Incidence and Prognosis of Childhood Glaucoma

A Study of 63 Cases

MAGDA BARSOUM-HOMSY, MD, FRCS(C), LINE CHEVRETTE, MD, FRCS(C)

Abstract: Sixty-three consecutive cases (95 eyes) of glaucoma in children were studied. Glaucoma associated with congenital anomalies (group II) formed the largest group in this study. This accounted for 46% of the cases compared to primary congenital glaucoma (group I) that accounted for 22.2%. Secondary glaucoma (group Ill) occurred in 31.8%. The presenting signs and symptoms in group I were tearing and corneal edema. In 50% of the cases in groups II and Ill, diagnosis was made on a routine ophthalmologic examination. Surgery was performed in 95.8% of eyes in group I, 53.2% in group II, and 54.2% in group Ill. The best visual prognosis occurred in group I where 77.3% of affected eyes had visual acuity equal to or better than 20/50 with good pressure control in all. This was followed by group II where 41 .5 % had vision equal to or better than 20/50 and 41.4% had 20/200 vision or less. Intraocular pressure remained uncontrolled in 19.1% of this group. The worst prognosis and morbidity was found in group Ill where 30.5% of eyes had 20/50 vision or better and 47.8% had 20/200 vision or less. In group Ill, 33.3% had uncontrolled intraocular pres­sure. [Key words: glaucoma associated with congenital anomalies, primary con­genital glaucoma, secondary glaucoma, vision in childhood glaucoma.] Oph­thalmology 93:1323-1327, 1986

Glaucoma in infants and children has been classified into three groups: group I, primary congenital or infantile glaucoma; group II, glaucoma associated with congenital anomalies; and group III, glaucoma secondary to other ocular pathology. 1 The incidence of primary congenital glaucoma is reported to be 1:10,0002

; however, we have not come across any study that indicates the incidence of groups II and III. The purpose of this study is to evaluate the relative incidence of glaucoma in the three different groups, to describe and compare the associated clinical findings and treatment modalities, as well as to establish the prognosis in each group.

From the Department of Ophthalmology, Hopital Sainte-Justine, Universite de Montreal, Montreal, Quebec.

Presented at the Canadian Ophthalmological Society Meeting, June 1982.

Reprint requests to M. Barsoum-Homsy, MD, FRCS(C), Department of Ophthalmology, Hopital Sainte-Justine, 3175 Chemin Cote Sainte-Catherine, Montreal, PO, Canada H3T 1 C5.

MATERIALS AND METHODS

Sixty-three consecutive cases (95 eyes) of glaucoma in children, seen between 1975 and 1983 at the Pediatric Glaucoma Clinic ofHopital Ste-Justine, were included in this study. All observations were recorded by either one or both authors. Therapeutic decisions were made jointly. The follow-up period varied from 2 months to 10 years with an average of 4.4 years. The ocular family history, the age of onset and the presenting signs and symptoms of glaucoma were compiled. Visual acuity, corneal di­ameters, cup/disc ratio, refractive error, and intraocular pressure were obtained when possible before treatment, as well at the end of the follow-up period. Corneal di­ameters were measured by calipers with the patient under general anesthesia. Anesthesia was induced with oxygen, nitrous oxide, and halothane 1.5 to 2.0%. Atropine (10 mg/kg) was given intravenously. Tracheal intubation was performed and anesthesia was monitored with oxygen, halothane ( 1.5-2.0% ), and nitrous oxide (2-4 1) through

OPHTHALMOLOGY • OCTOBER 1986 • VOLUME 93 • NUMBER 10

Table 1. The Relative Incidence, Bilaterality, Sex, Age, and Clinical Presentation, in the Three Groups of Childhood Glaucoma

Variables

No. of cases (%) No. of eyes (%) Bilateral (%) Sex(%) Average age at diagnosis Clinical presentation

M =male.

