CASE REPORT

Uveitis–glaucoma–hyphema syndrome after uneventful placement of a 1-piece intraocular lens into the capsular bag

Nath, Siddharth MD, PhD; Rai, Amandeep S. MD, FRCSC

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doi: 10.1097/j.jcro.0000000000000064
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Abstract

Uveitis–glaucoma–hyphema (UGH) syndrome is an uncommon but potentially serious complication of cataract surgery. First reported in 1978, UGH occurs from chafing of the iris by an intraocular lens (IOL).1 Mechanical disruption of iris tissue leads to pigment dispersion, microhyphema, and release of leukocytes and protein into the anterior chamber. Blockage of the trabecular meshwork by debris increases intraocular pressure, resulting in glaucomatous changes. UGH classically presents with iris transillumination defects in the shape of IOL haptics, highlighting areas of chafing. Initial treatment involves lowering intraocular pressure with topical and systemic medications and using corticosteroids for management of anterior uveitis. Surgical intervention, consisting of IOL repositioning, exchange, or explantation, remains the mainstay for addressing the underlying etiology.2

Although originally described as a complication of first-generation IOLs positioned within the ciliary sulcus, UGH has also become associated with modern IOLs placed in the sulcus or those in a bag–sulcus tilted position. UGH syndrome with placement of a single-piece IOL into the capsular bag, however, remains exceedingly rare.2 In this study, we describe such a case, with UGH after routine in-the-bag placement of a modern single-piece IOL.

Patient Consent Statement

Written consent was obtained from the patient for publication of the case. No personally identifiable information is included in the case report. All imaging data have been anonymized.

CASE REPORT

A 55-year-old man underwent routine cataract surgery on his right eye. Preoperative biometry was unremarkable with an axial length of 24.86 mm, anterior chamber depth of 3.83 mm, and white-to-white distance of 12.00 mm as measured by Nidek AL-Scan. Intraoperative floppy iris syndrome was encountered, consistent with a history of tamsulosin use, but this was managed without complication. A 1-piece hydrophobic SA60AT 17.5-diopter (D) acrylic IOL (Alcon Laboratories, Inc.) was placed into the capsular bag; at the end of the surgery, placement of the IOL with a 360-degree capsular coverage of the optic and haptics was confirmed. The same-day and 1-week postoperative evaluations were within normal limits. At the 2-week postoperative visit, slitlamp examination revealed iris transillumination in the shape of IOL haptics and marked iritis, suggestive of UGH syndrome. Although the IOL appeared well-centered within the capsular bag, inadequate pupillary dilation precluded definitive confirmation of IOL placement. The patient was scheduled for surgery and taken to the operating room the following week.

Preoperative ultrasound biomicroscopy (UBM) was not suggestive of a sulcus-placed IOL (Figure 1), and we proceeded with surgical exploration. Intraoperatively, the IOL was indeed confirmed in-the-bag position with complete capsulorhexis overlap over the optic and both haptics in an intact capsular bag (Figure 1). Given the clinical course, the IOL was exchanged for an MA60AC 17.5 D 3-piece in-the-bag IOL (Alcon Laboratories, Inc.), and wounds were sealed.

F1
Figure 1.:
Preoperative UBM showing well-centered in-the-bag IOL in sagittal (a) and axial (b) views. Exploratory surgery (c) shows the IOL in an intact capsular bag with a 360-degree coverage of both haptics and the optic. UBM = ultrasound biomicroscopy

The postoperative period was unremarkable and sequelae of UGH resolved. At 4 weeks post-IOL exchange, UBM confirmed in-the-bag positioning of the 3-piece IOL, and there was no active inflammation, hyphema, or ocular hypertension. At 3 months post-IOL exchange, iris transillumination defects persisted but the eye was otherwise quiet (Figure 2).

F2
Figure 2.:
Post-IOL exchange the eye is quiet (a) with only persistent iris transillumination defects (b) in the shape of the IOL haptics.

