There are a number of surgical options available for treatment of the pediatric glaucomas including: goniotomy, trabeculotomy, trabeculectomy, trabeculectomy-otomy, tube implants, and cyclodestructive procedures. These should always be used with the understanding that a child is being treated, not an adult. The least destructive and minimally distorting surgical procedures should be done initially. This will minimize the potential for complications while making available other needed surgical procedures to be performed later for the child suffering from this “lifelong disease”. Just because a surgeon is able to perform a given procedure should not be the main criterion for choosing it over one that could be more beneficial in a given situation. In such a case, it would be beneficial for the most effective procedure to be done by another surgeon. One of the compelling reasons to establish 1, 2, or more recognized pediatric glaucoma surgeons in a center is that they are more likely to have a series of surgical options at their disposal and not just one or two.
Not many surgeons perform goniotomy, but with proper training, it is relatively simple to do under good conditions. It is the least invasive, most effective procedure for most infantile glaucomas. Goniotomy has a relatively high degree of success and few complications. When complications do occur they include: cyclodialysis, iridodialysis, synechiae, and in rare cases when the blade disrupts the lens capsule, cataract.
Goniotomy is comparable to trabeculotomy in success rate, although trabeculotomy has the disadvantage of causing much more tissue distortion and scarring. Goniotomy has the advantage of being a “low technology” procedure requiring just a few relatively low cost instruments and for that reason can be readily introduced to a community with limited resources. Unfortunately, some cases of pediatric glaucoma, especially those that present in an advanced state, are not amenable to goniotomy because this technique requires a relatively clear cornea for safe and effective application. This downside may be particularly problematic in developing countries where access to care is more difficult and patients frequently present later and with more advanced stages of disease.
A goniotomy - direct incision; B closer view of the angle during surgery
1. Although commonly done with a microscope, goniotomy may be done with 2-3x loupes and a co-axial headlight. If done with a microscope, the head of the scope must be able to tilt to 30-45 degrees, although the angle of maximum advantage between the child’s eye and the microscope may be attained by simply tilting the patient’s head away from the surgeon.
A microscope; B loupes
2. An operating goniolens for goniotomy (e.g. Swan-Jacob with handle (Ocular Instruments Item # OSJAG) or Barkan operating lens.)
3. A 25-gauge needle on a syringe (0.5-2.0 cc) – with viscoelastic, if possible. Ringer’s lactate, Balanced Salt SolutionR (BSS) or hydroxymethylcellulose are alternatives.
4. Two locking fixation forceps of your choice (e.g. Elschnig-O’Connor) to fixate the eye (non-locking forceps can be used by the assistant, but they make it more difficult to sustain constant pressure for longer times.)
Goniotomy can be done immediately after an EUA or as a specifically scheduled procedure. But for safety and a better view of the angle, it should be performed with a non-dilated pupil. Bilateral surgery may be done in one sitting, but a complete re-scrub of the patient and surgical team and re-draping of the patient is required. In addition, a second set of instruments should be used or the first set of instruments should be resterilized. These steps are required to decrease the possibility of cross-contamination with resulting endophthalmitis that could lead, in the worst case, to bilateral blindness.
The steps of goniotomy are shown in A-D
The nasal angle is normally done first simply because it is technically easier. The surgeon sits temporal to the eye and the assistant sits opposite, on the other side of the patient. The patient’s head is rotated away from the surgeon making it easier to visualize the nasal angle. Now, the goniolens is placed on the eye to ensure that the nasal angle can be viewed. The inferior rectus is then grasped at its insertion with one forcep and the eye depressed to allow easier access to the superior rectus, which is now fixed with the second forceps. The surgeon usually places these forceps. A speculum is not recommended because it can interfere with the forceps and other instruments. Usually, sufficient exposure is obtained without one. The forceps are handed over to the assistant who handles the forceps carefully so as to not obstruct the surgeon’s view.. It is important to have the assistant practice rotating the eye, while keeping the iris plane steady and parallel to the planned entry and path of the goniotomy blade across the anterior chamber. This will make it easier for the surgeon who has a limited depth of focus and very little room between the cornea and iris.
