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Management of bilateral anterior dislocation of a lens in a child with Marfan’s syndrome
Management of bilateral anterior dislocation of a lens in a child with Marfan’s syndrome Abhay R. Vasavada, MS, FRCS, M.R. Praveen, DO, MS, Chirag Desai, MS A 13-year-old boy with Marfan’s syndrome presented with bilateral, acute, complete anterior dislocation of the crystalline lens. The lens substance was aspirated intracapsularly after 2 linear capsulotomies were created. The empty capsular bag was removed from the eye as a vitrectome was used to severe the adhesions between the capsule and vitreous face through 1 paracentesis and a forceps was used to pull the capsule through the other paracentesis. Aphakia was corrected by glasses and contact lenses. Two years after surgery, the best corrected visual acuity was 20/32 in both eyes. J Cataract Refract Surg 2003; 29:609 – 613 © 2003 ASCRS and ESCRS
E
ctopia lentis in children poses a challenge to the ophthalmologist. Managing the anterior dislocation of the lens into the anterior chamber demands great skill as the lens is entirely displaced and there are no zonules. We report a case of bilateral, severe, acute anterior dislocation of the lens in a 13-year-old and how it was managed.
Case Report A 13-year-old boy was seen at our clinic in August 1997 for poor vision bilaterally. The refraction was ⫺8.00 diopters (D) sphere in the right eye and ⫺9.00 D sphere in the left eye with a best corrected visual acuity (BCVA) of 20/80 and 20/125, respectively. A slitlamp examination showed ectopia lentis with inferior and temporal subluxation of the lens and a small pupil. The intraocular pressure (IOP) was normal in both eyes. One week before his visit to our clinic, the patient had an episode of severe pain, redness, watering, and photophobia with sudden dimness of vision in the left eye. He was found to have severe inflammation with anterior dislocation of lens in
Accepted for publication May 20, 2002. From the Iladevi Cataract & IOL Research Centre, Raghudeep Eye Clinic, Ahmedabad, India. Dr. Rupal H. Trivedi, junior consultant, Iladevi Cataract & IOL Research Centre, Ahmedabad, India, provided assistance. Reprint requests to Abhay R. Vasavada, Iladevi Cataract & IOL Research Centre, Gurukul Road, Memnagar, Ahmedabad–380 052, India. E-mail: [email protected]. © 2003 ASCRS and ESCRS Published by Elsevier Science Inc.
the left eye, which was totally inflamed. His local physician prescribed glaucoma medications and advised him to maintain a supine position. The lens returned to its original position. His physician asked him to continue using pilocarpine 2% eyedrops in the left eye; we advised him to continue to do so and come in for an examination. On general examination, the patient was diagnosed with Marfan’s syndrome with an arm span length of 64 cm. An x-ray of the spine revealed osteoporosis of the dorsi lumbar spine and pelvis. There was compression and reduction in the height of the vertebral bodies in the lower dorsal and lumbar regions. There was no evidence of craniostenosis or osteoporosis of the knee joints. There was minimal genu valgus deformity of the the knee joints. No deformity of the hands or evidence of arachnodactyly, microcephalus, or metaphyseal cupping was noted. A 2-dimensional echocardiograph revealed mitral valve prolapse with slight mitral regurgitation and a mildly dilated aortic root. A chest x-ray appeared normal. There was no sugar in the urine. The results of other investigations such as a hemogram were normal. The platelet count was 593 k/L and the erythrocyte sedimentation rate, 65.0 mm/hour. Renal function tests were within normal limits, and a urine nitro-prusside test was negative, ruling out homocystinuria. The family history was unremarkable. Three months later, the patient reported to our clinic with severe inflammation in both eyes. A week before, he had had a similar episode of acute glaucoma for which he was successfully treated with a medical regimen. He was using pilocarpine eyedrops 2% twice daily in the left eye. The IOP was higher than 40 mm Hg in both eyes. At his local clinic, he was given an intravenous injection of mannitol 350 mL and prescribed oral acetazolamide (Diamox威) 250 mg 4 times a day and timolol maleate 0.5% eyedrops 2 times a day. The IOP after treatment was 36 mm Hg in both eyes. 0886-3350/03/$–see front matter doi:10.1016/S0886-3350(02)01529-8
