft. 6/6 (20/20) 0 Monocular

3 OS 10 yr. Idiopathic 50 6/15 (20/50) 6/6 (20/20) 0

4 00 12 mo. Idiopathic 38 6/30 (20/100) 6/6 (20/20) 0 Nystagmus

5 00 1 mo. Rubella 28 6/6 (20/20) 6/6 (20/20) O'

6 00 15 yr. Idiopathic 40 6/12 (20/40) 6/6 (20/20) 0 OS 36 6/12 (20/40) 6/6 (20/20) 0

7 00 7 mo. Down's syndrome 36 6/6 (20/20) O' CME by history

8 OS 3 yr. Idiopathic 4 6/18 (20/60) 6/6 (20/20) 0 Microphthalmos

9 00 25 yr. Idiopathic 11 6/18 (20/20) 6/6 (20/20) O'

10 00 1 mo. Idiopathic 12 6/6 (20/20) O' OS 6/6 (20/20) O'

11 00 29 yr. Marfan's syndrome 1 6/9 (20/30) 6/6 (20/20) 0 OS 27 yr. 26 6/12 (20/40) 6/6 (20/20) 0

12 00 5 yr. Idiopathic 5 6/12 (20/40) 6/6 (20/20) 0 Nystagmus OS 7 6/15 (20/50) 6/6 (20/20) 0 Nystagmus

13 00 4 yr. Idiopathic 1 6/9 (20/30) 6/6 (20/20) 0 OS 10 6/9 (20/30) 6/6 (20/20) 0

14 00 16 yr. Marfan's syndrome 13 6/9 (20/30) 6/6 (20/20) 0 OS 18 yr. 1 6/9 (20/30) 6/6 (20/20) 0

15 00 10 mo. Idiopathic 2 6/6 (20/20) 0 OS 4 mo. 7 LP 6/6 (20/20) 0 Retinal detachment

16 OS 5 mo. Mittendorf's dot 6/6 (20/20) 1.0+

17 00 18 yr. Idiopathic 6/7.5 (20/25) 6/6 (20/20) 0

• Angioscopy instead of angiography was performed. + One masked observer noted grade 1; the second masked observed noted grade O. 00 = right eye; OS = left eye. CME = cystoid macular edema.

obtained in 19 eyes, and angioscopy was done in five eyes. In patient 16 one masked observer noted grade 1 cystoid macular edema; the other observer noted grade O. Although the patient (no. 7) with Down's syndrome had a history of cystoid macular edema, no leakage was noted at the time of this study. In total, 23 of 25 eyes failed to show evidence of cystoid macular edema by fluorescein angiography or angioscopy.

DISCUSSION

Despite the extensive experience in congenital cataract surgery, no one procedure has gained wide acceptance because of inherent problems associated with each. The simple discission technique has an unacceptable rate of postoperative complications including lens swelling, re­tained lens material, glaucoma, and retinal detach­ment. II - 13 Although better results have been reported with

linear extraction, this technique has been associated with complications such as delayed formation of the anterior chamber, anterior synechiae and updrawn pupil, occlu­sion of the pupil, membrane formation, vitreous loss, glaucoma, and retinal detachment. 14,15 Similarly, intra­capsular extraction has produced complications in a very high percentage of cases. 16 Irrigation and aspiration, as well as phacoemulsification, have greatly reduced the rate of complications. I? Although the incidence of postop­erative glaucoma and retinal detachment has been reduced significantly, secondary membrane formation and vitreous loss have continued to be major problems. 18-21

The use of the automated vitrectomy instrument has been added to the surgical treatment of congenital cat­aracts. Calhoun and Harley22 have described a surgical approach through the limbus, and Peyman and co-work­ers8 have used the pars plicata approach. Both techniques have virtually eliminated secondary membrane formation and pupillary block. Furthermore, elective removal of the

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OPHTHALMOLOGY • OCTOBER 1983 • VOLUME 90 • NUMBER 10

7

I/) 6 Q)

>-Q)

5

0

... 4 Q)

.D 3 Fig 3. Age of patients with E congenital cataract at time of ::J

lensectomy and vitrectomy. Z 2

0-0.5 0.6-1 2-4 5-10 11-20 21-30

Age in years at time of surgery

vitreous in neonatal and juvenile eyes has not resulted in detectable problems in ocular maturation. Sequential ultrasound and refraction studies, for example, have shown normal enlargement of the neonatal globe despite pars plicta lensectomy and total vitrectomy at very early ages, even in microphthalmic eyes caused by rubella in­fection. 23

Peyman and colleagues9,10 suggested previously that the pars plicata approach was superior to the limbal ap­proach because (1) it avoided direct manipulation of the iris, (2) it minimized contact with the corneal endothe­lium, and (3) it permitted easy retrieval oflens fragments in the vitreous. Despite the inherent weaknesses of any retrospective study, the data presented here appear to suggest yet another advantage of the pars plicata ap­proach-the prevention of cystoid macular edema. We recognize a deficiency of our study, in that 50% of our patients were lost to follow-up and that follow-up ranged from 1 month to 7 years. Nevertheless, of the 24 patients examined by fluorescein studies, only one patient showed

6 !II Q)

5 >-Q) - 4 0

.... 3 Q)

.c E 2 :l Z

1-3 4-6 7-12 13-30

evidence of cystoid macular edema. Moreover, this was an angiographic diagnosis and not a clinical problem.

