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Abnormal Foveal Avascular Zone in Nanophthalmos
Abnormal Foveal Avascular Zone in Nanophthalmos Mark K. Walsh and Morton F. Goldberg PURPOSE:
To evaluate the foveas of nanophthalmic pa-
tients. Retrospective observational case series. METHODS: Four nanophthalmic patients examined between April 2005 and April 2006 were included. Visual acuity (VA), refractive correction, axial length, corneal diameter, presence or lack of foveal light reflex, as well as fluorescein angiograms (FAs), and optical coherence tomography (OCT) scans of the maculae were evaluated. RESULTS: None of the eight eyes had a foveal light reflex, corresponding to lack of a normal foveal pit on OCT. Fluorescein angiography showed no normal foveal avascular zones; all were either completely absent or small and rudimentary. CONCLUSIONS: Nanophthalmic patients rarely have best-corrected visual acuity (BCVA) better than 20/40 at any point in their lives, even with an absence of known complications, such as uveal effusion or glaucoma. In many patients, this visual deficiency may correspond to an absent or rudimentary foveal avascular zone and lack of a normal foveal pit. (Am J Ophthalmol 2007;143:1067–1068. © 2007 by Elsevier Inc. All rights reserved.) DESIGN:
P
ATIENTS WITH NANOPHTHALMOS, A RARE BILATERAL
disorder, have short axial lengths, usually less than 20 mm. The condition is often associated with extreme hyperopia, usually greater than ⫹8.00 diopters. Characteristics include microcornea, shallow anterior chamber, thickened sclera, and a high lens/globe volume ratio. Patients are predisposed to angle closure, uveal effusion, and retinochoroidal folds. The best-corrected visual acuity (BCVA) is rarely better than 20/40 at any point in patients’ lives, even with an absence of uveal effusion or glaucoma.1–5 The reason for this poor visual acuity (VA) has not previously been understood. Between April 2005 and April 2006, we examined four nanophthalmic patients, all of whom are included in this retrospective review. Their VA, refractive correction, axial length, corneal diameter, presence or lack of a foveal light reflex, fluorescein angiograms (FAs), and optical coherence tomography (OCT) scans are reported herein.
Supplemental Material available at AJO.com. Accepted for publication Jan 24, 2007. From the Wilmer Ophthalmological Institute, Johns Hopkins School of Medicine, Baltimore, Maryland. Inquiries to Morton F. Goldberg, Woods Bldg. 276, Wilmer Ophthalmological Institute, 600 N. Wolfe Street, Baltimore, MD 21287; e-mail: [email protected] 0002-9394/07/$32.00
©
2007 BY
● CASE 1: A 70-year-old white male with axial lengths of 16.14 mm right eye (OD), 15.97 mm left eye (OS), and horizontal corneal diameters of 10 mm to 10.5 mm both eyes (OU). His refraction prior to cataract surgery was ⫹16.50 ⫹ 0.50 x 175 OD and ⫹15.75 ⫹1.00 x 178 OS. His best-recorded VA from 1998 to March 2006 was 20/40⫹1 OD and 20/50 OS. He had cataract surgery OU (May 6, 1999 OD and February 26, 1998 OS) and has a history of neovascular age-related macular degeneration OU with submacular fibrosis OD. FA showed an absent or small, rudimentary foveal avascular zone (FAZ) OU (Figure 1) and occult choroidal neovascularization OS (see Supplemental Figure 1 available at AJO.com). OCT on March 7, 2006 demonstrated lack of a normal foveal contour/pit OS (Figure 2). ● CASE 2:
A 58-year-old white female with axial lengths of 17.43 mm OD, 17.77 mm OS, and horizontal corneal diameters of 10 mm to 10.5 mm OU. Her best-recorded VA was 20/30 OD and 20/50 OS (records from 1993 to March 14, 2006). She has undergone cataract surgery OU (December 6, 2004 OD and June 30, 2003 OS) with persistent postoperative choroidal folds involving the maculae OU. FA showed an absent or small, rudimentary FAZ OU and choroidal folds OS (Supplemental Figure 1). OCT demonstrated a lack of a normal foveal contour/pit OU (see Supplemental Figure 2 available at AJO.com).
