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Macular Pseudoholes
Macular Pseudoholes Clinical Features and Accuracy of Diagnosis Richard H. Fish, MD, Rajiv Anand, MD, David]. Izbrand, MD Background: Epimacular membrane with pseudohole is an important vitreomacular disorder that belongs in the differential diagnosis of impending and established macular hole. To better characterize this lesion, the authors attempted to identify various features of eyes with epimacular membrane and pseudohole. Methods: Demographic, clinical, photographic, and fluorescein angiographic data for 14 eyes with epimacular membrane and pseudohole were reviewed. Horizontal and vertical diameters of the pseudoholes were measured, and the original diagnosis was recorded for each eye. Fluorescein angiography was performed in 11 eyes. Results: The mean age of patients with macular pseudoholes was 61.6 years, and median visual acuity for pseudohole eyes was 20/30. Mean horizontal and vertical diameters of the pseudoholes were 384 and 41 0 ~m, respectively. None of the eyes with pseudoholes had the characteristic ophthalmoscopic features associated with full-thickness macular holes or impending macular holes. Results of fluorescein angiography showed three eyes with increased tortuosity or abnormal straightening of the perifoveal vessels; three eyes with a foveal window defect; and three eyes with late leakage from the perifoveal vessels. The original diagnosis of the initial examining physician was correct in only 43% of eyes with epimacular membrane and pseudohole. Conclusion: Epimacular membrane with pseudohole may be an underdiagnosed lesion and commonly mistaken for impending macular hole, full-thickness hole, or lamellar hole. These data may be of use as more patients are being considered for recently advocated surgical treatments for impending and established macular hole. Ophthalmology 1992;99: 1665-1670
Full-thickness macular holes are a relatively common cause of central visual loss and are usually readily recognized on clinical examination. Because of the recent
Originally received: April 13, 1992. Manuscript accepted: May 21 , 1992. From the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas. Presented in part at the 9th Annual Meeting of the Vitreous Society, Paris, France, September 1991. Supported in part by an unrestricted research grant from Research to Prevent Blindness, Inc, New York, New York. Reprint requests to Richard H. Fish, MD, Department of Ophthalmology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75235.
increase in awareness regarding the pathogenesis and treatment of idiopathic macular holes, impending holes, and arrested holes,l-6 it has become more critical to carefully differentiate among these lesions and to distinguish them from lesions that resemble macular holes. Some eyes with epimacular membrane may develop contraction of the membrane with a sharply demarcated baring of the foveolar area that simulates the appearance of a macular hole. 7,s Because a faint, diaphanous epimacular membrane may be found in some eyes with a full-thickness hole or impending hole, epimacular membrane with a pseudohoJe may be readily mistaken for these entities. We report the results of a retrospective analysis of epimacular membranes with pseudoholes. Quantitative features such as visual acuity and measured diameters of the pseudohoJes are addressed, and the diagnostic accuracy
1665
Ophthalmology
Volume 99, Number 11, November 1992
is reported with attention to false-positive results (overdiagnosis) and false-negative results (underdiagnosis).
Materials and Methods Color photography and fluorescein angiography records of patients seen at the Ophthalmology Clinic of Parkland Memorial Hospital and the faculty practice at the University of Texas Southwestern Medical Center were searched for the years 1982 to 1991. All photographic and clinical records of patients with the diagnosis of macular hole, impending macular hole, partial thickness or lamellar hole, macular cyst, and macular pseudohole were reviewed. The original diagnosis made by the initial examining physician was recorded for each eye. In the majority of cases, the original diagnosis was made with the benefit of fluorescein angiography. Patients were contacted for a repeat examination, including visual acuity, biomicroscopic examination with a contact lens, and fluorescein angiography. Color stereoscopic photographs, fluorescein angiograms, and clinical records from the original and all subsequent examinations were reviewed for each eye by two retina specialists (RHF and RA). Specific photographic, angiographic, and clinical features were used to assign an actual diagnosis to each eye. Macular pseudoholes were diagnosed in eyes with clearly visible epimacular membranes with retinal striae and a defect within the substance of the epimacular membrane. A spectrum of findings was noted in eyes with epimacular membrane and pseudohole. These included a barely visible epimacular membrane with a well-demarcated pseudohole (Fig 1); focal areas of opacification or contraction of the epimacular membrane (Fig 2); and stretching or distortion of the pseudohole (Fig 3). Variable fluorescein angiographic features included abnormal tortuosity or straightening of the perifoveal
Figure 1. Fundus photograph of the left eye of patient 1 with epimacular membrane and pseudohole. Notice the presence of faint epiretinal membrane with retinal striae (arrowhead) and pseudohole (arrow). Visual acuity was 20/70 and improved to 20/30 after pars plana vitrectomy with membrane peeling.
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Figure 2. Fundus photograph of the right eye of patient 5 with epimacular membrane and pseudohole. Notice the clearly visible epiretinal membrane with focal area of condensed membrane (arrowhead) and pseudohole (arrow). Visual acuity remained stable at 20/20 over a follow-up period of 158 weeks.
capillaries due to the epimacular membrane (Fig 4). Unlike a full-thickness macular hole, a foveal transmission defect was not seen in the majority of pseudoholes (Fig 5). Demographic data, including age, sex, involved eye, and visual acuity, were recorded for each eye, and a magnifying reticle was used to measure the horizontal and vertical diameters of each pseudohole from the color or red-free photographs.
