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Macular Changes With Pit Of Optic Disc*
NOTES, CASES, INSTRUMENTS
1070
MACULAR CHANGES W I T H P I T O F OPTIC DISC* FLTJORESCEIN PHOTOGRAPHY ALBIN W.
JOHNSON,
J. LAWTON S M I T H ,
M.D. M.D.
AND LEONARD M .
HART
Durham, North Carolina
Although the association of macular le sions with congenital pits of the optic discs has been reported,1"3 this is the first such case to be studied by the technique of in travenous fluorescein fundus photography. CASE REPORT
This 30-year-old white man complained of pain less loss of vision in the left eye for 18 months. Previously, vision in the left eye had always been better than in the right. On examination, the corrected visual acuity was 20/50 in the right eye and hand movements in the left. External and biomicroscopic examinations of both eyes were normal but for the presence of a dense mature cataract in the left eye. The intra ocular pressures were normal. On ophthalmoscopy, the right fundus was well visualized and showed an oval, gray discolored area along the temporal border of the disc. This initially appeared convex. With the Hruby lens, however, it was seen to be definitely concave and covered with a grayish veil. It was a typical pit of the optic disc. The macula showed a grayish yellow, finely pigmented, minimally elevated disciform degeneration. The left fundus could not be visualized. The visual field in the right eye showed a small nasal enlargement of the blindspot to 1/1000 white, consistent with the anatomic location of the pit, and a small central scotoma to 3/1000 red. An intracapsular cataract extraction, using alpha chymotrypsin, was performed. The postoperative course was uncomplicated and six weeks later the vision in the left eye with an aphakic correction was 20/30.
are small invaginations, into the substance of the optic nervehead, of rudimentary ret inal tissue, nerve fiber material and pigmented tissue, suggestive of pigment epi thelium, with some supporting glial tissues. The pits apparently have no age or sex pref erence, and vary from one third to one eighth of the disc in diameter. They usually are round or oval but can be triangular or furrowlike. Typically, they have a grayshaded color. They almost always occur in the inferior temporal portion of the disc and more than half the cases show some type of field defect, either enlargement of the blindspot or arcuate scotoma. The pits have been thought to be either pathways of cilioretinal arteries where atro phy of the periarterial tissues has occurred, or abnormal, small colobomas, or an abnor mality of the primitive epithelium of the papilla with persistence of the optic-stalk lumen. Kranenburg 2 reviewed the literature through 1959 and found that about one fourth of all the reported cases of pits of the disc have shown some type of macular abnormality. In his series of 24 cases, 30
DISCUSSION
Pits of the optic disc are rare, only 72
cases being reported through 1942. Of these, eight were bilateral.4 Pathologically, the pits * From the Division of Ophthalmology, Duke University Medical Center, and the Department of Medical Illustration, Veterans Administration Hos pital. This work was supported in part by grant 2B-S232 from the National Institute of Neurolog ical Diseases and Blindness.
Fig. 1 (Johnson, Smith and Hart). Photograph of patient's right fundus, showing the pit of the optic disc and macular degeneration. The two cen tral black spots on each of the illustrations are artefacts.
NOTES, CASES, INSTRUMENTS
1071
Fig. 2 (Johnson, Smith and Hart). Photograph of patient's right fundus, showing fluorescence of retinal vasculature following the injection of fluorescein in an antecubital vein.
Fig. 3 (Johnson, Smith and Hart). Photographs of patient's left fundus, showing fluorescence of retinal vasculature following the injection of fluo rescein in an antecubital vein.
