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Congenital Epithelial Cyst of The IRIS Stroma*
UVEITIS AND HYPERURICEMIA
It has no consistent effect on the serum or urine uric acid levels. An oral dose of 0.5 mg. may be given every hour until the pain is relieved or until nausea, vomiting or di arrhea appear. One to two mg. is then given daily. One hundred units of ACTH-gel given intramuscularly may help terminate an attack, especially when seen late. The response to steriods is less consistent in these cases than with other methods of ther apy, as again the case discussed shows. Uricosuric drugs are not indicated unless the patient has either tophi, serum uric acid levels consistently above 8.0 mg. percent, or frequent attacks of acute gout despite ade quate doses of colchicine. Although a lowpurine diet does not reduce the serum urate levels to any great extent, the small reduc tions obtained in this manner may be the deciding factor in preventing some attacks. It has been pointed out6 that, in spite of the severe insult to the eye, such as this case exemplifies, relatively complete resolu tion generally occurs in inflammation due
291
to gout, suggesting a chemical etiology. Finally, although this is not a common condition, it would seem that gout should be included in the vast number of etiologic possibilities in the evaluation of the uveal tract (at least to the extent of determining the serum uric acid in cases which seem resistant to the usual therapy), where it might account for some of the cases considered idiopathic. A family history of gout or rheumatism, renal calculi, monoarticular arthritis, an increased serum uric acid in the absence of renal disease, tophi, and/or a response to colchicine7 lead to a strongly presumptive diagnosis of a gouty etiology. SUMMARY
A case of severe, generalized, plastic uveitis, associated with an elevation in the serum uric acid and responsive to colchicine, is presented. The case is discussed as it relates to recent concepts of gout. 636 Church Street.
REFERENCES
1. Hogan, M. J., and Zimmerman, L. E.: Ophthalmic Pathology. Philadelphia, Saunders, 1962. 2. McWilliams, J. R.: Ocular findings in gout: Report of a case of conjunctival tophi. Am. J. Ophth., 35:1778-1783, 1952. 3. Falls, H. F.: Clinical detection of the genetic carrier state in ophthalmic pathology. Am. J. Ophth., 37:848, 1954. 4. Wyngaarden, J. B., and Jones, O. W.: The pathogenesis of gout. Med. Clin. N. Am., 45:1241-1256, 1961. 5. Seegmiller, J. E.: The present-day treatment of gout. Med. Clin. N. Am., 45:1259-1271, 1961. 6. Duke-Elder, S.: Textbook of Ophthalmology. St. Louis, Mosby, 1941, v. 3, pp. 2381-2383. 7. Hogan, M. J., Kimura, S. J., and Thygeson, P.: Uveitis in association with rheumatism. AMA Arch. Ophth., 57:407-409, 1957.
CONGENITAL E P I T H E L I A L CYST O F T H E IRIS STROMA* B E R T H A A. KLIEIST, M.D.,
AND GEORGE S. T A N N E R ,
M.D.
Chicago, Illinois
Spontaneous or idiopathic cysts of the iris are principally of two kinds, stromal and intraepithelial. The latter appears to be the more common variety and its pathogene* From the Division of Ophthalmic Surgery, The University of Chicago. This work was done under a grant from the Douglas Smith Foundation for Medical Research of the University of Chicago.
sis is understood. It arises from incomplete obliteration or re-establishment of the space between the two leaves of the secondary optic vesicle, usually with some additional proliferation of the neuro-ectodermal ele ments of these layers. It may occur at any place from the pupillary border of the iris, where it may represent an open and dis-
292
BERTHA A. KLIEN AND GEORGE S. TANNER
a less striking layer of flat cells with the appearance of endothelium. While the former type of lining is undeniably of ectodermal origin, the character of the latter has been debated. Some consider the possi bility of an entirely mesoblastic origin by proliferation of iris endothelium into and along a cavity which might have arisen from closure of an iris crypt (Streiff,2 SchmidtRimpler,4 Terrien, 5 Lagrange 6 ), others be lieve it to arise from surface epithelium (Thiel 7 ) thus considering both types of linings to be derived from similar sources.