CASE REPORTS: VASAVADA
Our examination showed a visual acuity of counting fingers at 2 m in the right eye and counting fingers close to face in the left eye. The IOP was 50 mm Hg and 54 mm Hg, respectively. The anterior segment had severe corneal epithelial and stromal edema. The lens in the anterior chamber was touching the corneal endothelium and was anteriorly dislocated. The other details could not be well visualized. The patient was given an intravenous injection of mannitol 20% (300 mL). After treatment, the IOP was 25.8 mm Hg in both eyes. The patient was also given levobunolol eyedrops (Betagan威) twice daily in both eyes. The pupil was semidilated and fixed. The IOP was 46.9 mm Hg in the right eye and 27.2 mm Hg in the left eye. Another intravenous injection of mannitol was administered, and the Betagan eyedrops were continued. Visual acuity dropped to accurate light projection in both eyes. The right eye was operated on first (Figure 1) using a closed-chamber technique. Two limbal paracenteses were made on either side. Sodium hyaluronate 1.4% (Healon GV威) was injected into the anterior chamber to push the lens posterior, creating a space between it and the corneal endothelium. This maneuver failed to create a comfortable working space for manipulation. A slit knife was placed inside the lens through 2 paracenteses to create 2 linear capsulotomies in the area of the equator. The lens was stabilized with an iris spatula during this procedure (Figure 2). Lens aspiration was performed intracapsularly using bimanual irrigation/aspiration (I/A) through the capsulotomies in a well-controlled linear manner (Figure 3). The machine settings were vacuum, 300 mg Hg; aspiration flow, 20 cc/minute; and bottle height, 60 cm above the patient’s eye. Care was taken to continue the procedure entirely within the capsular bag. The 2 linear capsulotomies functioned as strong margins of a capsulorhexis, which achieved a closed bag and helped restrict turbulence in the bag, preventing damage to the corneal endothelium. They also helped with vitreous hydration, preventing vitreous disturbance. After complete removal of the lens substance, the empty capsular bag was left in the anterior chamber. The bag was removed gradually through 1 paracentesis with a forceps while through the other incision, a vitrector was used to cut the adhesions between the posterior capsule and the vitreous (Figure 4). There was no vitreous disturbance before the bag was removed. An elective vitrectomy was performed to sever the adhesions between the capsular bag and vitreous face. The capsular bag was not pulled out of the eye in a single stroke. Instead, a forceps was introduced through the paracentesis to grasp the empty bag. Another forceps was used to help introduce the vitrectome port behind the bag, with the port facing the anterior vitreous. Simultaneously, the anterior vitrectomy was performed and the empty capsular bag pulled with a forceps. The surgeon made sure the vitrectomy preceded the pull to reduce traction on the vitreous face and peripheral retina. He continued severing the adhesions through the vitrectome 610
Figure 1. (Vasavada) Preoperative photograph of complete anterior dislocation of the crystalline lens.
Figure 2. (Vasavada) Stabilization of the lens with an iris spatula during linear capsulotomy.
Figure 3. (Vasavada) Intracapsular aspiration of the lens.
J CATARACT REFRACT SURG—VOL 29, MARCH 2003
CASE REPORTS: VASAVADA
Figure 4. (Vasavada) Simultaneous removal of the bag with a vitrectome and forceps.
