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Complications of endocapsular tension ring implantation in a child with Marfan’s syndrome
Complications of endocapsular tension ring implantation in a child with Marfan’s syndrome Thomas S. Dietlein, MD, Philipp C. Jacobi, MD, Walter Konen, MD, Gu¨nter K. Krieglstein, MD ABSTRACT A 4-year-old boy with Marfan’s syndrome had severe visual impairment after subluxation of the crystalline lens with bisection of the pupil. In the first eye, a capsular tension ring and an intraocular lens (IOL) were uneventfully placed in the capsular bag after phacoemulsification. During implantation of the endocapsular tension ring in the second eye, an inadvertent tear of the anterior capsule occurred, and the posterior chamber IOL was placed in the sulcus. In both eyes, severe lens epithelial proliferation with secondary IOL decentration developed postoperatively. Several surgical revisions were necessary to keep the IOL within the optical axis. In the eye with the sulcus-implanted IOL, the endocapsular tension ring was markedly decentered. J Cataract Refract Surg 2000; 26:937–940 © 2000 ASCRS and ESCRS
T
he capsular bag tension ring is reported to be an elegant tool for the surgical management of zonular dialysis in phacoemulsification and intraocular lens (IOL) implantation. The clinical experience with the tension ring in adult cataract surgery shows that intraoperative and postoperative IOL centration is positively influenced in cases of zonulysis.1– 4 Furthermore, the high risk of vitreous loss associated with zonulysis might also be reduced. We report the intraoperative and postoperative complications of endocapsular ring and IOL implantation in a young child with extensive bilateral subluxation of the crystalline lens.
Accepted for publication November 30, 1999. From the Department of Ophthalmology, University of Cologne, Cologne, Germany. Reprint requests to Thomas S. Dietlein, MD, Department of Ophthalmology, University of Cologne, Joseph-Stelzmann-Strasse 9, D-50931 Ko¨ln, Germany. © 2000 ASCRS and ESCRS Published by Elsevier Science Inc.
Case Report A 4-year-old boy with Marfan’s syndrome was referred for visual deterioration in his right eye. His mother also had Marfan’s syndrome and bilateral lens subluxation. On initial examination, the boy had a visual acuity of 20/400 in the right eye and 20/63 in the left eye. Because the pupil in the right eye was centrally bisected by the upwardly subluxated lens (Figure 1), an improvement by optical correction was not feasible. The anterior chamber in both eyes was otherwise normal. An A-scan showed an axial length of 24.0 mm in both eyes. The parents were given detailed information on treatment alternatives. The decision was made to perform cataract surgery with implantation of an IOL and an endocapsular tension ring. After a capsulorhexis was performed, the lens was punched with a needle via the pars plana to keep a central position intraoperatively. The lens was then aspirated via the anterior chamber. After the endocapsular tension ring (Morcher type 14; 10.0 to 12.5 mm) was implanted, a posterior chamber IOL (Morcher 65C, 22.0 diopter [D], 13.0 mm overall, 6.0 mm optic) was placed in the capsular bag. Despite the implantation of an endocapsular tension ring, the capsular bag moved upward during surgery. 0886-3350/00/$–see front matter PII S0886-3350(00)00318-7
CASE REPORTS: DIETLEIN
Figure 1. (Dietlein) The crystalline lens has moved upward because of the large extent of zonulysis. Refractive correction would not improve visual acuity as the optical axis is bisected by the lens margin.
