History

• 69-year-old female, post-operative day 1 following Trabeculectomy with mitomycin C for pseudoexfoliative glaucoma OD
• Patient complains of severe, sharp pain in operative eye
• Associated with headache, nausea, vomiting and grossly decreased vision

Exam Findings

• VA: Hand Motions (pre-op 20/25) OD, 20/30 OS
• IOP: 42, 10
• Pupils: Sluggish OD, Round and Reactive OS, no RAPD
• EOM full OU
• Anterior Segment: elevated bleb, 1+ microcystic edema, diffuse shallow anterior chamber with 1+ cell OD; WNL OS
• Dilated Fundus Examination: 0.7 with inferior thinning OD, 0.5, sharp, pink OS. Macula, vessels, periphery WNL OU. Vitreous hemorrhage OD.

Differential Diagnosis

• Shallow chamber after trabeculectomy with high IOP
• Suprachoroidal hemorrhage
• Aqueous misdirection
• Choroidal effusion
• Pupillary block
• Shallow chamber after trabeculectomy with low IOP
• Bleb leak
• Excessive filtration
• Choroidal effusion
• Retinal detachment
• Cyclodialysis
• Ciliary body shutdown

Additional Investigations

Diagnosis

• Aqueous misdirection

Pathophysiology

• Aqueous misdirection (malignant glaucoma) is a rare condition characterized by elevated intraocular pressure and shallow anterior chamber without pupillary block due to an abnormal relationship between the ciliary process, lens and anterior hyaloid. It most commonly occurs in the early post-operative period but can occur months to years after surgery or in patients with no prior operations. The exact mechanism is still unknown.

Treatment

• Medical Therapy – first-line with resolution in 50 percent of cases
• Cycloplegic drugs (atropine)
• Topical beta-blocker and alpha agonists
• Oral acetazolamide
• Hyperosmotic agents (glycerol, mannitol)
• Miotics are contraindicated
• Laser Therapy – second-line treatment
  • Nd: YAG laser capsulotomy with disruption of anterior hyaloid face
  • Transscleral cyclodiode laser photocoagulation
• Surgical treatment – definitive treatment
• Transcorneal needling through iridotomy or posterior sclerotomy
• Iridectomy-hyaloido-zonulectomy with anterior vitrectomy (pseudophakic patients)
• Core vitrectomy-phacoemulsification-complete vitrectomy with iridectomy-hyaloidotomy-zonulectomy (phakic patients)

Prognosis and Future Directions

• Visual prognosis tends to be good if diagnosed and properly treated early in disease course
• Fellow eye is at increased risk of developing aqueous misdirection and some advocate for prophylactic vitrectomy prior to intraocular surgery in fellow eye
• Current research directed at modifying surgical methods and technique; for example, vitrectomy-phacoemulsification-vitrectomy is a relatively new surgical approach where core vitrectomy is performed prior to phacoemulsification to relieve posterior pressure.

References

• Arya SK, et al. Malignant glaucoma as a complication of Nd:YAG laser posterior capsulotomy. Ophthalmic Surg Lasers Imaging. May-Jun 2004;35(3):248-50.
• Bitrian E, Caprioli J. Pars plana anterior vitrectomy, hyaloido-zonulectomy, and iridectomy for aqueous humor misdirection. Am J Ophthalmol. July 2010;150(1)82-7.
• Brown RH, et al. Neodymium: YAG Vitreous Surgery for Phakic and Pseudophakic Malignant Glaucoma. Arch Ophthalmol. 1986 Oct;104(10):1464-6.
• Chandler PA, Simmons RJ, Grant WM. Malignant glaucoma. Medical and surgical treatment. Am J Ophthalmol. 1968 Sep;66(3):495-502.
• Chaudhry NA, et al. Pars plana vitrectomy during cataract surgery for prevention of aqueous misdirection in high-risk fellow eyes. Am J Ophthalmol. Mar 2000;129(3):387-8.
• Dorairaj S, et al. Diagnosis and Management of Malignant Glaucoma. EyeNet Magazine. April 2010:37-39.
• Francis BA, Wong RM, Minckler DS. Slit-lamp needle revision for aqueous misdirection after trabeculectomy. J Glaucoma. June 2002;11(3):183-8.
• Mastropasqua L, et al. Aqueous misdirection syndrome: A complication of YAG posterior capsulotomy. J Cataract Refract Surg. 20 (1994):563-5.
• Ruben S, et al. Malignant glaucoma and its management. British Journal of Ophthalmol. Feb 1997; 81(2):163-7.
• Sharma A, et al. Vitrectomy-Phacoemulsification-Vitrectomy for the Management of Aqueous Misdirection Syndrome in Phakic Eyes. Ophthalmology. Nov 2006;113(11):1968-73.
• Stumpf TH, et al. Transscleral cyclodiode laser photocoagulation in the treatment of aqueous misdirection syndrome. Ophthalmology. Nov 2008;115(11):2058-61.

Contact

• Malvin D. Anders, MD, Professor of Clinical Ophthalmology, Keck School of Medicine of USC, and Chief of Ophthalmology, LAC+USC Medical Center, manders@dhs.lacounty.gov
• Arman Zaman, MD, PGY-3 Ophthalmology resident, arman.zaman@med.usc.edu

Section Editors

• Vivek Patel, MD, Associate Professor of Clinical Ophthalmology, Program Director, vivek.patel@med.usc.edu
• Jesse Berry, MD, Assistant Professor of Clinical Ophthalmology, Associate Program Director, jesse.berry@med.usc.edu

Produced by: Monica Chavez, John Daniel, Joseph Yim and Dr. Vivek Patel