while progressively pulling the bag from the eye. Swiping the iris spatula across the pupillary margins ensured and confirmed the absence of vitreous incarceration in the anterior chamber. A similar procedure was repeated in the left eye 1 week later. No iridectomy was performed in either eye. One day postoperatively, a slitlamp examination showed diffuse corneal stromal edema, a deep anterior chamber, a round pupil, and multiple peripheral patches of iris atrophy in both eyes. A good red reflux was noted on retroillumination. One week postoperatively, persistent stromal edema was present from the longstanding anterior dislocation of the lens and its contact with the corneal endothelium. Iris atrophic patches caused by a continuous IOP rise were noted. The anterior chamber was deep and the pupil round and reactive bilaterally. A good red reflux was noted on ret-
roillumination. The IOP was 12.2 mm Hg in the right eye and 17.3 mm Hg in the left eye. The patient was placed on a contact lens trial. The BCVA was 20/80 in both eyes with a refraction of ⫹11.00 ⫹1.50 ⫻ 90 in the right eye and ⫹11.00 D sphere in the left eye. At 1 month, both eyes had a clear cornea, deep anterior chamber, iris atrophic patches, 3.0 mm round pupil, no vitreous in the anterior chamber, and surgical aphakia (Figure 5). On fundus examination, both eyes had a cup-to-disc ratio of 0.3 with a healthy appearing, pink neuroretinal rim and no splinter hemorrhages on the optic disc. The IOP was 9 mm Hg in both eyes. The BCVA was 20/50 with ⫹11.0 D sphere in the right eye and 20/50 with ⫹12.00 ⫹1.00 ⫻ 90 in the left eye. At 1 year, the anterior segment was quiet and the IOP was 12 mm Hg in both eyes. The BCVA in both eyes was 20/32 with a refraction of ⫹10.00 ⫹2.00 ⫻ 100 in the right eye and ⫹11.00 ⫹1.00 ⫻ 80 with a near add of ⫹2.00 D sphere in the left eye. The fundus was within normal limits bilaterally. At 2 years, the anterior segment was quiet and the IOP was 9 mm Hg in both eyes. The BCVA was 20/32 bilaterally with a refraction of ⫹10.00 ⫹2.00 ⫻ 100 in the right eye and ⫹11.00 D ⫹1.00 ⫻ 80 with a near add of ⫹2.00 D sphere in the left eye (Figure 6). The fundus was within normal limits in both eyes.
Discussion Treating ectopia lentis with anterior dislocation of the lens in children is challenging. Scleral IOL fixation is the first option.1 It takes skill to surgically manage the anterior dislocation of the lens into the anterior chamber as the lens is entirely displaced and there are no zonules. There are 2 major challenges when removing the lens: (1) preserving the bag through a closed system and
Figure 5. (Vasavada) One-month postoperative follow-up photographs show patches of iris atrophy. Left: Right eye. Right: Left eye. J CATARACT REFRACT SURG—VOL 29, MARCH 2003
611
CASE REPORTS: VASAVADA
Figure 6. (Vasavada) Two-year postoperative follow-up photographs show gross patches of iris atrophy. Left: Right eye. Right: Left eye.
avoiding damage to the corneal endothelium and other anterior segment structures as well as vitreous disturbance and (2) correction of aphakia. Early studies of the surgical removal of subluxated crystalline lenses reported a high incidence of surgical complications and relatively poor visual outcomes.2 However, the development of closed-system techniques significantly improved the prognosis and visual outcomes of the procedure.3,4 The easy option would be to open the eye to remove the cataract intracapsularly and perform an open-sky anterior vitrectomy. However, this approach had drawbacks, limitations, and complications.5 Another popular option is bimanual vitrectomy followed by lensectomy; that is, removing the entire lens with the capsular bag. The major drawback of this technique is that it generates more turbulence in the eye and the incidence of corneal endothelial damage by movement of the lens fragments is significantly high. This could be serious in already compromised eyes with an injured endothelium. The 2 linear capsulotomies created in the equatorial region produced a closed, empty bag within the closed chamber. This restricted turbulence in the bag and protected the corneal endothelium. Conventional anterior capsulotomy was not feasible as the Healon GV injection failed to create an adequate and safe working space for manipulation. We decided to create the 2 capsulotomies at opposite ends to produce a second closed chamber within the anterior chamber, restricting turbulence intracapsularly and protecting the corneal endothelium. 612
We believe this was helpful during lens aspiration as the corneal endothelium was already compromised as a result of sustained high IOP over a long period. It also reduced the possibility of disturbing the vitreous during lens removal. Linear capsulotomies at the equator of the lens are routinely performed during pars plana lensectomy, as is removal of lens material from within the bag, keeping the anterior capsule intact. The entire lens was removed in the bag to protect the corneal endothelium and avoid vitreous hydration. Restricting the fluid dynamics inside the bag allowed rapid and thorough lens aspiration. This left an empty capsular bag, and I/A was good. There was no peripheral extension of the capsulotomies during the procedure, perhaps because of the patient’s young age, inflamed capsule, and minimal parameter settings. The empty capsular bag was not pulled out of the eye in a single stroke. Instead, a forceps was introduced through the paracentesis to grasp the empty bag and another was used to introduce the vitrectome port and place it behind the bag, with the port facing the anterior vitreous. Anterior vitrectomy and withdrawal of the bag from the eye with a forceps were performed simultaneously. We made sure the vitrectomy preceded the pull to reduce potential traction on the vitreous base.6 Patients with Marfan’s syndrome have an increased incidence of retinal detachment.7 This combination of vitrectomy and removal of the bag was continued until the entire bag was out of the eye. This was key to reducing traction on the vitreous base.