Results A total of 75 eyes of 67 patients were originally included for study. Eighteen eyes were excluded because the patients were lost to follow-up, and the color photographs or flu-
Figure 3. Fundus photograph of the right eye of patient 12 with epimacular membrane and pseudohole. Notice the severe opacification of epiretinal membrane with distortion and oblique orientation of the pseudohole (arrow). Visual acuity was 20/40, deteriorated to 20/70, and improved to 20/30 after pars plana vitrectomy and membrane peeling.
Fish et al . Macular Pseudoholes
Figure 4. Arteriovenous phase fluorescein angiogram from the left eye of patient 1 with epimacular membrane and pseudohole. Notice the abnormal straightening of perifoveal vessels (arrow).
Figure 5. Arteriovenous phase fluorescein angiogram of the right eye of patient 5 with epimacular membrane and pseudohole shows slight hypofluorescence corresponding to localized area of condensed fibrosis (arrow) and absence of foveal window defect.
orescein angiograms were not of sufficient quality to accurately diagnose the macular lesion. In addition, 6 eyes were excluded because of a prior history of ocular trauma. Fourteen of the 51 remaining eyes were judged to have
an epimacular membrane with pseudo hole and are the basis ofthis report. Other actual diagnoses included macular hole, impending macular hole, cystoid macular edema, nonspecific pigmentary changes, and cilioretinal
Table 1. Demographic, Clinical, and Angiographic Features of 14 Eyes with Epimacular Membrane and Pseudohole Angiographic Features
Eye
Length of Follow-up (wks)
Initial Visual Acuity
Follow-up Visual Acuity
Abnormal Filling
as
216
20/70
20/30
+
58/F 64/F
aD aD
92 141
20/20 20/20
20/20 20/30
4 5
72/F 47/F
as aD
43 158
20/25 20/20
20/30 20/20
6
63/F
as
0
20/40
NA
+
+
7 8 9 10 11 12
71/F 71/F 69/F 52/F 61/F 41/F
aD aD aD as aD aD
169 0 20 50 14 31
20/20 20/50 20/80 20/30 20/60 20/40
20/25 NA 20/SO 20/40 20/SO 20/30
NA
NA
13
63/F
aD
0
20/30
NA
14
69jM
as
9
20/SO
20/80
Mean Median
61.6 37
20/30
20/30
Age (yrs)/ Sex
61jM 2 3
Patient No.
Late Leakage
+
+ NA NA
NA NA
+
Window Defect
Diameter of Pseudohole (!Lm) Horizontal
Vertical
Original Diagnosis
429
357
+
500 412
500 500
+
225 388
225 466
161
375
NA
404
+
250 300 509 692 255
317 250 810 226 346 638
491
464
353
265
Epimacular membrane/ pseudohole Impending hole Epimacular membrane/ pseudohole Impending hole Epimacular membrane/ pseudohole Epimacular membrane/ lamellar hole Lamellar hole Full-thickness hole Cystoid macular edema Lamellar hole Full-thickness hole Epimacular membrane/ pseudohole Epimacular membrane/ pseudohole Epimacular membrane/ pseudohole
384
410
NA NA
as = left eye; aD = right eye; NA = not available.
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Ophthalmology Volume 99, Number 11, November 1992 artery occlusion. During the course of the study, 11 (79%) of the 14 pseudohole eyes were re-examined by a retinal specialist. The demographic and clinical features of eyes with epimacular membrane and pseudohole appear in Table 1. There were 12 female and 2 male patients with epimacular membrane and pseudohole. The mean age of patients was 61.6 years (range, 41 to 72 years). The median visual acuity for pseudohole eyes was 20/30 (range, 20/ 20 to 20/80). Seven (50%) of 14 eyes had visual acuity of 20/30 or better; 3 eyes (21 %) had visual acuity of 20/40 or 20/50; and 4 eyes (29%) had visual acuity of 20/60 to 20/80. No eye had visual acuity worse than 20/80. Follow-up data were available for 11 patients (79%), with follow-up time ranging from 9 to 216 weeks, and an overall median follow-up of37 weeks. Of the 11 eyes with follow-up data available, follow-up visual acuity was the same as initial visual acuity in 4 eyes (36%), decreased by 1 or 2 lines in 5 eyes (46%), and improved by 1 or more lines in 2 eyes (27%). One of the eyes with improved visual acuity was in a patient who had pars plana vitrectomy and membrane peeling for the epimacular membrane. Overall, follow-up visual acuity was within 2 lines ofinitial acuity in 10 eyes (91%). None ofthe eyes with follow-up data available had worsening of visual acuity greater than 2 Snellen lines during the follow-up period. The majority of pseudoholes were elliptical in shape, with the long and short axes oriented in the horizontal and vertical meridians. A few pseudoholes had oblique orientations with respect to the long and short axes. The mean horizontal diameter of the pseudohole was 384 #Lm, while the mean vertical diameter of the pseudohole was 410 #Lm. There was no correlation between the size of the pseudohole (i.e., horizontal or vertical diameter) and visual acuity. None of the eyes with pseudoholes had the features frequently associated with a full-thickness hole-a limited cuff of subretinal fluid surrounding the hole, yellow-white deposits at the base of the hole, or an operculum suspended in front of the hole. Similarly, no pseudohole eye had features associated with impending macular hole-a foveolar detachment, yellow ring or dot in the foveal center, or cystic changes within the macula. All 14 eyes with pseudoholes had a clearly visible epiretinal membrane, either clinically or on color or red-free photographs. Major angiographic features for eyes with epimacular membrane and pseudohole appear in Table 1. Of the 11 patients who had fluorescein angiograms, only 3 (27%) demonstrated a window defect with early choroidal hyperfluorescence that faded in the late phase of the angiogram. Abnormal vascular filling with tortuosity or abnormal straightening of perifoveal vessels was seen in 3 eyes (27%) (Fig 4); late leakage of dye also was seen in 3 pseudohole eyes (27%). The original diagnosis of the initial examining physician for the 14 eyes with epimacular membrane and pseudohole also appears in Table 1. In the 51 eyes with a macular hole or related condition that were eligible for study, there were 7 instances in which epimacular membrane with pseudohole was applied as the original diag-
