This document summarizes macular hole formation and treatment. It describes the stages of macular hole development classified by Gass. Vitrectomy surgery involving fluid-gas exchange has been shown to successfully reattach the macula and improve vision in 58-73% of cases. The author reports high success rates for macular hole closure and visual improvement with vitrectomy using various tamponades and adjuncts like internal limiting membrane peeling. Face down positioning after surgery is important for compliance and optimal outcomes.

1. Macular hole Narciso F. Atienza, Jr. MD, DPBO Michael Shea Vitreo-Retina Fellow, University of Toronto St. Michael’s Hospital (2002-2004) Chief Retina Service: Cardinal Santos Medical Center

2. First described by Knapp (1869) and Noyes (1870) First coined by Ogilve (1900) Initially thought as untreatable. Patho-physiology unknown.

3. Factors inciting macular hole formation Vitreous syneresis Posterior vitreous separation Cystoid macular edema Previous ocular surgery Inflammatory process Traumatic blunt ocular injury Accidental laser injury Lightning Electrical shock High Myopia

4. Theory on Macular hole formation Lister (1924) Stated the importance of the vitreous in the pathogenesis.

5. Tangential traction on the macula Remnant posterior vitreous membrane on the macula with contractile cells. Focal shrinkage of foveal vitreous cortex Tractional elevation of the Henle’s nerve fiber layer. Intraretinal foveolar cyst formation. “ Unroofing” of the cyst. Gass JDM. Idiopathic senile macular hole: its early stages and pathogenesis. Arch Ophthalmol 1988: 106:629-639.

6. Hydration theory Together with peri-foveal traction, hydration of the edges of the hole causes the bridge to expand, increasing the size of the hole. Tornambe, P. Macular Hole Genesis: The Hydration Theory. Retina: 23 (3) June 2003 421-424

7. Other theories in macular hole formation Retinal/choroidal ischemia theory Affected by RPE dysfunction and possible intraretinal fluid accumulation in the fovea Involutional retinal thinning

8. Incidence and Risk factors (?) Incidence 0.05% Female predominance Lack of Estrogen use Bilateral in 3 to 22% Risk factors History of glaucoma Increased plasma fibrinogen

9. Gass classification

10. Stage 1 - localized shrinkage of prefoveal cortical vitreous, tractional shallow detachment of the foveola (loss of the normal foveolar depression and light reflex), retinal striae, Lack of Watzke sign. Stage 1A - small yellow spot (250-300 mm) Stage 1B - foveal detachment progresses, a yellow halo forms

11. Stage 1

12. Stage 2 - minute holes form near the center of the detached fovea. This is not an inevitable process. In 50% of cases, the vitreofoveal attachment spontaneously separates. Followed by restoration of the normal foveal depression and improved visual acuity.

13. Stage 2

14. Stage 3 – full thickness macular hole greater than 450 um in size, with no posterior vitreous separation. Most common presentation in the clinics Yellow deposits at the level of the retinal pigment epithelium Cuff of subretinal fluid Operculum Cystoid macular edema Positive Watzke’s sign

15. Stage 3

16. Stage 4 – full thickness macular hole with a posterior vitreous detachment

17. Stage 4

18. The Watzke-Allen test Slitlamp biomicroscopy The laser aiming beam test.

19. Questions asked (1) Is it possible to reattach the retina around the macular hole? (2) If it is reattached, will the patient's central vision improve?

20. Vitrectomy and fluid/gas exchange Kelly, EK, and Wendel, RT. Vitreous surgery for idiopathic macular holes: results of a pilot study, Arch Ophthalmol 109:654, 1991

21. In 30 (58%) of 52 patients, successful reattachment of the detached macula. In 22 (73%) of the 30 patients in whom the macula was successfully reattached, there was an improvement in visual acuity of two lines or better. In the 22 patients in whom reattachment of the macular hole was not obtained, there was no significant improvement in visual acuity.

22. Personal experience 91 cases macular hole surgery (since 7/2004) 76 patients 62 female vs 14 male patients 15 patients (bilateral) VA (CF 4 feet - 20/60)

23. 80 cases – phakic 68 - PPV alone 15 - PPV + phaco IOL 11 cases - pseudophakic Tamponade 55 cases - C3F8 36 cases - Silicone oil

24. 80 patients (90%) - successful hole closure in one surgery 71 patients- improvement in BCVA (more than 2 lines) 6 cases - did not close 2 cases had re-operation (closed after 2nd surgery)

25. Conclusions Importance of compliance (Face down positioning) Combined surgery Does not affect closure rate Tamponade No direct relationship between gas and oil (too small for comparison)

26. Observation 100% of patients will claim compliance Face down position Sign of compliance 41/101 (40%)

27. Post-operative course 15 developed cataract within 2 years (3 months - 2 years) No retinal detachments 3 cases of high IOP Failure to close 6 cases (1 case still had ILM, 4 cases patients did not position)

28. Technical modifications ILM peeling - 91% - 100% No face down requirement - 79%

29. Surgical adjuncts Transforming growth factor 91% vs 53% (Smiddy) Recombinant TGF-beta 78% vs 61% (Thompson) Autologous platelet 94% vs 81% (Paques)

30. “ If you don’t have complications, then you haven’t operated enough” Dr. Michael Shea 1st Fellow of Charles Schepens 1st Retina Surgeon in Canada (U of Toronto)