With the lens on the cornea, the angle is checked for visibility. If the angle is not seen well, the corneal epithelium may be scraped off using a blade or rubbed off after applying sterile glycerin. The epithelium just anterior to the limbus should be left undisturbed and in cases of aniridia, the epithelium should never be scraped off. The syringe-needle unit is grasped like a pen and is oriented to make it parallel to the iris. With the angle in view and the eye securely fixated, the 25-gauge needle, bevel-up on the air-free syringe, enters the anterior chamber through peripheral clear cornea at the temporal side (3 o’clock right eye and 9 o’clock left eye) under direct vision. Once the needle is 1/4 - 1/3 across the AC, the lens is moved slightly toward the surgeon making it possible for him / her to follow the tip of the needle into the angle under direct vision. The angle structures to identify are blood vessels in the angle that loop upward and then backward to the iris root stroma. A cut in the filmy tissue just anterior to the loop of the blood vessels is ideal positioning. If blood vessels are not seen, the cut is made into the filmy tissue just anterior to the iris root stroma. Short sweeps of the needle in one direction or the other will commonly produce a white line on the sclera as the iris drops backward. The 25-gauge needle should be inserted no further than one quarter of the bevel length. At no time should the surgeon feel resistance on the needle as the cut is made. Technical evidence of a successful goniotomy is visual and not tactile. A gritty feeling at the needle tip in primary glaucoma indicates that the tip of the needle is too deep and may result in bleeding. In Axenfeld-Rieger, and in uveitis some grittiness may be felt as the needle knife is cutting tissue in the angle. The goniolens technique will enable about 4 clock hours of goniotomy (2 to the right and 2 to the left) before the eye is rotated one way, and then the other, by the assistant. Access to additional clock hours in the angle is accomplished with a smooth and slow rotation of the eye by the assistant – maintaining proper iris plane stability at all times. While the assistant is rotating the eye, the needle should be removed from the angle, but remain over the nasal iris.
When the procedure is completed, withdrawal of the needle is done swiftly to avoid any touch of the iris or crystalline lens. Viscoelastic or other fluid in the syringe may be injected prior to needle withdrawal, or it can be done if the AC shallows during surgery. Fluid may also be inserted after the cutting instrument is removed from the eye. The AC usually shallows or becomes totally flat after goniotomy, and then spontaneously reforms in about 15-30 minutes. This should be prevented or minimized when doing surgery on a Sturge-Weber patient in order to prevent bleeding. Steroid and antibiotic ointment can be instilled at the conclusion of surgery and the eye is patched and a shield is placed.
The patient should be seen on the first postoperative day and then every few days for a week or two according to availability. Post-op care is routinely with steroids and antibiotic drops, but if no inflammation is encountered steroids may be omitted. Drops rather than ointment are easier to instill in the awake infant. Special care should be exercised to avoid trauma to the eye during the immediate post-operative period. Patients can demonstrate a variety of responses in the first few days to weeks. One to two months after surgery an EUA may be done to check for results including: clearing of the cornea, stable or reduced cupping of the optic nerve, and reduction in the IOP. In small children, reversal of the cupping is often seen. A second (temporal or inferior) goniotomy may be performed later to further improve the eye pressure. This is done if any of the criteria mentioned earlier indicating need for surgery are noted, showing no or little success with the first surgery or if there are signs of progression of the disease.
The white line indicates successful goniotomy with the iris plane falling back exposing the angle.
Goniotomy is used for primary infantile glaucoma but may also be used for late-onset infantile glaucoma, congenital rubella syndrome, neurofibromatosis, Sturge-Weber syndrome, uveitic glaucoma, aphakic glaucoma with goniodysgenesis, and some juvenile glaucomas with varying degrees of success. The success rate of goniotomy is remarkable but, like all other medical and surgical options for this disease, results may not be life-long. Success is highest with infantile glaucoma treated between 3 months and 1 year and is between 70-90% effective after 1-2 procedures. In neurofibromatosis and newborn primary infantile glaucoma the success rate is poor.