Fig. 1 (Klien and Tanner). Right eye of a girl, aged seven years. Congenital intraepithelial cyst of the ciliary body with circumscribed lenticular opacity, which extended from the embryonic nu cleus to the posterior surface of the lens. At this place the convex curvature of the posterior lenticu lar surface became slightly concave, indicating a partial coloboma of the lens at this site. No other anomalies were present.
tended v. Scily's sinus, to the pars plana of the ciliary body. It may be acquired in the course of many different intraocular patho logic processes and thus be found in adult eyes, or it may be congenital. A congenital origin can be established with certainty in the presence of associated defects, such as a lenticular opacity or a lenticular coloboma. A definitely congenital intraepithelial cyst associated with a partial coloboma of the lens was observed by Yudkin 1 in a 16-yearold girl, another is illustrated in Figure 1. Less well understood is the pathogenesis of the congenital stromal cysts, of which a small number was reported and discussed around the turn of the century (11 cases, studied also histopathologically, collected by Streiff,2 Rados 3 ). They lie, as the name im plies, embedded in the stroma of the iris and may be lined with a stratified epithelium sometimes containing goblet cells, or with
Concerning these sources two theories exist. One considers the possibility of cysts derived from misplaced islets of surface ec toderm, which could reach the intraocular location at or near the time the lens vesicle separates from the surface ectoderm at the nine mm. stage (Streiff,2 Nadal 8 ). The other considers the possibility of a neuro-ectodermal origin from cells of the anterior leaf of the secondary optic vesicle, left over from differentiation of the iris muscles (Juselius, 9 Bergemann 10 ). The proponents of the latter concept interpret the cells, containing a mucoid substance, which are found in the epithelial lining of such cysts not as goblet cells but as epithelial cells undergoing mucoid degeneration. Regarding the second theory it should be stated that unused neuro-ectodermal cell ele ments are common in the iris and may give rise to a variety of morphologic pictures (Klien 11 ), but none! of these is characterized by even a rudimentary tendency for cyst formation or anomalous differentiation into secreting goblet cells despite the pluripotential quality of the mother cells. An origin of the intrastromal cysts from misplaced islets of surface ectoderm seems more prob able and would have a counterpart in the cases of aberrant intraocular lacrimai gland (Christensen,12 Hunter 13 ), particularly those in which no communication with extrabulbar regions could be found (Bruce 14 ). R E P O R T OF A CASE
History. One month prior to the first ocular ex amination of a girl, aged four and one-half months,
EPITHELIAL CYST OF IRIS STROMA a "growth" in her left eye was noted by the parents. This lesion increased in size, accompanied by marked lacrimation, during the past few weeks. The child was the first born of healthy parents. A full-term pregnancy had been followed by spon taneous uncomplicated delivery. There was no birth trauma or subsequent ocular injury, and no mater nal illness during gestation. Findings. Examination of the left eye under general anesthesia revealed a slight net-shaped bulbar conjunctival injection, circumscribed stippling of the corneal surface in the superior nasal quad rant, corresponding to an oval lesion in the anterior chamber which touched the posterior corneal sur face in this area. The lesion appeared to be a trans parent stromal cyst of the iris (fig. 2), which pro jected forward and to an equal extent backward into the posterior chamber, touching the lens. Its temporal border came within one mm. of the pupil lary margin and limited pupillary dilation in this quadrant. The lens was clear, the fundus normal. A short tag of a persistent hyaloid vessel was attached to the optic disc. There was no defect, irregularity or scarring of the bulbar walls which might have raised suspicion of a communication between the intraocular lesion and extrabulbar regions. The right eye was normal, the iris was grayish blue in each eye, the tactile tension