Group I

14 (22.2) 24 (25.26) 10 (71.4) M (71.4) 10.6 mo (2 wk-5.2 yr) Corneal haze, tearing

a Bain circuit with a fresh gas flow of 6 1/minute. The cup/disc ratio was evaluated by judgmental quantitative estimates offundus photographs in all patients whose me­dia were clear. In those with corneal edema, cup/disc ratio was evaluated by direct and/or indirect ophthalmoscopy when possible. Cycloplegic refraction was performed with 1% cyclopentolate, 1 drop instilled twice in each eye with a five-minute interval between instillations. Intraocular applanation pressures were recorded under general anes­thesia, or when the patient's cooperation permitted, under topical anesthetic drops. Intraocular applanation tension of up to 24 mmHg was considered as adequate tension control with or without medication, when coupled with the absence of signs of disease progression such as increase in optic disc cupping, and/or corneal diameters. Aniso­metropia was defined as a spherical difference of 2.00 diopters (D) or a cylindrical difference of 1.50 D or more. To compare the three groups, standard analysis of variants was performed. Multiple comparisons were done using Scheffe's procedure.

RESULTS

The clinical findings in each group are shown in Tables 1 and 2. Group I included two affected brothers with a

Table 2. Summary of Clinical Findings in the Three Groups of Childhood Glaucoma

Group I Group II Group Ill Variables (no. of eyes) (no. of eyes) (no. of eyes)

Corneal diameter (24)* (26)* (24) (mm) 13.62 ± 0.76 12.2 ± 1.1 12.0 ± 1.0

Disc cupping Initial examination (24) (41) (16)

0.51 ± 0.34 0.43 ± 0.29 0.28 ± 0.33 Final examination (24) (42) (14)

0.41 ± 0.28 0.46 ± 0.28 0.29 ± 0.33 Applanation pressure

(mmHg) Initial examination (21) (23) (19)

21.8 ± 7.4t 29.7 ± 8.7 38.4 ± 8.6 Final examination (21) (42) (19)

15.6 ± 3.3 20.7 ± 6.8 19.3 ± 4.9

• Corneal diameter in patients under 3 years of age. t Mean intraocular pressure under general anesthesia.

1324

29 (46) 47 (49.5) 18 (62) M (51.7)

Group II

4.1 yr (6 wk-12 yr) Routine examination (58.6%),

corneal haze

20 (31.8) 24 (25.26) 4 (20)

M (70)

Group Ill

7.3 yr (4 wk-17.5 yr) Painful red eye (50%), routine

examination (45.8%)

negative family history of glaucoma. The main presenting signs and symptoms in this group, listed by frequency, were: cloudy cornea (8 cases), tearing (7 cases), ruptured Descemet's membrane (6 cases), increased corneal di­ameter (5 cases), and photophobia (4 cases). All patients in this group were treated surgically except for one patient with bilateral glaucoma (Table 3). This patient underwent surgery in one eye, whereas the signs and symptoms of glaucoma in the second eye regressed under topical an­tiglaucoma therapy alone. Eventually, medical treatment was stopped without recurrence of glaucoma in that eye.

In group I, the number of operations performed to ob­tain satisfactory control of the intraocular pressure aver­aged 2.1 per eye. The initial surgical procedure of choice was a goniotomy except where significant opacification ofthe cornea prevented adequate visualization of the an­gle. In such cases, trabeculotomy was performed as an initial procedure. Trabeculectomy was performed in this group after two goniotomies or trabeculotomies were un­successful in controlling the intraocular pressure (Table 4). Nineteen eyes were surgically controlled within the first year of diagnosis. Four eyes decompensated and de­veloped a recurrence of their disease 12 to 14 months after the initial surgery. One patient had a recurrence of his glaucoma 4;5 years after a successful second surgical procedure. No immediate or late surgical complications were noted in this group except for a small hyphema im­mediately after goniotomy or trabeculotomy in all cases. This usually disappeared within 24 hours.

