- Email: [email protected]
The Management of Retinal Detachments Associated With Choroidal Colobomas by Vitreous Surgery
The Management of Retinal Detachments Associated With Choroidal Colobomas by Vitreous Surgery
Anne Hanneken, M.D., Eugene de Juan, Jr., M . D . , and Brooks W. McCuen Π, M . D .
We u s e d v i t r e o u s s u r g e r y t o t r e a t s e v e n patients (eight eyes) with complicated retinal detachments associated with choroidal colo bomas. All eyes had large choroidal colobo m a s and no e v i d e n c e of p e r i p h e r a l retinal breaks. Small, atrophic breaks were detected in five of t h e e y e s a n d w e r e l o c a t e d i n t h e b a s e of t h e c o l o b o m a in f o u r o f t h e five e y e s . A d junctive surgical techniques were necessary and i n c l u d e d c y a n o a c r y l a t e r e t i n o p e x y in four eyes, s i l i c o n e oil t a m p o n a d e i n five e y e s , a n d r e t i n e c t o m y in t w o e y e s . R e t i n a l r e a t t a c h m e n t w a s u l t i m a t e l y a t t a i n e d i n s e v e n of t h e e i g h t eyes. T h e n u m b e r of s u r g i c a l p r o c e d u r e s r a n g e d f r o m o n e t o five, w i t h a n a v e r a g e o f three. Postoperative visual acuity of the eyes that underwent anatomically successful pro cedures ranged from 2 0 / 1 0 0 to light p e r c e p tion. Proliferative v i t r e o r e t i n o p a t h y was the m o s t f r e q u e n t c a u s e of r e d e t a c h m e n t , o c c u r r i n g in six o f t h e e i g h t e y e s .
A CHOROIDAL COLOBOMA IS 3 Congenital l e s i o n c h a r a c t e r i z e d b y t h e a b s e n c e of n o r m a l r e t i n a , retinal pigment epithelium, and choroid. This d e v e l o p m e n t a l a n o m a l y is c o m m o n l y l o c a t e d in the i n f e r o n a s a l r e g i o n o f t h e f u n d u s a n d is c a u s e d b y t h e failure o f c l o s u r e of t h e fetal fissure. It is often a s s o c i a t e d w i t h s e v e r a l o t h e r o c u l a r a b n o r m a l i t i e s , i n c l u d i n g iris c o l o b o m a , loss of inferior lens zonules, cataract, and o p t i c disk a b n o r m a l i t i e s . T h e p r e v a l e n c e o f r e t i n a l
Accepted for publicatior\ Nov. 26, 1990. From the Whittier Institute for Diabetes and Endocri nology, La Jolla, California (Dr. Hanneken); and Depart ment of Ophthalmology, Duke University Eye Center, Durham, North Carolina (Drs. de Juan and McCuen). This study was supported in part by the Adler Founda tion, New York, New York (Dr. McCuen). Dr. Hanneken was a 1988-1990 Heed Ophthalmic Fellow. Reprint requests to Brooks W. McCuen, M.D., Depart ment of Ophthalmology, Duke University Medical Cen ter, Box 3802, Durham, NC 27710.
d e t a c h m e n t in e y e s w i t h c h o r o i d a l c o l o b o m a s h a s b e e n r e p o r t e d to b e from 2 3 % to 4 2 % . ' · The use of scleral buckling t e c h n i q u e s to treat r e t i n a l d e t a c h m e n t s in e y e s w i t h c h o r o i d a l c o l o b o m a s h a s r e s u l t e d in l o w r a t e s o f a n a t o m i c success, varying b e t w e e n 3 5 % and 55%.'·' Vit r e o u s s u r g e r y h a s b e e n a d v o c a t e d as a n a l t e r n a t i v e to s c l e r a l b u c k l i n g s u r g e r y in t h e t r e a t m e n t of s e l e c t e d r e t i n a l d e t a c h m e n t s in e y e s w i t h large chorioretinal c o l o b o m a s , but there are little d a t a a v a i l a b l e on its efficacy.^·^ We u s e d v i t r e o u s s u r g e r y to t r e a t s e v e n p a t i e n t s ( e i g h t eyes) with retinal d e t a c h m e n t associated with choroidal coloboma.
Patients and IVIethods We r e v i e w e d t h e r e c o r d s o f s e v e n c o n s e c u t i v e patients (eight eyes) with retinal detachments and posterior colobomas treated by vitrectomy b e t w e e n 1 9 8 4 a n d 1 9 9 0 . T h e r e w e r e five m a l e s a n d t w o f e m a l e s , r a n g i n g in a g e f r o m 7 to 4 2 years. Six of the patients had bilateral retinal d e t a c h m e n t s . O f these six patients, only one u n d e r w e n t v i t r e o u s s u r g e r y in b o t h e y e s . T h e r e m a i n i n g five p a t i e n t s h a d d e v e l o p e d p h t h i s i s or w e r e n o t c a n d i d a t e s for s u r g e r y in t h e i r fellow eye. A m i n i m u m of six m o n t h s of followu p data w e r e a v a i l a b l e in all p a t i e n t s . T h e m e a n follow-up period was 2 6 m o n t h s . All p a t i e n t s h a d l a r g e p o s t e r i o r o c u l a r c o l o b o m a s a s s o c i a t e d w i t h iris c o l o b o m a s , i n f e r i o r lens opacities, absence of inferior lens zonules, and absence of retinal pigment epithelium and choroid. Patients with peripheral retinal breaks were treated with conventional scleral buckling s u r g e r y a n d w e r e n o t i n c l u d e d in t h i s s e r i e s . O n l y e y e s w i t h p o s t e r i o r h o l e s or e y e s w i t h a rhegmatogenous-appearing retinal detachment in w h i c h n o h o l e s c o u l d b e f o u n d w e r e c o n s i d e r e d c a n d i d a t e s for v i t r e c t o m y . P a t i e n t s w i t h retinal detachments associated with the morn ing glory syndrome, isolated optic nerve head c o l o b o m a , or o p t i c n e r v e pit w e r e e l i m i n a t e d
©AMERICAN JOURNAL OF OPHTHALMOLOGY 111:271-275, MARCH,
1991
271
272
AMERICAN JOURNAL OF OPHTHALMOLOGY