was soft and equal in the two eyes. The remaining physical examination was normal. Operative procedure. After a conjunctival in cision between the 10- and l-o'clock positions fourmm. from the limbus and dissection of the con junctiva, a limbal incision was made at the 12o'clock position. Attempts to grasp the smooth cyst walls proved futile, consequently the clear con tent of the cyst was partially emptied with a 27gauge needle. An iridectomy was then performed at the 12-o'clock position and much of the now easily grasped wall of the cyst was removed through the nasal part of the wound. Postoperative recovery was uneventful. The last examination of this eye, 14 months after the operation, revealed no lacrimation or signs of discomfort. The left globe was pale, the cornea transparent, the anterior chamber normally deep, the lens clear. The narrow total coloboma of the iris at the 12-o'clock position had straight, parallel out lines. An intermittent manifest left convergence was noted on near fixation and a test of refraction is planned for the near future. HlSTOLOGlC FINDINGS
The postoperative specimen was received in two portions, one consisting mainly of the walls of the cyst and adjacent iris, the other representing a piece of iris containing a small portion of sphincter muscle. Both were fixed in formalin and embedded in paraffin. The posterior wall of the cyst was intact
293
Fig. 2 (Klien and Tanner). Left eye of a girl, aged four months, showing congenital stromal epi thelial cyst of the iris. and consisted of moderately thinned iris, bulging backward and lined anteriorly with stratified epithelium whose thickness varied from two to five layers. There was no base ment membrane. Some of the epithelial cells were flat, others cubical or cylindrical, in terspersed with large cells of the appear ance of goblet cells. The latter stained deeply with the periodic acid-Schiff method, showing a finely granular cytoplasm and a marginal and, in most cells, well-preserved nucleus. They also gave a marked argentaffin reaction with Gomori's silver stain. Rem nants of the content of the cyst, of amor phous or stringy appearance, found in the neighborhood of or attached to these cells, also stained deeply with PAS, suggesting the secretory activity of true goblet cells ra ther than a mucoid degeneration (fig. 3 ) . No connection between the epithelial lining of the posterior wall and the well-visible dilator muscle could be found. The anterior wall of the cyst was present in collapsed remnants on each side. The epithelial lining was similar to that of the posterior wall. The outer half of the wall consisted mainly of extremely thinned iris stroma which could be definitely discerned as such by means of Gomori's trichrome stain (fig. 4). Small and large extensions öf
294
BERTHA A. KLIEN AND GEORGE S. TANNER JSMÎÊÈÎL
Fig. 3 (Klien and Tanner). Excised portion of cyst and adjacent iris, general view, same eye as in Figure 2. (P) Posterior wall of cyst, (G) Goblet cells. (R) Ramifications of cyst in iris stroma. (Periodic acid-Schiff, χ65.)
the cyst's epithelial lining could be followed into the adjacent iris stroma, producing outpouchings of the main lumen. There were abundant vessels, mainly capillaries, in the pupillary as well as in the ciliary stroma of the iris surrounding the cyst. Although the stroma was rich in nuclei, as characteristic for the infantile eye, no inflammatory cells were noted. COMMENT
Despite the surgeon's impression of hav
ing removed only part of the cyst, the pres ent status of the eye 14 months after the operation suggests its total removal. The posterior wall was certainly present in toto in the excised piece; the anterior wall was extremely thin and torn and the question, whether stromal remnants separated it everywhere from the cornea which it touched, could not be answered from study of the histologie specimen. The fact that the cornea remained normal after removal of the cyst makes a purely mechanical contact
Fig. 4 (Klien and Tanner). Excised portions of cyst, same eye as in Figures 2 and 3. (A) Collapsed anterior wall of cyst. (E) Inner epithelial portion of cyst wall. (S) Outer portion of cyst wall, consisting of extremely thinned iris stroma. (Gomori trichrome, X275.)