percent had changes suggestive of previous central serous retinopathy. Sugar 8 has re ported two more cases of pits of the disc with macular lesions, each with pigment at the macula. Kranenburg has proposed the theory that the pit, because of its location at the inferior temporal margin of the disc, interferes with the vascular and neural supply of the mac ula, and hence predisposes it to injury or de generation. Sugar suggests that aqueous humor in the vitreous space passes by way of the pit to the subretinal-retinal space and dissects in this space toward the macula, producing a picture like that of central serous retinopathy. Later the aqueous is ab sorbed, leaving irregular pigmentation at the macula. In order to validate the theory of altered vascularity of the macula due to the pit, fluorescein photographs of the fundi of the present patient were taken according to the technique reported by Novotny and Alvis5 and modified by Hart and David.6 With this technique, photographs were made with a Bausch and Lomb retinal camera with an electronic flash tube and power supply. The
light source which excites the fluorescence passes through a Wratten gelatin filter, No. 47, and a No. 56 Wratten filter is used as a barrier at the film plane. Maximum intensity of the fluorescence ap pears between 15 and 30 seconds following injection of 10 cc. of 10-percent fluorescein, and photographs of each fundus at its max imum fluorescence are shown in Figures 2 and 3. Because the fluorescein was injected manually and because the photographs could be taken only about every 10 seconds, the photographs of maximum fluorescence were at 14 seconds in the right eye and 25 sec onds in the left. In the left at no time was there greater fluorescence in the perimacular area than in Figure 3. The perimacular fluorescence in the right eye was present in the 14-second photograph and was still prominent one minute after injection. In essence, the left eye showed a normal vascular pattern, including the macular area. In the right eye, however, there was marked increase in fluorescence in the peri macular area and in a band running from the area of the pit toward the macula. This is highly suggestive that blood flow to the
1072
NOTES, CASES, INSTRUMENTS
macular area is increased rather than de creased in this condition. This would tend to invalidate the theory that the cause of the macular disease with the pits of the disc is due to interference with the blood supply of the macular area by the pit. Whether or not the increased vascularity is the cause of or the result of the macular degeneration, and whether the macular lesions are due to involvement of the papillomacular bundle by the pit are questions that remain to be an swered.
case of a pit of the optic disc with macular degeneration is presented. Photographs of the normal and affected eye following the in travenous injection of fluorescein illustrate an increased vascularity of the macular area of the affected eye, suggesting an increased rather than a decreased blood flow to the macula in this condition.
SUMMARY
The authors would like to acknowledge gratefully the aid of Dr. Noble J. David in obtaining the fluorescein photographs and suggestions made re garding this work by Dr. W. B. Anderson, Sr.
A brief discussion of pits of the optic disc and associated macular changes is given. A
Division of Ophthalmology.
REFERENCES
1. Halbertsma, K. T. A.: Craterlike hole and coloboma of the disc associated with changes at the macula. Brit. J. Ophth., 11:11, 1927. 2. Kranenburg, E. W. : Craterlike holes in the optic disc and central serous retinopathy. AMA Arch. Ophth., 64:912 (Dec.) 1960. 3. Sugar, H. S.: Congenital pits of the optic disc with acquired macular pathology. Am. J. Ophth., 53:307 (Feb.) 1962. 4. Greear, J. N.: Pits or craterlike holes in the optic disc. AMA Arch. Ophth., 28:467, 1942. 5. Novotny, H. R., and Alvis, D. L.: A method of photographing fluorescence in circulating blood in the human retina. Circulation, 24:82, 1961. 6. Hart, L. M., and David, N. J.: Serial photography of fluorescein dye in the retinal vessels. Pre sented at the Veterans Administration Medical Research Conference, Cincinnati, Ohio, December 5, 1961.
IRIS FORCEPS* J. W. JERVEY, JR., M.D. Greenville, South Carolina
in all directions. It is perfect for picking up a small prolapse in performing peripheral iridectomy.
This forceps is made on a standard blank with a round sharp cup measuring one mm. across at the end of each blade. It can be pushed into the angle from almost any di rection and the iris seized at any desired point and delivered for excision. There is little danger to the lens capsule, since the closed forceps has a smooth rounded surface * Presented to the 98th annual meeting of the American Ophthalmological Society, Hot Springs, Virginia, May, 1962. This instrument is available through the Storz Instrument Company, Saint Louis, Missouri.
