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Glaucoma Associated with Polycystic Kidney Disease
GLAUCOMA ASSOCIATED W I T H POLYCYSTIC KIDNEY DISEASE WILLIAM L.
BERKLEY,, CAPT. (MC)
New York
Glaucoma associated with polycystic kid ney disease is not prominently featured in the literature. Since the case report is the foundation of clinical medical literature, the report of a case of this nature with a brief discussion of some of its etiologic possibilities would appear to be in order. REPORT OF CASE
A. E. L., a white man, a bookkeeper, aged 52 years, was admitted to the U. S. Naval Hospital, St. Albans, New York, with a diagnosis of diffuse glomerulo-nephritis. His chief complaint was rapid loss of vision. History. A complete history could not be obtained from the patient due to his drowsy and partially disoriented condition. How ever, the following significant and reliable information regarding his case was obtained from his wife and from his family physician. For the past year the patient had experi enced intermittent painless hematuria and low back pains. Fourteen days prior to ad mission he experienced rather sudden loss of vision in the left eye. There was no as sociated ocular pain. Over a period of days the patient grew increasingly drowsy. Hema turia developed during this time and was pronounced upon admission to the hospital. The patient was treated for arterial hyper tension, degree unknown, six years prior to hospitalization. For several years his wife thought that he had some visual difficulty. This could well have been of a refractive nature or due to presbyopia. There was no history of syphilis, edema, or coma. The patient had no children. Both a brother and a sister had died in middle age of polycystic kidney disease. The affected brother had also experienced severe impair ment of vision during the terminal stages of his illness.
U.S.N.
Physical examination. The patient was ap parently well developed and attempted to co operate but was drowsy, disoriented, and at times incoherent. His skin was dry, sallow, and pasty in appearance but not jaundiced. The pulse rate was 80 beats per minute, temperature 100°F., and respiration was 20 per minute. Rye examination could not be thoroughly accomplished due to the patient's inability to cooperate. These findings were noted: Vision was: O.D., counting fingers at one foot; O.S., no light perception. There was partial ptosis of the left upper lid, otherwise the lids were normal. The extraocular muscles were intact. The lacrimal apparatus and the conjunctivas were normal. The corneas were transparent and showed normal luster. Both pupils were round, equal, and par tially dilated. The right pupil reacted to light but poorly to accommodation. The left did not react to light or accommodation. Ophthalmoscopic examination revealed the aqueous and vitreous to be clear. The lenses were normal in appearance. The fundi were clearly visualized. The right optic disc revealed sharply outlined margins, deep gen eralized cupping, and an abnormal lemonyellow color. The retinal arteries contained areas of mild spasticity and slight arteriovenous indentations and some generalized attenuation. The retina was otherwise clear and nor mal in appearance. There were no hemor rhages or exudates. The left fundus pre sented an appearance similar to the right except that the optic-disc changes were more pronounced. Tension measured by Schip'lz's tonometer was 18 mm. Hg in each eye. The visual field of the right eye was limited to a small area in the upper outer quadrant.
