Open-Angle Glaucoma Following Surgery for Congenital Cataracts Charles D. Phelps, MD, Nour I. Arafat, MD \s=b\ Eighteen patients who, years earlier, had undergone operations for congenital
cataracts
discovered to have
were
high
Intraocular pressures. Several had profound glaucomatous loss of vision. All had deep anterior chambers with flat iris
planes and wide open angles. The uveal meshwork
was
unusually
coarse
and
Nystagmus, aftercataracts, and small pupils made visual field testing and observation of pigmented
in
some cases.
optic discs difficult. Most
cases were
bilateral, which suggested a hereditary origin; some had a family history of glaucoma. In none did we observe signs of congenital glaucoma or rubella syn-
drome. The incidence of this disease is not yet
known,
but it is common enough in our to indicate lifetime surveillance for glaucoma in all patients who undergo congenital cataract surgery.
clinic
(Arch Ophthalmol 95:1985-1987, 1977)
period if flow of aqueous through the pupil is blocked by vitreous or lens remnants.' Another variety that has attracted little attention in the past is a
chronic
open-angle
form of
glau-
Unlike the first type, it develops slowly and may not become apparent until years or decades after the operation for congenital cataract. In this report we will describe 18 patients with the chronic open-angle type of glaucoma. Our purpose is to warn ophthalmologists of this poten-
coma.
tial, late complication of congenital
point out the importance of long-term follow-up, including periodic measurements of cataract surgery and to
intraocular pressure and examination of the optic discs, of all patients who undergo an operation for congenital cataracts.
DESCRIPTION OF PATIENTS is a well-known complication of surgery for congenital cataracts. The most dramatic variety is closed-angle glaucoma. It develops acutely during the early postoperative
Glaucoma Accepted
publication May 14, 1977. Ophthalmology, Iowa, Iowa City.
for
From the Department of
University of
Presented at the 126th annual convention of the American Medical Association, San Francisco, June 19, 1977. Reprint requests to Department of Ophthal-
mology, University Hospitals, 52242 (Dr Phelps).
Iowa
City,
IA
We have examined and treated 18 who developed open-angle glaucoma after an operation for congenital cataract. Ten patients were male, and eight were female. A family history of glaucoma was present in four patients: two sibling pairs. The father of one pair also had glaucoma following operations for congenital cataracts; he is not included in the present series because he was not examined by the authors. In none of the remaining 14 patients was there a family history of glaucoma; however, five had a family history of congenital cataracts. Cataracts were noted in all 18 patients
patients
after birth. The cataracts were described as nuclear in three patients, zonular in one, and complete in one; no description is recorded for the remainder. One patient had facial features of Hallermann-Streiff's syndrome, two had epilepsy, and one had mild congenital hearing loss with no other signs of rubella syndrome; the remaining 14 patients had no systemic abnormalities. Cataract operations were performed in both eyes of 17 patients and in one eye of one patient. The operations on eight patients were performed elsewhere, and for six of these, the type of operation, other than being extracapsular, is unknown. The initial cataract operation was a simple needling in 16 eyes of nine patients, linear extraction in five eyes of three patients, and intracapsular extraction in both eyes of one patient. One operation was performed in each eye of four patients; multiple operations were performed in each eye of ten patients; and the number of operations is unknown for four patients. Discission of a pupillary membrane was the most frequently performed secondary operation. In no patient was one of the soon
currently popular operative techniques (needling and aspiration, phacoemulsification, or roto-extraction) performed as a primary operation. The ages of patients at the time of their first operation for cataract ranged from 7 months to 34 years (Table). No ocular
abnormalities other than cataracts and poor visual acuity were noted in any patient before the first operation. Specifically, signs of congenital glaucoma, including corneal enlargement, corneal edema,
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
by the cataract operation. depended on the amount of aftercataract, nystagmus, and amblyopia, as well as the presence or absence of glaucomatous damage. It was 20/40 or better in and not caused Visual acuity
of Patients at Time of Cataract Operations, First Detection of IOP* Elevation, and Time Interval Between Last Cataract Operation and First Detection of IOP Elevation
Ages
at Time of Cataract Operations, yr '/s 1
Age When IOP
2, 19t 2, 3t 2, 3f 3, 4t
33 19 31 38 11 27 25
Ages
Patient 1
Elevation
Detected, yr
Interval, yr
8
glaucomatous damage. Nystagmus prevented steady fixation in 14 patients and made visual field testing unreliable. Fundus examination was often hampered by
14
16
28 34
aftercataracts, nystagmus, and small pupils. However, the Goldmann contact lens,
23 21
10 12 13 14 15 16 17 18
5, 7t 8,1 li9 10 10
14
14, 21t
48 24
34
36
15
45
se 16 17 32
22 33
•IOP indicates intraocular pressure. fFirst number indicates age at which the earliest operation for cataracts was who had more than one operation; second number, age at latest operation.
