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

Pseudocongenital Cortisone Glaucoma

Cortisone glaucoma in the newborn child in the first 2 years of age is relatively frequent.

Congenital obstruction of the lacrimal ducts, which is generally resolved with a needle to inject physiological solution in the upper and lower tear points and, more rarely, probing the tear duct with a Bangerter probe, does not require corticoids. Unfortunately, clinicians, family doctors, and pediatricians prescribe cortisone abundantly, with dexamethasone eye drops several times a day for months, and unleash an iatrogenic glaucoma known as pseudocongenital cortisone glaucoma. The same happens with other newborn children with uveitis, conjunctivitis, blepharitis, dacryocystitis, with equally disastrous results. It should be added that in some cases they coincide with a congenital glaucoma, with lacrimal duct obstruction and, in this case, the ophthalmologist must resolve both pathologies. Therefore, it is essential that any clinician or pediatrician treating a newborn with tearing or photophobia should immediately refer them to an ophthalmologist.

Symptoms

Signs and symptoms similar to those of congenital glaucoma appear at once: increased ocular pressure, increased axial length, deeper anterior chamber, and thinner crystalline lens. Sometimes there is an increase in corneal diameters in advanced cases with long-term treatment of 8 months or more and breakages of the Descemet membrane and endothelium. Glaucoma cupping of the optic disc can also be seen as in congenital glaucomas. This cortisone glaucoma is distinguished from pure congenital glaucoma by the chamber angle, which is absolutely normal.

The clinical picture is sometimes solved by suspending the corticoids, but this is rare. Generally it is necessary to operate, and this condition responds remarkably well to trabeculotomy.

If the chamber angle presents the elements of congenital glaucoma (type 1 or type 2 angle) with pathological mesodermal remnants, this is not a case of pseudocongenital cortisone glaucoma but of a congenital glaucoma in which corticoids have contributed to bringing it out or have worsened it. François published a comment on a 1973 study by Bietti questioning whether it was possible for one of the cases described by the latter to develop pseudocongenital cortisone

glaucoma with an increase in axial length. We are inclined to favor Bietti’s description, because in our clinical experience we have seen that the axial length continues increasing from birth to 4.5 years of age.

There are two fundamental studies on this topic in the literature: Gnad and Martenet’s, of Zurich, in 1973 [29] and that of Calixto, presented in the 95th meeting of the French Society of Ophthalmology, in 1984. The literature also contains other interesting studies on this subject: Alfano [30], Bietti [31], Mascaro et al. [32], Calixto and Cronemberg [33], and Kass et al. [34].

The following clinical history is an example of pseudocongenital cortisone glaucoma:

Clinical History No. 2

A 6-month-old male presented with conjunctivitis with photophobia and epiphora in the left eye at the age of 6 days. The pediatrician, without consulting the ophthalmologist, gave him hydrocortisone in eye drops, 0.5 g four times a day for 1 month and the following 3 months dexamethasone 0.1 g four times a day, also in eye drops. At 4 months, the mother noticed that the left eye was larger than the right eye and took him to the ophthalmologist, who referred him to the university hospital.

See Fig. 19.2 for echometry. Follow-up continued until the child reached 4 years of age. Ocular pressure was controlled at normal values and the echography showed good progress.

It is remarkable that in this case the examination of the fundus shows the optic disc with a cupping of 5/6 (Recas classification ) in the left eye as opposed to the normal cup of the other eye of 1/6. The visual field cannot be determined yet, and acuity is 20/30 in the unoperated eye and 20/40 in the operated eye.

Clinical History No. 3

A 6-month-old male presented with pseudocongenital cortisone glaucoma with a completely normal chamber angle. Trabeculotomy was done in the left eye.

For echometry results, see Fig. 19.3.

Clinical History No. 4

A 16-month-old male was treated for conjunctivitis and dacryocystitis of the newborn at the age of 10 months with cortisone drops, three or more times per day, and then for 6 months, once a day. The ophthalmologist performed a lacrimal probe. Photophobia and tearing persisted.

The child recovered in 1 week, the symptoms disappeared, and ocular pressure was regulated between 10 and 14 mmHg. Since age 7, a diurnal pressure curve has been made annually, which always shows normal values both in the mean and in the variability. The progression of the axial length since surgery is shown in Fig. 19.4a.

At age 11 (1991), the condition of the optic nerve can be seen in Fig. 19.4b and 19.4c. Remember that

the right eye was slightly larger than the left eye and had suffered more, as it presented tears in the paracentral endothelium and Descemet membrane. This led to a different surgical therapy since, while in the right eye only trabeculotomy was performed, in the left eye combined surgery was undertaken: trabeculotomy and trabeculectomy, despite the good visual acuity in both eyes (right eye, visual acuity, 20/200 with cylinder −0.50 at 10° and left eye, 20/200 with cyl. −0.50 at 170°). The optic disc cupping is asymmetric, barely 1/3 in the right eye and slightly more than 4/6 in the left eye.