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nosis by the initial examining physician. This diagnosis was correct in 6 eyes. The 1 false-positive case (14%) was an eye with chronic cystoid macular edema misdiagnosed as epimacular membrane with pseudohole. Of the 14 eyes with an actual epimacular membrane and pseudohole, only 6 were correctly diagnosed by the initial examining physician, with 8 (57%) false-negative results. Incorrect diagnoses that were applied to eyes that actually had pseudoholes included impending macular hole (2 cases), full-thickness macular hole (2 cases), lamellar hole (2 cases), epimacular membrane with lamellar hole (1 case), and cystoid macular edema (1 case).
Discussion Lesions that resemble various stages of macular hole formation are relatively common, although infrequently studied. 7- 9 Allen and Gass8 reported a series of pseudohole eyes in which 3 of 4 patients had initial visual acuity of 20/25 or better. Other associated features included wrinkling of the inner retinal surface, a faint light reflex from the retinal tissue in the base of the pseudohole, absence of a foveal reflex, and lack of the typical findings of a macular hole. 7•8 Fluorescein angiography was usually normal, with a few eyes demonstrating a hyperfluorescent window defect in the area of the pseudohole. A larger series of 11 patients with "epimacular holes" found similar demographic and clinical features, with a median visual acuity of 20/60. 10 The natural history of epimacular membrane with pseudohole is usually favorable, with most eyes retaining good visual acuity. In one case, an epimacular membrane spontaneously separated from the retina, revealing a normal-appearing macula and re-emphasizing the epiretinal nature of this process. 8 Our series of 14 eyes with epimacular membrane and pseudohole is the largest reported to date. The stability of visual acuity in our patients with this disorder compares favorably with previous studies. The median initial and follow-up visual acuities in pseudohole eyes was 20/30. Ten of the 11 eyes with follow-up data available (91 %) had follow-up visual acuity that was within 2 lines of initial acuity. One eye improved by more than 2 lines of acuity after pars plana vitrectomy and membrane peeling. Clinical and angiographic features also were similar to previous reports. Careful examination of the pseudohole eyes showed the presence of an epimacular membrane with retinal striae in all 14 eyes. This was an obvious finding in some eyes with opaque membranes, while other eyes had a more subtle cellophane-like epimacular membrane. There was a low incidence ofthe angiographic features commonly seen in severe macular pucker. Three of the 11 eyes (27%) that had fluorescein angiography showed abnormal tortuosity or straightening of the perifoveal vessels; 3 other eyes (27%) had late leakage in the macula. In addition, only 3 of the 11 eyes (27%) that had fluorescein angiograms showed a foveal window defect. The data from this series indicate that pseudoholes were frequently misdiagnosed. Six of the 7 cases (86%) in which
Fish et al . Macular Pseudoholes the original diagnosis of pseudohole was made were correct. This low false-positive rate suggests that when the diagnosis of epimacular membrane with pseudohole was made, it was usually correct. However, a false-negative diagnosis for this condition was seen in 8 of 14 eyes (57%), suggesting that pseudoholes were underdiagnosed. Two eyes in our series were originally believed to have fullthickness macular holes by the original examining physician. None of the 14 pseudohole eyes had evidence of a surrounding cuff of subretinal fluid, an overlying operculum, or yellow-white dots at the base of the pseudohole. Two other eyes in this series were misdiagnosed as impending macular holes. Both of these eyes maintained excellent visual acuity of 20/20 and 20/30 over a followup period of 92 and 43 weeks, respectively, and showed no sign of full-thickness hole formation. Features associated with impending holes were not appreciated in any of the pseudo hole eyes: foveolar detachment, a yellow ring or dot in the foveal center, or cystic changes in the surrounding fovea. Moreover, many patients with impending (stage 1) or early (stage 2) macular holes have a window defect visible on fluorescein angiography, a finding infrequently seen in the pseudohole eyes in this series. The mean horizontal and vertical diameters for pseudoholes-384 and 410 ~m, respectively-appear to be smaller than the