and Haab's striae, were absent during infancy and later in life. However, the intraocular pressure was measured in only two patients before the cataract operation. Elevated intraocular pressures (greater than 20 mm Hg) were discovered during a routine examination in 16 patients and during evaluation for sudden loss of visual acuity in two patients. The pressure was high in both eyes of 14 patients and in one eye of four patients. Three of the patients with unilateral pressure elevation had earlier lost their other eye from laceration, intraocular foreign body, or retinal detachment. The fourth was the patient who had had surgery for cataract in only one eye; her unoperated eye had a normal pressure.
The ages at which intraocular pressure were first detected ranged from 6 to 56 years (Table). The time interval between the last operation for congenital cataract and the first detection of an elevated intraocular pressure varied considerably; it was less than five years in three patients, five to ten years in six patients, 11 to 20 years in two patients, 21 to 30 years in four patients, and more than 30 years in three patients (Table). In many patients the pressure elevation may have been present for some time before its detection; earlier measurements of intraocular pressure were recorded in only six
elevations
patients.
performed
in
patients
The level of intraocular pressure when the elevation was first detected ranged from 22 to 42 mm Hg. It was initially less than 30 mm Hg in all but three patients. The eyes had deep anterior chambers and flat iris contours. With the occasional exception of a small anterior synechia at the site of an old surgical incision, all anterior chamber angles were wide open. In seven patients the angles appeared entirely normal. In nine patients the uveal
meshwork was unusually prominent: a thick layer of lacy interwoven, pigmented strands nearly obscured the scierai spur and extended to Schwalbe's line. In six four with prominent uveal meshwork, the angle recess was poorly developed, and the iris root inserted into the ciliary body just behind the scierai spur. We reexamined these patients on several occasions to be sure we were not missing extensive basal synechiae from old angle closure; however, the visibility of the scierai spur, the presence of iris processes bridging the angle, the uniform appearance of the angle throughout' its circumference, and the absence of signs of pupillary block reassured us that we were not in error. Prominent uveal meshwork and an anterior insertion of the iris were present in both eyes of the patient who had had a cataract operation and subsequent pressure elevation in only one eye, which provided further evidence that these goni-
patients, including
oscopic findings
were
congenital
in
12 eyes, 20/50 to 20/200 in 17 eyes, and less than 20/200 in four eyes. In many patients we could not accurately evaluate the visual fields and optic discs for
origin
which adheres to the cornea, could be used to steady nystagmoid eye movements and often allowed a biomicroscopic view of the optic disc when ophthalmoscopy was impossible. Glaucomatous optic nerve damage was definitely present in six patients, questionable in six more, and probably absent in six others. We treated all but two patients with pressure-lowering medication. Satisfactory pressure reduction was achieved in six patients with pilocarpine hydrochloride, two patients with echothiophate iodide, two patients with epinephrine hydrochloride, and three patients with aeetazolamide plus miotics. Two patients, both with normal optic nerves, had intraocular pressures over 30 mm Hg despite maximal tolerated medical treatment. One patient was treated surgically; he had a successful, Scheie thermoselerostomy in his only eye. Certain side effects of pressure-lowering medications were particularly troublesome in these patients. Miotics reduced visual acuity markedly when aftercataracts left dense opacities in the center of the pupil. Retinal detachment, possibly related to miotic treatment, occurred in one patient eight months after pilocarpine therapy was started and in another patient four weeks after pilocarpine therapy was changed to
echothiophate therapy. Epinephrine-induced macuiopathy, a cause for concern in these aphakic eyes, was not recognized but would be difficult to detect if visual acuity were
reduced from other
causes.