In addition, the good acuity with ocular dimensions at 11 years of age, above normal, indicates the emmetropization that takes place in eyes with glaucoma, which distends the ocular globe in the first months of life.

In summary, it is remarkable that with an almost equal mean defect in both eyes, the optic disc of the left eye, which is longer, showed much more cupping than the right eye’s disc, the axial length was greater and had tears in the Descemet membrane, i.e., the visual field in this case is not an index to know which eye is the most damaged, whereas the optic disc, the Descemet tears, and the axial length can provide this indication. It should also be emphasized that both eyes with a myopia below 1 diopter (D) had emmetropization.

In Fig. 19.5, the visual fields made with Octopus 2000 in 1991, show a greater mean defect (MD) in the left eye (4 dB).

The pathological anatomy also confirmed the diagnosis (Fig. 19.6).

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Late Congenital Glaucoma: Goniodysgenesis

The diagnosis of late congenital glaucoma is basically made through the chamber angle. These are children over 6 years of age, young people, or adults up to 45 years of age, presenting persistence of pathologic mesodermal tissue reaching at least to the spur, the trabecular meshwork, the height of the Schlemm canal, or even further up to the Schwalbe line. The most outstanding sign in gonioscopy is the absence of the ciliary body band, which may be total or partial. This is a fetal alteration of the development of the iridocorneal angle called goniodysgenesis and it is accompanied by a halt in the development of the superficial mesodermal layer of the iris that enables the radial vascular and avascular columns of the deep iris to be seen, and at the avascular columns, the black triangles can be seen corresponding to the posterior pigmentary epithelium of the iris. In general, peripheral atrophy of the superficial layer of the iris is visible.

At other times, there is mesodermal tissue, but its appearance is modified: it looks like a grayish white band. This led Busacca to name this metaplasia of the mesodermal tissue. Even though he includes it in child congenital glaucoma, we have rarely seen it there. Moreover, Busacca and Carvalho [1] describe metaplasia of the mesodermal tissue in 28 cases, 22 of whom were adults and only six were children.

Heredity is dominant in late congenital glaucomas, contrary to what occurs in pure congenital cases in

newborns. The most important difference with pure congenital cases in newborns is that in these cases the axial length is increased, and in late congenital glaucomas the axial length of the eye is normal.

Clinical experience shows that in addition to the cases we have described, in which the dimensions of the anterior segment are normal with an open angle, there are others that also have late onset but present enlargement of the cornea and at times of the eye as a whole. Heredity is dominant in these as well, but hypertension must surely have been present since childhood without giving rise to noticeable signs, because functionally the eye compensates in part for the defect [2]. Patients come late to consultation, with a transparent cornea, good vision, very high ocular pressure, and a highly damaged visual field.

Kniestedt et al.’s article [3] is useful to consult. We also advise reading the book Goniodysgenesis by Jerndal et al. [4], which is the most complete work on this topic, as well as the work of Boles Carenini [5].

It is very important to be able to recognize this clinical form of glaucoma, since, if the ocular pressure is not regulated, as occurs in some cases, surgery will be necessary, and special care will have to be taken in the location of the incision and in the transillumination (the Minsky maneuver), to avoid severe complications that are a consequence of the malformation of the chamber angle (Table 20.1).

Gloor’s team [3] found goniodysgenesis in 48%–77% of adults, in open angle glaucoma.

Figure 20.1a illustrates the chamber angle, where a new element, the pathological mesodermal remnants (1), appears, located between the outer and inner wall of the angle, in the recess of the angle, which are chestnut or brown in color.

In Fig. 20.1b, a goniophotograph of the angle shows

(1) pathological mesodermal remnants, (2) the last circular fold of the iris, and (3) the Schlemm canal. It is

quite clear that the mesodermal remnants completely hide the ciliary body band; therefore, goniodysgenesis is defined as a lack of visibility of the ciliary body band.

Figure 20.2a shows a diagram and 20.2b a goniophotograph of a normal angle. The iris processes can be seen, reaching the spur and leaving the ciliary body band perfectly visible behind in a bluish color. Figure 20.2c illustrates goniodysgenesis: the pathological mesodermal remnants can be seen hiding the entire ciliary body band.

The iris root is inserted, as can be seen in the upper part of Fig. 20.3, always in the internal face of the ciliary body, in hyperopic patients in the anterior part of the face, in emmetropic patients in the middle, and in myopics in the rear part, but always in the internal face of the ciliary body. The pathological congenital remnants occupy the recess of the angle in front of the iris root.

Even in type II congenital glaucomas, mistakenly called high iris insertion, which should be called apparent high insertion of the iris, since, as can be seen

in Fig. 20.3b, the root adheres to the lower wall of the angle, and when it is seen with the gonioscope, it seems to insert itself at the level of the Schwalbe line.

Kluyskens [6] wrote: “Most writers, even the most careful ones, observing the angle in cases of congenital glaucoma, have the impression that the iris is inserted higher than normal, but in fact it is not a case of an insertion abnormally high from the base of the iris, which is always in its normal position, but of the presence in the recess of the angle of an abnormal tissue that gives this false appearance.”