● CASE 3: A 54-year-old white male with axial lengths of 16.57 mm OD, 16.42 OS, and horizontal corneal diameters of 10 mm OU. His contact lens prescription was ⫹16.5 diopters OS. His best-recorded VA was 20/60 OD and 20/32-2 OS (records from April 2005 to March 27, 2006). He had combined cataract surgery and trabeculectomy OD on May 17, 2005. The patient developed uveal effusion and then had sclerectomy OD on January 3, 2006. FA showed an absent or small, rudimentary FAZ OS with macular window defects (Supplemental Figure 1). OCT showed absence of a normal foveal contour/pit OS (Supplemental Figure 2). ● CASE 4:
A 61-year-old white female with axial lengths of 18.3 mm OD, 18.16 mm OS, and horizontal corneal diameters of 10.5 mm to 11 mm OD and 10.5 mm OS. Her refractive error is ⫹8.75 ⫹0.50 ⫻ 180 OD, ⫹8.25 ⫹0.75 ⫻ 180 OS, and her best-recorded VA is 20/40 OD and 20/80 OS (records from September 1998 to April 17, 2006). On initial presentation to us, the patient had bilateral serous choroidal detachments. She underwent sclerectomy with vortex vein decompression OS (March 1999) and then repeat sclerectomy OS (April 1999). She developed subfoveal CNV OD in July 2005. FA showed an absent or small, rudimentary FAZ OS (Supplemental Figure 1), and her OCT demonstrated lack of a normal foveal contour/pit OD (Supplemental Figure 2; the macula was atrophic OS).
ELSEVIER INC. ALL
RIGHTS RESERVED.
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FIGURE 1. The fluorescein angiograms (FAs) of a normal patient with a normal foveal avascular zone (FAZ) and a nanophthalmic patient with a small, rudimentary FAZ (image on right of each pair ⴝ 3x magnification of fovea). The images on the left are from a normal 28-year-old female with a normal FAZ. Imaging of the right eye of nanophthalmic patient in Case 1 reveals a small, rudimentary FAZ (Right).
FIGURE 2. Foveal ocular coherence tomography (OCT) scans, which show lack of normal foveal contour in nanophthalmos. The image on the left is from a normal 37-year-old male showing a normal foveal pit/contour with mean subfield retinal thicknesses for this patient (circular diagram) and color code for percentiles in the normal population distribution. The OCT scan from patient in Case 1 shows a thickened macula and lack of a foveal pit OS (Right).
The abnormal foveal vascular architecture, in the form of an absent or rudimentary FAZ, is previously undescribed in nanophthalmos (based on a PubMed search), although it has been noted in posterior microphthalmos.6,7 This anomaly likely impacts foveal function, offering a novel explanation for the typically poor BCVA in at least some, if not all, nanophthalmic patients. This finding also supports the conventional belief that the normally avascular central fovea is important in obtaining central VA as good as 20/20 or better. Interestingly, in reports of posterior microphthalmos,6,7 no eye had VA better than 20/40. This information should assist clinicians in deciding when to perform cataract surgery in nanophthalmic patients, given that the abnormal foveal architecture may limit visual potential.
REFERENCES
1. Duke-Elder S. Anomalies in the size of the eye. In: Duke-Elder S, editor. System of Ophthalmology. Vol. III. St Louis, Missouri: Mosby, 1963;488 – 495. 2. Hirsch SE, Waltman SR, LaPiana FG. Bilateral nanophthalmos. Arch Ophthalmol 1973;89:353. 3. Cross HE, Yoder F. Familial nanophthalmos. Am J Ophthalmol 1976;300 –306. 4. Singh OS, Simmons RJ, Brockhurst RJ, Trempe CL. Nanophthalmos: a perspective on identification and therapy. Ophthalmology 1982;89:1006 –1012. 5. Serrano JC, Hodgkins PR, Taylor DS, Gole GA, Kriss A. The nanophthalmic macula. Br J Ophthalmol 1998;82:276 –279. 6. Spitznas M, Gerke E, Bateman JB. Hereditary posterior microphthalmos with papillomacular fold and high hyperopia. Arch Ophthalmol 1983;101:413– 417. 7. Khairallah M, Messaoud R, Zaouali S, Ben Yahia S, Ladjimi A, Jenzri S. Posterior segment changes associated with posterior microphthalmos. Ophthalmology 2002;109:569 – 574.
