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

Pathological Anatomy

in Pigmentary Glaucoma

Clinical History No. 14

A 33-year-old man came for consultation in 1974. He had worn glasses for myopia for 12 years, renewing them every 2 years. The ophthalmologists had never taken his ocular pressure. In 1971, he lost the vision in his left eye and optic neuritis was diagnosed. He is a medical doctor, and he consulted because at night he began to see iris halos around lights with the right eye, in which he still had sight.

He showed all the features of pigmentary glaucoma: Krukenberg spindle, typical chamber angle, atrophy of the iris pigment layer, trapezoidal anterior chamber, and slow photomotor reflex.

Gonioscopy showed a wide open angle with very pigmented trabecular meshwork at the height of the Schlemm canal and significant mesodermal remnants reaching the Schwalbe line.

The retina was normal in the left eye, while in the right eye a small bag of detachment was present at 7 o’ clock with a tear, as can be seen in Fig. 20.49. I performed photocoagulation at the edge of the detachment, on the undetached retina. Since he was a medical doctor and had nearly lost the left eye, I asked his permission to take out a larger trabeculectomy piece when performing the trabeculectomy in the left eye (Figs. 20.50, 20.51).

Clinical History No. 15

This is a 37-year-old man who had suffered from undiagnosed pigmentary glaucoma for more than 15 years. Despite the open angle, the first clinical manifestation were iris halos around lights, a very marked goniodysgenesis, and the pathological anatomy showed pathological mesodermal remnants, such as are found in late pigmentary glaucomas. The patient consulted in 1974. Bilateral glaucoma had been diagnosed in 1967 with ocular pressure at 40 mmHg in the right eye and 30 mmHg in the left eye and no surgery was indicated. At the time of the consultation, with maximum medication, we found the following data:

A trabeculectomy was done in both eyes, which has regulated the ocular pressure to date. The pathological anatomy of the trabeculectomy piece is very interesting. We will make a short summary of the following clinical history because we have found the same anatomopathological sign (Figs. 20.52, 20.53, 20.54).

Clinical History No. 16

This 46-year-old male presented bilateral pigmentary glaucoma with typical angle. He has two children, a 19-year-old boy and a girl aged 17, both with very pronounced pigmentary glaucoma and goniodysgenesis.

He was operated with bilateral trabeculectomy and his ocular pressure and pressure curve were regulated until 2000. The visual field defects did not progress. We also found a thrombus of pigment within a collector (Figs. 20.55, 20.56).

Clinical History No. 17

This 54-year-old male consulted for the first time in 1974. He was diagnosed in the right eye with retinal detachment operated 15 years earlier, which had not recovered. At the time of the consultation, he had 0 vision and atrophic detached retina. In the left eye, he had pigmentary glaucoma. His mother and brother were myopic, between −3 and −8 D, and his son had bilateral pigmentary glaucoma. He also had esophageal diverticulitis and cervical arthrosis.

As regards his ocular history, he had been operated 16 years before for retinal detachment in the right eye, but he did not recover his sight. Fourteen years before consultation, when he was 40, glaucoma was diagnosed in the left eye and it was treated with pilocarpine 2%, four times daily. Another ophthalmologist changed his treatment to beta-blockers 0.5%, twice daily and pilocarpine 1%, four times a day.

This patient presents pigmentary glaucoma, a disease expressed in a retinal detachment of the right eye. He had lost his sight after surgery. Two years later, glaucoma was discovered and treated erroneously with pilocarpine, which is contraindicated in pigmentary glaucoma for the possibility of provoking retinal detachment. His visual field stopped developing after the surgery, which regulated the daily pressure curve and he now maintains his visual field and his sight.

Clinical History No. 18

This 26-year-old male is the son of the preceding case history.

However, there is a slight diffuse depression, as can be seen in the Bebie cumulative defect curve. With medical therapy, the daily pressure curve was completely regulated and in 7 years of follow-up there has been no visual field defects.

Examining the peripheral retina showed five tears in the right eye, two at 12 o’ clock, one at 3 o’ clock, one at 4 o’ clock, and one at 5 o’ clock, and one in the left eye at 12 o’ clock, which were photocoagulated.

Etiopathogeny

Current ideas on the etiopathogeny of this form of -glaucoma can be summed up as follows:

Sugar thinks that the main factor producing the increase in ocular pressure is pigment dispersion. The

-congenital factor is secondary.

Nordmann considers that it is above all a congenital anomaly of the iris pigmentary epithelium, which leads to dispersion of the pigment that can obstruct drainage of the aqueous humor. This first anomaly is often associated with a second one, situated in the chamber angle. Briefly, like us, he believes that it is a

-late congenital glaucoma [66].

Malbrán describes eight cases in his work. All of these presented an absence of the ciliary body band hidden by the persistence of pathological mesoder-

-mal remnants and peripheral atrophy of the iris. Based on the cases I have presented in this chapter, I consider that this is late-onset congenital glaucoma (an anomaly in the chamber angle, myopia, dominant heredity). Secondarily, there is a pigment dispersion, which appears late in the progression of the disease.

The cases in which we have shown a great accumulation of pigment in the trabecular meshwork, hiding it completely, as well as the thrombi of pigment in the collectors, suggest that the causes producing the glaucoma are the congenital alteration of the angle and the pigment.

The pigmentary manifestation is a phenomenon that sets in later over the congenital component and, in most cases, demonstrates the disease. Even though this pigmentary manifestation is determined by heredity, it appears later, and its clinical manifestations, such as the pigmented ring of the trabecular meshwork at the Schlemm canal, the Krukenberg spindle, the atrophy of the epithelial layer of the iris, the pigmentation of the zonula, and the pigment rings on the posterior face of the lens are acquired manifestations that occur as the disease develops.

In homage to Jorge Malbrán, who was the first to discover that pigmentary glaucoma was a late congenital glaucoma, we will translate one of the most important parts of his work. Malbrán says:

Pathological mesodermal remnants are seen in nearly all cases of congenital glaucoma. François and Kluyskens take particular note of this and mention other authors observations in agreement. The uveal trabecular meshwork is abnormally developed. The embryonic mesodermal tissue, f ibrous or reticular in structure, cottony and silky in appearance, presents variable pigmentation. Galenga and Matteucci’s interpretation is little different from that of François, Kluysken and Barkan, recognizing that the peculiarities found at uveal trabecular meshwork level determine a high insertion of the iris with a tissue with the appearance and features of the iris itself, i.e. a completely undifferentiated tissue that, as we said earlier, gives the name of abnormally developed mesodermal tissue. [15]