Postoperatively, visual acuity was 20/100 in the right eye. Two weeks later, broad posterior synechias occurred because of massive lens epithelial proliferation and capsular bag shrinkage (Figure 2). An anterior chamber revision with synechiolysis and peripheral iridectomy were performed, after which the IOL was centrally located and visual acuity 20/32. Six months later, visual acuity in the second eye had worsened to 20/200 because of progressive lens subluxation. As the situation of the first eye was relatively stable, the same surgical procedure was performed in the second eye (e.g., cap-
Figure 2. (Dietlein) After cataract surgery with endocapsular ring and IOL implantation, marked posterior synechias with capsule bag shrinkage can be seen in the right eye.-
938
sulorhexis, lens punching, aspiration). However, during implantation of the capsular tension ring (Morcher type 14; 10.0 to 12.5 mm) in the capsular bag, a radial tear of the capsulorhexis occurred, so the IOL (same model as in right eye) was placed in the sulcus. During the initial postoperative period, the IOL was well centered and visual acuity recovered to 20/100. Three months later, the second eye developed extensive lens epithelial proliferation with posterior synechias. The capsular tension ring was still in the bag, but the entire capsular bag was upwardly decentered. Synechiolysis and discission of secondary membranes in the left eye were performed. Visual acuity improved to 20/32. During that time, visual acuity in the right eye was 20/40; however, the IOL in that eye had decentered into a nasoinferior position (Figure 3). The upper haptic pressed against the iris, and this haptic was fixated with a scleral suture. Six months later, visual acuity dropped to 20/200 in the left eye as a result of secondary epithelial membrane formation. A laser capsulotomy was not successful, and a surgical discission was performed. One year later, this surgical procedure was repeated in the left eye. Intraoperatively, it was noted that the capsular tension ring had moved upward, far more than the IOL, as a result of capsule shrinkage (Figure 4). After this last surgical intervention, visual acuity was 20/50 in the left eye and the upward decentration of the capsular tension ring diminished.
Discussion Several studies have reported the effective expansion and stabilization of the capsular bag by an endocapsular
Figure 3. (Dietlein) The IOL is decentered in the naso-inferior position despite the use of an endocapsular ring in the right eye after cataract surgery and secondary membrane discission.
J CATARACT REFRACT SURG—VOL 26, JUNE 2000
CASE REPORTS: DIETLEIN
Figure 4. (Dietlein) After several anterior chamber revisions, the sulcus-implanted IOL is relatively stable, while the endocapsular tension ring has moved upward behind the IOL and is in a position similar to that of the original crystalline lens.
ring when zonulysis or zonular weakness are present.1–5 Even several months after surgery, the tension ring can provide excellent IOL centration and positioning.4 In experimental studies, the tendency of secondary lens epithelial reproliferation was reduced by use of an endocapsular tension ring in cataract surgery.6 However, the use of an endocapsular tension ring in a young child with Marfan’s syndrome is extraordinary for 2 reasons. Secondary lens epithelial proliferation and capsule shrinkage are much more pronounced in young children than in adults older than 50 years. In addition, the degree of zonular dialysis in Marfan’s patients with early lens subluxation and bisection of the pupil might be more severe than in previously reported cases of tension ring implantation in adults.2,4 There might be different explanations for the complicated outcome in our case. On one hand, it is possible that strong traction by lens epithelial reproliferation on the IOL and the tension ring could have been reduced by the use of a special IOL (e.g., heparin modified or acrylic)7 or by performing a primary posterior capsulotomy and anterior vitrectomy.8 However, this procedure has certain risks, especially because little is known about the compatibility of these intraoperative measures with an endocapsular ring in children. It might be argued that the concept of a flexible tension ring in a growing eye with proliferative capacity of the surrounding tissue is not wise. The combination of strong lens epithelial proliferation and extremely