THIS STUDY WAS SUPPORTED BY THE GUERRIERI RETINAL Research Fund and the James T. Handa Research Award from the Wilmer Ophthalmological Institute, Baltimore, Maryland. The authors indicate no conflict of interest. Both authors were involved in design and conduct of study; collection, analysis and interpretation; and preparation of manuscript, review, and approval of the manuscript.
1068
AMERICAN JOURNAL
OF
OPHTHALMOLOGY
JUNE 2007
SUPPLEMENTAL FIGURE 1. Fluorescein angiograms (FAs) of nanophthalmic patients, each of which shows a small, rudimentary foveal avascular zone (FAZ; image on right of each pair ⴝ 3x magnification of fovea). The top left images are from a normal 28-year-old female with a normal FAZ. Imaging of the left eye of nanophthalmic Patient 1 reveals a small, rudimentary FAZ and occult CNV (Top right). Nanophthalmic Patient 2’s FA shows a small, rudimentary FAZ, juxtafoveal window defect (fades late without leakage), and superior choroidal folds (2nd row left). Patient 2’s left eye has a small, rudimentary FAZ and choroidal folds (2nd row right, images). The FA from nanophthalmic Patient 3 shows a small, rudimentary FAZ and window defects OS (fade late without leakage; 3rd row left). Nanophthalmic Patient 4 also has a small, rudimentary FAZ OS (3rd row right, images).
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SUPPLEMENTAL FIGURE 2. Foveal ocular coherence tomography (OCT) scans, which show lack of normal foveal contours in nanophthalmic patients. The top left image is from a normal 37-year-old male showing a normal foveal pit/contour with mean subfield retinal thicknesses for this patient (circular diagram) and color code for percentiles in the normal population distribution. Patient 2 had a thickened macula and lack of a foveal pit OD both prior to (top row right; OCT) and after development of a post-operative choroidal effusion (2nd row left, OCT) and OS (2nd row right). The OCT of Patient 3 also reveals a thickened macula and lack of a foveal pit OS (3rd row left). Patient 4 has a macula of normal thickness but also lacks a foveal pit OD (3rd row right).
1068.e2
AMERICAN JOURNAL
OF
OPHTHALMOLOGY
JUNE 2007
To evaluate the foveas of nanophthalmic pa-
tients. Retrospective observational case series. METHODS: Four nanophthalmic patients examined between April 2005 and April 2006 were included. Visual acuity (VA), refractive correction, axial length, corneal diameter, presence or lack of foveal light reflex, as well as fluorescein angiograms (FAs), and optical coherence tomography (OCT) scans of the maculae were evaluated. RESULTS: None of the eight eyes had a foveal light reflex, corresponding to lack of a normal foveal pit on OCT. Fluorescein angiography showed no normal foveal avascular zones; all were either completely absent or small and rudimentary. CONCLUSIONS: Nanophthalmic patients rarely have best-corrected visual acuity (BCVA) better than 20/40 at any point in their lives, even with an absence of known complications, such as uveal effusion or glaucoma. In many patients, this visual deficiency may correspond to an absent or rudimentary foveal avascular zone and lack of a normal foveal pit. (Am J Ophthalmol 2007;143:1067–1068. © 2007 by Elsevier Inc. All rights reserved.) DESIGN:
P
ATIENTS WITH NANOPHTHALMOS, A RARE BILATERAL
disorder, have short axial lengths, usually less than 20 mm. The condition is often associated with extreme hyperopia, usually greater than ⫹8.00 diopters. Characteristics include microcornea, shallow anterior chamber, thickened sclera, and a high lens/globe volume ratio. Patients are predisposed to angle closure, uveal effusion, and retinochoroidal folds. The best-corrected visual acuity (BCVA) is rarely better than 20/40 at any point in patients’ lives, even with an absence of uveal effusion or glaucoma.1–5 The reason for this poor visual acuity (VA) has not previously been understood. Between April 2005 and April 2006, we examined four nanophthalmic patients, all of whom are included in this retrospective review. Their VA, refractive correction, axial length, corneal diameter, presence or lack of a foveal light reflex, fluorescein angiograms (FAs), and optical coherence tomography (OCT) scans are reported herein.