corresponding diameters of true fullthickness macular holes and larger than impending holes. Horizontal and vertical diameters for full-thickness macular holes were reported in one series to be 485 and 459 ~m, respectively.3 Using confocal laser tomography, Bartsch and co-workers II found short axis diameters of macular holes ranging from 505 to 555 ~m and long axis diameters ranging from 520 to 775 ~m. Johnson and Gass3 found the horizontal and vertical diameters in eyes with early holes (stages 2A and 2B) to be 187 and 191 ~m, respectively. The measured diameters of the macular pseudohole, along with the presence of a clearly visible epimacular membrane with retinal striae, the relatively good visual acuity in patients with pseudo holes, and the low incidence of a window defect on fluorescein angiography, may be of value in helping to distinguish this lesion from other macular disorders. The pseudoholes in our series were commonly mistaken for full-thickness holes, lamellar holes, and impending holes. A review of the literature shows several conditions that, in retrospect, may have been epimacular membranes with pseudoholes, including macular pucker with a co-existent macular hole and normal visual acuity, 12 preretinal membrane with a macular cyst, 13 and congenital premacular fibrosis with lamellar hole. 14 The potential for misdiagnosis is probably greatest in those eyes in which a pseudohole forms within a relatively mild epimacular membrane (Fig 1). Recently, attention has been focused on surgical prevention4.5.15.16 and treatment6 of macular holes. Our findings of frequent misdiagnosis of epimacular membranes with pseudoholes, combined with the acknowledged difficulty that occasionally arises in differentiating among true macular holes, lamellar holes, and pseudo-
holes,2,5,9,17-21 and the large number of disparate terms used to connote it premacular hole condition, 1-3,9,22-27 suggest a need for caution in the diagnosis and treatment of the vitreomacular disorders. Epimacular membrane with pseudohole should be included in the differential diagnosis of full-thickness and impending macular hole. Every available means of evaluation including stereoscopic photography and quantitative measurement of the lesion with a reticle should be used to arrive at a correct diagnosis in these patients before surgical intervention is contemplated.
References I. Gass JDM. Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment, 3rd ed. Vol. 2. St. Louis: CV Mosby, 1987;684-93. 2. Gass JDM. Idiopathic senile macular hole: its early stages and pathogenesis. Arch Ophthalmol 1988; 106:629-39. 3. Johnson RN, Gass JDM. Idiopathic macular holes: observations, stages of formation, and implications for surgical intervention. Ophthalmology 1988;95:917-24. 4. Smiddy WE, Michels RG, Glaser BM, deBustros S. Vitrectomy for impending idiopathic macular holes. Am J Ophthalmol 1988; 105:371-6. 5. Jost BF, Hutton WL, Fuller DG, et al. Vitrectomy in eyes at risk for macular hole formation. Ophthalmology 1990;97: 843-7. 6. Kelly NE, Wendel RT. Vitreous surgery for idiopathic macular holes. Arch Ophthalmol 1991;109:654-9. 7. Gass JDM. Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment, 3rd ed. Vol. 2. St. Louis: CV Mosby, 1987;700-12. 8. Allen AW Jr, Gass JDM. Contraction of a perifoveal epiretinal membrane simulating a macular hole. Am J Ophthalmol 1976;82:684-91. 9. Gass JDM, Joondeph Be. Observations concerning patients with suspected impending macular holes. Am J Ophthalmol 1990; 109:638-46. 10. Mandelcom MS, Lipton N. Epi-macular holes: a cause of decreased vision in the elderly. Can J OphthalmoI1977;12: 182-7. II. Bartsch D-U, Intaglietta M, Bille JF, et al. Confocal laser tomographic analysis of the retina in eyes with macular hole formation and other focal macular diseases. Am J Ophthalmol 1989;108:277-87. 12. Maumenee AE. Further advances in the study ofthe macula. Arch Ophthalmol 1967;78:151-65. 13. Wise GN. Oinical features of idiopathic preretinal macular fibrosis. Schoenberg Lecture. Am J Ophthalmol 1975;79: 349-57. 14. Wise GN. Congenital preretinal macular fibrosis. Am J Ophthalmol 1975;79:363-5. 15. deBustros S. Early stages of macular holes: to treat or not to treat [editorial]? Arch Ophthalmol 1990; 108: 1085-6. 16. Margherio RR, Trese MT, Margherio AR, Cartright K. Surgical management of vitreomacular traction syndromes. Ophthalmology 1989;96: 1437-45. 17. Yaoeda H. Clinical observations on the macular hole. Nippon Ganka Gakkai Zasshi 1967;71:1723-36. 18. Gass JDM. Lamellar macular hole. A complication of cystoid macular edema after cataract extraction. Arch Ophthalmol 1976;94:793-800.
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Ophthalmology Volume 99, Number 11, November 1992 19. Aaberg TM. Macular holes: a review. Surv Ophthalmol 1970; 15: 139-62. 20. Morgan CM, Schatz H. Involutional macular thinning: a pre-macular hole condition. Ophthalmology 1986;93: 15361. 21. Margherio RR, Schepens CL. Macular breaks. I. Diagnosis, etiology, and observations. Am J Ophthalmol 1972;74:21932. 22. Morgan CM, Schatz H. Idiopathic macular holes. Am J Ophthalmol 1985;99:437-44. 23. McDonnell PJ, Fine SL, Hillis AI. Clinical features of idiopathic macular cysts and holes. Am J Ophthalmol 1982;93: 777-86.