1540
WILLIAM L. BERKLEY
ward from the level of the 12th rib on the left side. Skull plates showed areas of increased as well as decreased densities. The areas of de creased densities were numerous and pre sented small, O-shaped, punched-out appear ances. Studies at this point suggested as a most likely diagnosis either polycystic kidney dis ease or hypernephroma with metastases. Cystoscopic studies revealed no urethral obstruction. The bladder was filled with old Fig. 1 (Berkley). Photomicrograph of cross blood clots and bloody urine. The left uresection of globe, with globular posterior section, teral orifice contained a plug of clotted relatively small cornea, and pronounced generalized blood. The right orifice was normal in ap thinning of the sclera. pearance. Physical examination. The teeth were in Retrograde visualization of the kidneys extremely poor condition, containing many showed both to be abnormally large, with caries and much gingival infection. The ears, the left about twice the size of the right. nose and throat were normal. There was no The urologist stated that the left kidney, generalized glandular enlargement. The lungs seen by itself, would appear to contain a were clear. The heart was normal and the malignancy but, when compared with the pulse was full and regular. The blood pres right kidney, polycystic kidney disease would sure was 122/78 mm. Hg. seem to be the most likely diagnosis. The abdomen was soft but contained a During the remainder of his hospital stay, readily palpable, tender, large, irregular, but the eye findings did not change from those poorly defined mass in the left lower quad present on admission. The intraocular rant. The liver was enlarged, smooth and pressure remained at 18 to 20 mm. Hg slightly tender. (Schijzitz), and no retinal hemorrhages or The remainder of the physical examina exudate developed. tion, including genital, rectal, and neurologic The patient's general condition deterio studies, revealed no abnormal findings. rated rapidly with N.P.N. continuing to rise Laboratory examination showed the fol rapidly, reaching at one time the phenomenal lowing positive findings: figure of 600 mg. per 100 cc. He died on the Red blood count, 2,500,000—white blood 14th day of hospitalization. Clinical impres count, 17,000, with a differential count of sions at time of death were: (1) Uremia and 94-percent segmented neutrophils, four-per renal failure due to polycystic kidney dis cent lymphocytes, and three-percent mono- ease, and (2) blindness, possibly of central cytes. The Kahn test was negative and the origin due to uremia or optic atrophy. sedimentation rate was 35 mm. in one hour. Postmortem examination. The points es The N.P.N. was 151 mg. per 100 cc. of sential to an ophthalmic study of this case blood and the C 0 2 combining power was 15 are briefly enumerated. vol. percent. A urine specimen was grossly The kidneys were extremely enlarged, the bloody, heavily loaded with albumin, and had right weighing 2,370 gm. and the left 1,250 a specific gravity of 1,009. gm. They were grossly similar, both being A flat roentgen plate of the abdomen re nodular, multicystic, and blue-gray to dark vealed a kidney-shaped mass of approxi red in color. They presented the typical pic mately 4 by 6 by 7 inches extending down ture of polycystic kidneys.
GLAUCOMA AND POLYCYSTIC KIDNEY DISEASE
The left kidney yielded a positive culture of alpha streptococci and proteus vulgaris. The liver also contained many cysts. The eyes were removed and placed in Bouin's solution in preparation for special pathologic study by Dr. J. Arnold deVeer of Brooklyn. Upon completion of the study, Dr. deVeer returned the following compre hensive report: GROSS. The material consists of two rather large but otherwise grossly normal eyes and an entire optic chiasm. MICROSCOPIC. Both eyes show the same findings. Both are of the same size and con tours and are almost identical in all histologic details. There is generalized ectasia of the scleras which gives rather broad, globular posterior segments. In both there is flat recession of the optic discs which is accompanied by ectasia of the posterior scleral foramina and some distortion of the nerve at the level of the stretched lamina cribrosas. The retinas are very little detached by artefaction of fixation and subsequent han dling. This suggests a firmer attachment than usual, as is usually the case in glau coma. No definite adhesions of the retinas
Fig. 2 (Berkley). Photomicrograph, showing free and open chamber angle and the large open Schlemm's canal.
1541
are detected. There is no active inflammation in any of the tissues. The only other abnormalities are the fol lowing: Slight encroachment of conjunctival vasculature and connective tissue on the
Fig. 3 (Berkley). Photomicrograph showing typi cal glaucomatous cupping of the nervehead. Ectasia of the sclera, and especially of the lamina cribrosa, has resulted in marked widening of the intervaginal space.
corneas (slight peripheral degenerative pann u s ) ; an unusually compact structure of the angle tissue; absence of anterior synechias and, instead, recession of the roots of the irises behind the level of Schlemm's canals; unusually long, slitlike canals of Schlemm; relatively small corneas for eyes of this size. Both lenses appear normal. The optic chiasm was mordanted in chrome salts and stained by the Weigert stain for myelin. Sec tions in the horizontal midline show no demyelinization. The interpretation of these changes is not easy. It seems obvious that there have been glaucomatous changes in both eyes, possibly on the basis of structural abnormalities in the angle tissue. The cupping of the disc seems definitely attributable to glaucoma with stretching of the lamina cribrosa and widening of the base of the intervaginal space in each eye.