from this s t u d y . Two p a t i e n t s h a d m o d e r a t e l y severe learning disabilities, one patient had a cleft lip a n d p a l a t e , a n d o n e p a t i e n t h a d a family h i s t o r y o f o c u l a r c o l o b o m a s . We u s e d a s t a n d a r d t h r e e - p o r t v i t r e c t o m y procedure. Most patients received general an esthesia. Retrobulbar injections were generally a v o i d e d b e c a u s e o f t h e risk o f u n i n t e n t i o n a l l y penetrating ectatic sclera. L e n s e c t o m i e s w e r e p e r f o r m e d in all e y e s using the phacofragmentation unit and a s e c o n dary infusion line. After l e n s e c t o m y , c a n n u l a s w e r e p l a c e d in t h e s u p e r i o r q u a d r a n t s , t h e c o n tact l e n s ring was s u t u r e d in p l a c e , a n d t h e a x i a l vitrectomy was performed. T h e s u r g i c a l g o a l s w e r e to r e l e a s e v i t r e o r e t i n a l t r a c t i o n a n d to i d e n t i f y a n d s e a l t h e r e t i n a l breaks. Achieving these goals was challenging in t h i s g r o u p o f p a t i e n t s . T h e i n i t i a l a p p r o a c h i n v o l v e d s t r i p p i n g t h e c o r t i c a l v i t r e o u s from t h e r e t i n a up to t h e p o s t e r i o r b o r d e r o f t h e v i t r e o u s b a s e . T h i s w a s often difficult b e c a u s e of the a b s e n c e o f a c o m p l e t e posterior vitreous detachment and the strong adhesion of the v i t r e o u s over r e g i o n s o f d e t a c h e d r e t i n a . We attributed the unusually strong vitreoretinal i n t e r f a c e to t h e y o u n g a g e o f m a n y o f t h e patients. When peripheral vitreous could not be r e l e a s e d a d e q u a t e l y or p e r i p h e r a l r e t i n a l h o l e s were created, peripheral encircling buckles were placed. The second surgical objective consisted of identifying the posterior retinal breaks. The r e t i n a l b r e a k s w e r e u s u a l l y s m a l l n a r r o w s l i t s in regions of thin rudimentary retina within the c o l o b o m a t o u s c r a t e r . T h e y w e r e often l o c a t e d in a r e a s b e n e a t h a l o c a l i z e d p o s t e r i o r v i t r e o u s d e t a c h m e n t . A useful t e c h n i q u e to i d e n t i f y t h e l o c a t i o n o f t h e b r e a k s w a s to a s p i r a t e o v e r r e g i o n s o f r u d i m e n t a r y r e t i n a a n d o b s e r v e for the p r e s e n c e o f s c h l i e r e n ( s u b t l e w a v y l i n e s t h a t d e v e l o p w h e n c l e a r fluids o f different v i s c o s i ties m i x ) . T h e third s u r g i c a l o b j e c t i v e w a s to c l o s e a n d s e a l t h e r e t i n a l b r e a k s . T h e a t r o p h i c b r e a k s in t h e b a s e o f t h e c o l o b o m a w e r e often t r e a t e d with cyanoacrylate tissue adhesive. This p r o c e d u r e was p e r f o r m e d after a c o m p l e t e fluid-gas e x c h a n g e . For t h e a d h e s i v e to s o l i d i f y w e l l , it was n e c e s s a r y to r e m o v e all s u b r e t i n a l fluid in the b a s e o f t h e c o l o b o m a . T h i s w a s difficult in c a s e s in w h i c h t h e r e w a s a d e e p p o c k e t o f t r a p p e d s u b r e t i n a l fluid in t h e c o l o b o m a t o u s crater. A variety of other procedures were consid e r e d helpful a d j u n c t s . L o n g - a c t i n g t a m p o n a d e s
March, 1991
w i t h s i l i c o n e oil, sulfur h e x a f l u o r i d e , or perflu oropropane were always used. Inferior iridecto m i e s w e r e n o t n e c e s s a r y w h e n s i l i c o n e oil w a s u s e d b e c a u s e t h e p a t i e n t s h a d i n f e r i o r iris c o l o b o m a s . In all p a t i e n t s , a firm c h o r i o r e t i n a l a d h e s i o n w a s c r e a t e d at t h e e d g e o f t h e c o l o b o m a b y e i t h e r e n d o c r y o t h e r a p y or e n d o l a s e r p h o t o c o a g u l a t i o n . Two r o w s o f t r e a t m e n t w e r e p l a c e d along a s e g m e n t overlapping the edge of the c o l o b o m a a n d t h e n o r m a l r e t i n a a n d c h o r o i d in a n a t t e m p t to i s o l a t e t h e r u d i m e n t a r y r e t i n a in t h e c o l o b o m a from t h e r e m a i n i n g p e r i p h e r a l retina.
Results T h e r e s u l t s a r e s u m m a r i z e d in t h e T a b l e . T h e r e t i n a w a s s u c c e s s f u l l y r e a t t a c h e d in s e v e n o f the eight eyes (Figs. 1 and 2 ) . T h e total n u m b e r o f s u r g i c a l p r o c e d u r e s n e c e s s a r y to a c h i e v e r e t i n a l r e a t t a c h m e n t r a n g e d from o n e to five, with an average of three. Follow-up was avail a b l e for all p a t i e n t s a n d r a n g e d from six m o n t h s to s e v e n y e a r s . P o s t o p e r a t i v e v i s u a l a c u i t y in t h e s u c c e s s f u l l y r e a t t a c h e d e y e s v a r i e d from 2 0 / 1 0 0 to l i g h t p e r c e p t i o n . Retinal breaks were identified either preoper a t i v e l y o r i n t r a o p e r a t i v e l y i n five o f t h e e i g h t e y e s . F o u r o f t h e five b r e a k s w e r e f o u n d w i t h i n t h e c o l o b o m a t o u s c r a t e r , in t h i n r u d i m e n t a r y
TABLE DATA OF EIGHT EYES WITH RETINAL DETACHMENTS ASSOCIATED WITH CHOROIDAL COLOBOMAS
CASE NO.. AGE (YHS)
SURGICAL ADJUNCTS
1, 42 2, 35 3,24
Silicone Glue Silicone, retinectomy Glue Silicone Glue Silicone Glue, silicone, retinectomy
4. 35 5, 7 6, 7 7,25 8, 11
NO. OF PROCE VISUAL FOLLOW-UP DURES RESULTS ACUITY* (MOS)
4 1 3
Attached 20/400 Attached CF Attached 20/100
14 11 8
2 4 2 4 5
Attached Attached Detached Attached Attached
14 84 52 21 6
20/200 20/200 LP LP 20/400
•CF indicates counting fingers and LP indicates light percep tion.