The relative incidence of anisometropia, amblyopia,

Table 3. Treatment in Childhood Glaucoma

Type of Treatment Group I Group II Group Ill

Surgical 95.8% 53.2% 56.5% Average no. of surgery

per eye 2.1 2.3 1:2 Medical antiglaucoma

treatment after surgery 16.6% 88% 84.6%

Medical antiglaucoma treatment alone 4.2% 36.1% 43.5%

No treatment None 10.6% 4.2% Uncontrolled intraocular

pressure None 19.1% 33.3%

BARSOUM-HOMSY AND CHEVRETIE • CHILDHOOD GLAUCOMA

Table 4. Surgical Procedures in Childhood Glaucoma

Group I Group II Group Ill (23/24 eyes) (22/47 eyes) (13/23 eyes)

Goniotomy 25 18 T rabeculotomy 15 5 1 Trabeculectomy 7 19 8 Trabeculodialysis 2 Cyclocryotherapy 8 1 Peripheral iridectomy 4 Sector iridectomy

Table 5. The Relative Occurrence of Strabismus, Amblyopia, and Anisometropia in Childhood Glaucoma

Group I Group II Group Ill (%) (%) (%)

Strabismus 21.4 20 20 Amblyopia 78.6 31 5 Anisometropia 71.4 20

and strabismus is shown in Table 5. Table 6 shows the visual acuity at the end of the follow-up period. In group I, one patient with unilateral primary congenital glaucoma had 20/30 vision in the affected eye, with straight eyes and a 1 00-inch stereopsis. One patient was lost to follow­up one year after initial treatment.

The different ocular and systemic anomalies observed in group II are listed in Table 7. Mesodermal dysgenesis of the anterior segment was the most frequent anomaly in this group. A case of unilateral uveal coloboma asso­ciated with bilateral cleft lip and cleft palate as well as unilateral atresia of ala nasa on the same side as the uveal coloboma was included in this group. That patient had abnormal vascularization of the angle as well as goniodys­genesis. Diagnosis was made on a routine ophthalmologic examination in 17 cases (58.6%). Nine cases presented with tearing, corneal edema, increased corneal diameter, and photophobia. Two cases presented with headache and ocular pain. Unilateral myopia was the presenting sign in a 3-months-old patient with Sturge-Weber syndrome. The corneal diameter in 26 eyes in which glaucoma appeared before 3 years of age varied between 9.5 to 13.5 mm (mean, 12.2 mm ± 1.1) (Table 2).

Treatment in group II was initially medical and con­sisted of one or more of the following: epinephrine, ti­molol, pilocarpine, and/or acetazolamide (Table 3). Twenty-two of the 47 eyes (53.2%) required surgery be­cause medical treatment alone was not sufficient to control their glaucoma. After surgery, 19 eyes (88%) needed ad­ditional medical treatment to achieve adequate control of the intraocular pressure. In this group, 22 eyes under­went 51 operations, with an average of 2.3 operations per eye as outlined in Table 4. One patient with Sturge-Weber syndrome needed eight surgical procedures plus medical treatment to control the pressure. Two enucleations were performed due to intractable glaucoma, one in a case of

Table 6. Snellen Visual Acuity (Letters or Illiterate E) at the End of the Follow-Up Period in Childhood Glaucoma

Group I Group II Vision (22 eyes) (41 eyes)

20/20-20/50 77.3% 41.5% 20/60-20/100 13.6% 17.1% :::;20/200 9.1%* 26.8% Totally blind 14.6%

* P< 0.05.

Table 7. Ocular and Systemic Anomalies Associated with Group II Childhood Glaucoma

Pathology

lridocorneal dysgenesis Goniodysgenesis Rieger's syndrome Rieger's anomaly Axenfeld syndrome

Aniridia Sturge-Weber syndrome Uveal coloboma Neurofibromatosis Larsen syndrome

Total

Cases (49 eyes)

3 5 2 2 8 6 1 1 1

29

Group Ill (23 eyes)

30.5% 21.7% 8.7%

39.1%*

aniridia and the other in a case of uveal coloboma with complicated retinal detachment. Medical treatment alone was effective in 17 eyes (36.1% ), these included patients with Sturge-Weber syndrome, aniridia, Rieger's anomaly, and Larsen's syndrome. A 14-year-old patient with Sturge­Weber syndrome developed central retinal vein throm­bosis while under topical antiglaucoma therapy. Transient choroidal detachment after trabeculectomy occurred in a patient with Sturge-Weber syndrome and in one patient with Rieger's syndrome.

