Uveitis-glaucoma-hyphema syndrome secondary to an in-the-bag intraocular lens with capsular fibrosis: The perfect storm

Jiaru Liu; Andrei-Alexandru Szigiato; Rodolpho Takaishi Matsumoto; Paul Harasymowycz

doi:10.4103/ijo.IJO_2038_21

Users Online: 400

CASE REPORT

Year : 2022 | Volume : 2 | Issue : 3 | Page : 670-672

Uveitis-glaucoma-hyphema syndrome secondary to an in-the-bag intraocular lens with capsular fibrosis: The perfect storm

Jiaru Liu¹, Andrei-Alexandru Szigiato¹, Rodolpho Takaishi Matsumoto², Paul Harasymowycz³
¹ Department of Ophthalmology, University of Montreal, Montreal, Canada
² Department of Ophthalmology, University of Montreal, Montreal, Canada; Department of Ophthalmology, University of São Paulo, São Paulo, Brazil
³ Department of Ophthalmology, University of Montreal; Montreal Glaucoma Institute and Bellevue Ophthalmology Clinics, Montreal, Canada

Date of Submission	02-Aug-2021
Date of Acceptance	27-Apr-2022
Date of Web Publication	16-Jul-2022

Correspondence Address:
Dr. Jiaru Liu
1078 rue de la Sucrerie, Montreal, Quebec
Canada

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2038_21

Abstract

A patient developed pseudoexfoliative glaucoma and hyphema syndrome with an appropriately placed, in-the-bag single-piece lens. Ultrasound biomicroscopy showed a Soemmering ring, extensive fibrotic bands tethering the capsule to the ciliary body, and localized ciliary body traction. An anterior chamber washout, peeling of the fibrotic bands, and capsular phimosis correction were performed. Uveitis-glaucoma-hyphema syndrome may be caused by a nontilted in-the-bag lens, precipitated by the formation of tractional fibrotic bands, a Soemmering ring, and a predisposition to zonular laxity from pseudoexfoliative syndrome. Surgical peeling of the capsular fibrosis may be the most important factor in preventing recurrent hyphema.

Keywords: Intraocular lens, pseudoexfoliation, Soemmering ring, uveitis-glaucoma-hyphema syndrome, ultrasound biomicroscopy

How to cite this article:
Liu J, Szigiato AA, Matsumoto RT, Harasymowycz P. Uveitis-glaucoma-hyphema syndrome secondary to an in-the-bag intraocular lens with capsular fibrosis: The perfect storm. Indian J Ophthalmol Case Rep 2022;2:670-2

How to cite this URL:
Liu J, Szigiato AA, Matsumoto RT, Harasymowycz P. Uveitis-glaucoma-hyphema syndrome secondary to an in-the-bag intraocular lens with capsular fibrosis: The perfect storm. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 17];2:670-2. Available from: https://www.ijoreports.in/text.asp?2022/2/3/670/351134

Uveitis-glaucoma-hyphema (UGH) syndrome is a rare condition seen post-cataract surgery resulting from the mechanical trauma caused by intraocular lens (IOL) chafing. The triad of uveitis, glaucoma, and hyphema stems from the excoriation of the iris and ciliary body by haptic loops that trigger pigment dispersion, macrophage clogging, and cytokine activation, leading to persistent inflammation and the breakdown of the blood–aqueous barrier.^[1] Most of the recent reports of UGH syndrome are caused by posterior chamber intraocular lenses (PCIOL) outside of the capsular bag such as single-piece IOLs placed in the sulcus space, and more rarely, single-piece IOL inside the capsular bag.^[1] We explore a potentially novel mechanism for recurrent hyphema in a case of UGH syndrome in a patient with an in-the-bag single-piece IOL and discuss our recommendation for management.

Case Report

An 86-year-old Caucasian man presented with progressive painless decreased vision in the right eye for 8 months. He was known for pseudoexfoliative glaucoma and had previous implantation of an Ahmed glaucoma valve and cataract surgery 5 years prior to presentation. He also underwent a Descemet membrane endothelial keratoplasty (DMEK) 2 years ago in his right eye. His visual acuity (VA) was hand motions with an intraocular pressure (IOP) of 9 mmHg. The examination was notable for a 1 mm hyphema in the anterior chamber and a moderate vitreous hemorrhage (1+). The IOL was clear and well positioned in the capsular bag. Ultrasound biomicroscopy (UBM) was performed and showed a Soemmering's ring in the inferior and lateral peripheral capsular bag [[Figure 1]a, green arrow]. While the Soemmering's ring was close to the iris, there was little to no loss of the iris pigment epithelium [[Figure 1]b, red arrow]. The lower haptic was also enveloped in a fibrotic mass attached to the inferior sulcus space seen on UBM [[Figure 1]b, yellow arrow], tethering the ciliary body and causing a localized detachment [[Figure 1]c, blue arrow]. Despite these changes, the IOL-capsule complex rested below the iris plane without any distortion [Figure 1]d.