Supplemental Material available at AJO.com. Accepted for publication Jan 24, 2007. From the Wilmer Ophthalmological Institute, Johns Hopkins School of Medicine, Baltimore, Maryland. Inquiries to Morton F. Goldberg, Woods Bldg. 276, Wilmer Ophthalmological Institute, 600 N. Wolfe Street, Baltimore, MD 21287; e-mail: [email protected] 0002-9394/07/$32.00
©
2007 BY
● CASE 1: A 70-year-old white male with axial lengths of 16.14 mm right eye (OD), 15.97 mm left eye (OS), and horizontal corneal diameters of 10 mm to 10.5 mm both eyes (OU). His refraction prior to cataract surgery was ⫹16.50 ⫹ 0.50 x 175 OD and ⫹15.75 ⫹1.00 x 178 OS. His best-recorded VA from 1998 to March 2006 was 20/40⫹1 OD and 20/50 OS. He had cataract surgery OU (May 6, 1999 OD and February 26, 1998 OS) and has a history of neovascular age-related macular degeneration OU with submacular fibrosis OD. FA showed an absent or small, rudimentary foveal avascular zone (FAZ) OU (Figure 1) and occult choroidal neovascularization OS (see Supplemental Figure 1 available at AJO.com). OCT on March 7, 2006 demonstrated lack of a normal foveal contour/pit OS (Figure 2). ● CASE 2:
A 58-year-old white female with axial lengths of 17.43 mm OD, 17.77 mm OS, and horizontal corneal diameters of 10 mm to 10.5 mm OU. Her best-recorded VA was 20/30 OD and 20/50 OS (records from 1993 to March 14, 2006). She has undergone cataract surgery OU (December 6, 2004 OD and June 30, 2003 OS) with persistent postoperative choroidal folds involving the maculae OU. FA showed an absent or small, rudimentary FAZ OU and choroidal folds OS (Supplemental Figure 1). OCT demonstrated a lack of a normal foveal contour/pit OU (see Supplemental Figure 2 available at AJO.com).
● CASE 3: A 54-year-old white male with axial lengths of 16.57 mm OD, 16.42 OS, and horizontal corneal diameters of 10 mm OU. His contact lens prescription was ⫹16.5 diopters OS. His best-recorded VA was 20/60 OD and 20/32-2 OS (records from April 2005 to March 27, 2006). He had combined cataract surgery and trabeculectomy OD on May 17, 2005. The patient developed uveal effusion and then had sclerectomy OD on January 3, 2006. FA showed an absent or small, rudimentary FAZ OS with macular window defects (Supplemental Figure 1). OCT showed absence of a normal foveal contour/pit OS (Supplemental Figure 2). ● CASE 4:
A 61-year-old white female with axial lengths of 18.3 mm OD, 18.16 mm OS, and horizontal corneal diameters of 10.5 mm to 11 mm OD and 10.5 mm OS. Her refractive error is ⫹8.75 ⫹0.50 ⫻ 180 OD, ⫹8.25 ⫹0.75 ⫻ 180 OS, and her best-recorded VA is 20/40 OD and 20/80 OS (records from September 1998 to April 17, 2006). On initial presentation to us, the patient had bilateral serous choroidal detachments. She underwent sclerectomy with vortex vein decompression OS (March 1999) and then repeat sclerectomy OS (April 1999). She developed subfoveal CNV OD in July 2005. FA showed an absent or small, rudimentary FAZ OS (Supplemental Figure 1), and her OCT demonstrated lack of a normal foveal contour/pit OD (Supplemental Figure 2; the macula was atrophic OS).
ELSEVIER INC. ALL
RIGHTS RESERVED.
1067
FIGURE 1. The fluorescein angiograms (FAs) of a normal patient with a normal foveal avascular zone (FAZ) and a nanophthalmic patient with a small, rudimentary FAZ (image on right of each pair ⴝ 3x magnification of fovea). The images on the left are from a normal 28-year-old female with a normal FAZ. Imaging of the right eye of nanophthalmic patient in Case 1 reveals a small, rudimentary FAZ (Right).
FIGURE 2. Foveal ocular coherence tomography (OCT) scans, which show lack of normal foveal contour in nanophthalmos. The image on the left is from a normal 37-year-old male showing a normal foveal pit/contour with mean subfield retinal thicknesses for this patient (circular diagram) and color code for percentiles in the normal population distribution. The OCT scan from patient in Case 1 shows a thickened macula and lack of a foveal pit OS (Right).