DISCUSSION

Few cases of UGH in association with placement of a 1-piece lens in the capsular bag have been reported in the literature. Mechanisms for UGH with 1-piece IOLs into an intact bag include chafing of the iris by an optic displaced by an iridociliary cyst, a Soemmerring ring, large capsulorhexis with minimal optic overlap, pseudophacodonesis secondary to zonular laxity from pseudoexfoliation, and focal capsular fibrosis around haptics in the setting of anteriorly rotated ciliary processes in plateau iris configuration.2–5 A report by Itagaki et al. also described reverse pupillary block as a mechanism, with intraoperative floppy iris syndrome proposed as a predisposing factor.6

Probing further, we learned that our patient had sustained repeated head traumas as the victim of multiple street crimes, suggesting a mechanism for traumatic zonulopathy. No history of eye-rubbing was elicited. We postulate that weakened zonular fibers allowed the otherwise well-position IOL to become mobile and chafe the posterior iris intermittently, causing UGH. IOL mobility with intermittent iris chafing has been suggested as a possible mechanism for UGH, and in such cases, it is recommended that B-scan ultrasonography be considered for evaluation of the IOL in different head positions.7 An equally important mechanism to consider in our case, given the shadows of the iris in Figure 2, is reverse pupillary block. As discussed earlier, intraoperative floppy iris syndrome may be an important factor in the development of subsequent UGH syndrome.6 The mechanism for this remains unclear; however, it is proposed that a billowy iris intraoperatively may settle as a latent valve, increasing the rate of flow of aqueous into the anterior chamber. This contributes to pigment dispersion and glaucomatous changes, which may be considered equivalent to UGH syndrome.6,8–10 As our case involved intraoperative floppy iris syndrome, it is entirely plausible that the iris of our patient settled in a position to allow for such processes to occur.

As 3-piece IOLs such as the MA60AC IOL have haptics composed of rigid poly(methyl methacrylate), which are angled forward, increased stability within the capsular bag is expected in comparison with 1-piece IOLs such as the SA60AT IOL initially implanted. These haptics also serve to position the optic of the IOL further posterior into the capsular bag, decreasing the potential for chafing and pupillary block. Furthermore, 1-piece IOLs often have haptics with sharp edges, which may lead to uveal chafing with movement even with a well-positioned IOL. Given this increased stability and the proposed mechanisms of UGH syndrome, it is understandable that our patient's symptoms resolved after the IOL exchange.

Management of UGH syndrome with in-the-bag IOLs remains variable and highly dependent on the postulated mechanism. Within the literature, in another case where UGH syndrome was encountered with placement of a modern IOL into the bag secondary to zonular laxity, surgeons opted for placement of a capsular tension ring to redistribute forces within the capsular bag as IOL exchange was not possible because of capsular fibrosis.2 In this case, the UGH symptoms improved, although did not resolve completely, and the patient continued to require medical therapy. In a case where UGH syndrome was believed to be because of reverse pupillary block, the authors opted for a laser iridotomy, as this has previously been reported to be an effective measure.6

In our case, as we suspected zonular weakness to be the primary mechanism of UGH and our patient's clinical status permitted IOL exchange, we opted to do so as a 3-piece IOL offers improved stability and minimizes the potential for iris chafing in comparison with 1-piece IOLs. We caution surgeons that UGH syndrome may sometimes also be multifactorial–for instance, in our case, both zonular laxity and reverse pupillary block may have contributed to his presentation. Although a laser iridotomy may offer utility in relieving reverse pupillary block, it would not be sufficient to treat cases where other mechanisms may be at play. IOL exchange should remain the mainstay of treatment, if possible, to ensure complete resolution of symptoms in cases where mechanisms other than reverse pupil block are suspected. Recognizing that this may not be possible in all cases, however, surgeons should turn to the literature to consider less-invasive treatments as the first-line management, including medical management, laser iridotomy, and placement of capsular tension devices. Concomitant medical and surgical management may be required to treat recalcitrant cases of UGH syndrome. Clinicians should also consider implementing a multimodal imaging approach, including UBM and anterior segment optical coherence tomography to determine whether any plateau iris configuration or physical chafing of the iris by the IOL exist and to aid in surgical planning.

