Ординатура / Офтальмология / Английские материалы / The Glaucomas Volume 1 Pediatric Glaucomas_Sampaolesi, Zarate_2009

Case 9:

Late Congenital Glaucoma or Juvenile Glaucoma (Goniodysgenesis)

This male child was 7 years old and presented with late congenitalglaucoma(goniodysgenesis).Trabeculotomy had been performed in both eyes at 6 years of age.

As can be seen in the evolution diagram (Fig. 17.86), the right eye was regulated at between 18 and 20 mmHg, and the left eye was always around 10 mmHg, i.e.,, ap-

parently both eyes were regulated, but a deeper study showed that for the right eye, pressures of around 18– 20 mmHg were not the target pressure needed. As the patient lived in another city, the ophthalmologist who followed him up started to make daily pressure curves at the age of 10 years, and told us that the pressures at 6 a.m. in bed were 25, 28, 28, 30, 27, and 33 mmHg.

As can be seen in Fig. 17.87, the action over the years of the ocular pressure (peaks at 6 a.m.) gradually wore down the optic nerve in the right eye. At the center, the HRT shows a pathological rim volume. In the retinofluoresceinography in Fig. 17.85c, corresponding to the right eye, there are no more capillaries in the capillary border of the disc. In Fig. 17.85f, corresponding to the left eye, the capillaries are normal in this zone. In Fig. 17.85b, e, the HRT image corresponds to what was said above.

In Fig. 17.88, the HRT corresponds to both eyes shows the pathology of the right eye. Since the intraocular pressure went down with medication and the diurnal pressure curve regulated (Figs. 17.89, 17.90), it seems for the moment to have stopped the progress of the optic nerve lesion of right eye. Figure 17.87 of the right eye shows with the ophthalmoscope, a pathological excavation. With HRT the optic nerve is very damaged. Interestingly, the photograph on the right (fluorescein) shows absence of the capillaries in the periphery of the optic nerve and vascular hooks. The figure below shows a normal optic disc with capillaries and no hooks.

The visual fields with conventional and nonconventional perimetry are normal in both eyes.

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In 1979, Robin et al. [1] and in 1980, Morin and Bryars [2] published perimetry studies in children operated for congenital glaucoma using the Goldmann perimeter. In 1989, Tejeiro and Domínguez [3] performed the first visual fields in children operated for congenital glaucoma with computed perimetry, using the Octopus 500 and the 2000, program G1. The children studied were aged between 6 and 17 years. They found a generalized depression: MD = 62 dB. Fifty-eight percent had truly pathological values of MD greater than or equal to 4 dB. They had 15.2% loss of visual field, which, as the authors comment, is certainly small. They say that this good result stems from:

1.The children being operated within 3 days of diagnosis.

2.After surgery, every eye that had an ocular pressure greater than 16 mmHg was reoperated.

This work is extraordinary if we think that it was written 16 years ago and shows the authors’ profound knowledge of congenital glaucoma disease.

Sampaolesi and Casiraghi´s [4] study titled “Computerized visual fields in pediatric glaucoma” had findings similar to Domínguez’s study [3], i.e., a limited diffuse loss of sensitivity in children with pure congenital glaucoma. This good result stems from good early surgical treatment and frequent monitoring of the refraction, i.e., the three criteria described by Dominguez are respected. In refractory congenital glaucoma and in late congenital glaucoma, we found diffuse loss of sensitivity and scotomas. In these cases, the visual defects were greater and in some cases were at very advanced stages.

Material

In our 1990 study, we examined 46 eyes of 25 patients from three pediatric glaucoma groups, ranging from 6 to 21 years of age (Table 18.1). We divided the patients into three groups:

–Group 1: Pure congenital glaucomas;

–Group 2: Refractory congenital glaucomas;

–Group 3: Late congenital glaucoma (goniodysgenesis).

Method

The visual fields were examined with the Octopus 2000 perimeter, using both phases of the program G1 [5, 6] and analyzed with Octosmart including the Bebie curve [7]. In order to minimize learning effects, the number of visual fields performed was between two and five per eye [8].

Analysis of variance including the following elements was performed with both parametric and nonparametric tests: best corrected visual acuity in the 20/20 scale, refraction, corneal diameter, axial length, mean defect (MD), and corrected loss variance (CLV), and a reliability factor lower than 10. The visual fields were analyzed with Bebie cumulative frequency curve and classified as diffuse, scotomatous, or combined (diffuse plus scotomatous) loss.

To evaluate the normal threshold in the 10to 20-year-old age group [9], we carried out both phases of the G1 program on ten young normal subjects with a visual acuity of 1.0 20/20 and axial length between 23.50 and 24.50, with a normal daily pressure curve and no ocular or associated systemic pathology.

Results

Group 1: Pure Congenital Glaucoma

The prognosis in these cases is very good when surgery normalizes intraocular pressure. The eyes stop enlarging, the optic disc is normal, the visual acuity is good, and the visual field is normal or nearly normal (Fig. 18.1).

Group 3: Late Congenital Glaucoma

In Fig. 18.3 corresponding to the right eye, it can be seen that the optic nerve (ON) is in phase IV, the conventional visual field is normal, and the nonconventional visual field is in phase III. For the ophthalmologists who think that glaucoma is an alteration of the optic nerve (ON) and of the visual field and who work with conventional perimetry, the diagnosis in this case is ocular hypertension. For those who work with nonconventional perimetry, the diagnosis is glaucoma, since the ON lesion corresponds topographically to the visual field (VF) lesion made with nonconventional

frequency-doubling perimetry. Since in this patient the maximum medication, prostaglandins – carbon anhydrase inhibitors and beta blockers – did not regulate the pressure, we performed surgery: deep nonpenetrating sclerectomy and goniopuncture with Yag laser. The pressure regulated at 12 mmHg and the daily pressure curve was normal. We would stress here that this patient was on medication for 17 years while her ON and VF were deteriorating. We believe that she will not have the same fate as her glaucomatous predecessors.

The ON and the VF are generally the same as in group 2, but the defects are much greater.