Hypotony Maculopathy

Parisa Farhi, MD

Definition of Hypotony

Hypotony is statistically defined as IOP of less than 10.1 mm Hg, which is 2 standard deviations below the mean population IOP of 15.9 mm Hg.1 Clinical hypotony occurs when ocular function is decreased secondary to ocular hypotension. Many clinicians use IOP less than 5 mm Hg to define clinical hypotony.1,2 Hypotony maculopathy occurs when hypotony affects the macula, leading to structural and functional changes.1-4

History

Dr. Angelos Dellaporta has been credited for describing fundus changes associated with hypotony in 1955.2,5 J. Donald M. Gass, MD, coined the term hypotony maculopathy in 1972.1,2

Associated Signs

Associated findings may include ocular shrinkage (with reduction of the axial length and volume of the eye), optic disc swelling, choroidal effusion, retinal or choroidal folds, retinal vascular tortuosity, neuronal atrophy, collapse of the anterior chamber, anterior chamber flare, cataract, corneal edema, astigmatism, and visual acuity decline (Slide 1).1-7

Slide 1

Slide 1

Indocyanine green angiography (ICG), a study used to evaluate choroidal circulation, may show choroidal vascular dilation and tortuosity in the posterior pole in addition to scattered areas of hypofluorescence.8

Diagnosis

Diagnosis is based on findings of a hypotonic eye along with some of the above associated findings. Many authors define hypotony maculopathy by findings of IOP less than 5 mm Hg in addition to retinal/choroidal folds (particularly in the macula), vascular tortuosity, papilledema, and/or vision loss.9 However, hypotony is a relative state. The literature provides one case report of fundus changes suggestive of hypotony maculopathy but with statistically normal IOP in a patient with increased intracranial pressure.3

Optical coherence tomography (OCT) has recently shown potential to be a useful tool to diagnose occult hypotony maculopathy and to assess resolution of this condition.10,11

Etiology

Hypotony results when the normal pressure of the aqueous and vitreous humor in the eye is compromised. Hypotony may be secondary to one or more of the following:

One of the most common causes of hypotony maculopathy is trabeculectomy. Hypotony maculopathy accounts for 3% to 14.3% of post-trabeculectomy vision loss.1,9 Trabeculectomy is a fistulizing procedure aimed at treating glaucoma by reducing IOP. Antifibrotic agents such as mitomycin C and 5FU are used to decrease fibrosis of the bleb; however, the use of these agents is associated with a higher risk of hypotony maculopathy.6,17-18

Risk factors

In a retrospective study of 81 eyes with hypotony maculopathy, an age of younger than 60 years, myopia, and male gender were found to be significant risk factors for this condition.1 Diabetes and choroidal effusion were associated with a decreased risk of hypotony maculopathy.1 However, in a retrospective study of six patients with hypotony maculopathy, coronary artery disease, and systemic hypertension were associated with higher risk.9

In another retrospective study of 52 eyes, increased time of application of mitomycin C was associated with increased risk of hypotony. Neither age nor number of previous incisional eye surgeries was found to be a significant risk factor for development of hypotony. However, hypotonic eyes were found to have lower preoperative IOPs, on average.19

Treatment

Normalizing IOP might restore the functional and structural changes caused by hypotony. Treatment is usually initiated when visually significant deterioration occurs.20 For trabeculectomy-induced hypotony, decreasing filtration through the bleb may be curative.6 Bandage soft contact lens or collagen shields, eye patching, topical corticosteroids, tissue adhesives, diathermy, cryotherapy, intraocular perfluoropropane injection, autologous blood injection, trichloroacetic acid use, argon or neodymium:YAG laser-induced subconjunctival bleeding, argon photocoagulation of the surface of the bleb, intracameral viscoelastic injection, compression sutures, patch grafting the sclerostomy site, and cataract surgery are techniques used with variable success to treat an eye with post-trabeculectomy hypotony.6,10,18,20,21

For cases of cyclodialysis-induced hypotony, cycloplegia, argon laser trans-corneal photocoagulation, and transscleral cyclophotocoagulation have been used to close the cleft.15 For difficult-to-close clefts, ciliochoroidal argon laser endophotocoagulation, cryotherapy diathermy, translimbal and transscleral suture fixation, direct cyclopexy, anterior scleral buckling, combined vitrectomy, cryotherapy and gas tamponading, and phacoemulcification plus a capsular tension ring placement have been tried.14,15 In cases where normalization of IOP does not correct the structural and functional changes of an eye with hypotony maculopathy, vitrectomy and replacement of the vitreous with an agent that is heavier than water might improve vision.20

Prognosis

Normalization of IOP may or may not correct the structural or functional changes in an eye with hypotony maculopathy.20,23 Cases of significant acuity improvement have been described by normalizing the IOP despite prolonged hypotony maculopathy.24 However, chronic swelling of the retina can lead to damage to the ganglion or photoreceptor cells.3 Therefore, rapid intervention once the diagnosis has been made is advisable.

