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Capsular tension ring in a patient with Weill-Marchesani syndrome
Capsular tension ring in a patient with Weill-Marchesani syndrome Sarah A. Groessl, MD, Charles J. Anderson, MD
ABSTRACT
,
Weill-Marchesani patients with cataractous lenses may present a surgical challenge in the presence of zonular weakness and microsherophakia. A 52~year:-ald Weill Marchesani patient developed zonular dehiscence during capsule contraction after cataract extraction in her right eye. Use of a poly(methyl methacrylate) capsular tension ring in the second eye facilitated lens removal and intraocular lens placement. Postoperative results suggest the capsular tension ring provides long-term zonular stabilization by maintaining an internal force against the capsule. J Cataract Refract Surg 1998; 24:1164-1165
P
atients with Weill-Marchesani syndrome are char acterized by bradymorphia, normal mentation, and an autosomal recessive inheritance. 1 Microspherophakia and zonular degeneration are the most common oph thalmic findings with a 60% incidence of lens sublux ation. The subluxation is usually down and centraU Lenticular myopia is frequently a feature of micro spherophakia. Zonular weakness in these patients makes removal of nondislocated cataracts challenging. This report describes the use of a capsular tension ring that facilitates cataract removal and may improve visual outcome.
left. She was anisometropic with manifest refraction of -4.50 +0.75 X 84 in the right eye and plano + 1.25 X 102 in the left. Four-mirror gonioscopy revealed narrowed but open angles for 360 degrees. Slidamp examination showed iridodonesis in both eyes. She was spherophakic without lens displacement. Her fundus examination was unremarkable. A-scan biometry demonstrated an axial length of 22.09 mm in the right eye and 20.73 mm in the left eye. A physical examination revealed short stature, brachydactyly, and brachycephaly (Figure 1). During phacoemulsification in the right eye, a superior zonular dehiscence required sulcus intraocular lens (IOL)
Case Report A 52-year-old woman with Weill-Marchesani syndrome and blurred vision was evaluated for cataract extraction. Visual acuity was 20/200 in the right eye and 20/40 in the From the University ofWisconsin Department of Ophthalmology and Visual Sciences, Madison, Wisconsin, USA. Supported in part by an unrestricted grant ftom Research to Prevent Blindness Inc., New York, NY, and the Wisconsin Lions Eye Research Fund through the Department of Ophthalmology and Visual Sciences. Reprint requests to C. Joseph Anderson, MD, University ofWisconsin Department of Ophthalmology, 600 Highland Avenue, CSC F41336, Madison, Wisconsin 53792-3220, USA. 1164
Figure 1. (Groessl) Brachydactyly in a patient with Weill Marchesani syndrome.
J CATARACT REFRACT SURG-VOL 24, AUGUST 1998
CASE REPORTS: GROESSL
Figure 2. (Groessl) Left: The right eye 6 months after cataract extraction and 4 months after Nd:YAG capsulotomy. Note the capsule contraction and opacification. Right: The left eye 5 months after cataract extraction. Note the minimal capsule opacity and good intracapsular centration of the posterior chamber IOL.
placement with a superior iris-to-IOL fixation suture. The patient required a neodymium:YAG (Nd:YAG) posterior capsulotomy 1 month after surgery, and visual acuity in the operative eye recovered to 20/40. Subsequently, visual acuity in the left eye dropped to 20160 associated with nuclear sclerosis. The patient reported difficulty driving and requested cataract removal. A second procedure was planned using a capsular tension ring in the second eye. The device, a single-piece poly(methyl methacry late) (PMMA) incomplete circular ring (Marcher), had not obtained U.S. Food and Drug Administration premarket approval but was implanted on a compassionate-use basis. Informed consent and institutional review board approval were obtained. After continuous curvilinear capsulorhexis, the ring was placed in the capsular bag just posterior to the anterior capsule. Phacoemulsification was performed without zonular dehiscence or other complications. Postoperatively, no capsule contraction and minimal opacification occurred. Final visual acuity 6 months after surgery was 20/30 in both eyes.
PMMA device, can stabilize a capsule with dysfunc tional or missing zonules. The ring maintains the open bag during lens extraction by evenly distributing forces throughout the capsular bag. The ring remains in the bag postsurgically to improve IOL centration and provide continued zonular stability to prevent capsule contraction (Figure 2). 4•5
References 1. Streeten BW Pathology of the lens. In: Albert DM,
2.
3.
4.
Discussion Zonular weakness and lens subluxation charac terize the ocular findings of Weill-Marchesani syn drome.1-3 The capsular tension ring, a single-piece
5.