In this group, five eyes (10.6%) did not need any form oftreatment. This was due to the fact that these cases had signs of glaucoma consisting of asymetric cupping and asymetric corneal diameters. The cup/disc ratio in those eyes ranged between 0.2 to 0.6 (average, 0.4). However, on repeated examinations, no further changes were ob­served in these parameters, and the pressures recorded were always within normal range. These eyes were con­sidered to have had glaucoma sometime during their in­fancy and had undergone spontaneous normalization of their ocular pressure. Among these were patients with Sturge-Weber syndrome, Axenfeld anomaly, and Rieger's syndrome.

Visual acuity results in this group are shown in Table 6. Totally blind eyes were found in patients with aniridia, Rieger's syndrome, and chorioretinal coloboma associated with secondary retinal detachment.

Secondary glaucoma, group III, comprised 20 cases (24 eyes). The etiologic diagnosis is listed in Table 8. Patients

1325

OPHTHALMOLOGY • OCTOBER 1986 • VOLUME 93 • NUMBER 10

Table 8. Etiologic Diagnosis in Group Ill Secondary Glaucoma

Uveitis Aphakia

Pathology

Traumatic hyphema Congenital rubella Congenital rubella and aphakia Coats Retrolental fibroplasia Persistant hyperplastic primary vitreous Microphthalmus with congenital retinal fold

Total no. of cases

Cases {24 eyes)

6 5 2 1 2 1 1 1 1

20

with traumatic hyphema who presented with secondary glaucoma at time of the initial trauma were not included in this study, only those with late onset glaucoma were retained.

The presenting sign in this group was a red painful eye in ten cases (50%). Glaucoma was discovered on routine ophthalmologic examination in nine cases. A patient with congenital rubella syndrome presented at one month of age with unilateral increased corneal diameter and corneal edema. It is interesting to note that in an aphakic 3.5-year-old boy, glaucoma was suspected when on a routine visit his refraction showed a decrease of 10 D of hyperopia; the awake applanation pressure was 40 mmHg and the optic disc was normal with absence of cupping.

Treatment in this group was initially medical. One pa­tient was lost to follow-up; 13/23 eyes (56.5%) needed surgery (Table 3). A total of 16 operations were performed with an average of 1.2 operation per eye (Table 4). A congenital rubella patient, whose glaucoma was diagnosed and operated at one month of age, died two months after surgery from severe cardiovascular anomalies. Patients with Coats' disease and retrolental fibroplasia presented with acute angle-closure glaucoma and required peripheral basal iridectomies to achieve control of their pressure. One patient with bilateral uveitis presented with bilateral seclusio pupillae and iris bombe. Bilateral peripheral iri­dectomies were performed and additional medical therapy was needed to control the intraocular pressure. A patient with microphthalmus and congenital retinal fold devel­oped progressive shallowing of the anterior chamber and eventually angle-closure glaucoma, which was treated medically. Eleven of 13 operated cases needed additional medical antiglaucoma therapy to control their pressure. Medical treatment alone was successful in 10 of the 23 eyes. Only one patient did not require any treatment. This was due to the fact that glaucoma occurred in a blind eye and the patient was asymptomatic. Postoperative com­plications in this group included a transient choroidal de­tachment after trabeculectomy in a patient with bilateral aphakia and uveitis associated with rheumatoid arthritis. Another patient with rheumatoid arthritis, bilateral uve­itis, and glaucoma developed cataracts four months after bilateral trabeculectomies. Extracapsular cataract extrac-

1326

tions were performed and one eye went on to develop a retinal detachment 15 weeks after the surgery. Pressures were uncontrolled in 7 of the 21 treated eyes (33.3%). Visual results are shown in Table 6. Patients with total blindness had the following pathology: persistent hyper­plastic primary vitreous, Coats' disease, hyphema with neovascular glaucoma, uveitis with secondary retinal de­tachment, aphakic retinal detachment, retrolental fibro­plasia, and microphthalmus with congenital retinal fold. Amblyopia was present in a bilateral aphakic patient.