For more details and a modality for learning goniotomy see: Patel, HI, Levin AV: Developing a model system for teaching goniotomy. Ophthalmology 2005; 112(6): 968-973.
This procedure is the equivalent of goniotomy in success rate, but it differs from goniotomy in that it destroys or alters more tissue than a goniotomy. However, trabeculotomy does offer an alternative to goniotomy in the case of a cloudy cornea or where the surgeon is uncomfortable in performing goniotomy.
A simple set of instruments and supplies are required as for trabeculectomy. These include:
- A sharp, pointed knife to cut down on the canal of Schlemm
- A rounded knife that might be used in trabeculectomy e.g. #15 or better
- A set of trabeculotomes (right and left)
Right and left trabeculotomes
The conjunctiva is opened at the limbus employing a fornix-based flap of conjunctiva. Before this, fixation is achieved using a superior rectus bridle or peripheral corneal suture. An inferonasal or inferotemporal self-sealing paracentesis is recommended to deal with potential anterior chamber (AC) collapse that could occur part way through the procedure. Dissection of Tenon’s and episclera should be carried out to expose bare sclera. Assessment of the bare sclera at the limbus will help in finding the Canal of Schlemm (CS), but this is often difficult to identify using the location of blood in the CS. Aqueous veins, with half blood and half clear aqueous may suggest the CS location viewed from the scleral surface.
A half-thickness scleral flap of about 4x4 mm is then dissected, hinged at the limbus. If in doubt, a longer posterior dissection from the limbus may be needed to have access to a CS that has been displaced more than expected due to globe enlargement. This will reveal the “blue zone” between cornea and sclera. About 1 mm behind this zone is where the CS may be found.
Enlargement of the eye distorts normal anatomy so these hints may be very useful or not, as the surgeon looks for the CS. A radial incision in a dry field using a sharppointed blade under high magnification is then made in the scleral bed, scratching down carefully while spreading the incision walls until the circular scleral fibers near the CS are encountered. A small drop of aqueous indicates entrance into the CS. Under ideal circumstances, the concave inner wall of the CS will be seen reflecting back from the depths of the dissection. If the first attempt at finding CS fails, another radial incision can be attempted within the same scleral bed to either side of the first. Small circumferential cuts can be made to partially unroof the CS to the right and left of the incision. This is done to allow easier passage of the trabeculotomes into the CS.
Passage of one of the probes is attempted while stabilizing the globe. The probe is passed gently, parallel to the limbus. The fit of the probe in the canal is usually tight. This is normal. Extremely easy passage of the probe with no resistance may indicate a false passageway into the suprachoroidal space or AC. The probe improperly placed in this case will rotate freely posteriorly or into the AC where the probe can be rotated into view. With the probe fully inserted into the CS, it can be rotated into the anterior chamber while avoiding the iris root and Descemet’s. This is done by holding the tip firmly against the sclera. This will minimize the chances of disinserting the iris root or creating a Descemet’s tear. Slow rotation allows the tip to be just seen in the AC and at the same time be gradually withdrawn as the meshwork is torn to its greatest extent Rotation of the trabeculotome should be only to about 70 degrees, retaining a small bit of tissue immediately in line with the scleral wound. This will decrease the chances of loss of the AC. If successful, the other probe can be passed in a similar fashion without need to refill the AC. If it does collapse, the AC can be refilled with fluid or viscoelastic. Care should be taken to avoid damaging the corneal endothelium with the probe tip. Withdrawal of the tip should be in a radial direction on both occasions. A hyphema may occur from either side of the angle that has been torn. This will resorb in a few days.
Some surgeons prefer to pre-cannulate the CS in both directions (to the right and to the left) with a small, 2-inch segment of a stiff suture, such as 6-0 Prolene. This allows one to confirm the correct location within CS. For example, if the suture appears in the AC, it is placed too far anterior. This maneuver also assists with identification of the CS if one should lose AC depth during the first half of the procedure as the initial trabeculotome is rotated into the anterior chamber.