Figure 1: Ultrasound biomicroscopy of the right eye. (a) Soemmering's ring (green arrow) and (b) Soemmering's ring coming in close contact with the iris (red arrow) but not distorting its plane and no thinning of the iris pigment epithelium; fibrotic mass (yellow arrow); (c) ciliary body traction and detachment (blue arrow); and (d) in-the-bag IOL below the iris plane

Click here to view

Two weeks after his initial visit, our patient underwent surgical exploration assisted by endoscopy, including an anterior chamber washout, anterior vitrectomy, peeling of fibrotic membranes [Figure 2], and capsular tension reduction using radial relaxing incisions at the margins of the anterior capsulorhexis [Video 1][Additional file 1]. At the first postoperative month, his VA was hand motions and his IOP was 14 mmHg with no IOP-lowering medications. The IOL was centered and there was no recurrence of hyphema or vitreous hemorrhage for the last 18 months. His decreased VA was attributed to persistent corneal edema and the patient was scheduled for a revision of his DMEK graft failure.

Figure 2: Intraoperative view of fibrotic bands causing traction on the ciliary body (red arrow)

Click here to view

Discussion

Single case reports have proposed different mechanisms of UGH syndrome with in-the-bag IOLs, including individual presentations of PEX syndrome,^[2] capsular bag fibrosis,^[3] and Soemmering's ring formation.^[4] To our knowledge, this is the first reported case of UGH syndrome occurring in a patient with an in-the-bag PCIOL due to a combination of three risk factors: Soemmering's ring, severe capsular fibrosis, and PEX syndrome. A Sommereing's ring forms when a capsulorhexis larger than the IOL optic allows adhesion of the anterior and posterior capsules, where retained lens epithelial cells from initial incomplete cortical cleanup during cataract surgery proliferate and form a ring of cortical fibers. It is more commonly seen in IOLs with a haptic misplaced in the sulcus due to poor visualization or lens dislocation but could also develop beneath the capsule of in-the-bag IOLs. Bryant described a case of UGH syndrome with extensive fibrosis and a Soemmering's ring around the capsule of an in-the-bag PCIOL, tilting the entire haptic-capsule complex and leading to chafing of the misaligned haptic against the posterior surface of the iris.^[4] Zhang et al.^[3] also reported a case of UGH syndrome in the setting of a bulky, fibrotic in-the-bag IOL creating a plateau iris configuration and causing chafing and pseudoherniation of the ciliary processes. Unlike the first two cases, the haptic-capsule complex in our patient was not as tilted and did not exhibit clinical signs of iris chafing. There was also no plateau iris configuration seen on UBM. Sousa et al.^[2] described a case of in-the-bag single-piece IOL dislocation in a patient known for PEX syndrome, with subsequent development of UGH syndrome likely caused by prolonged haptic-induced trauma of the posterior iris. Although our patient's IOL remained in the bag and the posterior iris seemed intact, the zonular laxity from his preexisting exfoliative condition, combined with the weight of the IOL, was sufficient to cause damage to the ciliary body when the lens complex moved and caused intermittent chafing of the ciliary body and posterior iris.

Conclusion

In our patient with all three contributing factors, we believe that the bands of fibrosis were the most important cause of the hyphema, as removing them alone resulted in the resolution of the patient's symptoms. UBM was a useful diagnostic tool in identifying and localizing the fibrotic bands, Soemmering's ring, and zone of ciliary body detachment. These findings were confirmed by endoscopic examination in the peripheral sulcus. We also recommend the surgical peeling of capsular fibrotic material and removal of the Soemmering's ring to resolve and prevent further recurrence of the hyphema. The treating ophthalmologist may also consider exchanging the single piece lens for a three-piece IOL or to perform intrascleral intraocular lens fixation using the Yamane technique.^[5] Our case illustrates the aggregation of several risk factors leading to UGH syndrome occurring in a well-positioned, nontilted, in-the-bag IOL. In suspected cases, we prompt clinicians to investigate using UBM despite a normal haptic-capsule complex on routine dilated eye exam and to consider surgical exploration using endoscopy to aid in the visualization of the sulcus space.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Mihail Z, Georgiana C. Uveitis – glaucoma – hyphaema syndrome. General review. Rom J Ophtalmol 2017;61:11-7.
2.	Sousa D, Leal I, Faria M, Pinto L. A rare manifestation of uveitis-glaucoma-hyphema syndrome. J Curr Glaucoma Pr 2016;10:76-8.
3.	Zhang L, Hood CT, Vrabec JP, Cullen AL, Parrish EA, Moroi SE. Mechanisms for in-the-bag uveitis-glaucoma-hyphema syndrome. J Cart Refract Surg 2014;40:490-2.
4.	Bryant TK, Feinberg EE, Peeler CE. Uveitis – glaucoma – hyphema syndrome secondary to a Soemmerring ring. J Cataract Refract Surg 2017;43:985-7.
5.	Yamane S, Sato S, Maruyama-Inoue M, Kadonosono K. Flanged intrascleral intraocular lens fixation with double-needle technique. Ophthalmology 2017;124:1136-42.