Irvine was the first to recognize cystoid macular edema following rupture of the vitreous face and adherence of the vitreous to the wound.24 Nicholls stressed the occur­rence of macular edema after cataract extraction without the adherence of vitreous to the wound.25

,26 Gass and Norton described the characteristic fluorescein angiogram and slit-lamp biomicroscopic pattern of cystoid macular edema.27 Fluorescein angiography showed dilated peri­foveal and disc capillaries with leakage of protein-rich fluid into the retina and optic nerve head, Histopatho­logically, cystoid spaces were seen in the retina, especially in the outer plexiform layer.

The pathogenesis of cystoid macular edema remains uncertain, While inflammation appears to play a major role in its development, other factors are important.28

The frequency of cystoid macular edema increases in patients when complications occur during surgery or in the postoperative period. Retained cortical material, ad-

31-40 41-60 >60

Fig 4. Follow-up of congen­ital cataract cases after len­sectomy and vitrectomy,

Time of evaluation in months after surgery

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GILBARD, et al • EVALUATION FOR CYSTOID MACULOPATHY

Fig 5. Most recent and best corrected visual acuity of eyes having undergone pars pli­cata lensectomy and vitrec­tomy.

III Q)

>-Q)

-0

... Q)

.c E ::J

Z

8

7

6

5

4

3

2

20/20-20/30

20/40-20/60

20/70-20/200

C.F.­H.M.

N.L.P. not possible

Visual acuity of operated eyes

herence of vitreous to the wound, late rupture of the anterior hyaloid face, previous uveitis, and the implan­tation of intraocular lenses have been suggested as factors contributory to inflammation and the development of cystoid macular edema. Elderly patients and those with systemic vascular diseases such as diabetes and hyper­tension seem particularly susceptible.27,29-31 Recently, prostaglandin synthesis has been postulated to be im­portant in the development of cystoid macular edema.32-36

Stimulation or irritation by surgical trauma, for example, is believed to be associated with prostaglandin synthesis and release into the aqueous by the iris. Prostaglandins and other inflammatory mediators could diffuse through the vitreous, penetrate the internal limiting membrane of the retina, and induce inflammatory responses in the macular and disc capillaries, notably those of vasodilation and leakage.

It appears that pars plicata lensectomy is advantageous over limbal lensectomy in preventing cystoid maculo­pathy. Inflammation and prostaglandin release may pos­sibly be reduced following pars plicata lensectomy, be­cause iris manipUlation is lessened and the presence of residual cortex is reduced. Moreover, the pars plicata ap­proach avoids vitreous incarceration in a limbal wound, which is a known potentiator of cystoid macular edema. In this regard, Taylor noted a 23% incidence of vitreous incarceration after removal of congenital cataracts by a limbal lensectomy-vitrectomy approach.21 Finally, the pars plicata approach encompasses both an anterior and posterior vitrectomy; this is in contrast to just an anterior vitrectomy via limbal surgery. In a rabbit model that employed intravitreal injection of antigens, Zimmerman and Silverstein showed that the vitreous can heighten an inflammatory response by holding antigen in place as a depot with prolonged release.37 It is possible that the more complete vitrectomy asociated with the pars plicata ap­proach minimizes the depot of an antigen or the edema

inciting agent. Whatever the precise pathogenesis of cys­toid macular edema may be, the pars plicata approach for congenital cataract surgery appears to reduce this complication to an acceptably low level.

CONCLUSION

In conclusion, our study suggests that cystoid macular edema is not a common complication of pars plicata lensectomy and vitrectomy for congenital cataracts. The one retinal detachment in this series represents our only major complication to date. The avoidance of cystoid maculopathy, secondary membranes, and pupillary block argues strongly for pars plicata lensectomy and vitrectomy as the current procedure of choice in the treatment of congenital cataracts. The production of permanently clear optical media is another major advantage in the human eye during its "critical period" of visual maturation. Al­though the follow-up in our cases was reasonably long, we are pursuing additional longitudinal observations.

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OPHTHALMOLOGY • OCTOBER 1983 • VOLUME 90 • NUMBER 10

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