weak and fragile zonules predisposes to endocapsular ring dislocation and distortion. The size of the endocapsular tension ring can also play a crucial role in pediatric cataract surgery as it is designed for use in adults. Potentially, a radial tear of the capsulorhexis can occur after ring implantation, as in our patient, because the capsular bag of such a young child is too small for the ring. We cannot currently recommend the regular use of an endocapsular tension ring in young children with extensive zonular dialysis as the potential postoperative problems are significant; other surgical strategies have proven safe thus far.9 –12 However, modifications of endocapsular tension rings and of surgical technique might improve the potential use of the rings in pediatric cataract surgery. The use of a double Cionni ring, which allows scleral suturing of the endocapsular tension ring without violating the capsular bag, might be an option.3
References 1. Hara T, Hara T, Yamada Y. “Equator ring ” for maintenance of the completely circular contour of the capsular bag equator after cataract removal. Ophthalmic Surg 1991; 22:358 –359 2. Cionni RJ, Osher RH. Endocapsular ring approach to the subluxed cataractous lens. J Cataract Refract Surg 1995; 21:245–249 3. Cionni RJ, Osher RH. Management of profound zonular dialysis or weakness with a new endocapsular ring designed for scleral fixation. J Cataract Refract Surg 1998; 24:1299 –1306 4. 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 5. Strenn K, Menapace R, Vass C. Capsular bag shrinkage after implantation of an open-loop silicone lens and a poly(methyl methacrylate) IOL and capsule tension ring. J Cataract Refract Surg 1997; 23:1543–1547 6. Kugelberg U, Zetterstro¨m C, Lundgren B, et al. Aftercataract and ocular growth in newborn rabbit eyes implanted with a capsule tension ring. J Cataract Refract Surg 1997; 23:635– 640 7. Zetterstro¨m C, Kugelberg U, Oscarson C. Cataract surgery in children with capsulorhexis of anterior and posterior capsules and heparin-surface-modified intraocular lenses. J Cataract Refract Surg 1994; 20:599 – 601 8. Koch DD, Kohnen T. Retrospective comparison of techniques to prevent secondary cataract formation following posterior chamber intraocular lens implantation in infants and children. J Cataract Refract Surg 1997; 23: 657– 663
J CATARACT REFRACT SURG—VOL 26, JUNE 2000
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CASE REPORTS: DIETLEIN
9. Peyman GA, Raichand M, Goldberg MF, Ritacca D. Management of subluxated and dislocated lenses with vitrophage. Br J Ophthalmol 1979; 63:771–778 10. Plager DA, Parks MM, Helveston EM, Ellis FD. Surgical treatment of subluxated lenses in children. Ophthalmology 1992; 99:1018 –1021; discussion by AW Biglan, 1022–1023
940
11. Salehpour O, Lavy T, Leonard J, Taylor D. The surgical management of nontraumatic ectopic lenses. J Pediatr Ophthalmol Strabismus 1996; 33: 8 –13 12. Zetterstro¨m C, Lundvall A, Weeber H Jr, Jeeves M. Sulcus fixation without capsular support in children. J Cataract Refract Surg 1999; 25:776 –781
J CATARACT REFRACT SURG—VOL 26, JUNE 2000
T
he capsular bag tension ring is reported to be an elegant tool for the surgical management of zonular dialysis in phacoemulsification and intraocular lens (IOL) implantation. The clinical experience with the tension ring in adult cataract surgery shows that intraoperative and postoperative IOL centration is positively influenced in cases of zonulysis.1– 4 Furthermore, the high risk of vitreous loss associated with zonulysis might also be reduced. We report the intraoperative and postoperative complications of endocapsular ring and IOL implantation in a young child with extensive bilateral subluxation of the crystalline lens.
Accepted for publication November 30, 1999. From the Department of Ophthalmology, University of Cologne, Cologne, Germany. Reprint requests to Thomas S. Dietlein, MD, Department of Ophthalmology, University of Cologne, Joseph-Stelzmann-Strasse 9, D-50931 Ko¨ln, Germany. © 2000 ASCRS and ESCRS Published by Elsevier Science Inc.
