AMERICAN JOURNAL OF OPHTHALMOLOGY APRIL, 1952
VOLUME 35
NUMBER 4
SURGICAL TREATMENT OF CONGENITAL GLAUCOMA* A
NEW
CONTACT L E N S FOR
MANUEL
URIBE
G0NIOT0MY
TRONCOSO,
New
M.D.
York
In 1942 Otto Barkan 1-5 published an ac count of 17 successful operations for con genital glaucoma by the method of goniotomy or incision of the angle of the anterior chamber. The results in all these cases were very encouraging. Pressure was normalized in 16 of the 17 eyes and vision was main tained in 14. All cases were those of children with an average age of three months at the time of the operation (from eight days to two years). The good results were main tained from one to five years in these cases. As all previous experience with other methods in the surgical treatment of hydrophthalmia had been short of disastrous, Dr. Barkan's communication was received with great interest and attention. His results iwere confirmed by several authors: Allen,6 Rocha,7 Scheie,8 McKinney,9 Kluyskens,10 McArevey,11 and others. MECHANISM OF HYPERTENSION IN HYDROPHTHALMIA
Since the pathologic examination of eyes affected with this disease made by Horner (1880) and later by Cross (1896), Seefelder (1906), and others, it has been estab lished that the angle of the anterior chamber is obstructed by mesodermal tissues which failed to be absorbed at the proper time be fore the birth of the child. The presence of * From the Department of Ophthalmology, Col lege of Physicians and Surgeons, Columbia Univer sity, and the Institute of Ophthalmology of the Presbyterian Hospital.
Schlemm's canal has been variously de scribed as existing behind the obstruction or being entirely absent (Cross, 1896). It has been ascertained, however, that Schlemm's canal is present in the earlier stages of the disease in about 75 percent of the cases (An derson 12 ). Later it becomes clogged or dis placed and disappears in more than half of the specimens taken from children over two and one-half years of age. Anderson thinks that Schlemm's canal closes in later stages due to the distention of the eyeball by the increased intraocular pres sure. When this occurs, the eye is perma nently damaged and no operative treatment can restore the normal level of the intra ocular pressure. Barkan insists on the neces sity of performing the operation immediately after the first signs of hydrophthalmia are discovered. The good results obtained by this author undoubtedly can be ascribed to the section or rupture of the mesoblastic tissues filling the angle and covering Schlemm's canal, and the reestablishment of aqueous filtration through the trabeculum uncovered by the operation. According to Barkan, goniotomy, when performed early, has these advantages : (1) Of immediately reducing the intraocular pressure; (2) reducing the size of the eye ball before the distention produces perma nent pathologic changes and obstruction of Schlemm's canal; (3) restoration of vision and avoiding of blindness.
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MANUEL URIBE TRONCOSO
Before the advent of the gonioscope only pathologic specimens of hydrophthalmic eyes could detect the presence of mesoblastic tis sue and establish the obstruction of the angle. Later, gonioscopy, by direct examina tion of the angle, showed conclusively the extent and character of the embryonic tis sues covering the angle and the changes pro duced in its normal appearance.* With this method Troncoso,13 Sugar,14 and other observers found that the iris root appeared to have been drawn up and was inserted more or less higher, nearer the Schwalbe line at the end of the Descemet's membrane. The normal sinus of the angle had entirely disappeared. An attentive ob servation in the early stages shows that there are numerous fibers of an abnormal meshwork tissue which cover the trabeculum and change to a slanting wall the normal sinus of the angle. Sugar found the iris tissue rela tively transparent as he could see through it the reflection of the pigment epithelium from the ciliary body to the iris. Although mesoblastic tissue in the angle is pigmented and resembles, in places, a normal rarefied iris, it is important to understand that the root of the iris has remained in place and that the persistent meshwork fills the space between the iris and the scleral wall. That this is the case is proven, besides, by the results of goniotomy operations. When the aberrant meshwork is sectioned there is no hemorrhage but, if the iris tissue below is cut out, a large hemorrhage usually en sues, both from the iris vessels and, in some cases, from new vessels which are commonly present at the surface of the membrane. GONIOTOMY OPERATION
De Vincentis, in 1892, described, with the name of "incision of the angle of the ante♦With the slitlamp microscope, Juler was also able to see the meshwork stretching from the angle and back of the cornea to the iris and pointed to the deficiency of the iris stroma at the corresponding sector.