EPITHELIAL CYST OF IRIS STROMA probable. Only the future will tell if slowgrowing epithelial remnants remained em bedded in the stroma of the iris. T h e regu larity of the coloboma after more than a year encourages the assumption of a com plete removal of the epithelial cyst.
295
SUMMARY
T h e clinical and histopathologic findings in one of the rare cases of congenital stromal epithelial cyst of the iris and its possible pathogenesis are discussed. 950 East 59th Street (37).
REFERENCES
1. Yudkin, A.: Intraepithelial cyst. In Reese, A. B.: Tumors of the Eye. New York, Hoeber, 1951, p. 295. 2. Streiff, J. J. : Kryptenblatt und Kryptengrundblatt der Regenbogenhaut und die Entstehung der serösen Cysten der vorderen Seite der Iris. Arch. f. Augenh., 50:56, 1904. 3. Rados, A.: Ueber spontane Iriscysten. Arch. f. Ophth., 99:152, 1919. 4. Schmidt-Rimpler, H.: Zur Entstehung der serösen Iriscysten. Arch. f. Ophth., 35:147, 1889. 5. Terrien, F.: Étude sur les cystes de l'iris. Arch, ophtal., 21:651, 1901. 6. Lagrange, F.: Contribution a l'étude des kystes de l'iris. Arch, ophtal., 20:272, 1900. 7. Thiel, R. : Secundärglaukom nach Epithel-oder Endothel- einwanderung in die Vorderkammer. Klin. Monatsbl. Augenh., 137:705, 1960. 8. Nadal, R.: Note sur la pathogénie des kystes séreux congénitaux de l'iris. Arch, ophtal., 31:363, 1911. 9. Juselius, E.: Die spontanen Iriscysten, ihre Pathogenese und Entwicklung. Klin Monatsbl. Augenh., 46:300, 1908. 10. Bergemann, H.: Beitrag zu den angeborenen Iriscysten. Arch. f. Augenh., 66:37, 1910. 11. Klien, B.: The ciliary margin of the dilator muscle of the iris. Arch. Ophth., 15:985, 1936. 12. Christensen, L., and Anderson, E. D.: Aberrant intraocular adenomata and epithelization of the anterior chamber. Arch. Ophth., 48:19, 1952. 13. Hunter, W. S.: Aberrant intraocular lacrimai gland tissue. Brit. J. Ophth., 44:619 (Oct.) 1960. 14. Bruce, G.: Aberrant glandular tissue in iris. Tr. Am. Acad. Ophth., 56:47 (Jan.-Feb.) 1952.
A S T U D Y O F F U N D U S C H A N G E S IN T H E E A R L Y STAGES O F TAKAYASU-OHNISHI (PULSELESS) DISEASE* KlNNOSUKE HlROSE, M . D . Nagasaki, Japan INTRODUCTION
In 1908 Takayasu reported a case which had a peculiar wreathlike anastomosis of the central artery and vein of the retina around the optic disc. At the same time, Ohnishi mentioned the association of this specific al teration in the ocular fundus with pulseless ness of the radial artery. These two abnor malities may be considered the essential cri teria for the diagnosis of this vascular dis order known as Takayasu-Ohnishi's or pulseless disease. Specific ocular changes occasionally are not, however, seen in the early stages of the * This study was aided by grant No. G-247 of the National Council to Combat Blindness, Inc., New York, New York.