The abnormal foveal vascular architecture, in the form of an absent or rudimentary FAZ, is previously undescribed in nanophthalmos (based on a PubMed search), although it has been noted in posterior microphthalmos.6,7 This anomaly likely impacts foveal function, offering a novel explanation for the typically poor BCVA in at least some, if not all, nanophthalmic patients. This finding also supports the conventional belief that the normally avascular central fovea is important in obtaining central VA as good as 20/20 or better. Interestingly, in reports of posterior microphthalmos,6,7 no eye had VA better than 20/40. This information should assist clinicians in deciding when to perform cataract surgery in nanophthalmic patients, given that the abnormal foveal architecture may limit visual potential.
REFERENCES
1. Duke-Elder S. Anomalies in the size of the eye. In: Duke-Elder S, editor. System of Ophthalmology. Vol. III. St Louis, Missouri: Mosby, 1963;488 – 495. 2. Hirsch SE, Waltman SR, LaPiana FG. Bilateral nanophthalmos. Arch Ophthalmol 1973;89:353. 3. Cross HE, Yoder F. Familial nanophthalmos. Am J Ophthalmol 1976;300 –306. 4. Singh OS, Simmons RJ, Brockhurst RJ, Trempe CL. Nanophthalmos: a perspective on identification and therapy. Ophthalmology 1982;89:1006 –1012. 5. Serrano JC, Hodgkins PR, Taylor DS, Gole GA, Kriss A. The nanophthalmic macula. Br J Ophthalmol 1998;82:276 –279. 6. Spitznas M, Gerke E, Bateman JB. Hereditary posterior microphthalmos with papillomacular fold and high hyperopia. Arch Ophthalmol 1983;101:413– 417. 7. Khairallah M, Messaoud R, Zaouali S, Ben Yahia S, Ladjimi A, Jenzri S. Posterior segment changes associated with posterior microphthalmos. Ophthalmology 2002;109:569 – 574.
THIS STUDY WAS SUPPORTED BY THE GUERRIERI RETINAL Research Fund and the James T. Handa Research Award from the Wilmer Ophthalmological Institute, Baltimore, Maryland. The authors indicate no conflict of interest. Both authors were involved in design and conduct of study; collection, analysis and interpretation; and preparation of manuscript, review, and approval of the manuscript.
1068
AMERICAN JOURNAL
OF
OPHTHALMOLOGY
JUNE 2007
SUPPLEMENTAL FIGURE 1. Fluorescein angiograms (FAs) of nanophthalmic patients, each of which shows a small, rudimentary foveal avascular zone (FAZ; image on right of each pair ⴝ 3x magnification of fovea). The top left images are from a normal 28-year-old female with a normal FAZ. Imaging of the left eye of nanophthalmic Patient 1 reveals a small, rudimentary FAZ and occult CNV (Top right). Nanophthalmic Patient 2’s FA shows a small, rudimentary FAZ, juxtafoveal window defect (fades late without leakage), and superior choroidal folds (2nd row left). Patient 2’s left eye has a small, rudimentary FAZ and choroidal folds (2nd row right, images). The FA from nanophthalmic Patient 3 shows a small, rudimentary FAZ and window defects OS (fade late without leakage; 3rd row left). Nanophthalmic Patient 4 also has a small, rudimentary FAZ OS (3rd row right, images).
VOL. 143, NO. 6
BRIEF REPORTS
1068.e1
SUPPLEMENTAL FIGURE 2. Foveal ocular coherence tomography (OCT) scans, which show lack of normal foveal contours in nanophthalmic patients. The top left image is from a normal 37-year-old male showing a normal foveal pit/contour with mean subfield retinal thicknesses for this patient (circular diagram) and color code for percentiles in the normal population distribution. Patient 2 had a thickened macula and lack of a foveal pit OD both prior to (top row right; OCT) and after development of a post-operative choroidal effusion (2nd row left, OCT) and OS (2nd row right). The OCT of Patient 3 also reveals a thickened macula and lack of a foveal pit OS (3rd row left). Patient 4 has a macula of normal thickness but also lacks a foveal pit OD (3rd row right).
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AMERICAN JOURNAL
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OPHTHALMOLOGY
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