rior chamber," an operation consisting of the section by a narrow knife of the tissues beyond the iris root and over the scleral wall. He thought this incision opened Schlemm's canal, and reestablished the outlets from the angle in cases of primary glaucoma. Otto Barkan, in 1936, revived this opera tion and greatly improved its technique by performing the section not in a blind way as De Vincentis had done, but under direct gonioscopic observation through a special contact glass which he devised. This contact glass had a flat shape and permitted examination of the angle through a binocular loupe worn by the surgeon, which magnified the images from four to five times. Barkan also claimed that it was possible to direct the point of the knife to Schlemm's canal and open it into the anterior chamber to relieve hypertension and reestablish the aqueous outflow. Later he used goniotomy for operations on hydrophthalmic eyes. Unfortunately, this contact lens did not stand up to expectations as immediately the surgeon raised it on one side to introduce the knife into the cornea, the liquid under the glass flowed out and air bubbles pene trated, obliterating the angle image. Besides, the presence of the glass prevents a good fixation of the eyeball by the surgeon, inas much as the glass conceals the limbus on the opposite side from where the knife is introduced. To avoid this difficulty, Barkan advises the fixation of the globe with two forceps held by an assistant, above and below the globe, while the surgeon punctures the cor nea. Barkan, and others, have lately discarded this contact glass for the operation and ad vises performing goniotomy in a blind way, keeping the knife parallel to the iris surface. Barkan also discontinued the operation for primary glaucoma, confining it to cases of hydrophthalmus in which, as mentioned be fore, it has produced excellent results. Ellis15 has modified Barkan's lens, reduc ing its size and changing its form to an egg-
SURGERY FOR CONGENITAL GLAUCOMA shaped area. He cuts the edges of this area vertically down so that the glass is applied higher upon the corneal surface, leaving, on either side, an uncovered space through which the knife can be inserted into the cornea. The glass is provided with a central rod which the surgeon presses down upon the globe to insure stability. Later Ellis tried to obliterate entirely the span between glass and cornea, the "water chamber," as he calls it, which he did not find necessary for clearness or magnifica tion of the image. In this way the contact lens lays directly over the surface of the cornea which has • been flooded with saline solution. In Ellis's three cases there were never "abrasions or cloudiness of the cor nea," but he mentions that some striations appeared over the membrane, induced by the flat curvature of the glass. The reduced size of the glass allows only the examination of one third or one fourth of the angle cir cumference. Ellis describes three cases of goniotomy in two of which hemorrhages were produced in the anterior chamber during the operation. This glass also has the disadvantage that an assistant is necessary to fix the eyeball with forceps during the intervention. MESORRHEXIS
In order to avoid the risk of wounding the iris and producing a large hemorrhage, several surgeons, instead of cutting the mesoblastic tissues with a knife, have tried to tear out the insertion of the meshwork from the scleral wall with a spatula introduced into the chamber, with which the iris is forcibly de pressed downward. Good results have been reported with this method in early cases of hydrophthalmia. A I R INJECTION
In 1945, Hughes and Cole advised filling the anterior chamber with air after with drawing the aqueous, thus permitting ob servation of the angle without using a con
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tact glass in operations for hydrophthalmia. They devised a new knife to which two syringes were connected—one for withdraw ing the aqueous and the other for injecting air. This method has not been used exten sively, as the image of the angle from an airfilled chamber is not very clear. Besides, the technique is not easy and the operation may be dangerous since, in some cases, the air injection has induced a certain amount of hypertension. SURGICAL CONTACT GLASS
To avoid the uncertainty and danger of making a blind incision of the tissues of the angle, I have devised a new contact glass which permits the introduction of the knife into the anterior chamber without disturbing the lens fluid, and with which the image of the angle, all around, is kept clear throughout the whole operation. This new glass has the advantage, too, of permitting the surgeon to fix the eyeball by himself, with forceps, grasping the limbus at the opposite side of the corneal puncture. The surgical contact glass is the same goniolens (both the old and the tubular models) used for gonioscopic examination on which two windows have been made in the glass on opposite sides and reaching the edge of the glass (fig. 1-A and B, m and m'). These windows are of different sizes and are covered with a rubberlike plastic mate rial. Through the smaller, round window (fig. 1-m) the knife can be introduced easily into the anterior chamber, perforating the membrane, and then puncturing the cornea. There is not any loss of fluid and no air bubbles can be introduced. The tip of the knife is seen perfectly and may be followed through the glass to any part of the angle which has to be sectioned. The membrane is elastic and after the knife is withdrawn it seals by itself. The larger and narrower window on the opposite side of the glass is also covered with a membrane (fig. 1-m'). This can be pushed