disease. This apparently is true when the diagnosis has been made only on the basis of absence of the arterial pulse in the upper portion of the body. Relatively little atten tion has been given to the retinal lesions so that they have assumed a position of con siderably less importance. In this paper, the significance of the characteristic fundus changes, especially in the early stages of Takayasu-Ohnishi's dis ease, will be discussed. R E V I E W OF T H E LITERATURE
T h e fundus changes described in the re ported cases of this disease include: ( 1 ) wreathlike anastomosis of the central artery and vein of the retina around the disc, ( 2 )
It has no consistent effect on the serum or urine uric acid levels. An oral dose of 0.5 mg. may be given every hour until the pain is relieved or until nausea, vomiting or di arrhea appear. One to two mg. is then given daily. One hundred units of ACTH-gel given intramuscularly may help terminate an attack, especially when seen late. The response to steriods is less consistent in these cases than with other methods of ther apy, as again the case discussed shows. Uricosuric drugs are not indicated unless the patient has either tophi, serum uric acid levels consistently above 8.0 mg. percent, or frequent attacks of acute gout despite ade quate doses of colchicine. Although a lowpurine diet does not reduce the serum urate levels to any great extent, the small reduc tions obtained in this manner may be the deciding factor in preventing some attacks. It has been pointed out6 that, in spite of the severe insult to the eye, such as this case exemplifies, relatively complete resolu tion generally occurs in inflammation due
291
to gout, suggesting a chemical etiology. Finally, although this is not a common condition, it would seem that gout should be included in the vast number of etiologic possibilities in the evaluation of the uveal tract (at least to the extent of determining the serum uric acid in cases which seem resistant to the usual therapy), where it might account for some of the cases considered idiopathic. A family history of gout or rheumatism, renal calculi, monoarticular arthritis, an increased serum uric acid in the absence of renal disease, tophi, and/or a response to colchicine7 lead to a strongly presumptive diagnosis of a gouty etiology. SUMMARY
A case of severe, generalized, plastic uveitis, associated with an elevation in the serum uric acid and responsive to colchicine, is presented. The case is discussed as it relates to recent concepts of gout. 636 Church Street.
REFERENCES
1. Hogan, M. J., and Zimmerman, L. E.: Ophthalmic Pathology. Philadelphia, Saunders, 1962. 2. McWilliams, J. R.: Ocular findings in gout: Report of a case of conjunctival tophi. Am. J. Ophth., 35:1778-1783, 1952. 3. Falls, H. F.: Clinical detection of the genetic carrier state in ophthalmic pathology. Am. J. Ophth., 37:848, 1954. 4. Wyngaarden, J. B., and Jones, O. W.: The pathogenesis of gout. Med. Clin. N. Am., 45:1241-1256, 1961. 5. Seegmiller, J. E.: The present-day treatment of gout. Med. Clin. N. Am., 45:1259-1271, 1961. 6. Duke-Elder, S.: Textbook of Ophthalmology. St. Louis, Mosby, 1941, v. 3, pp. 2381-2383. 7. Hogan, M. J., Kimura, S. J., and Thygeson, P.: Uveitis in association with rheumatism. AMA Arch. Ophth., 57:407-409, 1957.
CONGENITAL E P I T H E L I A L CYST O F T H E IRIS STROMA* B E R T H A A. KLIEIST, M.D.,
AND GEORGE S. T A N N E R ,
M.D.
Chicago, Illinois
Spontaneous or idiopathic cysts of the iris are principally of two kinds, stromal and intraepithelial. The latter appears to be the more common variety and its pathogene* From the Division of Ophthalmic Surgery, The University of Chicago. This work was done under a grant from the Douglas Smith Foundation for Medical Research of the University of Chicago.
sis is understood. It arises from incomplete obliteration or re-establishment of the space between the two leaves of the secondary optic vesicle, usually with some additional proliferation of the neuro-ectodermal ele ments of these layers. It may occur at any place from the pupillary border of the iris, where it may represent an open and dis-
292
BERTHA A. KLIEN AND GEORGE S. TANNER
a less striking layer of flat cells with the appearance of endothelium. While the former type of lining is undeniably of ectodermal origin, the character of the latter has been debated. Some consider the possi bility of an entirely mesoblastic origin by proliferation of iris endothelium into and along a cavity which might have arisen from closure of an iris crypt (Streiff,2 SchmidtRimpler,4 Terrien, 5 Lagrange 6 ), others be lieve it to arise from surface epithelium (Thiel 7 ) thus considering both types of linings to be derived from similar sources.