1542
WILLIAM L. BERKLEY COMMENTS
It is submitted that this represents a case of glaucoma resulting from congenital struc tural defect of the sclera in which the globe could not tolerate a normal tension without pathologic damage of the type resulting from hypertension in normal eyes. The case is presented for its interest as a case of glau coma resulting from congenital structural defects with the same underlying etiology as those causing the polycystic kidneys. Although studies and pathologic findings suggest that the structural defects involving the eyes, kidneys, liver, and skull probably had a common congenital origin, an exten sive but incomplete search of the literature does not reveal other similar cases suggesting such a connection. The nearest reference to a possible path ologic connection between glaucoma and polycystic kidney disease is suggested by Fuchs* when he speaks of cases of uremic amaurosis associated with ■albuminuric retinitis. Perhaps some cases placed in that cate gory could have represented the type of con dition presently under discussion. It seems probable, however, that this type of struc tural defect should be listed as another one * Fuchs, E.: Textbook of Ophthalmology (Duane). Philadelphia, Lippincott, 1917, ed. 5, p. 579.
of the many causes of the glaucoma syn drome. In this presentation we apparently see a case of glaucoma due not to increased in traocular pressure above that found in nor mal eyes, but rather due to generalized ectasia of the sclera which has resulted in an inability of the affected globe to withstand a normal intraocular pressure. The clinical syndrome such as the one herewith presented, although showing struc tural defects involving a number of organs including the eyes, naturally passes under the designation of polycystic kidney disease because of the overwhelming importance of the renal aspect of the condition. However, the question may well be asked, might it not be possible and indeed probable that in some cases the structural defects of the eyes are the prominent features with the kidneys only slightly affected? And if such should be the case, then could not some cases of this type, which in the past have passed as so-called low-tension glaucoma, in reality represented congenital structural defects of the sclera associated with the syndrome un der discussion ? The possibility that a previous abnormally high tension may have accounted for the findings presented in this case report cannot be wholly discounted. A.P.O. 676, c/o Postmaster, New York.
OPHTHALMIC MINIATURE
In all ophthalmias in which the discharge is greenish or livid and in which there is sleeplessness as well as severe pain in the temples, there is likely to occur ulceration of the eyeball. \n all such cases, perforation may follow. Hippocrates, circa 400 B.C. From the translation bv Francis Adams.
BERKLEY,, CAPT. (MC)
New York
Glaucoma associated with polycystic kid ney disease is not prominently featured in the literature. Since the case report is the foundation of clinical medical literature, the report of a case of this nature with a brief discussion of some of its etiologic possibilities would appear to be in order. REPORT OF CASE
A. E. L., a white man, a bookkeeper, aged 52 years, was admitted to the U. S. Naval Hospital, St. Albans, New York, with a diagnosis of diffuse glomerulo-nephritis. His chief complaint was rapid loss of vision. History. A complete history could not be obtained from the patient due to his drowsy and partially disoriented condition. How ever, the following significant and reliable information regarding his case was obtained from his wife and from his family physician. For the past year the patient had experi enced intermittent painless hematuria and low back pains. Fourteen days prior to ad mission he experienced rather sudden loss of vision in the left eye. There was no as sociated ocular pain. Over a period of days the patient grew increasingly drowsy. Hema turia developed during this time and was pronounced upon admission to the hospital. The patient was treated for arterial hyper tension, degree unknown, six years prior to hospitalization. For several years his wife thought that he had some visual difficulty. This could well have been of a refractive nature or due to presbyopia. There was no history of syphilis, edema, or coma. The patient had no children. Both a brother and a sister had died in middle age of polycystic kidney disease. The affected brother had also experienced severe impair ment of vision during the terminal stages of his illness.
U.S.N.
