CONSULTATION SECTION
Cataract Surgical Problem Edited by Samuel Masket, MD Online Video A 46-year-old man was referred for cataract management by his vitreoretinal specialist. He has a history of the rare oculo–dental–digital syndrome and has had multiple surgical procedures for cleft palate, syndactyly, and jaw reconstruction. From an ocular standpoint he has had poor vision throughout life, albeit functioning well as a sighted individual. However, visual function has gradually been reduced over the past 20 years as a result of progressive cataract formation. His ocular history includes nystagmus; bilateral microcornea; bilateral inferonasal coloboma of the iris, ciliary body, and choroid including the posterior pole; and large-angle esotropia, fixating with the strongly preferred right eye. The referring retinal specialist indicates that the posterior pole cannot be visualized but is unchanged from earlier, as imaged by ultrasonography. Eschewing surgery until the present, the patient has maintained pupil dilation with chronic topical atropine use and has functioned with aphakic spectacles by viewing around the cataracts through the aphakic area, taking advantage of the inferior nasal coloboma in the right eye. However, progressive zonulysis has induced inferior subluxation of the mature cataract in the right eye, interfering with that strategy (Figure 1). He now requests cataract surgery.
Figure 1. Slitlamp photomicrograph shows inferior subluxation of a mature cataract with a fibrotic anterior capsule, microcornea, and an inferonasal coloboma.
338
Q 2013 ASCRS and ESCRS Published by Elsevier Inc.
Current findings include a corrected distance visual acuity (CDVA) of finger counting at 3 feet in the right eye and finger counting at 1 foot in the left eye. The intraocular pressure is 14 mm Hg in both eyes. There is esotropia of approximately 70 prism diopters by light reflex with low-amplitude horizontal nystagmus in all gaze directions; fixates right eye. Slitlamp findings (Figure 1) in the right eye show microcornea (8.5 mm diameter), a deep anterior chamber, an inferonasal iris coloboma, and a mature cataract with modest inferior subluxation, as can be seen around the cataract superotemporally. There is moderate pseudophacodonesis and marked fibrosis of the central aspect of the anterior capsule. There is no view of the posterior segment, although the history suggests a large inferior coloboma and an attached retina. Findings in the left eye are similar. Biometry by A-scan ultrasonography shows an axial length (AL) of 25.12 mm in the right eye and 25.48 mm in the left eye. The keratometry (K) readings are 41.75/ 39.25 @ 25 and 43.87/42.12 @ 150, respectively. Given the patient’s desire for improved vision, what would you suggest as the best approach?
- This brunescent, subluxated lens with minimal, if any, zonular support and fibrotic anterior capsule in an anomalous eye is daunting. The fibrotic plaque, which appears to reach to the equator of the lens, makes it very unlikely that a continuous curvilinear capsulorhexis (CCC) will be possible, which is needed if one is to hope for a bag-sparing surgery with scleral fixation (my usual technique for subluxated cataracts). Use of a modified capsular tension ring (CTR) would likely not be possible even with femtosecond laser–assisted capsulotomy because capsule hooks could not be applied to an insecure capsule edge and the insertion of the ring itself would risk splitting the capsule across a cut fibrous scar. Although in a normal eye brunescence does not raise my blood pressure, this profoundly dense lens adds to the challenge of a phacoemulsification strategy, which I think would be fraught with risk given this constellation of findings. Because the patient was referred by a vitreoretinal surgeon, I presume the surgeon has ruled out a lensectomy. With such a loose lens, small-incision manual extracapsular surgery would be challenging 0886-3350/$ - see front matter http://dx.doi.org/10.1016/j.jcrs.2013.11.017
CONSULTATION SECTION
to perform without vitreous loss. Although intracapsular cataract extraction (ICCE) is anachronistic and rare and would require an incision that takes up much of the limbal real estate, it bears consideration and would probably be the default choice if the patient cannot live with his current status. The patient would be most lucky if nature would allow the lens to totally dislocate or we had the skill and the fortitude to couch it. The patient was accustomed to aphakic spectacle correction before the lens subluxated into the colobomatous space. This correction was possibly helpful in magnifying the image off the anomalous posterior pole, making the situation livable. With a microcornea and such disproportionate measurements, what intraocular lens (IOL) power to select would be a guesstimation at best. In my opinion, lens fixation to an abnormal colobomatous iris is not desirable, and a scleral-fixated or glued IOL would require a large vitrectomy, so the risk outweighs the benefit in the absence of macular function. An anterior chamber (AC) IOL is out of the question because of the microcornea. I would favor an aphakic corrective solution after ICCE is performed. Does anyone still have a cataract cryoprobe? At least we will not have to look for alpha-chymotrypsin. Lisa B. Arbisser, MD Bettendorf, Iowa, USA