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inward by the tip of the forceps to permit a good hold on the globe by the surgeon ma nipulating the instrument himself (fig. 1-B, Fx). There are two kinds of surgical goniolenses, the tubular and the ordinary diag nostic goniolens. F o r the tubular model (fig. 1-A and B ) , the tube has been displaced backward over the convex surface so as to give the observer a larger field of the oppo-
the eyeball. After instilling a few drops of cocaine, the surgical contact lens, previously washed with an antiseptic, cleaned with a wetting agent (zephiran, 1:1,000), and final ly rinsed in sterile water, is placed over the eye. In the tubular model, the cavity is filled through the tube with sterile water until all the air bubbles are expelled. T h e knife needle should have a sickle shape (preferably, Zieg-
B Fig. 1 (Troncoso). (A) Surgical goniolens provided with a tube which is inserted laterally to give a larger view of the angle. The two windows (m and m') on the opposite sides of the glass are covered with rubberlike membranes. (B) Schematic section showing the contact glass (C.G.) in place on the eye for operation. The knife-needle (K) lias penetrated through the membrane (m) and the cornea (C) and divided the mesoblastic tissue (Ms2) leaving the angle free at (Ms'). The curved arrow shows the direction of the knife movement. On the opposite side of the angle the fixation forceps (Fx) pushes the membrane inward and grasps the conjunctiva at the limbus to steady the eyeball. site side of the angle. In the ordinary gonio lens (fig. 2-C and D ) , the tube is missing, but the two windows on either side are cov ered with the same rubberlike elastic mem brane. This glass is more difficult to apply but once in place, after removing the air bubbles, it stays over the cornea better on account of the negative pressure it de velops.* T E C H N I Q U E OF GONIOTOMY W I T H CONTACT L E N S
T h e operation should be performed under general anesthesia in small children since its execution demands complete immobility of
ler's knife model) and its shank should be progressively thicker so as to obliterate com pletely the corneal wound. T h e small window of the glass should be turned toward the temporal side of the eye. It is advisable that at least two thirds of the circumference of the chamber should be sec tioned. However, the glass can be rotated so that the window faces any other side of the limbus when the surgeon wants to make the section in any other direction of the angle. With his left hand holding the
fixation
* The surgical goniolens is made by A. Oriani, 17 South Grove Street, Freeport, L.I., New York.
SURGERY FOR CONGENITAL GLAUCOMA forceps, the surgeon depresses the mem brane and grasps the corneal limbus. Before the puncture is made, an assistant should press the tube of the glass downward to pre vent its displacement with the forceps. H e should also see that the tube is filled with ad ditional water if any bubbles tend to pene trate into the glass. T h e older goniolens, without the tube, also
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membrane and the cornea, should be pushed straight, keeping it parallel to the iris sur face. W h e n it reaches the angle, the tip is directed into the aberrant meshwork which is punctured and then cut out to the desired extent around the circumference of the limbus, using semicircular movements of the handle (fig. 1-B). T h e section should be made about one mm. below the Schwalbe
C
D
Fig. 2 (Troncoso). (C) Surgical goniolens without tube, provided with two windows which are covered with rubberlike membranes. (D) Frontal aspect showing the two membranes filling the apertures on the opposite sides of the contact glass. provided with two windows and membranes (fig. 2 ) , may also be used to advantage, in asmuch as it has a strong negative pressure which helps to keep the glass over the globe during the operation and does not have to be pressed down by an assistant. T h r o u g h the contact lens, the angle is illuminated by the strong light of an electric torch and the surgeon, using a binocular loupe, examines the angle all around to de termine the position of the Schwalbe line which is the anterior border line of the angle. This line is sometimes pigmented and easy to recognize, but other times it is marked only by the difference in color between the mesodermic tissues and the cornea. The tip of the knife, after perforating the
line, always avoiding directing the downward toward the iris.