Physical examination. The patient was ap parently well developed and attempted to co operate but was drowsy, disoriented, and at times incoherent. His skin was dry, sallow, and pasty in appearance but not jaundiced. The pulse rate was 80 beats per minute, temperature 100°F., and respiration was 20 per minute. Rye examination could not be thoroughly accomplished due to the patient's inability to cooperate. These findings were noted: Vision was: O.D., counting fingers at one foot; O.S., no light perception. There was partial ptosis of the left upper lid, otherwise the lids were normal. The extraocular muscles were intact. The lacrimal apparatus and the conjunctivas were normal. The corneas were transparent and showed normal luster. Both pupils were round, equal, and par tially dilated. The right pupil reacted to light but poorly to accommodation. The left did not react to light or accommodation. Ophthalmoscopic examination revealed the aqueous and vitreous to be clear. The lenses were normal in appearance. The fundi were clearly visualized. The right optic disc revealed sharply outlined margins, deep gen eralized cupping, and an abnormal lemonyellow color. The retinal arteries contained areas of mild spasticity and slight arteriovenous indentations and some generalized attenuation. The retina was otherwise clear and nor mal in appearance. There were no hemor rhages or exudates. The left fundus pre sented an appearance similar to the right except that the optic-disc changes were more pronounced. Tension measured by Schip'lz's tonometer was 18 mm. Hg in each eye. The visual field of the right eye was limited to a small area in the upper outer quadrant.
1540
WILLIAM L. BERKLEY
ward from the level of the 12th rib on the left side. Skull plates showed areas of increased as well as decreased densities. The areas of de creased densities were numerous and pre sented small, O-shaped, punched-out appear ances. Studies at this point suggested as a most likely diagnosis either polycystic kidney dis ease or hypernephroma with metastases. Cystoscopic studies revealed no urethral obstruction. The bladder was filled with old Fig. 1 (Berkley). Photomicrograph of cross blood clots and bloody urine. The left uresection of globe, with globular posterior section, teral orifice contained a plug of clotted relatively small cornea, and pronounced generalized blood. The right orifice was normal in ap thinning of the sclera. pearance. Physical examination. The teeth were in Retrograde visualization of the kidneys extremely poor condition, containing many showed both to be abnormally large, with caries and much gingival infection. The ears, the left about twice the size of the right. nose and throat were normal. There was no The urologist stated that the left kidney, generalized glandular enlargement. The lungs seen by itself, would appear to contain a were clear. The heart was normal and the malignancy but, when compared with the pulse was full and regular. The blood pres right kidney, polycystic kidney disease would sure was 122/78 mm. Hg. seem to be the most likely diagnosis. The abdomen was soft but contained a During the remainder of his hospital stay, readily palpable, tender, large, irregular, but the eye findings did not change from those poorly defined mass in the left lower quad present on admission. The intraocular rant. The liver was enlarged, smooth and pressure remained at 18 to 20 mm. Hg slightly tender. (Schijzitz), and no retinal hemorrhages or The remainder of the physical examina exudate developed. tion, including genital, rectal, and neurologic The patient's general condition deterio studies, revealed no abnormal findings. rated rapidly with N.P.N. continuing to rise Laboratory examination showed the fol rapidly, reaching at one time the phenomenal lowing positive findings: figure of 600 mg. per 100 cc. He died on the Red blood count, 2,500,000—white blood 14th day of hospitalization. Clinical impres count, 17,000, with a differential count of sions at time of death were: (1) Uremia and 94-percent segmented neutrophils, four-per renal failure due to polycystic kidney dis cent lymphocytes, and three-percent mono- ease, and (2) blindness, possibly of central cytes. The Kahn test was negative and the origin due to uremia or optic atrophy. sedimentation rate was 35 mm. in one hour. Postmortem examination. The points es The N.P.N. was 151 mg. per 100 cc. of sential to an ophthalmic study of this case blood and the C 0 2 combining power was 15 are briefly enumerated. vol. percent. A urine specimen was grossly The kidneys were extremely enlarged, the bloody, heavily loaded with albumin, and had right weighing 2,370 gm. and the left 1,250 a specific gravity of 1,009. gm. They were grossly similar, both being A flat roentgen plate of the abdomen re nodular, multicystic, and blue-gray to dark vealed a kidney-shaped mass of approxi red in color. They presented the typical pic mately 4 by 6 by 7 inches extending down ture of polycystic kidneys.