Fig. 1 (Jervey). The iris forceps.
1070
MACULAR CHANGES W I T H P I T O F OPTIC DISC* FLTJORESCEIN PHOTOGRAPHY ALBIN W.
JOHNSON,
J. LAWTON S M I T H ,
M.D. M.D.
AND LEONARD M .
HART
Durham, North Carolina
Although the association of macular le sions with congenital pits of the optic discs has been reported,1"3 this is the first such case to be studied by the technique of in travenous fluorescein fundus photography. CASE REPORT
This 30-year-old white man complained of pain less loss of vision in the left eye for 18 months. Previously, vision in the left eye had always been better than in the right. On examination, the corrected visual acuity was 20/50 in the right eye and hand movements in the left. External and biomicroscopic examinations of both eyes were normal but for the presence of a dense mature cataract in the left eye. The intra ocular pressures were normal. On ophthalmoscopy, the right fundus was well visualized and showed an oval, gray discolored area along the temporal border of the disc. This initially appeared convex. With the Hruby lens, however, it was seen to be definitely concave and covered with a grayish veil. It was a typical pit of the optic disc. The macula showed a grayish yellow, finely pigmented, minimally elevated disciform degeneration. The left fundus could not be visualized. The visual field in the right eye showed a small nasal enlargement of the blindspot to 1/1000 white, consistent with the anatomic location of the pit, and a small central scotoma to 3/1000 red. An intracapsular cataract extraction, using alpha chymotrypsin, was performed. The postoperative course was uncomplicated and six weeks later the vision in the left eye with an aphakic correction was 20/30.
are small invaginations, into the substance of the optic nervehead, of rudimentary ret inal tissue, nerve fiber material and pigmented tissue, suggestive of pigment epi thelium, with some supporting glial tissues. The pits apparently have no age or sex pref erence, and vary from one third to one eighth of the disc in diameter. They usually are round or oval but can be triangular or furrowlike. Typically, they have a grayshaded color. They almost always occur in the inferior temporal portion of the disc and more than half the cases show some type of field defect, either enlargement of the blindspot or arcuate scotoma. The pits have been thought to be either pathways of cilioretinal arteries where atro phy of the periarterial tissues has occurred, or abnormal, small colobomas, or an abnor mality of the primitive epithelium of the papilla with persistence of the optic-stalk lumen. Kranenburg 2 reviewed the literature through 1959 and found that about one fourth of all the reported cases of pits of the disc have shown some type of macular abnormality. In his series of 24 cases, 30
DISCUSSION
Pits of the optic disc are rare, only 72
cases being reported through 1942. Of these, eight were bilateral.4 Pathologically, the pits * From the Division of Ophthalmology, Duke University Medical Center, and the Department of Medical Illustration, Veterans Administration Hos pital. This work was supported in part by grant 2B-S232 from the National Institute of Neurolog ical Diseases and Blindness.
Fig. 1 (Johnson, Smith and Hart). Photograph of patient's right fundus, showing the pit of the optic disc and macular degeneration. The two cen tral black spots on each of the illustrations are artefacts.
NOTES, CASES, INSTRUMENTS
1071
Fig. 2 (Johnson, Smith and Hart). Photograph of patient's right fundus, showing fluorescence of retinal vasculature following the injection of fluorescein in an antecubital vein.
Fig. 3 (Johnson, Smith and Hart). Photographs of patient's left fundus, showing fluorescence of retinal vasculature following the injection of fluo rescein in an antecubital vein.