COMMENT
Other reports of glaucoma following
extracapsular operations for congenital cataracts implicate pupillary block with angle closure as the mecha-
nism in almost every case.'-a While Chandler and Grant,' stressed that pupillary block is the usual mechanism, they mentioned "quite exceptional cases" in which numerous operations for congenital cataract were followed by open angle glaucoma. The
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
trabecular meshwork in these unusual cases was covered by granular-pigmented debris. All of our patients had open anterior chamber angles. Years or even decades following operations for congenital cataracts, intraocular pressures
became
examined
no
relationship
high. Because
we
have
eye pathologically, the between the cataracts,
their surgery, and the subsequent glaucoma is unclear. However, we have considered several possible rela-
tionships.
mention One possibility, which only to disclaim it, is that these eyes originally had angle-closure glaucoma that resulted from pupillary block and subsequently developed a "pseudoangle"-an angle lined by peripheral anterior synechiae but seemingly we
open to casual view. Careful gonioscopic examination refutes this hypothesis: the chamber angles of these patients were unquestionably open. The scierai spur was always visible, although in some cases it was partially obscured by dense uveal meshwork and iris processes. A second possibility, more difficult to disprove, is that these patients,
simply by chance, had two unrelated diseases: congenital cataracts and primary open-angle glaucoma. Supporting this hypothesis is the bilateral nature of the glaucoma. Against it is
the young age of many of the patients
and the absence of a family history of glaucoma in all but the two pairs of siblings. Also, we are impressed that the number of patients with this syndrome is large in relation to the number of operations performed in our hospital for congenital cataracts. Very rough calculations, in fact, suggest that the incidence of open-
angle glaucoma following congenital
cataract surgery may be several times the incidence of open-angle glaucoma in the general population. We plan an epidemiologic study to define the inci-
dence
more
exactly.
A third possibility is that the cataracts and the glaucoma are both mani-
festations of an undescribed congenital ocular syndrome. Supporting
this hypothesis is the atypical ap-
pearance of the anterior chamber angle in those eyes in which the uveal
meshwork was unusually prominent, and the iris root inserted anteriorly. However, the late onset of clinical manifestations of glaucoma, the absence of signs of congenital glaucoma, and the normal appearance of the chamber angle in some eyes weigh against this hypothesis. A fourth possibility is that the operation for congenital cataract caused the glaucoma. In almost all cases, the first operation for cataract left a large amount of lens cortex to fluff up and eventually absorb. Postoperative inflammation or toxic effects of degenerating lens protein may have caused trabecular damage. Other investigators have demonstrated by tonography a reduction of aqueous outflow
following extracapsular
cataract
ex-
tractions.''1 The mechanism of the glaucoma may not have been the same in all of our patients. Nevertheless, the clinical presentation was quite uniform. Typically, the patient had one or more operations early in life for cataracts; a clear visual axis was obtained; aphakic glasses were fitted; the condition was thought to be stable by the patient and his physician; and for many years, the patient was not examined by an
ophthalmologist. Asymptomatic glaucoma began at some time during this period but was not recognized by the patient. If loss of vision occurred, it either went unnoticed or was attributed to aftercataract. Three of our patients lost all useful vision in one eye before glaucoma was
suspected. Fortunately, we have patient lose useful vision after
even had no
was started. It is obvious that preservation of vision, in this as in all forms of glaucoma, depends upon early diagnosis and treatment. Therefore, we recommend that all patients who undergo operations for
treatment
and an anteriorly inserted iris root should be observed with special care for future development of glaucoma. If glaucoma is detected, it must be treated energetically but with awareness of the potential hazards. Adverse effects of pressure-lowering medications in young aphakic eyes include reduction of visual acuity when lens remnants occupy the pupillary zone, miotic-induced retinal detachment,
and, possibly, epinephrine-induced maculopathy. The usual yardsticks by
which efficacy of treatment is measured may be missing: visual fields are often unreliable because of nystagmus; the optic disc is often hidden from view by aftercataracts and small or displaced pupils. Measurement of intraocular pressure may be the only way to assess effectiveness of treatment.