DISCUSSION

Though primary congenital glaucoma is believed to be the most frequent type of pediatric glaucoma, we found that glaucoma associated with congenital anomalies (group II) was the largest group in this study and accounted for 46% of the cases.2 This was followed by group III (31.8% ); the smallest group was group I (22.2% ). It has been claimed by Becker that the average ophthalmologist is unlikely to see more than one new case of primary congenital glaucoma in five years of practice. 1 Because primary congenital glaucoma represented a fifth of the total number of childhood glaucoma cases in our study, we can therefore assume that the average ophthalmologist is likely to see one new case of childhood glaucoma per year, taking into consideration that there may be several factors that might influence this incidence. Also, because primary congenital glaucoma in the general population is estimated to be 1: 10,000, we believe that this figure is in fact close to 1 in 2,000 with regards to all types of glaucoma in childhood.2

The incidence of group III may even be higher than is shown in this study. This may be due to the following factors: the older age bracket in this group (7 years) on initial diagnosis makes intraocular pressure readings easier to record. Therefore, these patients are more readily di­agnosed and treated by the general ophthalmologist, and all cases are not necessarily referred to a specialized center for pediatric glaucoma. Also, cases such as glaucoma (sec­ondary to eight-ball hemorrage, pupillary block by a sub­luxated lens, and intraocular tumors, whose glaucoma problem is resolved by treating the initial pathology) are not referred to the glaucoma clinic.

In this study, we have found a higher incidence of af­fected males. This is particularly evident in groups I and III (Table 1 ). Biglan and Hiles also found a male prepon­derance in 65% of their study.3 Bilaterality is more often observed in groups I and II. This is in agreement with other reports with regard to bilaterality in primary con­genital glaucoma. 3•

4

The presenting signs and symptoms in group I as re­ported by different authors were tearing and corneal cloudiness.2

•3•5 The average corneal diameter in group I

was 13.6 mm, which is a 2-mm increase over the average normal corneal diameters in infants by 1 year of age.6

•7

This is in accordance with other reports. 2•3

•5 Corneal di­

ameter measurement in group I is compared to group II patients in whom glaucoma was diagnosed before 3 years

BARSOUM-HOMSY AND CHEVRETTE • CHILDHOOD GLAUCOMA

of age. The difference in corneal diameter measurements between groups I and II is statistically significant (P < 0.0001). The larger corneal diameter in group I may be due to the fact that in this group, the tearing is more often misdiagnosed as blocked tear ducts and patients are consequently referred later in the course of the disease. However, in group II, patients younger than 3 years of age, having ocular and/or systemic anomalies such as an­iridia, Sturge-Weber syndrome, and mesodermal dysgen­esis, were often referred to us before any signs of glaucoma were present. Consequently, glaucoma was diagnosed earlier in group II, as soon as the first signs of decompen­sation appeared and before the occurrence of marked changes in corneal diameter.

Approximately 50% of the cases in groups II and III were asymptomatic and were diagnosed during a routine ophthalmologic examination. It is therefore important to recognize the different ocular and systemic syndromes that are associated with glaucoma in childhood, as well as the different ocular pathology leading to secondary glaucoma. It is imperative to follow those patients closely with pe­riodic pressure measurements, sequential cycloplegic re­fractions, fundus examination, and optic disc photogra­phy. Refractive changes towards myopia should trigger the diagnosis of glaucoma in susceptible patients.5•

8

The initial intraocular pressure in group I cannot be compared with the other two groups because in this group, initial pressures were recorded under general anesthesia and therefore do not bear the same absolute value as those taken without sedation. However, all the intraocular ap­planation pressure measurements at the end of the follow­up period in the three groups were recorded in the awake state. To compare the three groups, standard analysis of variants was performed and multiple comparisons were done using Scheffe's procedure. There was a significant difference in the final intraocular pressure between group I and the other two groups (P < 0.005). Group I had the lowest intraocular pressure with a mean of 15.6 ± 3.3 mmHg. This may reflect the better control and prognosis of the glaucoma in this group.