Fig. 1 (Klien and Tanner). Right eye of a girl, aged seven years. Congenital intraepithelial cyst of the ciliary body with circumscribed lenticular opacity, which extended from the embryonic nu cleus to the posterior surface of the lens. At this place the convex curvature of the posterior lenticu lar surface became slightly concave, indicating a partial coloboma of the lens at this site. No other anomalies were present.
tended v. Scily's sinus, to the pars plana of the ciliary body. It may be acquired in the course of many different intraocular patho logic processes and thus be found in adult eyes, or it may be congenital. A congenital origin can be established with certainty in the presence of associated defects, such as a lenticular opacity or a lenticular coloboma. A definitely congenital intraepithelial cyst associated with a partial coloboma of the lens was observed by Yudkin 1 in a 16-yearold girl, another is illustrated in Figure 1. Less well understood is the pathogenesis of the congenital stromal cysts, of which a small number was reported and discussed around the turn of the century (11 cases, studied also histopathologically, collected by Streiff,2 Rados 3 ). They lie, as the name im plies, embedded in the stroma of the iris and may be lined with a stratified epithelium sometimes containing goblet cells, or with
Concerning these sources two theories exist. One considers the possibility of cysts derived from misplaced islets of surface ec toderm, which could reach the intraocular location at or near the time the lens vesicle separates from the surface ectoderm at the nine mm. stage (Streiff,2 Nadal 8 ). The other considers the possibility of a neuro-ectodermal origin from cells of the anterior leaf of the secondary optic vesicle, left over from differentiation of the iris muscles (Juselius, 9 Bergemann 10 ). The proponents of the latter concept interpret the cells, containing a mucoid substance, which are found in the epithelial lining of such cysts not as goblet cells but as epithelial cells undergoing mucoid degeneration. Regarding the second theory it should be stated that unused neuro-ectodermal cell ele ments are common in the iris and may give rise to a variety of morphologic pictures (Klien 11 ), but none! of these is characterized by even a rudimentary tendency for cyst formation or anomalous differentiation into secreting goblet cells despite the pluripotential quality of the mother cells. An origin of the intrastromal cysts from misplaced islets of surface ectoderm seems more prob able and would have a counterpart in the cases of aberrant intraocular lacrimai gland (Christensen,12 Hunter 13 ), particularly those in which no communication with extrabulbar regions could be found (Bruce 14 ). R E P O R T OF A CASE
History. One month prior to the first ocular ex amination of a girl, aged four and one-half months,
EPITHELIAL CYST OF IRIS STROMA a "growth" in her left eye was noted by the parents. This lesion increased in size, accompanied by marked lacrimation, during the past few weeks. The child was the first born of healthy parents. A full-term pregnancy had been followed by spon taneous uncomplicated delivery. There was no birth trauma or subsequent ocular injury, and no mater nal illness during gestation. Findings. Examination of the left eye under general anesthesia revealed a slight net-shaped bulbar conjunctival injection, circumscribed stippling of the corneal surface in the superior nasal quad rant, corresponding to an oval lesion in the anterior chamber which touched the posterior corneal sur face in this area. The lesion appeared to be a trans parent stromal cyst of the iris (fig. 2), which pro jected forward and to an equal extent backward into the posterior chamber, touching the lens. Its temporal border came within one mm. of the pupil lary margin and limited pupillary dilation in this quadrant. The lens was clear, the fundus normal. A short tag of a persistent hyaloid vessel was attached to the optic disc. There was no defect, irregularity or scarring of the bulbar walls which might have raised suspicion of a communication between the intraocular lesion and extrabulbar regions. The right eye was normal, the iris was grayish blue in each eye, the tactile tension