Vol. I l l , No. 3
Retinal Detachments and Choroidal Colobomas
273
F i g . 1 (Hanneken, de Juan, and McCuen). Case 3 . Retinal detachment associated with choroidal coloboma. Left, Fundus of the left eye demonstrating a recurrent retinal detachment with subretinal membranes after initial vitrectomy and fluid-gas exchange. Right, Fundus of the same eye after revision of vitrectomy, retinectomy, removal of subretinal membranes, endocryotherapy, and silicone oil tamponade.
r e t i n a . M o s t p a t i e n t s h a d o n l y o n e b r e a k in t h e coloboma, although one patient had two. Peripheral encircling scleral buckles were p r e s e n t in five o f t h e e i g h t e y e s at t h e final f o l l o w - u p e x a m i n a t i o n . Two o f t h e p a t i e n t s were initially seen with peripheral b u c k l e s al r e a d y in p l a c e , a n d t h r e e p a t i e n t s r e c e i v e d s c l e r a l b u c k l e s d u r i n g o u r s u r g e r y to s u p p o r t the posterior vitreous b a s e . C y a n o a c r y l a t e r e t i n o p e x y w a s u s e d to c l o s e a n d s e a l t h e p o s t e r i o r b r e a k s in four o f t h e five e y e s in w h i c h h o l e s w e r e f o u n d . All o f t h e h o l e s t r e a t e d w i t h g l u e w e r e c l o s e d s u c c e s s f u l l y . In o n e o f the p a t i e n t s , h o w e v e r , t h e glue d i d n o t adhere adequately to the retina and was partial ly r e m o v e d ( C a s e 4 ) . T h i s p r o b l e m w a s c a u s e d by r e s i d u a l s u b r e t i n a l fluid in t h e b a s e o f t h e c o l o b o m a that p r e v e n t e d a t i g h t a d h e s i o n b e tween the retina and the glue. S i l i c o n e oil w a s u s e d in five e y e s to a c h i e v e a l o n g - t e r m t a m p o n a d e . In four of t h e five e y e s , the s i l i c o n e oil w a s r e m o v e d after s e v e r a l m o n t h s . In o n e o f t h e s e p a t i e n t s , h o w e v e r , t h e e y e r e d e t a c h e d after s i l i c o n e oil r e m o v a l , a n d the s i l i c o n e oil h a d to b e r e p l a c e d ( C a s e 8 ) . The most c o m m o n postoperative complica tion was the development of proliferative vitre oretinopathy. Six of the eight eyes developed p r o l i f e r a t i v e v i t r e o r e t i n o p a t h y , r a n g i n g from f u l l - t h i c k n e s s fixed r e t i n a l f o l d s in two q u a d r a n t s to fixed r e t i n a l folds o f c l o s e d f u n n e l s h a p e in four q u a d r a n t s . Two e y e s d e v e l o p e d
significant tractional subretinal m e m b r a n e s , both of which required removal before the r e t i n a c o u l d b e r e a t t a c h e d . S e v e r e a n t e r i o r vit r e o u s b a s e c o n t r a c t i o n o c c u r r e d in a n o t h e r p a t i e n t a n d l e d to t h e u l t i m a t e f a i l u r e o f r e a t t a c h ment surgery (Case 6 ) . A s e v e r e fibrin p u p i l l a r y m e m b r a n e o c c u r r e d p o s t o p e r a t i v e l y in o n e p a t i e n t ( C a s e 7). T h i s m e m b r a n e did n o t r e s o r b w i t h c o r t i c o s t e r o i d treatment and required surgical removal.
Discussion In t h e a b s e n c e o f p e r i p h e r a l r e t i n a l b r e a k s , v i t r e o u s s u r g e r y is t h e t r e a t m e n t o f c h o i c e i n managing retinal d e t a c h m e n t associated with choroidal colobomas. Previously, scleral buck ling t e c h n i q u e s alone have b e e n used, but the rates of anatomic r e a t t a c h m e n t have been l o w . ^ ' In 1 9 8 3 , G o n v e r s " r e p o r t e d t h e s u c c e s s ful u s e o f t h e s i l i c o n e oil t a m p o n a d e to t r e a t o n e eye w i t h a r e t i n a l d e t a c h m e n t a s s o c i a t e d with a choroidal c o l o b o m a . Since then, addi tional reports of retinal detachment caused by chorioretinal colobomas and treated by vitre ous surgery have b e e n few.' T h e r e l a t i v e l y p o o r s u r g i c a l r e s u l t s in t h e s e c a s e s a r e c a u s e d b y s e v e r a l k e y a n a t o m i c fea t u r e s t h a t m a k e t h e s e e y e s u n i q u e l y different from o t h e r e y e s w i t h r e t i n a l d e t a c h m e n t . O n e
274
AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 2 (Hanneken, de Juan, and McCuen). Case 8 . B-scan ultrasound image demonstrating a retinal detachment associated with a large posterior colobo ma (horizontal orientation).
of t h e s e f e a t u r e s is t h e p r e s e n c e o f s m a l l slits h a p e d r e t i n a l b r e a k s in t h e b a s e o f t h e c o l o b o m a . T h e r e t i n a l b r e a k s in t h e s e e y e s a r e often difficult to i d e n t i f y b o t h p r e o p e r a t i v e l y a n d intraoperatively. The retina overlying the colo b o m a is e x t r e m e l y a t r o p h i c , t h i n , a n d t r a n s p a r e n t . T h e a b s e n c e of n o r m a l r e t i n a l p i g m e n t e p i t h e l i u m a n d c h o r o i d r e d u c e s the c o n t r a s t a n d c o n t r i b u t e s to t h e p r o b l e m o f v i s u a l i z i n g the b r e a k . T h e b e s t i n t r a o p e r a t i v e t e c h n i q u e for i d e n t i f y i n g t h e s e b r e a k s w i t h i n t h e b a s e o f the c o l o b o m a is to a s p i r a t e fluid a b o v e t h e c o l o b o m a a n d to l o o k for s c h l i e r e n as s u b r e t i n a l fluid l e a k s t h r o u g h the r e t i n a l b r e a k . W i t h t h i s t e c h n i q u e , we w e r e a b l e to i d e n t i f y or c o n f i r m m o s t o f t h e b r e a k s in o u r s e r i e s . H o w e v e r , we w e r e still u n a b l e to i d e n t i f y p r i m a r y r e t i n a l b r e a k s in t h r e e o f our p a t i e n t s . The absence of a posterior vitreous detach m e n t is a n o t h e r c o m p l i c a t i n g feature in t h e s e eyes. Although the typical r h e g m a t o g e n o u s de tachment contains a nearly complete posterior vitreous detachment, our cases demonstrated virtually no separation between the cortical v i t r e o u s a n d the r e t i n a . T h e o n l y a r e a w h e r e t h e v i t r e o u s w a s s e p a r a t e d from t h e r e t i n a w a s over t h e c o l o b o m a , w h e r e a s h a l l o w , p a r t i a l , posterior vitreous separation was frequently p r e s e n t . T h i s s e p a r a t i o n w a s focal, h o w e v e r , a n d firm v i t r e o r e t i n a l a d h e s i o n w a s p r e s e n t over t h e r e m a i n d e r o f t h e c o l o b o m a a n d t h e more peripheral retina. The absence of a posterior vitreous detach m e n t c a u s e s c o n s i d e r a b l e t e c h n i c a l difficulty d u r i n g s u r g e r y . T h i s difficulty o c c u r s w h e n at t e m p t i n g to strip firmly a t t a c h e d c o r t i c a l v i t r e o u s from d e t a c h e d r e t i n a . W e c o n s i d e r it i m p o r tant to s e p a r a t e the p o s t e r i o r v i t r e o u s from t h e r e t i n a to r e d u c e the p o s s i b i l i t y o f p o s t o p e r a t i v e