study of these patients leaves unanswered questions. What is many the incidence of open-angle glaucoma Our
following congenital surgery? How soon after the
cataract cataract does the intraocular pressure surgery become elevated? Is the mechanism of
the pressure elevation the same in all cases? Does open-angle glaucoma occur after modern extracapsular procedures in which most of the lens cortex is removed during the initial operation, or is it limited to older surgical procedures in which lens cortex is left to fluff up and eventually absorb? Is late open-angle glaucoma following cataract extraction a phenomenon restricted to congenital cataract, or does it also occur after adult cataract surgery? Answers to these questions, when they become available, will clarify the relationship of the cataracts, the surgery, and the glaucoma; perhaps these answers will point the way to a rational prophylaxis.
congenital cataract be carefully examined for signs of glaucoma. Patients
should not be dismissed from followup after successful cataract surgery, but should be periodically brought back for reexamination. Each examination should include measurement of intraocular pressure and a careful examination of the optic discs. Patients in whom gonioscopy reveals a heavily pigmented uveal meshwork
References 1. Chandler coma.
PA, Grant WM: Lectures on Glau-
Philadelphia, Lea & Febiger, 1965, pp 367\x=req-\
375. 2. Fox SA:
Postoperative glaucoma.
Ophthalmol 16:585-608, 1936.
Arch
3. Miller JE, Keskey GR, Becker B: Cataract extraction and aqueous outflow. Arch Ophthalmol 58:401-406, 1957. 4. Lee PF, Trotter RR: Tonographic and gonioscopic studies before and after cataract extraction. Arch Ophthalmol 58:407-416, 1957.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
cataracts
discovered to have
were
high
Intraocular pressures. Several had profound glaucomatous loss of vision. All had deep anterior chambers with flat iris
planes and wide open angles. The uveal meshwork
was
unusually
coarse
and
Nystagmus, aftercataracts, and small pupils made visual field testing and observation of pigmented
in
some cases.
optic discs difficult. Most
cases were
bilateral, which suggested a hereditary origin; some had a family history of glaucoma. In none did we observe signs of congenital glaucoma or rubella syn-
drome. The incidence of this disease is not yet
known,
but it is common enough in our to indicate lifetime surveillance for glaucoma in all patients who undergo congenital cataract surgery.
clinic
(Arch Ophthalmol 95:1985-1987, 1977)
period if flow of aqueous through the pupil is blocked by vitreous or lens remnants.' Another variety that has attracted little attention in the past is a
chronic
open-angle
form of
glau-
Unlike the first type, it develops slowly and may not become apparent until years or decades after the operation for congenital cataract. In this report we will describe 18 patients with the chronic open-angle type of glaucoma. Our purpose is to warn ophthalmologists of this poten-
coma.
tial, late complication of congenital
point out the importance of long-term follow-up, including periodic measurements of cataract surgery and to
intraocular pressure and examination of the optic discs, of all patients who undergo an operation for congenital cataracts.
DESCRIPTION OF PATIENTS is a well-known complication of surgery for congenital cataracts. The most dramatic variety is closed-angle glaucoma. It develops acutely during the early postoperative
Glaucoma Accepted
publication May 14, 1977. Ophthalmology, Iowa, Iowa City.
for
From the Department of
University of
Presented at the 126th annual convention of the American Medical Association, San Francisco, June 19, 1977. Reprint requests to Department of Ophthal-
mology, University Hospitals, 52242 (Dr Phelps).