J CATARACT REFRACT SURG—VOL 29, MARCH 2003
CASE REPORTS: VASAVADA
A planned vitrectomy was performed not because of vitreous loss but rather to sever the adhesions between the capsular bag and vitreous face caused by longstanding inflammation. An alternative would be to remove the capsule by a 2-port anterior chamber vitrectomy. However, we believe this could result in incomplete removal of the capsular bag as it might inadvertently sink into the vitreous cavity because of high turbulence in the anterior chamber. No iridectomy was performed, and the minimal trauma inflicted during surgery prevented pupillary block. We were surprised to see that the pupils were round and reactive to light even though the IOP was elevated. We attribute this to the patient’s young age, at which the tissues are highly resistant to insult. The patient was left aphakic in both eyes. A contact lens trial was discussed with his parents, and thorough counseling was done. The patient’s lifestyle and chances of compliance did not make contact lenses a suitable option. We do not know the long-term consequences of primary IOL implantation in children. Another important constraint was the inflamed eyes with corneal endothelial decompensation and the possibility of persistent postoperative glaucoma. An additional problem was bilateral anterior lens dislocation. For these reasons, we were reluctant to fixate an IOL in both eyes. We gave the patient several options for correcting the aphakia including a contact lens trial, corneal refractive surgery, and secondary IOL implantation. With an empty but intact capsular bag, we had 2 options: primary scleral fixation2 and secondary scleral fixation. We decided against primary scleral fixation as the patient was a child with bilateral dislocated lenses and eyes already compromised by glaucoma and inflammation. If we had aimed for primary scleral fixation, this could have been achieved by converting the 2 linear capsulotomies into a continuous curvilinear capsulo-
rhexis followed by scleral fixation of the capsular bag and Cionni capsular tension ring and IOL.8,9 The main prerequisite is conversion of the linear capsulotomies to a single capsulorhexis. We considered this option but found it to be too demanding. The other theoretical consideration would be scleral fixation of the IOL. In summary, the patient had satisfactory subjective and objective outcomes. He has the option of correcting the aphakia in the future with a contact lens, secondary IOL implantation, or corneal refractive surgery.
References 1. Lam DSC, Ng JSK, Fan DSP, et al. Short-term results of scleral intraocular lens fixation in children. J Cataract Refract Surg 1998; 24:1474 –1479 2. Jarrett WH II. Dislocation of the lens; a study of 166 hospitalized cases. Arch Ophthalmol 1967; 78:289 –296 3. Hakin KN, Jacobs M, Rosen P, et al. Management of the subluxed crystalline lens. Ophthalmology 1992; 99:542– 545 4. Peyman GA, Raichand M, Goldberg MF, Ritacca D. Management of subluxated and dislocated lenses with the vitrophage. Br J Ophthalmol 1979; 63:771–778 5. Morley MG. Pars plana lensectomy for primary extraction and removal of lens fragments. In: Steinert RF, ed, Cataract Surgery: Technique, Complications, & Management. Philadelphia, PA, Saunders, 1995; chap 15, 192– 195 6. Nelson LB, Maumenee IH. Ectopia lentis. Surv Ophthalmol 1982; 27:143–160 7. Cross HE, Jensen AD. Ocular manifestations in the Marfan syndrome and homocystinuria. Am J Ophthalmol 1973; 75:405–420 8. 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 9. Lam DSC, Young AL, Leung ATS, et al. Scleral fixation of a capsular tension ring for severe ectopia lentis. J Cataract Refract Surg 2000; 26:609 –612
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E
ctopia lentis in children poses a challenge to the ophthalmologist. Managing the anterior dislocation of the lens into the anterior chamber demands great skill as the lens is entirely displaced and there are no zonules. We report a case of bilateral, severe, acute anterior dislocation of the lens in a 13-year-old and how it was managed.