The optic disc cupping on initial examination was greatest in group I followed by groups II and III (Table 2). This difference was not statistically significant. Only in group I did the mean optic disc cupping regress at the end of the follow-up period (P < 0.05). This has also been observed by other authors.3

•5

Medical treatment alone was sufficient irt controlling the pressure in 36.1% of eyes in group II and 43.5% in group Ill, whereas 95.8% in group I required surgery. In a patient with bilateral primary glaucoma, one eye had regression of symptoms while under medical antiglau­coma therapy alone. This has been previously recorded in a case with Rieger's anomaly but not in primary in­fantile glaucoma.9 Contrary to group I and II in which goniotomy and trabeculotomy were the surgical proce­dures of choice, trabeculectomy was the most common procedure in group III.

Group III seems to bear the worst prognosis because ocular pressure remained uncontrolled in -33.3% of the

eyes, and 47.8% had 20/200 vision or less (Table 6). Using the chi-square test, group III has a statistically higher in­cidence of totally blind eyes compared to group II (P < 0.05) and thus carries the highest morbidity of the glau­comas in children. Group I has the best prognosis. Only in group I did the optic disc cupping decrease significantly during the course of the disease (P < 0.05). Also, all pa­tients in this group have well-controlled intraocular pres­sure, and only 9.1% have vision of 20/200 or less. Chi­square tests were conducted to assess the difference be­tween the number of eyes with vision of 20/200 or less in the three groups. Group I had the best visual outcome compared to group II and III (P < 0.05). Although ani­sometropia and amblyopia were present in more than 70% of the cases in group I, 77.3% of eyes had final vision of 20/50 or better. Our good visual results can be attributed to the prompt optical correction of ametropia and ani­sometropia, as well as the vigorous treatment of potential amblyopia.

Close follow~up at regular intervals of one to three months is mandatory in all glaucoma patients. This creates a good doctor-to-patient contact, making it easy to record ocular pressure without sedation as early as 2 to 3 years of age. Individual and expert attention is given to each and every problem including amblyopia and anisometro­pia. Early diagnosis of the first signs of glaucoma, or de­compensation of the latter and the institution of prompt treatment are essential in order to prevent the occurrence of irreversible permanent visual damage.

ACKNOWLEDGMENTS

The authors thank Mr. Gilles Ducharme, MSc, for the statis­tical analysis cif the data in this article.

REFERENCES

1. Kolker AE, Hetherington J Jr, eds. Becker-Shaffer's Diagnosis and Therapy of the Glaucomas, 3d ed. St Louis: CV Mosby, 1970; 258-96.

2. Walton DS. Primary congenital open angle glaucoma: a study of the anterior segment abnormalities. Trans Am Ophthalmol Soc 1979; 77: 746-68.

3. Biglan AW, Hiles DA. the visual results following infantile glaucoma surgery. J Pediatr Ophthalmol Strabismus 1979; 16:377-81.

4. Gencik A, Kadasi L, GenCikova A, Gerinec A. Notes on the genetics of congenital glaucoma. Ophthalmologica 1979; 179:209-13.

5. Broughton WL, Parks MM. An analysis of treatment of congenital

glaucoma by goniotomy. Ani J Ophthalmol 1981; 91:566-72. 6. Hetherington J Jr. Congenital glaucoma. In: Duane TD, ed. Clinical

Ophthalmology. Philadelphia: Harper & Row, 1978; Volume 3, Chapter 51.

7. The Ophthalmologic Staff of the Hospital for Sick Children, Toronto. The Eye in Childhood. Chicago: Year Book, 1967; 270-1.

8. Haas J. Principles and problems of therapy in congenital glaucoma. Invest Ophthalmol1968; 7:140-6.

9. Leib ML, Saheb NN, Little JM. Rieger's anomaly with congenital glau­coma; a case presentation of postnatal anterior segment maturation. Can J Ophthalmol1979; 14:137-41.

1327