Iowa
City,
IA
We have examined and treated 18 who developed open-angle glaucoma after an operation for congenital cataract. Ten patients were male, and eight were female. A family history of glaucoma was present in four patients: two sibling pairs. The father of one pair also had glaucoma following operations for congenital cataracts; he is not included in the present series because he was not examined by the authors. In none of the remaining 14 patients was there a family history of glaucoma; however, five had a family history of congenital cataracts. Cataracts were noted in all 18 patients
patients
after birth. The cataracts were described as nuclear in three patients, zonular in one, and complete in one; no description is recorded for the remainder. One patient had facial features of Hallermann-Streiff's syndrome, two had epilepsy, and one had mild congenital hearing loss with no other signs of rubella syndrome; the remaining 14 patients had no systemic abnormalities. Cataract operations were performed in both eyes of 17 patients and in one eye of one patient. The operations on eight patients were performed elsewhere, and for six of these, the type of operation, other than being extracapsular, is unknown. The initial cataract operation was a simple needling in 16 eyes of nine patients, linear extraction in five eyes of three patients, and intracapsular extraction in both eyes of one patient. One operation was performed in each eye of four patients; multiple operations were performed in each eye of ten patients; and the number of operations is unknown for four patients. Discission of a pupillary membrane was the most frequently performed secondary operation. In no patient was one of the soon
currently popular operative techniques (needling and aspiration, phacoemulsification, or roto-extraction) performed as a primary operation. The ages of patients at the time of their first operation for cataract ranged from 7 months to 34 years (Table). No ocular
abnormalities other than cataracts and poor visual acuity were noted in any patient before the first operation. Specifically, signs of congenital glaucoma, including corneal enlargement, corneal edema,
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
by the cataract operation. depended on the amount of aftercataract, nystagmus, and amblyopia, as well as the presence or absence of glaucomatous damage. It was 20/40 or better in and not caused Visual acuity
of Patients at Time of Cataract Operations, First Detection of IOP* Elevation, and Time Interval Between Last Cataract Operation and First Detection of IOP Elevation
Ages
at Time of Cataract Operations, yr '/s 1
Age When IOP
2, 19t 2, 3t 2, 3f 3, 4t
33 19 31 38 11 27 25
Ages
Patient 1
Elevation
Detected, yr
Interval, yr
8
glaucomatous damage. Nystagmus prevented steady fixation in 14 patients and made visual field testing unreliable. Fundus examination was often hampered by
14
16
28 34
aftercataracts, nystagmus, and small pupils. However, the Goldmann contact lens,
23 21
10 12 13 14 15 16 17 18
5, 7t 8,1 li9 10 10
14
14, 21t
48 24
34
36
15
45
se 16 17 32
22 33
•IOP indicates intraocular pressure. fFirst number indicates age at which the earliest operation for cataracts was who had more than one operation; second number, age at latest operation.
and Haab's striae, were absent during infancy and later in life. However, the intraocular pressure was measured in only two patients before the cataract operation. Elevated intraocular pressures (greater than 20 mm Hg) were discovered during a routine examination in 16 patients and during evaluation for sudden loss of visual acuity in two patients. The pressure was high in both eyes of 14 patients and in one eye of four patients. Three of the patients with unilateral pressure elevation had earlier lost their other eye from laceration, intraocular foreign body, or retinal detachment. The fourth was the patient who had had surgery for cataract in only one eye; her unoperated eye had a normal pressure.
The ages at which intraocular pressure were first detected ranged from 6 to 56 years (Table). The time interval between the last operation for congenital cataract and the first detection of an elevated intraocular pressure varied considerably; it was less than five years in three patients, five to ten years in six patients, 11 to 20 years in two patients, 21 to 30 years in four patients, and more than 30 years in three patients (Table). In many patients the pressure elevation may have been present for some time before its detection; earlier measurements of intraocular pressure were recorded in only six
elevations
patients.