- This patient requires cataract removal with IOL implantation (as determined from the biometry). Conventional phacoemulsification may not be possible due to the large subluxation and dense cataract. We would plan for ICCE with a glued transscleral-fixated posterior chamber (PC) IOL in one sitting.1 The problem in this condition is the large wound required for cataract removal because the lens may be much larger
339
than the microcornea. This can lead to sudden globe decompression and suprachoroidal hemorrhage. However, it can be managed by creating a scleral tunnel rather than a straight incision and using preplaced sutures to help to close the globe rapidly if suprachoroidal hemorrhage occurs. Under sterile conditions and peribulbar anesthesia, 2 limbus-based, partial-thickness scleral flaps (2.5 mm 2.5 mm) are made diagonally apart. Infusion is maintained through a 23-gauge trocar infusion system, a superior scleral tunnel incision is created with a keratome, and two 20-gauge sclerotomies are made under the flaps 1.0 to 1.5 mm from the limbus. The cataract is removed by ICCE using a lens vectis; nylon sutures are placed in the main wound immediately after lens removal. Closed-chamber vitrectomy is performed to remove the vitreous that has entered the AC. A 3-piece foldable IOL with poly(methyl methacrylate) (PMMA) haptics and 6.0 mm optic is introduced through the scleral tunnel wound using an injector (Figure 2, a). Closed-chamber IOL insertion is performed. The glued IOL forceps is passed through the sclerotomy, and the tip of the leading haptic is grasped and externalized. The haptic is held by an assistant or silicone tires.2 The second haptic is then flexed into the AC and pulled through the opposite sclerotomy by the handshake technique.3 When both haptics are externalized under the flaps, they are tucked into the limbus-parallel intralamellar Scharioth scleral tunnels made with a 26-gauge needle at the point of haptic externalization. The main wound is closed with 10-0 monofilament nylon and the trocar infusion removed. Air is injected into the AC, fibrin glue injected under the scleral flaps, and local pressure applied. The conjunctiva is closed with glue. An AC or iris-claw IOL may not be ideal due to the microcornea and iris coloboma. Because sutured scleral-fixated IOLs can cause pseudophacodonesis and posterior segment changes, we prefer a glued
Figure 2. a: Three-piece foldable IOL is injected via the scleral tunnel, and the leading haptic is received by the forceps through the sclerotomy in a microcornea eye. Note the closed chamber maintained by suturing the main wound. The white reflex is due to the fundus coloboma in the eye. b: The oversized haptic is trimmed intraoperatively.
J CATARACT REFRACT SURG - VOL 40, FEBRUARY 2014
Cataract Surgical Problem Edited by Samuel Masket, MD Online Video A 46-year-old man was referred for cataract management by his vitreoretinal specialist. He has a history of the rare oculo–dental–digital syndrome and has had multiple surgical procedures for cleft palate, syndactyly, and jaw reconstruction. From an ocular standpoint he has had poor vision throughout life, albeit functioning well as a sighted individual. However, visual function has gradually been reduced over the past 20 years as a result of progressive cataract formation. His ocular history includes nystagmus; bilateral microcornea; bilateral inferonasal coloboma of the iris, ciliary body, and choroid including the posterior pole; and large-angle esotropia, fixating with the strongly preferred right eye. The referring retinal specialist indicates that the posterior pole cannot be visualized but is unchanged from earlier, as imaged by ultrasonography. Eschewing surgery until the present, the patient has maintained pupil dilation with chronic topical atropine use and has functioned with aphakic spectacles by viewing around the cataracts through the aphakic area, taking advantage of the inferior nasal coloboma in the right eye. However, progressive zonulysis has induced inferior subluxation of the mature cataract in the right eye, interfering with that strategy (Figure 1). He now requests cataract surgery.
Figure 1. Slitlamp photomicrograph shows inferior subluxation of a mature cataract with a fibrotic anterior capsule, microcornea, and an inferonasal coloboma.
338
Q 2013 ASCRS and ESCRS Published by Elsevier Inc.
Current findings include a corrected distance visual acuity (CDVA) of finger counting at 3 feet in the right eye and finger counting at 1 foot in the left eye. The intraocular pressure is 14 mm Hg in both eyes. There is esotropia of approximately 70 prism diopters by light reflex with low-amplitude horizontal nystagmus in all gaze directions; fixates right eye. Slitlamp findings (Figure 1) in the right eye show microcornea (8.5 mm diameter), a deep anterior chamber, an inferonasal iris coloboma, and a mature cataract with modest inferior subluxation, as can be seen around the cataract superotemporally. There is moderate pseudophacodonesis and marked fibrosis of the central aspect of the anterior capsule. There is no view of the posterior segment, although the history suggests a large inferior coloboma and an attached retina. Findings in the left eye are similar. Biometry by A-scan ultrasonography shows an axial length (AL) of 25.12 mm in the right eye and 25.48 mm in the left eye. The keratometry (K) readings are 41.75/ 39.25 @ 25 and 43.87/42.12 @ 150, respectively. Given the patient’s desire for improved vision, what would you suggest as the best approach?