March, 1991
v i t r e o u s c o n t r a c t i o n l e a d i n g to r e c u r r e n t r e t i nal detachment. A n i m p o r t a n t c o n s i d e r a t i o n in t h e t r e a t m e n t o f t h i s g r o u p o f p a t i e n t s is t h e c h o i c e a n d u s e o f c h o r i o r e t i n a l a d h e s i v e m o d a l i t i e s to t r e a t r e t i n a l b r e a k s . T r a d i t i o n a l l y , b r e a k s are t r e a t e d with thermal irritation of the retinal pigment e p i t h e l i u m a n d r e t i n a to c r e a t e c h o r i o r e t i n a l scarring and adhesion. Within the c o l o b o m a , h o w e v e r , t h e r e t i n a l p i g m e n t e p i t h e l i u m is a b normal and frequently absent. Laser p h o t o c o agulation, cryotherapy, and diathermy do not r e s u l t in g o o d c h o r i o r e t i n a l a d h e s i o n s w i t h i n this r e g i o n . O t h e r a l t e r n a t i v e s a r e n e c e s s a r y . C y a n o a c r y l a t e retinopexy*'^ is a useful t e c h n i q u e for c l o s i n g a t r o p h i c b r e a k s in t h e b a s e o f t h e c o l o b o m a . For t h e a d h e s i v e t o s o l i d i f y , t h e g l u e must b e a p p l i e d after all r e s i d u a l fluid h a s b e e n r e m o v e d from t h e v i t r e o u s c a v i t y b y fluidgas exchange and internal drainage of subreti n a l fluid. P o o r c h o r i o r e t i n a l a d h e s i o n w a s e n c o u n t e r e d in o n e p a t i e n t ( C a s e 4 ) in w h o m t h e rudimentary retina within the coloboma could n o t b e c o m p l e t e l y flattened a g a i n s t t h e s c l e r a . S i l i c o n e oil w a s u s e d in five o f t h e e i g h t e y e s to a c h i e v e a l o n g - t e r m t a m p o n a d e . It w a s u l t i m a t e l y r e m o v e d in four o f t h e five e y e s . B e c a u s e one of these eyes developed a redetachment after t h e r e m o v a l o f s i l i c o n e o i l , h o w e v e r , w e are n o w m o r e c a u t i o u s w h e n c o n s i d e r i n g w h e t h e r to r e m o v e it. E n d o l a s e r r e t i n o p e x y o r e n d o c r y o t h e r a p y is a p p l i e d a l o n g t h e m a r g i n o f t h e c o l o b o m a to c r e a t e a b o r d e r o f c h o r i o r e t i n a l a d h e s i o n a n d to s e p a r a t e n o r m a l r e t i n a from a b n o r m a l r e t i n a . T h i s t r e a t m e n t w a s u s e d to l i m i t t h e e x t e n s i o n o f p o t e n t i a l r e c u r r e n t s u b r e t i n a l fluid in t h e b a s e of t h e c o l o b o m a . The most c o m m o n cause of retinal redetach m e n t in t h e s e c a s e s w a s p r o l i f e r a t i v e v i t r e o r e t i n o p a t h y . S e v e n o f t h e e i g h t e y e s in o u r s e r i e s r e d e t a c h e d , a n d t h e d e t a c h m e n t in all but o n e o f t h e s e e y e s w a s c a u s e d b y t r a n s v i t r e a l , cir c u m f e r e n t i a l , or s u b r e t i n a l t r a c t i o n a l m e m branes. Severe anterior vitreous base contrac t i o n a c c o u n t e d for t h e u l t i m a t e failure in o n e p a t i e n t ( C a s e 6 ) . T h e r e a s o n for t h e h i g h p r e v a l e n c e o f p r o l i f e r a t i v e v i t r e o r e t i n o p a t h y is n o t c l e a r , b u t it m a y b e r e l a t e d to t h e r e l a t i v e l y y o u n g a g e o f t h e p a t i e n t s , t h e c h r o n i c i t y of t h e d e t a c h m e n t s , or t h e e x t e n t o f l a s e r or c r y o therapy. Although multiple operations may be neces s a r y , v i t r e o u s s u r g e r y p r o v i d e s an effective treatment of complicated retinal detachments in p a t i e n t s w i t h c h o r o i d a l c o l o b o m a s . T h e r i s k s
Retinal Detachments and Choroidal Colobomas
Vol. I l l , No. 3
are great in t h e s e p a t i e n t s b e c a u s e t h e y a r e often m o n o c u l a r , t h e p r o c e d u r e is difficult, a n d the p r e v a l e n c e o f p r o l i f e r a t i v e v i t r e o r e t i n o p a thy is h i g h . F o r t u n a t e l y , h o w e v e r , t h e u s e o f vitreous surgical techniques has improved the a n a t o m i c a n d v i s u a l r e s u l t s in t h e s e c o m p l i c a t ed cases.
References 1. Jesberg, D. O., and Schepens, C. L.: Retinal de tachment associated with coloboma of the choroid. Arch. Ophthalmol. 65:163, 1 9 6 1 .
275
2. Patnaik, B., and Kalsi, R.: Retinal detachment with coloboma of the choroid. Indian J. Ophthalmol. 29:345, 1 9 8 1 . 3. Wang, K., and Hilton, G. F.: Retinal detachment associated with coloboma of the choroid. Trans. Am. Ophthalmol. Soc. 83:49, 1 9 8 5 . 4. Gonvers, M.: Temporary use of silicone oil in the treatment of special cases of retinal detachment. Ophthalmologica 187:202, 1 9 8 3 . 5. McCuen, B. W., II, Hida, T., and Sheta, S. M.: Transvitreal cyanoacrylate retinopexy in the man agement of complicated retinal detachment. Am. J. Ophthalmol. 104:127, 1987. 6. Sheta, S. M., Hida, T., and McCuen, B. W., II: Cyanoacrylate tissue adhesive in the management of recurrent retinal detachment caused by macular hole. Am. J. Ophthalmol. 109:28, 1 9 9 0 .
OPHTHALMIC MINIATURE There was one point that my father had noted about L i n c o l n ' s p h y s i o g n o my that 1 have never seen recorded. It was that the left eye, from time to time, looked queer and then suddenly " c r o s s e d , " i.e., turned up. At the time when my father told m e this it did not strike m e as having the least i m p o r t a n c e . Older grown, however, and b e c o m e an o c u l i s t I was struck one day by the recollection and then by the m e a n i n g of the fact itself. Lincoln had b e e n the victim of hyperphoria (a t e n d e n c y of one eye upward) with, now and then, a m o m e n t a r y hypertropia (actual turning of one eye upward). S u c h a condition, as all oculists k n o w , gives rise to an intense form of eyestrain and is one of the c o m m o n e s t causes of deep and protracted m e l a n c h o l y — t h e c h r o n i c , i n e x p r e s s i b l e blues. Here, then, was the probable explanation of the well-known L i n c o l n i a n depression of spirits which lasted, off and on, until his death. T h o m a s Hall S h a s t i d , My Father Knew Lincoln The Nation 1 2 8 : 2 2 7 , 1 9 2 9
Anne Hanneken, M.D., Eugene de Juan, Jr., M . D . , and Brooks W. McCuen Π, M . D .
