The reported pain, blurred vision, episodes of anterior segment Tyndall phenomenon, pressure spikes, and erythrocytes in the anterior aqueous in the left eye of this patient strongly suggest the presence of the full triad of the UGH syndrome. This syndrome was first presented in 1977 by Ellingson in a patient with an aphakic AC IOL.1 However, it has also been described years after the implantation of PC IOLs.2
The biomicroscopic photograph of the left eye shows a slightly inferiorly displaced 3-piece PMMA lens and a centrally clear posterior capsule. Also, a smooth-edged round peripheral iridectomy can be seen. The edge of the anterior capsule can be seen on the inferior side of the IOL only. It is likely a D-shaped anterior envelope capsulotomy was performed and most likely followed by extracapsular cataract extraction. The incision scar seems too large for a first-generation phacoemulsification procedure. During expression of the nucleus, a limited superior zonulysis or posterior capsule rupture occurred. The peripheral superior iridectomy was probably made to prevent pupillary block glaucoma due to vitreous prolapse. The 3-piece acrylic IOL was probably implanted with both haptics in the sulcus. The superior haptic presumably moved slightly anteriorly as a result of irregular anterior capsule remnants or vitreous prolapse, causing unstable sulcus fixation. Anterior segment optical coherence tomography would give more accurate information on the position of the IOL in relation to the back of the iris and possible tilting of the IOL.
I would presume that the prolonged chafing of 1 or both haptics against the posterior iris and possibly the ciliary body could cause recurrent microscopic bleeding in any patient. The slight peripheral iris atrophy that can be seen temporally could also be an indication of prolonged iris erosion. However, it is very likely that the bleeding was exacerbated by the use of an anticoagulant or aspirin-type medication, which is often used by elderly patients. The patient could also have a bleeding disorder.
If there is no way to reduce the tendency toward bleeding and the patient is motivated to have surgery, the IOL could be removed. I would create a 6.0 mm superior incision just peripheral to the limbus with 2 additional 1.5 mm stab incisions on either side. The explantation of the IOL should be straightforward with an untoothed capsule forceps and a mydriatic pupil. I do not expect adhesions of the IOL to the capsule; however, any vitreous loss could be managed by anterior vitrectomy through the pupil opening as well as via the peripheral iridotomy. Then, 0.5 cc of 3- to 4-times diluted pilocarpine 2.0% can be instilled in the anterior chamber followed by horizontal implantation of a rigid aphakic Artisan-style iris-fixated AC IOL with needle enclavation. Fortunately, this eye already has a peripheral iridectomy, so no extra precautions against acute pupillary block glaucoma have to be taken, as is usually necessary with iris-fixated IOLs.
In this eye, a 3-piece PMMA IOL with open loops has obviously been placed in the ciliary sulcus. Through the coloboma at 12 o’clock and between 5 o’clock and 8 o’clock, the anterior capsule appears fused to the clear posterior capsule, with regeneratory Soemmering-type PCO having formed in the capsular fornix. The optic is decentered inferiorly, potentially indicating zonular dehiscence. Significant decentration may result with even small zonular defects when a loop tip penetrates the gap and advances until stopped by the haptic junction after counterclockwise IOL rotation. Alternatively, such decentration may result from haptic deformation during IOL insertion.
In this case, the haptic loop is reported to be temporarily visible in the coloboma. This speaks against haptic loop dislocation through an inferior zonular defect because the stuck loop would not allow further rotation of the IOL. Also, the IOL fixated in such a manner would not likely cause recurrent bleeding. Instead, the IOL apparently spins in a sulcus that is larger than the haptic span, causing uveal chafe or making the loop ends irritate or impale the ciliary processes.
Therefore, any surgical approach must aim at stable IOL fixation. In principle, there are 2 options; that is, an IOL exchange through a 7.0 mm or larger incision for an Artisan iris-fixated PMMA IOL or exchange for a foldable PC IOL with 14.0 mm haptic specifically designed for sulcus fixation through a 3.5 mm or larger incision after the PMMA optic is dissected with Steinmetz scissors. With the large preexisting superior incision, the latter would be preferred. Alternatively, the existing IOL could be repositioned and fixated by (1) optic capture within a secondary posterior capsulorhexis created beneath the optic, (2) loop suturing to the iris, or (3) intrascleral fixation of the loop ends.
I decided for option 2 because of its simplicity. Two tangential paracenteses were created at 11 o’clock and 1 o’clock. The aqueous was replaced with a cohesive OVD. The IOL was rotated 180 degrees to free the inferior loop from potential zonular incarceration plus 2 clock hours until the loop presented in the peripheral coloboma. The straight needle of a 10-0 polypropylene suture was entered through the temporal paracentesis, passed behind the loop exposed in the coloboma to penetrate the nasal ankle of the coloboma, and externalized through the nasal paracentesis. The suture was grasped with a hook between where it exited the iris and approached the nasal paracentesis, and the loop thus created was externalized through the temporal paracentesis. A knot was created by threading the proximal suture end through the externalized suture loop, was internalized and tightened by gently pulling on both suture ends before the IOL optic recentered, and was trimmed with intraocular scissors. After acetylcholine pupil constriction, the OVD was rinsed out and the paracentesis closed by hydration. Because the iris periphery does not move when the size of the pupil changes (Figure 2), the suture-fixated IOL will remain in place, just like the claw-haptics of an Artisan-style IOL. If this minimally invasive intrascleral fixation had been unsuccessful, I would have resorted to option 1 to avoid bleeding from needle perforation of vessel-rich ciliary tissue.
Rupert Menapace MD
1. Ellingson FT. Complications with the Choice Mark VIII anterior chamber lens implant (uveitis-glaucoma-hyphema). Am Intraocular Implant Soc J
2. Van Liefferinge I, Van Oye R, Kestelyn P. Uveitis-glaucoma-hyphaema syndrome: a late complication of posterior chamber lenses. Bull Soc Belge Ophtalmol
. 1994;252:61-65. discussion by A Galand, 66.