Case Report A 13-year-old boy was seen at our clinic in August 1997 for poor vision bilaterally. The refraction was ⫺8.00 diopters (D) sphere in the right eye and ⫺9.00 D sphere in the left eye with a best corrected visual acuity (BCVA) of 20/80 and 20/125, respectively. A slitlamp examination showed ectopia lentis with inferior and temporal subluxation of the lens and a small pupil. The intraocular pressure (IOP) was normal in both eyes. One week before his visit to our clinic, the patient had an episode of severe pain, redness, watering, and photophobia with sudden dimness of vision in the left eye. He was found to have severe inflammation with anterior dislocation of lens in
Accepted for publication May 20, 2002. From the Iladevi Cataract & IOL Research Centre, Raghudeep Eye Clinic, Ahmedabad, India. Dr. Rupal H. Trivedi, junior consultant, Iladevi Cataract & IOL Research Centre, Ahmedabad, India, provided assistance. Reprint requests to Abhay R. Vasavada, Iladevi Cataract & IOL Research Centre, Gurukul Road, Memnagar, Ahmedabad–380 052, India. E-mail: [email protected]. © 2003 ASCRS and ESCRS Published by Elsevier Science Inc.
the left eye, which was totally inflamed. His local physician prescribed glaucoma medications and advised him to maintain a supine position. The lens returned to its original position. His physician asked him to continue using pilocarpine 2% eyedrops in the left eye; we advised him to continue to do so and come in for an examination. On general examination, the patient was diagnosed with Marfan’s syndrome with an arm span length of 64 cm. An x-ray of the spine revealed osteoporosis of the dorsi lumbar spine and pelvis. There was compression and reduction in the height of the vertebral bodies in the lower dorsal and lumbar regions. There was no evidence of craniostenosis or osteoporosis of the knee joints. There was minimal genu valgus deformity of the the knee joints. No deformity of the hands or evidence of arachnodactyly, microcephalus, or metaphyseal cupping was noted. A 2-dimensional echocardiograph revealed mitral valve prolapse with slight mitral regurgitation and a mildly dilated aortic root. A chest x-ray appeared normal. There was no sugar in the urine. The results of other investigations such as a hemogram were normal. The platelet count was 593 k/L and the erythrocyte sedimentation rate, 65.0 mm/hour. Renal function tests were within normal limits, and a urine nitro-prusside test was negative, ruling out homocystinuria. The family history was unremarkable. Three months later, the patient reported to our clinic with severe inflammation in both eyes. A week before, he had had a similar episode of acute glaucoma for which he was successfully treated with a medical regimen. He was using pilocarpine eyedrops 2% twice daily in the left eye. The IOP was higher than 40 mm Hg in both eyes. At his local clinic, he was given an intravenous injection of mannitol 350 mL and prescribed oral acetazolamide (Diamox威) 250 mg 4 times a day and timolol maleate 0.5% eyedrops 2 times a day. The IOP after treatment was 36 mm Hg in both eyes. 0886-3350/03/$–see front matter doi:10.1016/S0886-3350(02)01529-8
CASE REPORTS: VASAVADA