percent had changes suggestive of previous central serous retinopathy. Sugar 8 has re ported two more cases of pits of the disc with macular lesions, each with pigment at the macula. Kranenburg has proposed the theory that the pit, because of its location at the inferior temporal margin of the disc, interferes with the vascular and neural supply of the mac ula, and hence predisposes it to injury or de generation. Sugar suggests that aqueous humor in the vitreous space passes by way of the pit to the subretinal-retinal space and dissects in this space toward the macula, producing a picture like that of central serous retinopathy. Later the aqueous is ab sorbed, leaving irregular pigmentation at the macula. In order to validate the theory of altered vascularity of the macula due to the pit, fluorescein photographs of the fundi of the present patient were taken according to the technique reported by Novotny and Alvis5 and modified by Hart and David.6 With this technique, photographs were made with a Bausch and Lomb retinal camera with an electronic flash tube and power supply. The
light source which excites the fluorescence passes through a Wratten gelatin filter, No. 47, and a No. 56 Wratten filter is used as a barrier at the film plane. Maximum intensity of the fluorescence ap pears between 15 and 30 seconds following injection of 10 cc. of 10-percent fluorescein, and photographs of each fundus at its max imum fluorescence are shown in Figures 2 and 3. Because the fluorescein was injected manually and because the photographs could be taken only about every 10 seconds, the photographs of maximum fluorescence were at 14 seconds in the right eye and 25 sec onds in the left. In the left at no time was there greater fluorescence in the perimacular area than in Figure 3. The perimacular fluorescence in the right eye was present in the 14-second photograph and was still prominent one minute after injection. In essence, the left eye showed a normal vascular pattern, including the macular area. In the right eye, however, there was marked increase in fluorescence in the peri macular area and in a band running from the area of the pit toward the macula. This is highly suggestive that blood flow to the
1072
NOTES, CASES, INSTRUMENTS
macular area is increased rather than de creased in this condition. This would tend to invalidate the theory that the cause of the macular disease with the pits of the disc is due to interference with the blood supply of the macular area by the pit. Whether or not the increased vascularity is the cause of or the result of the macular degeneration, and whether the macular lesions are due to involvement of the papillomacular bundle by the pit are questions that remain to be an swered.
case of a pit of the optic disc with macular degeneration is presented. Photographs of the normal and affected eye following the in travenous injection of fluorescein illustrate an increased vascularity of the macular area of the affected eye, suggesting an increased rather than a decreased blood flow to the macula in this condition.
SUMMARY
The authors would like to acknowledge gratefully the aid of Dr. Noble J. David in obtaining the fluorescein photographs and suggestions made re garding this work by Dr. W. B. Anderson, Sr.
A brief discussion of pits of the optic disc and associated macular changes is given. A
Division of Ophthalmology.
REFERENCES
1. Halbertsma, K. T. A.: Craterlike hole and coloboma of the disc associated with changes at the macula. Brit. J. Ophth., 11:11, 1927. 2. Kranenburg, E. W. : Craterlike holes in the optic disc and central serous retinopathy. AMA Arch. Ophth., 64:912 (Dec.) 1960. 3. Sugar, H. S.: Congenital pits of the optic disc with acquired macular pathology. Am. J. Ophth., 53:307 (Feb.) 1962. 4. Greear, J. N.: Pits or craterlike holes in the optic disc. AMA Arch. Ophth., 28:467, 1942. 5. Novotny, H. R., and Alvis, D. L.: A method of photographing fluorescence in circulating blood in the human retina. Circulation, 24:82, 1961. 6. Hart, L. M., and David, N. J.: Serial photography of fluorescein dye in the retinal vessels. Pre sented at the Veterans Administration Medical Research Conference, Cincinnati, Ohio, December 5, 1961.
IRIS FORCEPS* J. W. JERVEY, JR., M.D. Greenville, South Carolina
in all directions. It is perfect for picking up a small prolapse in performing peripheral iridectomy.
This forceps is made on a standard blank with a round sharp cup measuring one mm. across at the end of each blade. It can be pushed into the angle from almost any di rection and the iris seized at any desired point and delivered for excision. There is little danger to the lens capsule, since the closed forceps has a smooth rounded surface * Presented to the 98th annual meeting of the American Ophthalmological Society, Hot Springs, Virginia, May, 1962. This instrument is available through the Storz Instrument Company, Saint Louis, Missouri.
Fig. 1 (Jervey). The iris forceps.