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24. Akiba J, Yoshida A, Trempe CL. Risk of developing a macular hole. Arch Ophthalmol 1990; 108: 1088-90. 25. Bronstein MA, Trempe CL, Freeman HM. Fellow eyes of eyes with macular holes. Am J Ophthalmol 1981;92:75761. 26. Hauch TL, Straatsma BR, Kreiger AE, Hayreh MMS. Macular holes: etiology and visual function. In: Ryan SJ, Dawson AK, Little HL, eds. Retinal Diseases. Orlando: Grone & Stratton, 1985;Chap. 28. 27. Akiba J, Quiroz MA, Trempe CL. Role of posterior vitreous detachment in idiopathic macular holes. Ophthalmology 1990;97: 1610-13.
Full-thickness macular holes are a relatively common cause of central visual loss and are usually readily recognized on clinical examination. Because of the recent
Originally received: April 13, 1992. Manuscript accepted: May 21 , 1992. From the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas. Presented in part at the 9th Annual Meeting of the Vitreous Society, Paris, France, September 1991. Supported in part by an unrestricted research grant from Research to Prevent Blindness, Inc, New York, New York. Reprint requests to Richard H. Fish, MD, Department of Ophthalmology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75235.
increase in awareness regarding the pathogenesis and treatment of idiopathic macular holes, impending holes, and arrested holes,l-6 it has become more critical to carefully differentiate among these lesions and to distinguish them from lesions that resemble macular holes. Some eyes with epimacular membrane may develop contraction of the membrane with a sharply demarcated baring of the foveolar area that simulates the appearance of a macular hole. 7,s Because a faint, diaphanous epimacular membrane may be found in some eyes with a full-thickness hole or impending hole, epimacular membrane with a pseudohoJe may be readily mistaken for these entities. We report the results of a retrospective analysis of epimacular membranes with pseudoholes. Quantitative features such as visual acuity and measured diameters of the pseudohoJes are addressed, and the diagnostic accuracy
1665
Ophthalmology
Volume 99, Number 11, November 1992
is reported with attention to false-positive results (overdiagnosis) and false-negative results (underdiagnosis).
Materials and Methods Color photography and fluorescein angiography records of patients seen at the Ophthalmology Clinic of Parkland Memorial Hospital and the faculty practice at the University of Texas Southwestern Medical Center were searched for the years 1982 to 1991. All photographic and clinical records of patients with the diagnosis of macular hole, impending macular hole, partial thickness or lamellar hole, macular cyst, and macular pseudohole were reviewed. The original diagnosis made by the initial examining physician was recorded for each eye. In the majority of cases, the original diagnosis was made with the benefit of fluorescein angiography. Patients were contacted for a repeat examination, including visual acuity, biomicroscopic examination with a contact lens, and fluorescein angiography. Color stereoscopic photographs, fluorescein angiograms, and clinical records from the original and all subsequent examinations were reviewed for each eye by two retina specialists (RHF and RA). Specific photographic, angiographic, and clinical features were used to assign an actual diagnosis to each eye. Macular pseudoholes were diagnosed in eyes with clearly visible epimacular membranes with retinal striae and a defect within the substance of the epimacular membrane. A spectrum of findings was noted in eyes with epimacular membrane and pseudohole. These included a barely visible epimacular membrane with a well-demarcated pseudohole (Fig 1); focal areas of opacification or contraction of the epimacular membrane (Fig 2); and stretching or distortion of the pseudohole (Fig 3). Variable fluorescein angiographic features included abnormal tortuosity or straightening of the perifoveal
Figure 1. Fundus photograph of the left eye of patient 1 with epimacular membrane and pseudohole. Notice the presence of faint epiretinal membrane with retinal striae (arrowhead) and pseudohole (arrow). Visual acuity was 20/70 and improved to 20/30 after pars plana vitrectomy with membrane peeling.
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Figure 2. Fundus photograph of the right eye of patient 5 with epimacular membrane and pseudohole. Notice the clearly visible epiretinal membrane with focal area of condensed membrane (arrowhead) and pseudohole (arrow). Visual acuity remained stable at 20/20 over a follow-up period of 158 weeks.
capillaries due to the epimacular membrane (Fig 4). Unlike a full-thickness macular hole, a foveal transmission defect was not seen in the majority of pseudoholes (Fig 5). Demographic data, including age, sex, involved eye, and visual acuity, were recorded for each eye, and a magnifying reticle was used to measure the horizontal and vertical diameters of each pseudohole from the color or red-free photographs.
Results A total of 75 eyes of 67 patients were originally included for study. Eighteen eyes were excluded because the patients were lost to follow-up, and the color photographs or flu-
Figure 3. Fundus photograph of the right eye of patient 12 with epimacular membrane and pseudohole. Notice the severe opacification of epiretinal membrane with distortion and oblique orientation of the pseudohole (arrow). Visual acuity was 20/40, deteriorated to 20/70, and improved to 20/30 after pars plana vitrectomy and membrane peeling.
Fish et al . Macular Pseudoholes
Figure 4. Arteriovenous phase fluorescein angiogram from the left eye of patient 1 with epimacular membrane and pseudohole. Notice the abnormal straightening of perifoveal vessels (arrow).