Our examination showed a visual acuity of counting fingers at 2 m in the right eye and counting fingers close to face in the left eye. The IOP was 50 mm Hg and 54 mm Hg, respectively. The anterior segment had severe corneal epithelial and stromal edema. The lens in the anterior chamber was touching the corneal endothelium and was anteriorly dislocated. The other details could not be well visualized. The patient was given an intravenous injection of mannitol 20% (300 mL). After treatment, the IOP was 25.8 mm Hg in both eyes. The patient was also given levobunolol eyedrops (Betagan威) twice daily in both eyes. The pupil was semidilated and fixed. The IOP was 46.9 mm Hg in the right eye and 27.2 mm Hg in the left eye. Another intravenous injection of mannitol was administered, and the Betagan eyedrops were continued. Visual acuity dropped to accurate light projection in both eyes. The right eye was operated on first (Figure 1) using a closed-chamber technique. Two limbal paracenteses were made on either side. Sodium hyaluronate 1.4% (Healon GV威) was injected into the anterior chamber to push the lens posterior, creating a space between it and the corneal endothelium. This maneuver failed to create a comfortable working space for manipulation. A slit knife was placed inside the lens through 2 paracenteses to create 2 linear capsulotomies in the area of the equator. The lens was stabilized with an iris spatula during this procedure (Figure 2). Lens aspiration was performed intracapsularly using bimanual irrigation/aspiration (I/A) through the capsulotomies in a well-controlled linear manner (Figure 3). The machine settings were vacuum, 300 mg Hg; aspiration flow, 20 cc/minute; and bottle height, 60 cm above the patient’s eye. Care was taken to continue the procedure entirely within the capsular bag. The 2 linear capsulotomies functioned as strong margins of a capsulorhexis, which achieved a closed bag and helped restrict turbulence in the bag, preventing damage to the corneal endothelium. They also helped with vitreous hydration, preventing vitreous disturbance. After complete removal of the lens substance, the empty capsular bag was left in the anterior chamber. The bag was removed gradually through 1 paracentesis with a forceps while through the other incision, a vitrector was used to cut the adhesions between the posterior capsule and the vitreous (Figure 4). There was no vitreous disturbance before the bag was removed. An elective vitrectomy was performed to sever the adhesions between the capsular bag and vitreous face. The capsular bag was not pulled out of the eye in a single stroke. Instead, a forceps was introduced through the paracentesis to grasp the empty bag. Another forceps was used to help introduce the vitrectome port behind the bag, with the port facing the anterior vitreous. Simultaneously, the anterior vitrectomy was performed and the empty capsular bag pulled with a forceps. The surgeon made sure the vitrectomy preceded the pull to reduce traction on the vitreous face and peripheral retina. He continued severing the adhesions through the vitrectome 610
Figure 1. (Vasavada) Preoperative photograph of complete anterior dislocation of the crystalline lens.
Figure 2. (Vasavada) Stabilization of the lens with an iris spatula during linear capsulotomy.
Figure 3. (Vasavada) Intracapsular aspiration of the lens.
J CATARACT REFRACT SURG—VOL 29, MARCH 2003
CASE REPORTS: VASAVADA
Figure 4. (Vasavada) Simultaneous removal of the bag with a vitrectome and forceps.