It is also possible, using a longer piece of the same suture to perform a 360° trabeculotomy although this is more difficult for most surgeons. This is done by threading the suture for 360° degrees and then pulling the suture tight to incise the trabecular meshwork as the suture loop passes across the AC. A 360° trabeculotomy should only be attempted as an initial procedure and the tip of the suture should be blunted by application of light cautery to the tip. Attempting this technique in eyes that have had prior angle surgery may be more likely to create a false passage into the posterior chamber or sub-retinal space.
Repair of the sclera and conjunctiva is performed as in a trabeculectomy but, because this is probably being done in a child, an absorbable suture is preferred. The flap should be closed tightly. The radial incision used to identify CS may or may not be closed. The conjunctiva can be closed with 8-0 vicryl suture as well.
Trabeculotomy shown in several steps
If the trabeculotomy is not successful, the procedure may be converted to a trabeculectomy. But if mitomycin or 5FU is not used, the result is likely to fail in the long term.
The procedure may also be combined as a trabeculectomy-otomy. Well-respected authors (Mandal AK and Netland PA) have written of this procedure and claim good results in their patients (2/3 “successful” IOP levels at 6 years) but as with other procedures, success is often not life-long.
The eye is dressed and post-operative care conducted as described above for goniotomy. In addition cycloplegic drops are used.
For further detail the reader can consult Hamel P, Levin AV: Glaucoma surgical techniques in children: from past to future (part 2) Techniques in Ophthalmology 2004; 2(1): 21-30.
Surgeons who manage pediatric glaucomas should strive to become comfortable with both goniotomy and trabeculotomy surgery techniques. These are complementary procedures. Goniotomy requires a clear cornea and provides excellent access to the nasal and temporal angle structures. Conversely, trabeculotomies may be performed on cloudy or even opaque corneas and provide excellent access to superior and temporal angle portions of the angle. In cases of congenital or infantile glaucoma, it is preferable to open the entire angle prior to proceeding to other, less physiologic surgical interventions such as: tube implants, trabeculectomy, or cyclodestruction. Complete angle surgery employing goniotomy/trabeculotomy may require two or more separate surgeries to accomplish.
As briefly mentioned above, the likelihood of success over the long-term with trabeculectomy is lower in aphakia and in infants / children due to scarring leading to a non-functioning bleb. The success rate has been reported as being higher in Indian patients, but in most other countries healing properties of children lead to failure in nearly every case due to scarring at the episcleral level.
Antimetabolites have been used with some success, but the fear of thin-walled blebs is always present. This occurs in 33 to 66% after 18 months using 0.2% or 0.4% mitomycin-C. This is a concern especially in countries where a large portion of the children live in an agricultural setting and where the chance of contamination/infection is high. As with most developing world countries, follow-up is unpredictable at best, and even when successful; most support staff in remote locations may not be able to assess post-operative eyes effectively. This may result in post-operative failure after uneventful and successful surgery due to unrecognized problems such as endophthalmitis, cataracts, corneal decompensation, or flat anterior chamber.
For most patients a fornix based conjunctival flap results in a broader and far superior drainage area and lessens the future incidence of bleb revisions for thinning blebs. Every effort should be made to apply the antimetabolite drug exclusively to the sclera posterior to the scleral flap in the hope of establishing aqueous flow away from the limbus. This will further decrease the chances of a thin-walled bleb. Parents must understand that observation of the eye is very important even years later, and peripheral support staff (doctors and nurses) must be consulted at any time if doubts exist about the eye status.
Tube Implant Procedures
If angle surgery is unsuccessful or if the child has a form of glaucoma that is not typically amenable to angle surgery (aphakic/pseudophakic glaucoma without goniodysgenesis, Axenfeld-Rieger with prominent iridocorneal strands); tube implant procedures may be necessary. Like trabeculectomy, tube implants provide an alternative outflow path that bypasses the CS, and implants also provide a bleb well posterior to the limbus. The procedure is easy to learn and is very effective at providing rapid and substantial lowering of IOP in children of all ages. Additionally, tube implantation does not require the application of antimetabolite compounds although excessive postoperative inflammation can lead to failure of bleb permeability and a rise in IOP unless the inflammation is specifically and aggressively suppressed.