was soft and equal in the two eyes. The remaining physical examination was normal. Operative procedure. After a conjunctival in cision between the 10- and l-o'clock positions fourmm. from the limbus and dissection of the con junctiva, a limbal incision was made at the 12o'clock position. Attempts to grasp the smooth cyst walls proved futile, consequently the clear con tent of the cyst was partially emptied with a 27gauge needle. An iridectomy was then performed at the 12-o'clock position and much of the now easily grasped wall of the cyst was removed through the nasal part of the wound. Postoperative recovery was uneventful. The last examination of this eye, 14 months after the operation, revealed no lacrimation or signs of discomfort. The left globe was pale, the cornea transparent, the anterior chamber normally deep, the lens clear. The narrow total coloboma of the iris at the 12-o'clock position had straight, parallel out lines. An intermittent manifest left convergence was noted on near fixation and a test of refraction is planned for the near future. HlSTOLOGlC FINDINGS
The postoperative specimen was received in two portions, one consisting mainly of the walls of the cyst and adjacent iris, the other representing a piece of iris containing a small portion of sphincter muscle. Both were fixed in formalin and embedded in paraffin. The posterior wall of the cyst was intact
293
Fig. 2 (Klien and Tanner). Left eye of a girl, aged four months, showing congenital stromal epi thelial cyst of the iris. and consisted of moderately thinned iris, bulging backward and lined anteriorly with stratified epithelium whose thickness varied from two to five layers. There was no base ment membrane. Some of the epithelial cells were flat, others cubical or cylindrical, in terspersed with large cells of the appear ance of goblet cells. The latter stained deeply with the periodic acid-Schiff method, showing a finely granular cytoplasm and a marginal and, in most cells, well-preserved nucleus. They also gave a marked argentaffin reaction with Gomori's silver stain. Rem nants of the content of the cyst, of amor phous or stringy appearance, found in the neighborhood of or attached to these cells, also stained deeply with PAS, suggesting the secretory activity of true goblet cells ra ther than a mucoid degeneration (fig. 3 ) . No connection between the epithelial lining of the posterior wall and the well-visible dilator muscle could be found. The anterior wall of the cyst was present in collapsed remnants on each side. The epithelial lining was similar to that of the posterior wall. The outer half of the wall consisted mainly of extremely thinned iris stroma which could be definitely discerned as such by means of Gomori's trichrome stain (fig. 4). Small and large extensions öf
294
BERTHA A. KLIEN AND GEORGE S. TANNER JSMÎÊÈÎL
Fig. 3 (Klien and Tanner). Excised portion of cyst and adjacent iris, general view, same eye as in Figure 2. (P) Posterior wall of cyst, (G) Goblet cells. (R) Ramifications of cyst in iris stroma. (Periodic acid-Schiff, χ65.)
the cyst's epithelial lining could be followed into the adjacent iris stroma, producing outpouchings of the main lumen. There were abundant vessels, mainly capillaries, in the pupillary as well as in the ciliary stroma of the iris surrounding the cyst. Although the stroma was rich in nuclei, as characteristic for the infantile eye, no inflammatory cells were noted. COMMENT
Despite the surgeon's impression of hav
ing removed only part of the cyst, the pres ent status of the eye 14 months after the operation suggests its total removal. The posterior wall was certainly present in toto in the excised piece; the anterior wall was extremely thin and torn and the question, whether stromal remnants separated it everywhere from the cornea which it touched, could not be answered from study of the histologie specimen. The fact that the cornea remained normal after removal of the cyst makes a purely mechanical contact
Fig. 4 (Klien and Tanner). Excised portions of cyst, same eye as in Figures 2 and 3. (A) Collapsed anterior wall of cyst. (E) Inner epithelial portion of cyst wall. (S) Outer portion of cyst wall, consisting of extremely thinned iris stroma. (Gomori trichrome, X275.)