References

  1. Schubert HD. Postsurgical hypotony: Relationship to fistulization, inflammation, chorioretinal lesions, and the vitreous. Surv Ophthalmol. 1996;41:97-125.

  2. Fannin LA, Schiffman JC, Budenz DL. Risk factors for hypotony maculopathy. Ophthalmology. 2003;110:1185-1191.

  3. Faingold D, Francis CJ, Buys YM. Hypotony maculopathy and papilledema after trabeculectomy in a patient with pseudotumor cerebri. J Glaucoma. 2003;12:374-378.

  4. Blumenthal EZ, Morales A. Resolution of hypotony maculopathy. Arch Ophthalmol. 2003;121:1502-1503.

  5. Dellaporta A. Fundus changes in postoperative hypotony. Am J Ophthalmol. 1955;40:780-785.

  6. Bashford KP, Shafranov G, Shields MB. Bleb revision for hypotony maculopathy after trabeculectomy. J Glaucoma. 2004;13:256-260.

  7. Azuara-Blanco A, Katz LJ. Dysfunctional filtering blebs. Surv Ophthalmol. 1998;43:93-1265.

  8. Masaoka N, Sawada K, Komatsu T, et al. Indocyanine green angiographic findings in 3 patients with traumatic hypotony maculopathy. Jpn J Ophthalmol. 2000;44:283-289.

  9. Costa VP, Smith M, Spaeth GL, et al. Loss of visual acuity after trabeculectomy. Ophthalmology. 1993;100:599-612.

  10. Budenz DL, Schwartz K, Gedde S. Occult hypotony maculopathy diagnosed with optical coherence tomography. Arch Ophthalmol. 2005;123:113-114.

  11. Kokame GT, de Leon MD, Tanji T. Serous retinal detachment and cystoid macular edema in hypotony maculopathy. Am J Ophthalmol. 2001;131:384-386.

  12. Deramo VA, Haupert CL, Fekrat S, Postel EA. Hypotony caused by scleral buckle erosion in Marfan syndrome. Am J Ophthalmol. 2001;132:429-431.

  13. Takaya K, Suzuki Y, Nakazawa M. Four cases of hypotony maculopathy caused by traumatic cyclodialysis and treated by vitrectomy, cryotherapy, and gas tamponade. Graefes Arch Clin Exp Ophthalmol. 2006;244:855-858. Epub 2005 Oct 19.

  14. Mandava N, Kahook MY, Mackenzie DL, Olson JL. Anterior scleral buckling procedure for cyclodialysis cleft with chronic hypotony. Ophthalmic Surg Lasers Imaging. 2006;37:151-153.

  15. Yuen NS, Hui SP, Woo DC. New methods of surgical repair for 360-degree cyclodialysis. J Cataract Refract Surg. 2006;32:13-17.

  16. Bainbridge JW, Raina J, Shah SM, et al. Ocular complications of intravenous cidofovir for cytomegalovirus retinitis in patients with AIDS. Eye. 1999;13:353-356.

  17. Suner IJ, Greenfield DS, Miller MP, et al. Hypotony maculopathy after filtering surgery with mitomycin C. Incidence and treatment. Ophthalmology. 1997;104:207-215.

  18. Shields MB, Scroggs MW, Sloop CM, Simmons RB. Clinical and histopathologic observations concerning hypotony after trabeculectomy with adjunctive mitomycin C. J Ophthalmol. 1993;116:673-863.

  19. Zacharia PT, Deppermann SR, Schuman JS. Ocular hypotony following trabeculectomy with mitomycin C. Am J Ophthalmol. 1993;116:314-326.

  20. Duker JS, Schuman JS. Successful surgical treatment of hypotony maculopathy following trabeculectomy with topical mitomycin C. Ophthalmic Surg. 1994;25:463-465.

  21. Ascaso FJ, Loras E, Cristobal JA. Combination of Nd:Yag laser-induced subconjunctival bleeding and intracameral viscoelastic injection to treat hypotony maculopathy. Ophthalmic Surg Lasers. 2002:33:504-507.

  22. Akova YA, Dursun D, Aydin P, et al. Management of hypotony maculopathy and a large filtering bleb after trabeculectomy with mitomycin C: Success with argon laser therapy. Ophthalmic Surg Lasers. 2000;31:491-494.

  23. Cohen SM, Flynn HW Jr, Palmberg PF, et al. Treatment of hypotony maculopathy after trabeculectomy. Ophthalmic Surg Lasers. 1995;26:435-441.

  24. Delgado MF, Daniels S, Pascal S, Dickens CJ. Hypotony maculopathy: Improvement of visual acuity after 7 years. Am J Ophthalmol. 2001;132:931-933.