Case Report A 4-year-old boy with Marfan’s syndrome was referred for visual deterioration in his right eye. His mother also had Marfan’s syndrome and bilateral lens subluxation. On initial examination, the boy had a visual acuity of 20/400 in the right eye and 20/63 in the left eye. Because the pupil in the right eye was centrally bisected by the upwardly subluxated lens (Figure 1), an improvement by optical correction was not feasible. The anterior chamber in both eyes was otherwise normal. An A-scan showed an axial length of 24.0 mm in both eyes. The parents were given detailed information on treatment alternatives. The decision was made to perform cataract surgery with implantation of an IOL and an endocapsular tension ring. After a capsulorhexis was performed, the lens was punched with a needle via the pars plana to keep a central position intraoperatively. The lens was then aspirated via the anterior chamber. After the endocapsular tension ring (Morcher type 14; 10.0 to 12.5 mm) was implanted, a posterior chamber IOL (Morcher 65C, 22.0 diopter [D], 13.0 mm overall, 6.0 mm optic) was placed in the capsular bag. Despite the implantation of an endocapsular tension ring, the capsular bag moved upward during surgery. 0886-3350/00/$–see front matter PII S0886-3350(00)00318-7
CASE REPORTS: DIETLEIN
Figure 1. (Dietlein) The crystalline lens has moved upward because of the large extent of zonulysis. Refractive correction would not improve visual acuity as the optical axis is bisected by the lens margin.
Postoperatively, visual acuity was 20/100 in the right eye. Two weeks later, broad posterior synechias occurred because of massive lens epithelial proliferation and capsular bag shrinkage (Figure 2). An anterior chamber revision with synechiolysis and peripheral iridectomy were performed, after which the IOL was centrally located and visual acuity 20/32. Six months later, visual acuity in the second eye had worsened to 20/200 because of progressive lens subluxation. As the situation of the first eye was relatively stable, the same surgical procedure was performed in the second eye (e.g., cap-
Figure 2. (Dietlein) After cataract surgery with endocapsular ring and IOL implantation, marked posterior synechias with capsule bag shrinkage can be seen in the right eye.-
938
sulorhexis, lens punching, aspiration). However, during implantation of the capsular tension ring (Morcher type 14; 10.0 to 12.5 mm) in the capsular bag, a radial tear of the capsulorhexis occurred, so the IOL (same model as in right eye) was placed in the sulcus. During the initial postoperative period, the IOL was well centered and visual acuity recovered to 20/100. Three months later, the second eye developed extensive lens epithelial proliferation with posterior synechias. The capsular tension ring was still in the bag, but the entire capsular bag was upwardly decentered. Synechiolysis and discission of secondary membranes in the left eye were performed. Visual acuity improved to 20/32. During that time, visual acuity in the right eye was 20/40; however, the IOL in that eye had decentered into a nasoinferior position (Figure 3). The upper haptic pressed against the iris, and this haptic was fixated with a scleral suture. Six months later, visual acuity dropped to 20/200 in the left eye as a result of secondary epithelial membrane formation. A laser capsulotomy was not successful, and a surgical discission was performed. One year later, this surgical procedure was repeated in the left eye. Intraoperatively, it was noted that the capsular tension ring had moved upward, far more than the IOL, as a result of capsule shrinkage (Figure 4). After this last surgical intervention, visual acuity was 20/50 in the left eye and the upward decentration of the capsular tension ring diminished.
Discussion Several studies have reported the effective expansion and stabilization of the capsular bag by an endocapsular
Figure 3. (Dietlein) The IOL is decentered in the naso-inferior position despite the use of an endocapsular ring in the right eye after cataract surgery and secondary membrane discission.
J CATARACT REFRACT SURG—VOL 26, JUNE 2000
CASE REPORTS: DIETLEIN
Figure 4. (Dietlein) After several anterior chamber revisions, the sulcus-implanted IOL is relatively stable, while the endocapsular tension ring has moved upward behind the IOL and is in a position similar to that of the original crystalline lens.