GLAUCOMA AND POLYCYSTIC KIDNEY DISEASE
The left kidney yielded a positive culture of alpha streptococci and proteus vulgaris. The liver also contained many cysts. The eyes were removed and placed in Bouin's solution in preparation for special pathologic study by Dr. J. Arnold deVeer of Brooklyn. Upon completion of the study, Dr. deVeer returned the following compre hensive report: GROSS. The material consists of two rather large but otherwise grossly normal eyes and an entire optic chiasm. MICROSCOPIC. Both eyes show the same findings. Both are of the same size and con tours and are almost identical in all histologic details. There is generalized ectasia of the scleras which gives rather broad, globular posterior segments. In both there is flat recession of the optic discs which is accompanied by ectasia of the posterior scleral foramina and some distortion of the nerve at the level of the stretched lamina cribrosas. The retinas are very little detached by artefaction of fixation and subsequent han dling. This suggests a firmer attachment than usual, as is usually the case in glau coma. No definite adhesions of the retinas
Fig. 2 (Berkley). Photomicrograph, showing free and open chamber angle and the large open Schlemm's canal.
1541
are detected. There is no active inflammation in any of the tissues. The only other abnormalities are the fol lowing: Slight encroachment of conjunctival vasculature and connective tissue on the
Fig. 3 (Berkley). Photomicrograph showing typi cal glaucomatous cupping of the nervehead. Ectasia of the sclera, and especially of the lamina cribrosa, has resulted in marked widening of the intervaginal space.
corneas (slight peripheral degenerative pann u s ) ; an unusually compact structure of the angle tissue; absence of anterior synechias and, instead, recession of the roots of the irises behind the level of Schlemm's canals; unusually long, slitlike canals of Schlemm; relatively small corneas for eyes of this size. Both lenses appear normal. The optic chiasm was mordanted in chrome salts and stained by the Weigert stain for myelin. Sec tions in the horizontal midline show no demyelinization. The interpretation of these changes is not easy. It seems obvious that there have been glaucomatous changes in both eyes, possibly on the basis of structural abnormalities in the angle tissue. The cupping of the disc seems definitely attributable to glaucoma with stretching of the lamina cribrosa and widening of the base of the intervaginal space in each eye.
1542
WILLIAM L. BERKLEY COMMENTS
It is submitted that this represents a case of glaucoma resulting from congenital struc tural defect of the sclera in which the globe could not tolerate a normal tension without pathologic damage of the type resulting from hypertension in normal eyes. The case is presented for its interest as a case of glau coma resulting from congenital structural defects with the same underlying etiology as those causing the polycystic kidneys. Although studies and pathologic findings suggest that the structural defects involving the eyes, kidneys, liver, and skull probably had a common congenital origin, an exten sive but incomplete search of the literature does not reveal other similar cases suggesting such a connection. The nearest reference to a possible path ologic connection between glaucoma and polycystic kidney disease is suggested by Fuchs* when he speaks of cases of uremic amaurosis associated with ■albuminuric retinitis. Perhaps some cases placed in that cate gory could have represented the type of con dition presently under discussion. It seems probable, however, that this type of struc tural defect should be listed as another one * Fuchs, E.: Textbook of Ophthalmology (Duane). Philadelphia, Lippincott, 1917, ed. 5, p. 579.
of the many causes of the glaucoma syn drome. In this presentation we apparently see a case of glaucoma due not to increased in traocular pressure above that found in nor mal eyes, but rather due to generalized ectasia of the sclera which has resulted in an inability of the affected globe to withstand a normal intraocular pressure. The clinical syndrome such as the one herewith presented, although showing struc tural defects involving a number of organs including the eyes, naturally passes under the designation of polycystic kidney disease because of the overwhelming importance of the renal aspect of the condition. However, the question may well be asked, might it not be possible and indeed probable that in some cases the structural defects of the eyes are the prominent features with the kidneys only slightly affected? And if such should be the case, then could not some cases of this type, which in the past have passed as so-called low-tension glaucoma, in reality represented congenital structural defects of the sclera associated with the syndrome un der discussion ? The possibility that a previous abnormally high tension may have accounted for the findings presented in this case report cannot be wholly discounted. A.P.O. 676, c/o Postmaster, New York.
OPHTHALMIC MINIATURE
In all ophthalmias in which the discharge is greenish or livid and in which there is sleeplessness as well as severe pain in the temples, there is likely to occur ulceration of the eyeball. \n all such cases, perforation may follow. Hippocrates, circa 400 B.C. From the translation bv Francis Adams.