Although it remains a rare complication, with the ubiquity of in-the-bag placement of single-piece IOLs, surgeons should be prepared to recognize and treat UGH swiftly to minimize the potential for permanent vision loss. As our case demonstrated, IOL position at the slitlamp and intraoperatively may be deceiving, and clinicians should consider a thorough social history to accompany their preoperative medical evaluation. Management of UGH syndrome after in-the-bag placement of an IOL should be guided by the surgeon’s suspicion of the underlying mechanism.

WHAT WAS KNOWN

  • Uveitis–glaucoma–hyphema (UGH) syndrome is an uncommon but serious complication of cataract surgery, most commonly associated with early IOLs or placement of an IOL in the ciliary sulcus.
  • UGH syndrome has not previously been shown to occur with a modern 1-piece IOL placed into the capsular bag.

WHAT THIS PAPER ADDS

  • UGH syndrome may occur even with placement of a modern IOL into the capsular bag.
  • Routine diagnostic techniques such as ultrasound biomicroscopy and even exploratory surgery may be deceiving in cases of UGH syndrome with contemporary 1-piece in-the-bag IOLs.
  • Clinicians should ensure an exhaustive social history that accompanies the medical history to identify any potential insults to the zonular apparatus and key anterior segment structures.

REFERENCES

1. Ellingson FT. The uveitis-glaucoma-hyphema syndrome associated with the Mark VIII anterior chamber lens implant. J Am Intraocul Implant Soc 1978;4:50–53
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3. Foroozan R, Tabas JG, Moster ML. Recurrent microhyphema despite intracapsular fixation of a posterior chamber intraocular lens. J Cataract Refract Surg 2003;29:1632–1635
4. Bryant TK, Feinberg EE, Peeler CE. Uveitis-glaucoma-hyphema syndrome secondary to a Soemmerring ring. J Cataract Refract Surg 2017;43:985–987
5. Razeghinejad MR, Havens SJ. Large capsulorhexis related uveitis-glaucoma-hyphema syndrome managed by intraocular lens implant exchange and gonioscopy assisted transluminal trabeculotomy. J Ophthalmic Vis Res 2019;14:215–218
6. Itagaki H, Kunikata T, Hiratsuka K, Saito J, Oshika T. Reverse pupillary block associated with pigment dispersion syndrome after in-the-bag intraocular lens implantation. J Cataract Refract Surg 2013;39:1925–1928
7. Wu N, Zhang H, Chen B, Ding W. A novel application of B-ultrasonography at various head positions in the diagnosis of untypical uveitis-glaucoma-hyphema (UGH) syndrome: a case report. Medicine (Baltimore) 2019;98:e13891
8. Mendez-Hernandez C, Fernandez-Barrientos Y, Fernandez-Vidal A, Martinez-de-la-Casa JM, Saenz-Frances F, Garcia-Feijoo J. Secondary pigment dispersion syndrome after in-the-bag AcrySof intraocular lens SN60AT implantation. Can J Ophthalmol 2008;43:120–121
9. Boutboul S, Letaief I, Lalloum F, Puech M, Borderie V, Laroche L. Pigmentary glaucoma secondary to in-the-bag intraocular lens implantation. J Cataract Refract Surg 2008;34:1595–1597
10. Fernandez-Bahamonde JL, Roman-Rodriguez CF. Pigment dispersion with elevation of intraocular pressure after bilateral phacoemulsification with implantation of acrylic intra-ocular lenses. Semin Ophthalmol 2010;25:36–38
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