ring when zonulysis or zonular weakness are present.1–5 Even several months after surgery, the tension ring can provide excellent IOL centration and positioning.4 In experimental studies, the tendency of secondary lens epithelial reproliferation was reduced by use of an endocapsular tension ring in cataract surgery.6 However, the use of an endocapsular tension ring in a young child with Marfan’s syndrome is extraordinary for 2 reasons. Secondary lens epithelial proliferation and capsule shrinkage are much more pronounced in young children than in adults older than 50 years. In addition, the degree of zonular dialysis in Marfan’s patients with early lens subluxation and bisection of the pupil might be more severe than in previously reported cases of tension ring implantation in adults.2,4 There might be different explanations for the complicated outcome in our case. On one hand, it is possible that strong traction by lens epithelial reproliferation on the IOL and the tension ring could have been reduced by the use of a special IOL (e.g., heparin modified or acrylic)7 or by performing a primary posterior capsulotomy and anterior vitrectomy.8 However, this procedure has certain risks, especially because little is known about the compatibility of these intraoperative measures with an endocapsular ring in children. It might be argued that the concept of a flexible tension ring in a growing eye with proliferative capacity of the surrounding tissue is not wise. The combination of strong lens epithelial proliferation and extremely
weak and fragile zonules predisposes to endocapsular ring dislocation and distortion. The size of the endocapsular tension ring can also play a crucial role in pediatric cataract surgery as it is designed for use in adults. Potentially, a radial tear of the capsulorhexis can occur after ring implantation, as in our patient, because the capsular bag of such a young child is too small for the ring. We cannot currently recommend the regular use of an endocapsular tension ring in young children with extensive zonular dialysis as the potential postoperative problems are significant; other surgical strategies have proven safe thus far.9 –12 However, modifications of endocapsular tension rings and of surgical technique might improve the potential use of the rings in pediatric cataract surgery. The use of a double Cionni ring, which allows scleral suturing of the endocapsular tension ring without violating the capsular bag, might be an option.3
References 1. Hara T, Hara T, Yamada Y. “Equator ring ” for maintenance of the completely circular contour of the capsular bag equator after cataract removal. Ophthalmic Surg 1991; 22:358 –359 2. Cionni RJ, Osher RH. Endocapsular ring approach to the subluxed cataractous lens. J Cataract Refract Surg 1995; 21:245–249 3. Cionni RJ, Osher RH. Management of profound zonular dialysis or weakness with a new endocapsular ring designed for scleral fixation. J Cataract Refract Surg 1998; 24:1299 –1306 4. 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 5. Strenn K, Menapace R, Vass C. Capsular bag shrinkage after implantation of an open-loop silicone lens and a poly(methyl methacrylate) IOL and capsule tension ring. J Cataract Refract Surg 1997; 23:1543–1547 6. Kugelberg U, Zetterstro¨m C, Lundgren B, et al. Aftercataract and ocular growth in newborn rabbit eyes implanted with a capsule tension ring. J Cataract Refract Surg 1997; 23:635– 640 7. Zetterstro¨m C, Kugelberg U, Oscarson C. Cataract surgery in children with capsulorhexis of anterior and posterior capsules and heparin-surface-modified intraocular lenses. J Cataract Refract Surg 1994; 20:599 – 601 8. Koch DD, Kohnen T. Retrospective comparison of techniques to prevent secondary cataract formation following posterior chamber intraocular lens implantation in infants and children. J Cataract Refract Surg 1997; 23: 657– 663
J CATARACT REFRACT SURG—VOL 26, JUNE 2000
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CASE REPORTS: DIETLEIN
9. Peyman GA, Raichand M, Goldberg MF, Ritacca D. Management of subluxated and dislocated lenses with vitrophage. Br J Ophthalmol 1979; 63:771–778 10. Plager DA, Parks MM, Helveston EM, Ellis FD. Surgical treatment of subluxated lenses in children. Ophthalmology 1992; 99:1018 –1021; discussion by AW Biglan, 1022–1023
940
11. Salehpour O, Lavy T, Leonard J, Taylor D. The surgical management of nontraumatic ectopic lenses. J Pediatr Ophthalmol Strabismus 1996; 33: 8 –13 12. Zetterstro¨m C, Lundvall A, Weeber H Jr, Jeeves M. Sulcus fixation without capsular support in children. J Cataract Refract Surg 1999; 25:776 –781
J CATARACT REFRACT SURG—VOL 26, JUNE 2000