point
MESORRHEXIS
W h e n the observer finds, through gonioscopic examination, that several new vessels have developed in the iris surface near the angle, it seems advisable to avoid the risk of hemorrhage by tearing down the aberrant mesoblastic tissue instead of dividing it with the knife. The operation is conducted as described before but, when the knife needle reaches the angle, it is turned around and the blunt side of the edge is applied and pressed down forcibly, scraping down the whole mesoblast. This usually gives way and is torn down
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from its insertion to the inner sclera.
OTHER OPERATIONS IN THE ANGLE
operation described by Scheie, which con sists of dividing the limbus immediately above the Schwalbe line from the inside to the outside, could be more easily performed with the contact lens than in a blind way as it is done now. I think that, in the future, other operations, such as reverse cyclodialysis, could be performed from inside, cutting the scleral spur through the ciliary-body band with a k n i f e a n d s e p a r a t i n g t h e t i s s u e s from the
The surgical contact glass may be applied also to the performance of other operations on the angle in addition to goniotomy. The
sclera until the suprachoroidal space is reached. 500 West End Avenue (24).
RELAPSES
Sometimes goniotomy fails the first time and the intraocular pressure remains high, In these cases a second or third operation should be performed at convenient timeintervals, until the permanent reduction of hypertension is obtained.
REFERENCES
1. Barkan, O.: Operation for congenital glaucoma. Am. J. Ophth., 25 :552 (May) 1942. 2. : Goniotomy for congenital glaucoma. J.A.M.A., 133:526-533 (Feb.) 1947. 3. : Cyclodialysis, multiple or single, with air injection: An operative technique for chronic glaucoma. California Med., 67:78-83 (Aug.) 1947. 4. : Technic of goniotomy for congenital glaucoma. Tr. Am. Acad. Ophth., 52:210-226 (Jan.Feb.) 1948. 5. : Goniotomy for relief of congenital glaucoma. Brit. J. Ophth., 32:701-728 (Sept.) 1948. 6. Allen, T. D.: Bilateral congenital glaucoma and cataract: Goniotomy and needling: Case. Tr. Am. Ophth. Soc, 42:308-315, 1944; Am. J. Ophth., 28:388-392 (Apr.) 1945. 7. Rocha, J. M.: Results of surgical therapy. Arq. Instituto Penido Burnier, 6:367-380 (July) 1942. 8. Scheie, H. G.: Goniotomy in treatment of congenital glaucoma. Arch, ophth., 42:266-283 (Sept.) 1949; Tr. Am. Ophth. Soc, 47 :115-137, 1949. 9. McKinney, J. W.: Goniotomy for congenital glaucoma. J. Tennessee M.A., 42:277-283 (Aug.) 1949. 10. Kluyskens, J.: Congenital glaucoma. Rep. Belgian Opht. Soc, Feb., 1950. 11. McArevey, J. B.: Treatment of congenital glaucoma. Brit. J. Ophth., 34:568-573 (Sept.) 1950. 12. Anderson, J. R.: Hydrophthalmia or Congenital Glaucoma. London, Cambridge Univ. Press, 1939. 13. Troncoso, M. U.: A Treatise on Gonioscopy. Philadelphia, Davis, 1939. 14. Sugar, H. S.: Practical applications of gonioscopy to surgery. Am. J. Ophth., 25 :663-671 (June) 1942. 15. Ellis, O. H.: New goniotomy lens. Am. J. Ophth., 27 :1258-1265 (Nov.) 1944.
OPHTHALMIC MINIATURE
The disease termed glaucoma consists essentially in an alteration of the component parts of the vitreous humor, accompanied by derangement of structure of the hyaloid membrane, of the retina, and tunica choroidea, the vessels of which are always more or less in a varicose state. Guthrie, Lecture on the Operative Surgery of the Eye, 1830,