while progressively pulling the bag from the eye. Swiping the iris spatula across the pupillary margins ensured and confirmed the absence of vitreous incarceration in the anterior chamber. A similar procedure was repeated in the left eye 1 week later. No iridectomy was performed in either eye. One day postoperatively, a slitlamp examination showed diffuse corneal stromal edema, a deep anterior chamber, a round pupil, and multiple peripheral patches of iris atrophy in both eyes. A good red reflux was noted on retroillumination. One week postoperatively, persistent stromal edema was present from the longstanding anterior dislocation of the lens and its contact with the corneal endothelium. Iris atrophic patches caused by a continuous IOP rise were noted. The anterior chamber was deep and the pupil round and reactive bilaterally. A good red reflux was noted on ret-
roillumination. The IOP was 12.2 mm Hg in the right eye and 17.3 mm Hg in the left eye. The patient was placed on a contact lens trial. The BCVA was 20/80 in both eyes with a refraction of ⫹11.00 ⫹1.50 ⫻ 90 in the right eye and ⫹11.00 D sphere in the left eye. At 1 month, both eyes had a clear cornea, deep anterior chamber, iris atrophic patches, 3.0 mm round pupil, no vitreous in the anterior chamber, and surgical aphakia (Figure 5). On fundus examination, both eyes had a cup-to-disc ratio of 0.3 with a healthy appearing, pink neuroretinal rim and no splinter hemorrhages on the optic disc. The IOP was 9 mm Hg in both eyes. The BCVA was 20/50 with ⫹11.0 D sphere in the right eye and 20/50 with ⫹12.00 ⫹1.00 ⫻ 90 in the left eye. At 1 year, the anterior segment was quiet and the IOP was 12 mm Hg in both eyes. The BCVA in both eyes was 20/32 with a refraction of ⫹10.00 ⫹2.00 ⫻ 100 in the right eye and ⫹11.00 ⫹1.00 ⫻ 80 with a near add of ⫹2.00 D sphere in the left eye. The fundus was within normal limits bilaterally. At 2 years, the anterior segment was quiet and the IOP was 9 mm Hg in both eyes. The BCVA was 20/32 bilaterally with a refraction of ⫹10.00 ⫹2.00 ⫻ 100 in the right eye and ⫹11.00 D ⫹1.00 ⫻ 80 with a near add of ⫹2.00 D sphere in the left eye (Figure 6). The fundus was within normal limits in both eyes.
Discussion Treating ectopia lentis with anterior dislocation of the lens in children is challenging. Scleral IOL fixation is the first option.1 It takes skill to surgically manage the anterior dislocation of the lens into the anterior chamber as the lens is entirely displaced and there are no zonules. There are 2 major challenges when removing the lens: (1) preserving the bag through a closed system and
Figure 5. (Vasavada) One-month postoperative follow-up photographs show patches of iris atrophy. Left: Right eye. Right: Left eye. J CATARACT REFRACT SURG—VOL 29, MARCH 2003
611
CASE REPORTS: VASAVADA
Figure 6. (Vasavada) Two-year postoperative follow-up photographs show gross patches of iris atrophy. Left: Right eye. Right: Left eye.
avoiding damage to the corneal endothelium and other anterior segment structures as well as vitreous disturbance and (2) correction of aphakia. Early studies of the surgical removal of subluxated crystalline lenses reported a high incidence of surgical complications and relatively poor visual outcomes.2 However, the development of closed-system techniques significantly improved the prognosis and visual outcomes of the procedure.3,4 The easy option would be to open the eye to remove the cataract intracapsularly and perform an open-sky anterior vitrectomy. However, this approach had drawbacks, limitations, and complications.5 Another popular option is bimanual vitrectomy followed by lensectomy; that is, removing the entire lens with the capsular bag. The major drawback of this technique is that it generates more turbulence in the eye and the incidence of corneal endothelial damage by movement of the lens fragments is significantly high. This could be serious in already compromised eyes with an injured endothelium. The 2 linear capsulotomies created in the equatorial region produced a closed, empty bag within the closed chamber. This restricted turbulence in the bag and protected the corneal endothelium. Conventional anterior capsulotomy was not feasible as the Healon GV injection failed to create an adequate and safe working space for manipulation. We decided to create the 2 capsulotomies at opposite ends to produce a second closed chamber within the anterior chamber, restricting turbulence intracapsularly and protecting the corneal endothelium. 612
We believe this was helpful during lens aspiration as the corneal endothelium was already compromised as a result of sustained high IOP over a long period. It also reduced the possibility of disturbing the vitreous during lens removal. Linear capsulotomies at the equator of the lens are routinely performed during pars plana lensectomy, as is removal of lens material from within the bag, keeping the anterior capsule intact. The entire lens was removed in the bag to protect the corneal endothelium and avoid vitreous hydration. Restricting the fluid dynamics inside the bag allowed rapid and thorough lens aspiration. This left an empty capsular bag, and I/A was good. There was no peripheral extension of the capsulotomies during the procedure, perhaps because of the patient’s young age, inflamed capsule, and minimal parameter settings. The empty capsular bag was not pulled out of the eye in a single stroke. Instead, a forceps was introduced through the paracentesis to grasp the empty bag and another was used to introduce the vitrectome port and place it behind the bag, with the port facing the anterior vitreous. Anterior vitrectomy and withdrawal of the bag from the eye with a forceps were performed simultaneously. We made sure the vitrectomy preceded the pull to reduce potential traction on the vitreous base.6 Patients with Marfan’s syndrome have an increased incidence of retinal detachment.7 This combination of vitrectomy and removal of the bag was continued until the entire bag was out of the eye. This was key to reducing traction on the vitreous base.