- This brunescent, subluxated lens with minimal, if any, zonular support and fibrotic anterior capsule in an anomalous eye is daunting. The fibrotic plaque, which appears to reach to the equator of the lens, makes it very unlikely that a continuous curvilinear capsulorhexis (CCC) will be possible, which is needed if one is to hope for a bag-sparing surgery with scleral fixation (my usual technique for subluxated cataracts). Use of a modified capsular tension ring (CTR) would likely not be possible even with femtosecond laser–assisted capsulotomy because capsule hooks could not be applied to an insecure capsule edge and the insertion of the ring itself would risk splitting the capsule across a cut fibrous scar. Although in a normal eye brunescence does not raise my blood pressure, this profoundly dense lens adds to the challenge of a phacoemulsification strategy, which I think would be fraught with risk given this constellation of findings. Because the patient was referred by a vitreoretinal surgeon, I presume the surgeon has ruled out a lensectomy. With such a loose lens, small-incision manual extracapsular surgery would be challenging 0886-3350/$ - see front matter http://dx.doi.org/10.1016/j.jcrs.2013.11.017
CONSULTATION SECTION
to perform without vitreous loss. Although intracapsular cataract extraction (ICCE) is anachronistic and rare and would require an incision that takes up much of the limbal real estate, it bears consideration and would probably be the default choice if the patient cannot live with his current status. The patient would be most lucky if nature would allow the lens to totally dislocate or we had the skill and the fortitude to couch it. The patient was accustomed to aphakic spectacle correction before the lens subluxated into the colobomatous space. This correction was possibly helpful in magnifying the image off the anomalous posterior pole, making the situation livable. With a microcornea and such disproportionate measurements, what intraocular lens (IOL) power to select would be a guesstimation at best. In my opinion, lens fixation to an abnormal colobomatous iris is not desirable, and a scleral-fixated or glued IOL would require a large vitrectomy, so the risk outweighs the benefit in the absence of macular function. An anterior chamber (AC) IOL is out of the question because of the microcornea. I would favor an aphakic corrective solution after ICCE is performed. Does anyone still have a cataract cryoprobe? At least we will not have to look for alpha-chymotrypsin. Lisa B. Arbisser, MD Bettendorf, Iowa, USA
- This patient requires cataract removal with IOL implantation (as determined from the biometry). Conventional phacoemulsification may not be possible due to the large subluxation and dense cataract. We would plan for ICCE with a glued transscleral-fixated posterior chamber (PC) IOL in one sitting.1 The problem in this condition is the large wound required for cataract removal because the lens may be much larger
339
than the microcornea. This can lead to sudden globe decompression and suprachoroidal hemorrhage. However, it can be managed by creating a scleral tunnel rather than a straight incision and using preplaced sutures to help to close the globe rapidly if suprachoroidal hemorrhage occurs. Under sterile conditions and peribulbar anesthesia, 2 limbus-based, partial-thickness scleral flaps (2.5 mm 2.5 mm) are made diagonally apart. Infusion is maintained through a 23-gauge trocar infusion system, a superior scleral tunnel incision is created with a keratome, and two 20-gauge sclerotomies are made under the flaps 1.0 to 1.5 mm from the limbus. The cataract is removed by ICCE using a lens vectis; nylon sutures are placed in the main wound immediately after lens removal. Closed-chamber vitrectomy is performed to remove the vitreous that has entered the AC. A 3-piece foldable IOL with poly(methyl methacrylate) (PMMA) haptics and 6.0 mm optic is introduced through the scleral tunnel wound using an injector (Figure 2, a). Closed-chamber IOL insertion is performed. The glued IOL forceps is passed through the sclerotomy, and the tip of the leading haptic is grasped and externalized. The haptic is held by an assistant or silicone tires.2 The second haptic is then flexed into the AC and pulled through the opposite sclerotomy by the handshake technique.3 When both haptics are externalized under the flaps, they are tucked into the limbus-parallel intralamellar Scharioth scleral tunnels made with a 26-gauge needle at the point of haptic externalization. The main wound is closed with 10-0 monofilament nylon and the trocar infusion removed. Air is injected into the AC, fibrin glue injected under the scleral flaps, and local pressure applied. The conjunctiva is closed with glue. An AC or iris-claw IOL may not be ideal due to the microcornea and iris coloboma. Because sutured scleral-fixated IOLs can cause pseudophacodonesis and posterior segment changes, we prefer a glued
Figure 2. a: Three-piece foldable IOL is injected via the scleral tunnel, and the leading haptic is received by the forceps through the sclerotomy in a microcornea eye. Note the closed chamber maintained by suturing the main wound. The white reflex is due to the fundus coloboma in the eye. b: The oversized haptic is trimmed intraoperatively.
J CATARACT REFRACT SURG - VOL 40, FEBRUARY 2014