Figure 5. Arteriovenous phase fluorescein angiogram of the right eye of patient 5 with epimacular membrane and pseudohole shows slight hypofluorescence corresponding to localized area of condensed fibrosis (arrow) and absence of foveal window defect.
orescein angiograms were not of sufficient quality to accurately diagnose the macular lesion. In addition, 6 eyes were excluded because of a prior history of ocular trauma. Fourteen of the 51 remaining eyes were judged to have
an epimacular membrane with pseudo hole and are the basis ofthis report. Other actual diagnoses included macular hole, impending macular hole, cystoid macular edema, nonspecific pigmentary changes, and cilioretinal
Table 1. Demographic, Clinical, and Angiographic Features of 14 Eyes with Epimacular Membrane and Pseudohole Angiographic Features
Eye
Length of Follow-up (wks)
Initial Visual Acuity
Follow-up Visual Acuity
Abnormal Filling
as
216
20/70
20/30
+
58/F 64/F
aD aD
92 141
20/20 20/20
20/20 20/30
4 5
72/F 47/F
as aD
43 158
20/25 20/20
20/30 20/20
6
63/F
as
0
20/40
NA
+
+
7 8 9 10 11 12
71/F 71/F 69/F 52/F 61/F 41/F
aD aD aD as aD aD
169 0 20 50 14 31
20/20 20/50 20/80 20/30 20/60 20/40
20/25 NA 20/SO 20/40 20/SO 20/30
NA
NA
13
63/F
aD
0
20/30
NA
14
69jM
as
9
20/SO
20/80
Mean Median
61.6 37
20/30
20/30
Age (yrs)/ Sex
61jM 2 3
Patient No.
Late Leakage
+
+ NA NA
NA NA
+
Window Defect
Diameter of Pseudohole (!Lm) Horizontal
Vertical
Original Diagnosis
429
357
+
500 412
500 500
+
225 388
225 466
161
375
NA
404
+
250 300 509 692 255
317 250 810 226 346 638
491
464
353
265
Epimacular membrane/ pseudohole Impending hole Epimacular membrane/ pseudohole Impending hole Epimacular membrane/ pseudohole Epimacular membrane/ lamellar hole Lamellar hole Full-thickness hole Cystoid macular edema Lamellar hole Full-thickness hole Epimacular membrane/ pseudohole Epimacular membrane/ pseudohole Epimacular membrane/ pseudohole
384
410
NA NA
as = left eye; aD = right eye; NA = not available.
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Ophthalmology Volume 99, Number 11, November 1992 artery occlusion. During the course of the study, 11 (79%) of the 14 pseudohole eyes were re-examined by a retinal specialist. The demographic and clinical features of eyes with epimacular membrane and pseudohole appear in Table 1. There were 12 female and 2 male patients with epimacular membrane and pseudohole. The mean age of patients was 61.6 years (range, 41 to 72 years). The median visual acuity for pseudohole eyes was 20/30 (range, 20/ 20 to 20/80). Seven (50%) of 14 eyes had visual acuity of 20/30 or better; 3 eyes (21 %) had visual acuity of 20/40 or 20/50; and 4 eyes (29%) had visual acuity of 20/60 to 20/80. No eye had visual acuity worse than 20/80. Follow-up data were available for 11 patients (79%), with follow-up time ranging from 9 to 216 weeks, and an overall median follow-up of37 weeks. Of the 11 eyes with follow-up data available, follow-up visual acuity was the same as initial visual acuity in 4 eyes (36%), decreased by 1 or 2 lines in 5 eyes (46%), and improved by 1 or more lines in 2 eyes (27%). One of the eyes with improved visual acuity was in a patient who had pars plana vitrectomy and membrane peeling for the epimacular membrane. Overall, follow-up visual acuity was within 2 lines ofinitial acuity in 10 eyes (91%). None ofthe eyes with follow-up data available had worsening of visual acuity greater than 2 Snellen lines during the follow-up period. The majority of pseudoholes were elliptical in shape, with the long and short axes oriented in the horizontal and vertical meridians. A few pseudoholes had oblique orientations with respect to the long and short axes. The mean horizontal diameter of the pseudohole was 384 #Lm, while the mean vertical diameter of the pseudohole was 410 #Lm. There was no correlation between the size of the pseudohole (i.e., horizontal or vertical diameter) and visual acuity. None of the eyes with pseudoholes had the features frequently associated with a full-thickness hole-a limited cuff of subretinal fluid surrounding the hole, yellow-white deposits at the base of the hole, or an operculum suspended in front of the hole. Similarly, no pseudohole eye had features associated with impending macular hole-a foveolar detachment, yellow ring or dot in the foveal center, or cystic changes within the macula. All 14 eyes with pseudoholes had a clearly visible epiretinal membrane, either clinically or on color or red-free photographs. Major angiographic features for eyes with epimacular membrane and pseudohole appear in Table 1. Of the 11 patients who had fluorescein angiograms, only 3 (27%) demonstrated a window defect with early choroidal hyperfluorescence that faded in the late phase of the angiogram. Abnormal vascular filling with tortuosity or abnormal straightening of perifoveal vessels was seen in 3 eyes (27%) (Fig 4); late leakage of dye also was seen in 3 pseudohole eyes (27%). The original diagnosis of the initial examining physician for the 14 eyes with epimacular membrane and pseudohole also appears in Table 1. In the 51 eyes with a macular hole or related condition that were eligible for study, there were 7 instances in which epimacular membrane with pseudohole was applied as the original diag-
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nosis by the initial examining physician. This diagnosis was correct in 6 eyes. The 1 false-positive case (14%) was an eye with chronic cystoid macular edema misdiagnosed as epimacular membrane with pseudohole. Of the 14 eyes with an actual epimacular membrane and pseudohole, only 6 were correctly diagnosed by the initial examining physician, with 8 (57%) false-negative results. Incorrect diagnoses that were applied to eyes that actually had pseudoholes included impending macular hole (2 cases), full-thickness macular hole (2 cases), lamellar hole (2 cases), epimacular membrane with lamellar hole (1 case), and cystoid macular edema (1 case).