Jakibiec FA, eds, Principles and Practice of Ophthalmol ogy. Philadelphia, PA, WB Saunders Co, 1994; 2231 Merin S. Inherited Eye Diseases; Diagnosis and Clinical Management. New York, NY, Marcel Dekker Inc, 1991; 128-129 Yanoff M. Pathology of cataract. In: Bellows JG, ed, Cataract and Abnormalities of the Lens. New York, NY, Grune and Stratten Inc, 1975; 186 Cionni RJ, Osher RH. Endocapsular ring approach to the subluxated cataractous lens. J Cataract Refract Surg 1995; 21:245-249 Gimbel HV, Sun R, Heston JP. Management of zonular dialysis in phacoemulsification and IOL implantation using the capsular tension ring. Ophthalmic Surg Lasers 1997; 28:273-281
J CATARACT REFRACf SURG-VOL 24, AUGUST 1998
1165
ABSTRACT
,
Weill-Marchesani patients with cataractous lenses may present a surgical challenge in the presence of zonular weakness and microsherophakia. A 52~year:-ald Weill Marchesani patient developed zonular dehiscence during capsule contraction after cataract extraction in her right eye. Use of a poly(methyl methacrylate) capsular tension ring in the second eye facilitated lens removal and intraocular lens placement. Postoperative results suggest the capsular tension ring provides long-term zonular stabilization by maintaining an internal force against the capsule. J Cataract Refract Surg 1998; 24:1164-1165
P
atients with Weill-Marchesani syndrome are char acterized by bradymorphia, normal mentation, and an autosomal recessive inheritance. 1 Microspherophakia and zonular degeneration are the most common oph thalmic findings with a 60% incidence of lens sublux ation. The subluxation is usually down and centraU Lenticular myopia is frequently a feature of micro spherophakia. Zonular weakness in these patients makes removal of nondislocated cataracts challenging. This report describes the use of a capsular tension ring that facilitates cataract removal and may improve visual outcome.
left. She was anisometropic with manifest refraction of -4.50 +0.75 X 84 in the right eye and plano + 1.25 X 102 in the left. Four-mirror gonioscopy revealed narrowed but open angles for 360 degrees. Slidamp examination showed iridodonesis in both eyes. She was spherophakic without lens displacement. Her fundus examination was unremarkable. A-scan biometry demonstrated an axial length of 22.09 mm in the right eye and 20.73 mm in the left eye. A physical examination revealed short stature, brachydactyly, and brachycephaly (Figure 1). During phacoemulsification in the right eye, a superior zonular dehiscence required sulcus intraocular lens (IOL)
Case Report A 52-year-old woman with Weill-Marchesani syndrome and blurred vision was evaluated for cataract extraction. Visual acuity was 20/200 in the right eye and 20/40 in the From the University ofWisconsin Department of Ophthalmology and Visual Sciences, Madison, Wisconsin, USA. Supported in part by an unrestricted grant ftom Research to Prevent Blindness Inc., New York, NY, and the Wisconsin Lions Eye Research Fund through the Department of Ophthalmology and Visual Sciences. Reprint requests to C. Joseph Anderson, MD, University ofWisconsin Department of Ophthalmology, 600 Highland Avenue, CSC F41336, Madison, Wisconsin 53792-3220, USA. 1164
Figure 1. (Groessl) Brachydactyly in a patient with Weill Marchesani syndrome.
J CATARACT REFRACT SURG-VOL 24, AUGUST 1998
CASE REPORTS: GROESSL
Figure 2. (Groessl) Left: The right eye 6 months after cataract extraction and 4 months after Nd:YAG capsulotomy. Note the capsule contraction and opacification. Right: The left eye 5 months after cataract extraction. Note the minimal capsule opacity and good intracapsular centration of the posterior chamber IOL.
placement with a superior iris-to-IOL fixation suture. The patient required a neodymium:YAG (Nd:YAG) posterior capsulotomy 1 month after surgery, and visual acuity in the operative eye recovered to 20/40. Subsequently, visual acuity in the left eye dropped to 20160 associated with nuclear sclerosis. The patient reported difficulty driving and requested cataract removal. A second procedure was planned using a capsular tension ring in the second eye. The device, a single-piece poly(methyl methacry late) (PMMA) incomplete circular ring (Marcher), had not obtained U.S. Food and Drug Administration premarket approval but was implanted on a compassionate-use basis. Informed consent and institutional review board approval were obtained. After continuous curvilinear capsulorhexis, the ring was placed in the capsular bag just posterior to the anterior capsule. Phacoemulsification was performed without zonular dehiscence or other complications. Postoperatively, no capsule contraction and minimal opacification occurred. Final visual acuity 6 months after surgery was 20/30 in both eyes.
PMMA device, can stabilize a capsule with dysfunc tional or missing zonules. The ring maintains the open bag during lens extraction by evenly distributing forces throughout the capsular bag. The ring remains in the bag postsurgically to improve IOL centration and provide continued zonular stability to prevent capsule contraction (Figure 2). 4•5
References 1. Streeten BW Pathology of the lens. In: Albert DM,
2.
3.
4.
Discussion Zonular weakness and lens subluxation charac terize the ocular findings of Weill-Marchesani syn drome.1-3 The capsular tension ring, a single-piece
5.
Jakibiec FA, eds, Principles and Practice of Ophthalmol ogy. Philadelphia, PA, WB Saunders Co, 1994; 2231 Merin S. Inherited Eye Diseases; Diagnosis and Clinical Management. New York, NY, Marcel Dekker Inc, 1991; 128-129 Yanoff M. Pathology of cataract. In: Bellows JG, ed, Cataract and Abnormalities of the Lens. New York, NY, Grune and Stratten Inc, 1975; 186 Cionni RJ, Osher RH. Endocapsular ring approach to the subluxated cataractous lens. J Cataract Refract Surg 1995; 21:245-249 Gimbel HV, Sun R, Heston JP. Management of zonular dialysis in phacoemulsification and IOL implantation using the capsular tension ring. Ophthalmic Surg Lasers 1997; 28:273-281
J CATARACT REFRACf SURG-VOL 24, AUGUST 1998
1165