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An Unusual Spontaneous Acute Deepening of the Anterior Chamber

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 53-year-old woman presenting with a unilateral mild cortical and nuclear cataract associated with 20/32 visual acuity in her left eye was planning to undergo phacoemulsification. A few days before surgery, she noticed a sudden decrease in her left visual acuity measured at 20/80. Her eye specialist then observed a reverse pupillary block in her left eye and referred the patient to our department. At presentation, no other remarkable medical history was recorded and no precipitating factors identified. Slitlamp examination showed a unilateral deepening of the anterior chamber associated with signs of anterior uveitis (ie, keratic precipitates and severe aqueous flare, 3+) and posterior synechiae between the iris and the lens. In addition, fundus examination revealed an inferior retinal detachment without any associated visible tear. Intraocular pressure was 15 mm Hg in the right eye and 9 mm Hg in the left eye. Anterior segment optical coherence tomography images and fundus photographs of both eyes are shown in Figure 1.

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Iris retraction syndrome

D. Schedule combined phacoemulsification-vitrectomy surgery

Iris retraction syndrome is a rare entity initially described by Campbell1 in 1984. The proposed pathogenesis is that the retinal pigment epithelium (RPE) excessively absorbs aqueous humor and acts as a pump in the posterior segment. This overabsorption could be secondary to exposure of RPE through a peripheral retinal tear, but retinal breaks, although present in most cases, are not always noted.2 The misdirected flow of aqueous humor can lead to an inversion of the pressure gradient between the anterior and posterior chambers. This in turn can lead to a reverse pupillary block and its associated proinflammatory consequences.3 The associated exudative retinal detachment could be related to the absorption of the aqueous humor by the RPE toward the suprachoroidal space and to inflammation.4 The aim of the treatment is, one the one hand, to lyse posterior synechiae between the iris and the lens and, on the other hand, to drain the subretinal fluid and to treat the tear—when found—through pars plana vitrectomy. In some cases, medical treatment alone with topical atropine and steroids may be sufficient.2 Peripheral iridotomy (choice A) might be a dangerous option, as the iris is wedged to the lens and would not modify the misdirection of the aqueous humor flow. Sclerotomy (choice B) to drain fluid likely would not be appropriate, as the initial mechanism is not related to uveal effusion. Corticosteroid therapy (choice C) typically would not be useful, as the primary cause is not inflammatory.1,4 Iris retraction syndrome requires surgical management (choice D) to stop misdirected flow of aqueous humor toward the posterior segment. A topical treatment combining atropine and corticosteroids can be prescribed while awaiting surgery.

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Article Information

Corresponding Author: Marie-Noëlle Delyfer, MD, PhD, Vitreo-retinal Unit, Ophthalmology Department, Bordeaux University Hospital, Place Amélie Raba-Léon, 33000 Bordeaux, France (marie-noelle.delyfer@chu-bordeaux.fr).

Published Online: May 25, 2023. doi:10.1001/jamaophthalmol.2023.1824

Conflict of Interest Disclosures: Dr Delyfer reported personal fees from AbbVie, Bayer, Horus Pharma, Novartis, Roche, and Théa. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Campbell  DG .  Iris retraction associated with rhegmatogenous retinal detachment syndrome and hypotony. a new explanation.   Arch Ophthalmol. 1984;102(10):1457-1463. doi:10.1001/archopht.1984.01040031177013PubMedGoogle ScholarCrossref
2.
Geyer  O , Neudorfer  M , Rothkoff  L , Michaeli-Cohen  A , Lazar  M .  Iris retraction syndrome associated with nonrhegmatogenous retinal detachment.   Acta Ophthalmol Scand. 1998;76(5):617-619. doi:10.1034/j.1600-0420.1998.760522.xPubMedGoogle ScholarCrossref
3.
Goebel  C , Crabtree  GS , Altaweel  MM .  Management of iris retraction syndrome with heterochromia and retinal detachment.   Case Rep Ophthalmol. 2021;12(3):809-815. doi:10.1159/000519148PubMedGoogle ScholarCrossref
4.
Tabibian  D , Hoogewoud  F , Mavrakanas  N , Schutz  JS .  Misdirected aqueous flow in rhegmatogenous retinal detachment: a pathophysiology update.   Surv Ophthalmol. 2015;60(1):51-59. doi:10.1016/j.survophthal.2014.07.002PubMedGoogle ScholarCrossref
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Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 credit toward the CME [and Self-Assessment requirements] of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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