Discussion Lesions that resemble various stages of macular hole formation are relatively common, although infrequently studied. 7- 9 Allen and Gass8 reported a series of pseudohole eyes in which 3 of 4 patients had initial visual acuity of 20/25 or better. Other associated features included wrinkling of the inner retinal surface, a faint light reflex from the retinal tissue in the base of the pseudohole, absence of a foveal reflex, and lack of the typical findings of a macular hole. 7•8 Fluorescein angiography was usually normal, with a few eyes demonstrating a hyperfluorescent window defect in the area of the pseudohole. A larger series of 11 patients with "epimacular holes" found similar demographic and clinical features, with a median visual acuity of 20/60. 10 The natural history of epimacular membrane with pseudohole is usually favorable, with most eyes retaining good visual acuity. In one case, an epimacular membrane spontaneously separated from the retina, revealing a normal-appearing macula and re-emphasizing the epiretinal nature of this process. 8 Our series of 14 eyes with epimacular membrane and pseudohole is the largest reported to date. The stability of visual acuity in our patients with this disorder compares favorably with previous studies. The median initial and follow-up visual acuities in pseudohole eyes was 20/30. Ten of the 11 eyes with follow-up data available (91 %) had follow-up visual acuity that was within 2 lines of initial acuity. One eye improved by more than 2 lines of acuity after pars plana vitrectomy and membrane peeling. Clinical and angiographic features also were similar to previous reports. Careful examination of the pseudohole eyes showed the presence of an epimacular membrane with retinal striae in all 14 eyes. This was an obvious finding in some eyes with opaque membranes, while other eyes had a more subtle cellophane-like epimacular membrane. There was a low incidence ofthe angiographic features commonly seen in severe macular pucker. Three of the 11 eyes (27%) that had fluorescein angiography showed abnormal tortuosity or straightening of the perifoveal vessels; 3 other eyes (27%) had late leakage in the macula. In addition, only 3 of the 11 eyes (27%) that had fluorescein angiograms showed a foveal window defect. The data from this series indicate that pseudoholes were frequently misdiagnosed. Six of the 7 cases (86%) in which
Fish et al . Macular Pseudoholes the original diagnosis of pseudohole was made were correct. This low false-positive rate suggests that when the diagnosis of epimacular membrane with pseudohole was made, it was usually correct. However, a false-negative diagnosis for this condition was seen in 8 of 14 eyes (57%), suggesting that pseudoholes were underdiagnosed. Two eyes in our series were originally believed to have fullthickness macular holes by the original examining physician. None of the 14 pseudohole eyes had evidence of a surrounding cuff of subretinal fluid, an overlying operculum, or yellow-white dots at the base of the pseudohole. Two other eyes in this series were misdiagnosed as impending macular holes. Both of these eyes maintained excellent visual acuity of 20/20 and 20/30 over a followup period of 92 and 43 weeks, respectively, and showed no sign of full-thickness hole formation. Features associated with impending holes were not appreciated in any of the pseudo hole eyes: foveolar detachment, a yellow ring or dot in the foveal center, or cystic changes in the surrounding fovea. Moreover, many patients with impending (stage 1) or early (stage 2) macular holes have a window defect visible on fluorescein angiography, a finding infrequently seen in the pseudohole eyes in this series. The mean horizontal and vertical diameters for pseudoholes-384 and 410 ~m, respectively-appear to be smaller than the corresponding diameters of true fullthickness macular holes and larger than impending holes. Horizontal and vertical diameters for full-thickness macular holes were reported in one series to be 485 and 459 ~m, respectively.3 Using confocal laser tomography, Bartsch and co-workers II found short axis diameters of macular holes ranging from 505 to 555 ~m and long axis diameters ranging from 520 to 775 ~m. Johnson and Gass3 found the horizontal and vertical diameters in eyes with early holes (stages 2A and 2B) to be 187 and 191 ~m, respectively. The measured diameters of the macular pseudohole, along with the presence of a clearly visible epimacular membrane with retinal striae, the relatively good visual acuity in patients with pseudo holes, and the low incidence of a window defect on fluorescein angiography, may be of value in helping to distinguish this lesion from other macular disorders. The pseudoholes in our series were commonly mistaken for full-thickness holes, lamellar holes, and impending holes. A review of the literature shows several conditions that, in retrospect, may have been epimacular membranes with pseudoholes, including macular pucker with a co-existent macular hole and normal visual acuity, 12 preretinal membrane with a macular cyst, 13 and congenital premacular fibrosis with lamellar hole. 14 The potential for misdiagnosis is probably greatest in those eyes in which a pseudohole forms within a relatively mild epimacular membrane (Fig 1). Recently, attention has been focused on surgical prevention4.5.15.16 and treatment6 of macular holes. Our findings of frequent misdiagnosis of epimacular membranes with pseudoholes, combined with the acknowledged difficulty that occasionally arises in differentiating among true macular holes, lamellar holes, and pseudo-
holes,2,5,9,17-21 and the large number of disparate terms used to connote it premacular hole condition, 1-3,9,22-27 suggest a need for caution in the diagnosis and treatment of the vitreomacular disorders. Epimacular membrane with pseudohole should be included in the differential diagnosis of full-thickness and impending macular hole. Every available means of evaluation including stereoscopic photography and quantitative measurement of the lesion with a reticle should be used to arrive at a correct diagnosis in these patients before surgical intervention is contemplated.