performed
in
patients
The level of intraocular pressure when the elevation was first detected ranged from 22 to 42 mm Hg. It was initially less than 30 mm Hg in all but three patients. The eyes had deep anterior chambers and flat iris contours. With the occasional exception of a small anterior synechia at the site of an old surgical incision, all anterior chamber angles were wide open. In seven patients the angles appeared entirely normal. In nine patients the uveal
meshwork was unusually prominent: a thick layer of lacy interwoven, pigmented strands nearly obscured the scierai spur and extended to Schwalbe's line. In six four with prominent uveal meshwork, the angle recess was poorly developed, and the iris root inserted into the ciliary body just behind the scierai spur. We reexamined these patients on several occasions to be sure we were not missing extensive basal synechiae from old angle closure; however, the visibility of the scierai spur, the presence of iris processes bridging the angle, the uniform appearance of the angle throughout' its circumference, and the absence of signs of pupillary block reassured us that we were not in error. Prominent uveal meshwork and an anterior insertion of the iris were present in both eyes of the patient who had had a cataract operation and subsequent pressure elevation in only one eye, which provided further evidence that these goni-
patients, including
oscopic findings
were
congenital
in
12 eyes, 20/50 to 20/200 in 17 eyes, and less than 20/200 in four eyes. In many patients we could not accurately evaluate the visual fields and optic discs for
origin
which adheres to the cornea, could be used to steady nystagmoid eye movements and often allowed a biomicroscopic view of the optic disc when ophthalmoscopy was impossible. Glaucomatous optic nerve damage was definitely present in six patients, questionable in six more, and probably absent in six others. We treated all but two patients with pressure-lowering medication. Satisfactory pressure reduction was achieved in six patients with pilocarpine hydrochloride, two patients with echothiophate iodide, two patients with epinephrine hydrochloride, and three patients with aeetazolamide plus miotics. Two patients, both with normal optic nerves, had intraocular pressures over 30 mm Hg despite maximal tolerated medical treatment. One patient was treated surgically; he had a successful, Scheie thermoselerostomy in his only eye. Certain side effects of pressure-lowering medications were particularly troublesome in these patients. Miotics reduced visual acuity markedly when aftercataracts left dense opacities in the center of the pupil. Retinal detachment, possibly related to miotic treatment, occurred in one patient eight months after pilocarpine therapy was started and in another patient four weeks after pilocarpine therapy was changed to
echothiophate therapy. Epinephrine-induced macuiopathy, a cause for concern in these aphakic eyes, was not recognized but would be difficult to detect if visual acuity were
reduced from other
causes.
COMMENT
Other reports of glaucoma following
extracapsular operations for congenital cataracts implicate pupillary block with angle closure as the mecha-
nism in almost every case.'-a While Chandler and Grant,' stressed that pupillary block is the usual mechanism, they mentioned "quite exceptional cases" in which numerous operations for congenital cataract were followed by open angle glaucoma. The
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
trabecular meshwork in these unusual cases was covered by granular-pigmented debris. All of our patients had open anterior chamber angles. Years or even decades following operations for congenital cataracts, intraocular pressures
became
examined
no
relationship
high. Because
we
have
eye pathologically, the between the cataracts,
their surgery, and the subsequent glaucoma is unclear. However, we have considered several possible rela-
tionships.
mention One possibility, which only to disclaim it, is that these eyes originally had angle-closure glaucoma that resulted from pupillary block and subsequently developed a "pseudoangle"-an angle lined by peripheral anterior synechiae but seemingly we
open to casual view. Careful gonioscopic examination refutes this hypothesis: the chamber angles of these patients were unquestionably open. The scierai spur was always visible, although in some cases it was partially obscured by dense uveal meshwork and iris processes. A second possibility, more difficult to disprove, is that these patients,
simply by chance, had two unrelated diseases: congenital cataracts and primary open-angle glaucoma. Supporting this hypothesis is the bilateral nature of the glaucoma. Against it is
the young age of many of the patients
and the absence of a family history of glaucoma in all but the two pairs of siblings. Also, we are impressed that the number of patients with this syndrome is large in relation to the number of operations performed in our hospital for congenital cataracts. Very rough calculations, in fact, suggest that the incidence of open-
angle glaucoma following congenital
cataract surgery may be several times the incidence of open-angle glaucoma in the general population. We plan an epidemiologic study to define the inci-
dence
more
exactly.