We u s e d v i t r e o u s s u r g e r y t o t r e a t s e v e n patients (eight eyes) with complicated retinal detachments associated with choroidal colo bomas. All eyes had large choroidal colobo m a s and no e v i d e n c e of p e r i p h e r a l retinal breaks. Small, atrophic breaks were detected in five of t h e e y e s a n d w e r e l o c a t e d i n t h e b a s e of t h e c o l o b o m a in f o u r o f t h e five e y e s . A d junctive surgical techniques were necessary and i n c l u d e d c y a n o a c r y l a t e r e t i n o p e x y in four eyes, s i l i c o n e oil t a m p o n a d e i n five e y e s , a n d r e t i n e c t o m y in t w o e y e s . R e t i n a l r e a t t a c h m e n t w a s u l t i m a t e l y a t t a i n e d i n s e v e n of t h e e i g h t eyes. T h e n u m b e r of s u r g i c a l p r o c e d u r e s r a n g e d f r o m o n e t o five, w i t h a n a v e r a g e o f three. Postoperative visual acuity of the eyes that underwent anatomically successful pro cedures ranged from 2 0 / 1 0 0 to light p e r c e p tion. Proliferative v i t r e o r e t i n o p a t h y was the m o s t f r e q u e n t c a u s e of r e d e t a c h m e n t , o c c u r r i n g in six o f t h e e i g h t e y e s .
A CHOROIDAL COLOBOMA IS 3 Congenital l e s i o n c h a r a c t e r i z e d b y t h e a b s e n c e of n o r m a l r e t i n a , retinal pigment epithelium, and choroid. This d e v e l o p m e n t a l a n o m a l y is c o m m o n l y l o c a t e d in the i n f e r o n a s a l r e g i o n o f t h e f u n d u s a n d is c a u s e d b y t h e failure o f c l o s u r e of t h e fetal fissure. It is often a s s o c i a t e d w i t h s e v e r a l o t h e r o c u l a r a b n o r m a l i t i e s , i n c l u d i n g iris c o l o b o m a , loss of inferior lens zonules, cataract, and o p t i c disk a b n o r m a l i t i e s . T h e p r e v a l e n c e o f r e t i n a l
Accepted for publicatior\ Nov. 26, 1990. From the Whittier Institute for Diabetes and Endocri nology, La Jolla, California (Dr. Hanneken); and Depart ment of Ophthalmology, Duke University Eye Center, Durham, North Carolina (Drs. de Juan and McCuen). This study was supported in part by the Adler Founda tion, New York, New York (Dr. McCuen). Dr. Hanneken was a 1988-1990 Heed Ophthalmic Fellow. Reprint requests to Brooks W. McCuen, M.D., Depart ment of Ophthalmology, Duke University Medical Cen ter, Box 3802, Durham, NC 27710.
d e t a c h m e n t in e y e s w i t h c h o r o i d a l c o l o b o m a s h a s b e e n r e p o r t e d to b e from 2 3 % to 4 2 % . ' · The use of scleral buckling t e c h n i q u e s to treat r e t i n a l d e t a c h m e n t s in e y e s w i t h c h o r o i d a l c o l o b o m a s h a s r e s u l t e d in l o w r a t e s o f a n a t o m i c success, varying b e t w e e n 3 5 % and 55%.'·' Vit r e o u s s u r g e r y h a s b e e n a d v o c a t e d as a n a l t e r n a t i v e to s c l e r a l b u c k l i n g s u r g e r y in t h e t r e a t m e n t of s e l e c t e d r e t i n a l d e t a c h m e n t s in e y e s w i t h large chorioretinal c o l o b o m a s , but there are little d a t a a v a i l a b l e on its efficacy.^·^ We u s e d v i t r e o u s s u r g e r y to t r e a t s e v e n p a t i e n t s ( e i g h t eyes) with retinal d e t a c h m e n t associated with choroidal coloboma.
Patients and IVIethods We r e v i e w e d t h e r e c o r d s o f s e v e n c o n s e c u t i v e patients (eight eyes) with retinal detachments and posterior colobomas treated by vitrectomy b e t w e e n 1 9 8 4 a n d 1 9 9 0 . T h e r e w e r e five m a l e s a n d t w o f e m a l e s , r a n g i n g in a g e f r o m 7 to 4 2 years. Six of the patients had bilateral retinal d e t a c h m e n t s . O f these six patients, only one u n d e r w e n t v i t r e o u s s u r g e r y in b o t h e y e s . T h e r e m a i n i n g five p a t i e n t s h a d d e v e l o p e d p h t h i s i s or w e r e n o t c a n d i d a t e s for s u r g e r y in t h e i r fellow eye. A m i n i m u m of six m o n t h s of followu p data w e r e a v a i l a b l e in all p a t i e n t s . T h e m e a n follow-up period was 2 6 m o n t h s . All p a t i e n t s h a d l a r g e p o s t e r i o r o c u l a r c o l o b o m a s a s s o c i a t e d w i t h iris c o l o b o m a s , i n f e r i o r lens opacities, absence of inferior lens zonules, and absence of retinal pigment epithelium and choroid. Patients with peripheral retinal breaks were treated with conventional scleral buckling s u r g e r y a n d w e r e n o t i n c l u d e d in t h i s s e r i e s . O n l y e y e s w i t h p o s t e r i o r h o l e s or e y e s w i t h a rhegmatogenous-appearing retinal detachment in w h i c h n o h o l e s c o u l d b e f o u n d w e r e c o n s i d e r e d c a n d i d a t e s for v i t r e c t o m y . P a t i e n t s w i t h retinal detachments associated with the morn ing glory syndrome, isolated optic nerve head c o l o b o m a , or o p t i c n e r v e pit w e r e e l i m i n a t e d
©AMERICAN JOURNAL OF OPHTHALMOLOGY 111:271-275, MARCH,
1991
271
272
AMERICAN JOURNAL OF OPHTHALMOLOGY