References I. Gass JDM. Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment, 3rd ed. Vol. 2. St. Louis: CV Mosby, 1987;684-93. 2. Gass JDM. Idiopathic senile macular hole: its early stages and pathogenesis. Arch Ophthalmol 1988; 106:629-39. 3. Johnson RN, Gass JDM. Idiopathic macular holes: observations, stages of formation, and implications for surgical intervention. Ophthalmology 1988;95:917-24. 4. Smiddy WE, Michels RG, Glaser BM, deBustros S. Vitrectomy for impending idiopathic macular holes. Am J Ophthalmol 1988; 105:371-6. 5. Jost BF, Hutton WL, Fuller DG, et al. Vitrectomy in eyes at risk for macular hole formation. Ophthalmology 1990;97: 843-7. 6. Kelly NE, Wendel RT. Vitreous surgery for idiopathic macular holes. Arch Ophthalmol 1991;109:654-9. 7. Gass JDM. Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment, 3rd ed. Vol. 2. St. Louis: CV Mosby, 1987;700-12. 8. Allen AW Jr, Gass JDM. Contraction of a perifoveal epiretinal membrane simulating a macular hole. Am J Ophthalmol 1976;82:684-91. 9. Gass JDM, Joondeph Be. Observations concerning patients with suspected impending macular holes. Am J Ophthalmol 1990; 109:638-46. 10. Mandelcom MS, Lipton N. Epi-macular holes: a cause of decreased vision in the elderly. Can J OphthalmoI1977;12: 182-7. II. Bartsch D-U, Intaglietta M, Bille JF, et al. Confocal laser tomographic analysis of the retina in eyes with macular hole formation and other focal macular diseases. Am J Ophthalmol 1989;108:277-87. 12. Maumenee AE. Further advances in the study ofthe macula. Arch Ophthalmol 1967;78:151-65. 13. Wise GN. Oinical features of idiopathic preretinal macular fibrosis. Schoenberg Lecture. Am J Ophthalmol 1975;79: 349-57. 14. Wise GN. Congenital preretinal macular fibrosis. Am J Ophthalmol 1975;79:363-5. 15. deBustros S. Early stages of macular holes: to treat or not to treat [editorial]? Arch Ophthalmol 1990; 108: 1085-6. 16. Margherio RR, Trese MT, Margherio AR, Cartright K. Surgical management of vitreomacular traction syndromes. Ophthalmology 1989;96: 1437-45. 17. Yaoeda H. Clinical observations on the macular hole. Nippon Ganka Gakkai Zasshi 1967;71:1723-36. 18. Gass JDM. Lamellar macular hole. A complication of cystoid macular edema after cataract extraction. Arch Ophthalmol 1976;94:793-800.
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Ophthalmology Volume 99, Number 11, November 1992 19. Aaberg TM. Macular holes: a review. Surv Ophthalmol 1970; 15: 139-62. 20. Morgan CM, Schatz H. Involutional macular thinning: a pre-macular hole condition. Ophthalmology 1986;93: 15361. 21. Margherio RR, Schepens CL. Macular breaks. I. Diagnosis, etiology, and observations. Am J Ophthalmol 1972;74:21932. 22. Morgan CM, Schatz H. Idiopathic macular holes. Am J Ophthalmol 1985;99:437-44. 23. McDonnell PJ, Fine SL, Hillis AI. Clinical features of idiopathic macular cysts and holes. Am J Ophthalmol 1982;93: 777-86.
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24. Akiba J, Yoshida A, Trempe CL. Risk of developing a macular hole. Arch Ophthalmol 1990; 108: 1088-90. 25. Bronstein MA, Trempe CL, Freeman HM. Fellow eyes of eyes with macular holes. Am J Ophthalmol 1981;92:75761. 26. Hauch TL, Straatsma BR, Kreiger AE, Hayreh MMS. Macular holes: etiology and visual function. In: Ryan SJ, Dawson AK, Little HL, eds. Retinal Diseases. Orlando: Grone & Stratton, 1985;Chap. 28. 27. Akiba J, Quiroz MA, Trempe CL. Role of posterior vitreous detachment in idiopathic macular holes. Ophthalmology 1990;97: 1610-13.