J CATARACT REFRACT SURG—VOL 29, MARCH 2003
CASE REPORTS: VASAVADA
A planned vitrectomy was performed not because of vitreous loss but rather to sever the adhesions between the capsular bag and vitreous face caused by longstanding inflammation. An alternative would be to remove the capsule by a 2-port anterior chamber vitrectomy. However, we believe this could result in incomplete removal of the capsular bag as it might inadvertently sink into the vitreous cavity because of high turbulence in the anterior chamber. No iridectomy was performed, and the minimal trauma inflicted during surgery prevented pupillary block. We were surprised to see that the pupils were round and reactive to light even though the IOP was elevated. We attribute this to the patient’s young age, at which the tissues are highly resistant to insult. The patient was left aphakic in both eyes. A contact lens trial was discussed with his parents, and thorough counseling was done. The patient’s lifestyle and chances of compliance did not make contact lenses a suitable option. We do not know the long-term consequences of primary IOL implantation in children. Another important constraint was the inflamed eyes with corneal endothelial decompensation and the possibility of persistent postoperative glaucoma. An additional problem was bilateral anterior lens dislocation. For these reasons, we were reluctant to fixate an IOL in both eyes. We gave the patient several options for correcting the aphakia including a contact lens trial, corneal refractive surgery, and secondary IOL implantation. With an empty but intact capsular bag, we had 2 options: primary scleral fixation2 and secondary scleral fixation. We decided against primary scleral fixation as the patient was a child with bilateral dislocated lenses and eyes already compromised by glaucoma and inflammation. If we had aimed for primary scleral fixation, this could have been achieved by converting the 2 linear capsulotomies into a continuous curvilinear capsulo-
rhexis followed by scleral fixation of the capsular bag and Cionni capsular tension ring and IOL.8,9 The main prerequisite is conversion of the linear capsulotomies to a single capsulorhexis. We considered this option but found it to be too demanding. The other theoretical consideration would be scleral fixation of the IOL. In summary, the patient had satisfactory subjective and objective outcomes. He has the option of correcting the aphakia in the future with a contact lens, secondary IOL implantation, or corneal refractive surgery.
References 1. Lam DSC, Ng JSK, Fan DSP, et al. Short-term results of scleral intraocular lens fixation in children. J Cataract Refract Surg 1998; 24:1474 –1479 2. Jarrett WH II. Dislocation of the lens; a study of 166 hospitalized cases. Arch Ophthalmol 1967; 78:289 –296 3. Hakin KN, Jacobs M, Rosen P, et al. Management of the subluxed crystalline lens. Ophthalmology 1992; 99:542– 545 4. Peyman GA, Raichand M, Goldberg MF, Ritacca D. Management of subluxated and dislocated lenses with the vitrophage. Br J Ophthalmol 1979; 63:771–778 5. Morley MG. Pars plana lensectomy for primary extraction and removal of lens fragments. In: Steinert RF, ed, Cataract Surgery: Technique, Complications, & Management. Philadelphia, PA, Saunders, 1995; chap 15, 192– 195 6. Nelson LB, Maumenee IH. Ectopia lentis. Surv Ophthalmol 1982; 27:143–160 7. Cross HE, Jensen AD. Ocular manifestations in the Marfan syndrome and homocystinuria. Am J Ophthalmol 1973; 75:405–420 8. 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 9. Lam DSC, Young AL, Leung ATS, et al. Scleral fixation of a capsular tension ring for severe ectopia lentis. J Cataract Refract Surg 2000; 26:609 –612
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