from this s t u d y . Two p a t i e n t s h a d m o d e r a t e l y severe learning disabilities, one patient had a cleft lip a n d p a l a t e , a n d o n e p a t i e n t h a d a family h i s t o r y o f o c u l a r c o l o b o m a s . We u s e d a s t a n d a r d t h r e e - p o r t v i t r e c t o m y procedure. Most patients received general an esthesia. Retrobulbar injections were generally a v o i d e d b e c a u s e o f t h e risk o f u n i n t e n t i o n a l l y penetrating ectatic sclera. L e n s e c t o m i e s w e r e p e r f o r m e d in all e y e s using the phacofragmentation unit and a s e c o n dary infusion line. After l e n s e c t o m y , c a n n u l a s w e r e p l a c e d in t h e s u p e r i o r q u a d r a n t s , t h e c o n tact l e n s ring was s u t u r e d in p l a c e , a n d t h e a x i a l vitrectomy was performed. T h e s u r g i c a l g o a l s w e r e to r e l e a s e v i t r e o r e t i n a l t r a c t i o n a n d to i d e n t i f y a n d s e a l t h e r e t i n a l breaks. Achieving these goals was challenging in t h i s g r o u p o f p a t i e n t s . T h e i n i t i a l a p p r o a c h i n v o l v e d s t r i p p i n g t h e c o r t i c a l v i t r e o u s from t h e r e t i n a up to t h e p o s t e r i o r b o r d e r o f t h e v i t r e o u s b a s e . T h i s w a s often difficult b e c a u s e of the a b s e n c e o f a c o m p l e t e posterior vitreous detachment and the strong adhesion of the v i t r e o u s over r e g i o n s o f d e t a c h e d r e t i n a . We attributed the unusually strong vitreoretinal i n t e r f a c e to t h e y o u n g a g e o f m a n y o f t h e patients. When peripheral vitreous could not be r e l e a s e d a d e q u a t e l y or p e r i p h e r a l r e t i n a l h o l e s were created, peripheral encircling buckles were placed. The second surgical objective consisted of identifying the posterior retinal breaks. The r e t i n a l b r e a k s w e r e u s u a l l y s m a l l n a r r o w s l i t s in regions of thin rudimentary retina within the c o l o b o m a t o u s c r a t e r . T h e y w e r e often l o c a t e d in a r e a s b e n e a t h a l o c a l i z e d p o s t e r i o r v i t r e o u s d e t a c h m e n t . A useful t e c h n i q u e to i d e n t i f y t h e l o c a t i o n o f t h e b r e a k s w a s to a s p i r a t e o v e r r e g i o n s o f r u d i m e n t a r y r e t i n a a n d o b s e r v e for the p r e s e n c e o f s c h l i e r e n ( s u b t l e w a v y l i n e s t h a t d e v e l o p w h e n c l e a r fluids o f different v i s c o s i ties m i x ) . T h e third s u r g i c a l o b j e c t i v e w a s to c l o s e a n d s e a l t h e r e t i n a l b r e a k s . T h e a t r o p h i c b r e a k s in t h e b a s e o f t h e c o l o b o m a w e r e often t r e a t e d with cyanoacrylate tissue adhesive. This p r o c e d u r e was p e r f o r m e d after a c o m p l e t e fluid-gas e x c h a n g e . For t h e a d h e s i v e to s o l i d i f y w e l l , it was n e c e s s a r y to r e m o v e all s u b r e t i n a l fluid in the b a s e o f t h e c o l o b o m a . T h i s w a s difficult in c a s e s in w h i c h t h e r e w a s a d e e p p o c k e t o f t r a p p e d s u b r e t i n a l fluid in t h e c o l o b o m a t o u s crater. A variety of other procedures were consid e r e d helpful a d j u n c t s . L o n g - a c t i n g t a m p o n a d e s
March, 1991
w i t h s i l i c o n e oil, sulfur h e x a f l u o r i d e , or perflu oropropane were always used. Inferior iridecto m i e s w e r e n o t n e c e s s a r y w h e n s i l i c o n e oil w a s u s e d b e c a u s e t h e p a t i e n t s h a d i n f e r i o r iris c o l o b o m a s . In all p a t i e n t s , a firm c h o r i o r e t i n a l a d h e s i o n w a s c r e a t e d at t h e e d g e o f t h e c o l o b o m a b y e i t h e r e n d o c r y o t h e r a p y or e n d o l a s e r p h o t o c o a g u l a t i o n . Two r o w s o f t r e a t m e n t w e r e p l a c e d along a s e g m e n t overlapping the edge of the c o l o b o m a a n d t h e n o r m a l r e t i n a a n d c h o r o i d in a n a t t e m p t to i s o l a t e t h e r u d i m e n t a r y r e t i n a in t h e c o l o b o m a from t h e r e m a i n i n g p e r i p h e r a l retina.
Results T h e r e s u l t s a r e s u m m a r i z e d in t h e T a b l e . T h e r e t i n a w a s s u c c e s s f u l l y r e a t t a c h e d in s e v e n o f the eight eyes (Figs. 1 and 2 ) . T h e total n u m b e r o f s u r g i c a l p r o c e d u r e s n e c e s s a r y to a c h i e v e r e t i n a l r e a t t a c h m e n t r a n g e d from o n e to five, with an average of three. Follow-up was avail a b l e for all p a t i e n t s a n d r a n g e d from six m o n t h s to s e v e n y e a r s . P o s t o p e r a t i v e v i s u a l a c u i t y in t h e s u c c e s s f u l l y r e a t t a c h e d e y e s v a r i e d from 2 0 / 1 0 0 to l i g h t p e r c e p t i o n . Retinal breaks were identified either preoper a t i v e l y o r i n t r a o p e r a t i v e l y i n five o f t h e e i g h t e y e s . F o u r o f t h e five b r e a k s w e r e f o u n d w i t h i n t h e c o l o b o m a t o u s c r a t e r , in t h i n r u d i m e n t a r y
TABLE DATA OF EIGHT EYES WITH RETINAL DETACHMENTS ASSOCIATED WITH CHOROIDAL COLOBOMAS
CASE NO.. AGE (YHS)
SURGICAL ADJUNCTS
1, 42 2, 35 3,24
Silicone Glue Silicone, retinectomy Glue Silicone Glue Silicone Glue, silicone, retinectomy
4. 35 5, 7 6, 7 7,25 8, 11
NO. OF PROCE VISUAL FOLLOW-UP DURES RESULTS ACUITY* (MOS)
4 1 3
Attached 20/400 Attached CF Attached 20/100
14 11 8
2 4 2 4 5
Attached Attached Detached Attached Attached
14 84 52 21 6
20/200 20/200 LP LP 20/400
•CF indicates counting fingers and LP indicates light percep tion.
Vol. I l l , No. 3
Retinal Detachments and Choroidal Colobomas
273
F i g . 1 (Hanneken, de Juan, and McCuen). Case 3 . Retinal detachment associated with choroidal coloboma. Left, Fundus of the left eye demonstrating a recurrent retinal detachment with subretinal membranes after initial vitrectomy and fluid-gas exchange. Right, Fundus of the same eye after revision of vitrectomy, retinectomy, removal of subretinal membranes, endocryotherapy, and silicone oil tamponade.