A third possibility is that the cataracts and the glaucoma are both mani-
festations of an undescribed congenital ocular syndrome. Supporting
this hypothesis is the atypical ap-
pearance of the anterior chamber angle in those eyes in which the uveal
meshwork was unusually prominent, and the iris root inserted anteriorly. However, the late onset of clinical manifestations of glaucoma, the absence of signs of congenital glaucoma, and the normal appearance of the chamber angle in some eyes weigh against this hypothesis. A fourth possibility is that the operation for congenital cataract caused the glaucoma. In almost all cases, the first operation for cataract left a large amount of lens cortex to fluff up and eventually absorb. Postoperative inflammation or toxic effects of degenerating lens protein may have caused trabecular damage. Other investigators have demonstrated by tonography a reduction of aqueous outflow
following extracapsular
cataract
ex-
tractions.''1 The mechanism of the glaucoma may not have been the same in all of our patients. Nevertheless, the clinical presentation was quite uniform. Typically, the patient had one or more operations early in life for cataracts; a clear visual axis was obtained; aphakic glasses were fitted; the condition was thought to be stable by the patient and his physician; and for many years, the patient was not examined by an
ophthalmologist. Asymptomatic glaucoma began at some time during this period but was not recognized by the patient. If loss of vision occurred, it either went unnoticed or was attributed to aftercataract. Three of our patients lost all useful vision in one eye before glaucoma was
suspected. Fortunately, we have patient lose useful vision after
even had no
was started. It is obvious that preservation of vision, in this as in all forms of glaucoma, depends upon early diagnosis and treatment. Therefore, we recommend that all patients who undergo operations for
treatment
and an anteriorly inserted iris root should be observed with special care for future development of glaucoma. If glaucoma is detected, it must be treated energetically but with awareness of the potential hazards. Adverse effects of pressure-lowering medications in young aphakic eyes include reduction of visual acuity when lens remnants occupy the pupillary zone, miotic-induced retinal detachment,
and, possibly, epinephrine-induced maculopathy. The usual yardsticks by
which efficacy of treatment is measured may be missing: visual fields are often unreliable because of nystagmus; the optic disc is often hidden from view by aftercataracts and small or displaced pupils. Measurement of intraocular pressure may be the only way to assess effectiveness of treatment.
study of these patients leaves unanswered questions. What is many the incidence of open-angle glaucoma Our
following congenital surgery? How soon after the
cataract cataract does the intraocular pressure surgery become elevated? Is the mechanism of
the pressure elevation the same in all cases? Does open-angle glaucoma occur after modern extracapsular procedures in which most of the lens cortex is removed during the initial operation, or is it limited to older surgical procedures in which lens cortex is left to fluff up and eventually absorb? Is late open-angle glaucoma following cataract extraction a phenomenon restricted to congenital cataract, or does it also occur after adult cataract surgery? Answers to these questions, when they become available, will clarify the relationship of the cataracts, the surgery, and the glaucoma; perhaps these answers will point the way to a rational prophylaxis.
congenital cataract be carefully examined for signs of glaucoma. Patients
should not be dismissed from followup after successful cataract surgery, but should be periodically brought back for reexamination. Each examination should include measurement of intraocular pressure and a careful examination of the optic discs. Patients in whom gonioscopy reveals a heavily pigmented uveal meshwork
References 1. Chandler coma.
PA, Grant WM: Lectures on Glau-
Philadelphia, Lea & Febiger, 1965, pp 367\x=req-\
375. 2. Fox SA:
Postoperative glaucoma.
Ophthalmol 16:585-608, 1936.
Arch
3. Miller JE, Keskey GR, Becker B: Cataract extraction and aqueous outflow. Arch Ophthalmol 58:401-406, 1957. 4. Lee PF, Trotter RR: Tonographic and gonioscopic studies before and after cataract extraction. Arch Ophthalmol 58:407-416, 1957.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