EPITHELIAL CYST OF IRIS STROMA probable. Only the future will tell if slowgrowing epithelial remnants remained em bedded in the stroma of the iris. T h e regu larity of the coloboma after more than a year encourages the assumption of a com plete removal of the epithelial cyst.
295
SUMMARY
T h e clinical and histopathologic findings in one of the rare cases of congenital stromal epithelial cyst of the iris and its possible pathogenesis are discussed. 950 East 59th Street (37).
REFERENCES
1. Yudkin, A.: Intraepithelial cyst. In Reese, A. B.: Tumors of the Eye. New York, Hoeber, 1951, p. 295. 2. Streiff, J. J. : Kryptenblatt und Kryptengrundblatt der Regenbogenhaut und die Entstehung der serösen Cysten der vorderen Seite der Iris. Arch. f. Augenh., 50:56, 1904. 3. Rados, A.: Ueber spontane Iriscysten. Arch. f. Ophth., 99:152, 1919. 4. Schmidt-Rimpler, H.: Zur Entstehung der serösen Iriscysten. Arch. f. Ophth., 35:147, 1889. 5. Terrien, F.: Étude sur les cystes de l'iris. Arch, ophtal., 21:651, 1901. 6. Lagrange, F.: Contribution a l'étude des kystes de l'iris. Arch, ophtal., 20:272, 1900. 7. Thiel, R. : Secundärglaukom nach Epithel-oder Endothel- einwanderung in die Vorderkammer. Klin. Monatsbl. Augenh., 137:705, 1960. 8. Nadal, R.: Note sur la pathogénie des kystes séreux congénitaux de l'iris. Arch, ophtal., 31:363, 1911. 9. Juselius, E.: Die spontanen Iriscysten, ihre Pathogenese und Entwicklung. Klin Monatsbl. Augenh., 46:300, 1908. 10. Bergemann, H.: Beitrag zu den angeborenen Iriscysten. Arch. f. Augenh., 66:37, 1910. 11. Klien, B.: The ciliary margin of the dilator muscle of the iris. Arch. Ophth., 15:985, 1936. 12. Christensen, L., and Anderson, E. D.: Aberrant intraocular adenomata and epithelization of the anterior chamber. Arch. Ophth., 48:19, 1952. 13. Hunter, W. S.: Aberrant intraocular lacrimai gland tissue. Brit. J. Ophth., 44:619 (Oct.) 1960. 14. Bruce, G.: Aberrant glandular tissue in iris. Tr. Am. Acad. Ophth., 56:47 (Jan.-Feb.) 1952.
A S T U D Y O F F U N D U S C H A N G E S IN T H E E A R L Y STAGES O F TAKAYASU-OHNISHI (PULSELESS) DISEASE* KlNNOSUKE HlROSE, M . D . Nagasaki, Japan INTRODUCTION
In 1908 Takayasu reported a case which had a peculiar wreathlike anastomosis of the central artery and vein of the retina around the optic disc. At the same time, Ohnishi mentioned the association of this specific al teration in the ocular fundus with pulseless ness of the radial artery. These two abnor malities may be considered the essential cri teria for the diagnosis of this vascular dis order known as Takayasu-Ohnishi's or pulseless disease. Specific ocular changes occasionally are not, however, seen in the early stages of the * This study was aided by grant No. G-247 of the National Council to Combat Blindness, Inc., New York, New York.
disease. This apparently is true when the diagnosis has been made only on the basis of absence of the arterial pulse in the upper portion of the body. Relatively little atten tion has been given to the retinal lesions so that they have assumed a position of con siderably less importance. In this paper, the significance of the characteristic fundus changes, especially in the early stages of Takayasu-Ohnishi's dis ease, will be discussed. R E V I E W OF T H E LITERATURE
T h e fundus changes described in the re ported cases of this disease include: ( 1 ) wreathlike anastomosis of the central artery and vein of the retina around the disc, ( 2 )