r e t i n a . M o s t p a t i e n t s h a d o n l y o n e b r e a k in t h e coloboma, although one patient had two. Peripheral encircling scleral buckles were p r e s e n t in five o f t h e e i g h t e y e s at t h e final f o l l o w - u p e x a m i n a t i o n . Two o f t h e p a t i e n t s were initially seen with peripheral b u c k l e s al r e a d y in p l a c e , a n d t h r e e p a t i e n t s r e c e i v e d s c l e r a l b u c k l e s d u r i n g o u r s u r g e r y to s u p p o r t the posterior vitreous b a s e . C y a n o a c r y l a t e r e t i n o p e x y w a s u s e d to c l o s e a n d s e a l t h e p o s t e r i o r b r e a k s in four o f t h e five e y e s in w h i c h h o l e s w e r e f o u n d . All o f t h e h o l e s t r e a t e d w i t h g l u e w e r e c l o s e d s u c c e s s f u l l y . In o n e o f the p a t i e n t s , h o w e v e r , t h e glue d i d n o t adhere adequately to the retina and was partial ly r e m o v e d ( C a s e 4 ) . T h i s p r o b l e m w a s c a u s e d by r e s i d u a l s u b r e t i n a l fluid in t h e b a s e o f t h e c o l o b o m a that p r e v e n t e d a t i g h t a d h e s i o n b e tween the retina and the glue. S i l i c o n e oil w a s u s e d in five e y e s to a c h i e v e a l o n g - t e r m t a m p o n a d e . In four of t h e five e y e s , the s i l i c o n e oil w a s r e m o v e d after s e v e r a l m o n t h s . In o n e o f t h e s e p a t i e n t s , h o w e v e r , t h e e y e r e d e t a c h e d after s i l i c o n e oil r e m o v a l , a n d the s i l i c o n e oil h a d to b e r e p l a c e d ( C a s e 8 ) . The most c o m m o n postoperative complica tion was the development of proliferative vitre oretinopathy. Six of the eight eyes developed p r o l i f e r a t i v e v i t r e o r e t i n o p a t h y , r a n g i n g from f u l l - t h i c k n e s s fixed r e t i n a l f o l d s in two q u a d r a n t s to fixed r e t i n a l folds o f c l o s e d f u n n e l s h a p e in four q u a d r a n t s . Two e y e s d e v e l o p e d
significant tractional subretinal m e m b r a n e s , both of which required removal before the r e t i n a c o u l d b e r e a t t a c h e d . S e v e r e a n t e r i o r vit r e o u s b a s e c o n t r a c t i o n o c c u r r e d in a n o t h e r p a t i e n t a n d l e d to t h e u l t i m a t e f a i l u r e o f r e a t t a c h ment surgery (Case 6 ) . A s e v e r e fibrin p u p i l l a r y m e m b r a n e o c c u r r e d p o s t o p e r a t i v e l y in o n e p a t i e n t ( C a s e 7). T h i s m e m b r a n e did n o t r e s o r b w i t h c o r t i c o s t e r o i d treatment and required surgical removal.
Discussion In t h e a b s e n c e o f p e r i p h e r a l r e t i n a l b r e a k s , v i t r e o u s s u r g e r y is t h e t r e a t m e n t o f c h o i c e i n managing retinal d e t a c h m e n t associated with choroidal colobomas. Previously, scleral buck ling t e c h n i q u e s alone have b e e n used, but the rates of anatomic r e a t t a c h m e n t have been l o w . ^ ' In 1 9 8 3 , G o n v e r s " r e p o r t e d t h e s u c c e s s ful u s e o f t h e s i l i c o n e oil t a m p o n a d e to t r e a t o n e eye w i t h a r e t i n a l d e t a c h m e n t a s s o c i a t e d with a choroidal c o l o b o m a . Since then, addi tional reports of retinal detachment caused by chorioretinal colobomas and treated by vitre ous surgery have b e e n few.' T h e r e l a t i v e l y p o o r s u r g i c a l r e s u l t s in t h e s e c a s e s a r e c a u s e d b y s e v e r a l k e y a n a t o m i c fea t u r e s t h a t m a k e t h e s e e y e s u n i q u e l y different from o t h e r e y e s w i t h r e t i n a l d e t a c h m e n t . O n e
274
AMERICAN JOURNAL OF OPHTHALMOLOGY
Fig. 2 (Hanneken, de Juan, and McCuen). Case 8 . B-scan ultrasound image demonstrating a retinal detachment associated with a large posterior colobo ma (horizontal orientation).
of t h e s e f e a t u r e s is t h e p r e s e n c e o f s m a l l slits h a p e d r e t i n a l b r e a k s in t h e b a s e o f t h e c o l o b o m a . T h e r e t i n a l b r e a k s in t h e s e e y e s a r e often difficult to i d e n t i f y b o t h p r e o p e r a t i v e l y a n d intraoperatively. The retina overlying the colo b o m a is e x t r e m e l y a t r o p h i c , t h i n , a n d t r a n s p a r e n t . T h e a b s e n c e of n o r m a l r e t i n a l p i g m e n t e p i t h e l i u m a n d c h o r o i d r e d u c e s the c o n t r a s t a n d c o n t r i b u t e s to t h e p r o b l e m o f v i s u a l i z i n g the b r e a k . T h e b e s t i n t r a o p e r a t i v e t e c h n i q u e for i d e n t i f y i n g t h e s e b r e a k s w i t h i n t h e b a s e o f the c o l o b o m a is to a s p i r a t e fluid a b o v e t h e c o l o b o m a a n d to l o o k for s c h l i e r e n as s u b r e t i n a l fluid l e a k s t h r o u g h the r e t i n a l b r e a k . W i t h t h i s t e c h n i q u e , we w e r e a b l e to i d e n t i f y or c o n f i r m m o s t o f t h e b r e a k s in o u r s e r i e s . H o w e v e r , we w e r e still u n a b l e to i d e n t i f y p r i m a r y r e t i n a l b r e a k s in t h r e e o f our p a t i e n t s . The absence of a posterior vitreous detach m e n t is a n o t h e r c o m p l i c a t i n g feature in t h e s e eyes. Although the typical r h e g m a t o g e n o u s de tachment contains a nearly complete posterior vitreous detachment, our cases demonstrated virtually no separation between the cortical v i t r e o u s a n d the r e t i n a . T h e o n l y a r e a w h e r e t h e v i t r e o u s w a s s e p a r a t e d from t h e r e t i n a w a s over t h e c o l o b o m a , w h e r e a s h a l l o w , p a r t i a l , posterior vitreous separation was frequently p r e s e n t . T h i s s e p a r a t i o n w a s focal, h o w e v e r , a n d firm v i t r e o r e t i n a l a d h e s i o n w a s p r e s e n t over t h e r e m a i n d e r o f t h e c o l o b o m a a n d t h e more peripheral retina. The absence of a posterior vitreous detach m e n t c a u s e s c o n s i d e r a b l e t e c h n i c a l difficulty d u r i n g s u r g e r y . T h i s difficulty o c c u r s w h e n at t e m p t i n g to strip firmly a t t a c h e d c o r t i c a l v i t r e o u s from d e t a c h e d r e t i n a . W e c o n s i d e r it i m p o r tant to s e p a r a t e the p o s t e r i o r v i t r e o u s from t h e r e t i n a to r e d u c e the p o s s i b i l i t y o f p o s t o p e r a t i v e
March, 1991
v i t r e o u s c o n t r a c t i o n l e a d i n g to r e c u r r e n t r e t i nal detachment. A n i m p o r t a n t c o n s i d e r a t i o n in t h e t r e a t m e n t o f t h i s g r o u p o f p a t i e n t s is t h e c h o i c e a n d u s e o f c h o r i o r e t i n a l a d h e s i v e m o d a l i t i e s to t r e a t r e t i n a l b r e a k s . T r a d i t i o n a l l y , b r e a k s are t r e a t e d with thermal irritation of the retinal pigment e p i t h e l i u m a n d r e t i n a to c r e a t e c h o r i o r e t i n a l scarring and adhesion. Within the c o l o b o m a , h o w e v e r , t h e r e t i n a l p i g m e n t e p i t h e l i u m is a b normal and frequently absent. Laser p h o t o c o agulation, cryotherapy, and diathermy do not r e s u l t in g o o d c h o r i o r e t i n a l a d h e s i o n s w i t h i n this r e g i o n . O t h e r a l t e r n a t i v e s a r e n e c e s s a r y . C y a n o a c r y l a t e retinopexy*'^ is a useful t e c h n i q u e for c l o s i n g a t r o p h i c b r e a k s in t h e b a s e o f t h e c o l o b o m a . For t h e a d h e s i v e t o s o l i d i f y , t h e g l u e must b e a p p l i e d after all r e s i d u a l fluid h a s b e e n r e m o v e d from t h e v i t r e o u s c a v i t y b y fluidgas exchange and internal drainage of subreti n a l fluid. P o o r c h o r i o r e t i n a l a d h e s i o n w a s e n c o u n t e r e d in o n e p a t i e n t ( C a s e 4 ) in w h o m t h e rudimentary retina within the coloboma could n o t b e c o m p l e t e l y flattened a g a i n s t t h e s c l e r a . S i l i c o n e oil w a s u s e d in five o f t h e e i g h t e y e s to a c h i e v e a l o n g - t e r m t a m p o n a d e . It w a s u l t i m a t e l y r e m o v e d in four o f t h e five e y e s . B e c a u s e one of these eyes developed a redetachment after t h e r e m o v a l o f s i l i c o n e o i l , h o w e v e r , w e are n o w m o r e c a u t i o u s w h e n c o n s i d e r i n g w h e t h e r to r e m o v e it. E n d o l a s e r r e t i n o p e x y o r e n d o c r y o t h e r a p y is a p p l i e d a l o n g t h e m a r g i n o f t h e c o l o b o m a to c r e a t e a b o r d e r o f c h o r i o r e t i n a l a d h e s i o n a n d to s e p a r a t e n o r m a l r e t i n a from a b n o r m a l r e t i n a . T h i s t r e a t m e n t w a s u s e d to l i m i t t h e e x t e n s i o n o f p o t e n t i a l r e c u r r e n t s u b r e t i n a l fluid in t h e b a s e of t h e c o l o b o m a . The most c o m m o n cause of retinal redetach m e n t in t h e s e c a s e s w a s p r o l i f e r a t i v e v i t r e o r e t i n o p a t h y . S e v e n o f t h e e i g h t e y e s in o u r s e r i e s r e d e t a c h e d , a n d t h e d e t a c h m e n t in all but o n e o f t h e s e e y e s w a s c a u s e d b y t r a n s v i t r e a l , cir c u m f e r e n t i a l , or s u b r e t i n a l t r a c t i o n a l m e m branes. Severe anterior vitreous base contrac t i o n a c c o u n t e d for t h e u l t i m a t e failure in o n e p a t i e n t ( C a s e 6 ) . T h e r e a s o n for t h e h i g h p r e v a l e n c e o f p r o l i f e r a t i v e v i t r e o r e t i n o p a t h y is n o t c l e a r , b u t it m a y b e r e l a t e d to t h e r e l a t i v e l y y o u n g a g e o f t h e p a t i e n t s , t h e c h r o n i c i t y of t h e d e t a c h m e n t s , or t h e e x t e n t o f l a s e r or c r y o therapy. Although multiple operations may be neces s a r y , v i t r e o u s s u r g e r y p r o v i d e s an effective treatment of complicated retinal detachments in p a t i e n t s w i t h c h o r o i d a l c o l o b o m a s . T h e r i s k s
Retinal Detachments and Choroidal Colobomas
Vol. I l l , No. 3
are great in t h e s e p a t i e n t s b e c a u s e t h e y a r e often m o n o c u l a r , t h e p r o c e d u r e is difficult, a n d the p r e v a l e n c e o f p r o l i f e r a t i v e v i t r e o r e t i n o p a thy is h i g h . F o r t u n a t e l y , h o w e v e r , t h e u s e o f vitreous surgical techniques has improved the a n a t o m i c a n d v i s u a l r e s u l t s in t h e s e c o m p l i c a t ed cases.
References 1. Jesberg, D. O., and Schepens, C. L.: Retinal de tachment associated with coloboma of the choroid. Arch. Ophthalmol. 65:163, 1 9 6 1 .
275
2. Patnaik, B., and Kalsi, R.: Retinal detachment with coloboma of the choroid. Indian J. Ophthalmol. 29:345, 1 9 8 1 . 3. Wang, K., and Hilton, G. F.: Retinal detachment associated with coloboma of the choroid. Trans. Am. Ophthalmol. Soc. 83:49, 1 9 8 5 . 4. Gonvers, M.: Temporary use of silicone oil in the treatment of special cases of retinal detachment. Ophthalmologica 187:202, 1 9 8 3 . 5. McCuen, B. W., II, Hida, T., and Sheta, S. M.: Transvitreal cyanoacrylate retinopexy in the man agement of complicated retinal detachment. Am. J. Ophthalmol. 104:127, 1987. 6. Sheta, S. M., Hida, T., and McCuen, B. W., II: Cyanoacrylate tissue adhesive in the management of recurrent retinal detachment caused by macular hole. Am. J. Ophthalmol. 109:28, 1 9 9 0 .
OPHTHALMIC MINIATURE There was one point that my father had noted about L i n c o l n ' s p h y s i o g n o my that 1 have never seen recorded. It was that the left eye, from time to time, looked queer and then suddenly " c r o s s e d , " i.e., turned up. At the time when my father told m e this it did not strike m e as having the least i m p o r t a n c e . Older grown, however, and b e c o m e an o c u l i s t I was struck one day by the recollection and then by the m e a n i n g of the fact itself. Lincoln had b e e n the victim of hyperphoria (a t e n d e n c y of one eye upward) with, now and then, a m o m e n t a r y hypertropia (actual turning of one eye upward). S u c h a condition, as all oculists k n o w , gives rise to an intense form of eyestrain and is one of the c o m m o n e s t causes of deep and protracted m e l a n c h o l y — t h e c h r o n i c , i n e x p r e s s i b l e blues. Here, then, was the probable explanation of the well-known L i n c o l n i a n depression of spirits which lasted, off and on, until his death. T h o m a s Hall S h a s t i d , My Father Knew Lincoln The Nation 1 2 8 : 2 2 7 , 1 9 2 9