There are generally two types of tube implant: valved and non-valved. The valved form (e.g. Ahmed) offers immediate IOP reduction but obstructs free outflow from the device into the surrounding tissues thus maintaining physiological levels of IOP until the healing of tissues offers its own resistance to flow. If no valve were present, the IOP would be zero and hypotonous changes would inevitably occur in the eye. The nonvalved devices (e.g. Molteno, Baerveldt) offer no resistance and therefore the tubes must be temporarily occluded usually by sutures, removed after a time, or until the suture dissolves. This usually takes about 3-8 weeks during which time the IOP may be elevated, thus requiring treatment until the tube opens. It is rarely necessary to open the tube surgically but it may be necessary in some cases. There are conflicting reports that the non-valved devices lead to longer life for IOP reduction over those with valves. One must bear in mind the potential disadvantages of these implants. These include: opacification of cornea or lens if the tube tip lies in close proximity to these structures for a prolonged period of time. Also, erosion of the scleral patch graft and/or conjunctiva used to cover the tube, iritis, and if erosion occurs, endophthalmitis. Additionally, the expense of such implants may preclude their use in patients with limited resources. It is highly recommended to all surgeons that the tube be sutured loosely to the sclera with non-absorbable material, and donor sclera or other sources of tectonic grafts be used to reinforce the tube covering. This decreases the chances of erosion. Erosion and other complications associated with tube implantation procedures require surgeons to have a working knowledge of how to solve problems associated with the procedure.
- Same instruments as for a routine trabeculectomy procedure
- A tube implant device
- Sclera from an eye bank or other source (screened for infectious diseases including HIV, other sexually transmitted diseases, and hepatitis) or other patch material
- A 22- or 23-gauge needle for introduction of the tube into the AC
- 6-0 to 8-0 non-absorbable suture for securing the plate to the sclera permanently
- 8-0 non-absorbable suture to secure the tube to the sclera
- 8-0 vicryl or other absorbable suture to repair conjunctiva and donor material
A valved tube implant (Ahmed); B non-valved tube implant (Molteno)
Many different styles and sizes of tube implant devices exist but the basic implant technique is the same. A limbal peritomy is created, typically in the superior-temporal quadrant. Blunt dissection opens the potential space beneath Tenon’s capsule, taking care to avoid the rectus and oblique muscles. If the device is a “valved” implant, such as an Ahmed, it should be primed with Balanced Salt Solution® (BSS) or Ringer’s lactate to confirm that the valve is open. The tube plate(s) is then sutured in place using non-absorbable sutures, e.g. 6-0 black silk, so that the leading edge is 8-10 mm posterior to the limbus. Note that in children this may not be possible and distances of 5-7 mm are acceptable. In a microphthalmic eye, especially if less than 18mm in length, one must be careful that the plate does not touch the optic nerve. This can be accomplished by using a pediatric-sized device with anterior placement. Otherwise, adult size devices may be used. In some cases (e.g. Baerveldt), the implant plates can be trimmed to accommodate the smaller dimensions of some eyes or where retinal or other hardware complicates insertion.
A self-sealing paracentesis is always a good option so there is an alternative entry to the AC. The tube is then trimmed with bevel up so that when inserted it will extend 2-4 mm into the anterior chamber. A 22 or 23-gauge needle is used to enter the anterior chamber, just posterior to the limbus in a plane that is parallel to the iris. The tube is inserted through this opening such that it is touching neither the iris surface nor the corneal endothelium. If needed, air or viscoelastic may be used to reform the anterior chamber via the paracentesis, and may be left in the eye to help reduce the incidence of postoperative hypotony. As mentioned above, a means of occluding the tube in nonvalved implants is mandatory. Most commonly this is done using a 6-0 vicryl suture. The tube occlusion must be confirmed with Ringer’s lactate in a syringe using a 30 gauge needle or cannula.
The tube should be covered with a 4 x 6 mm patch of processed sclera or cornea (or with tissue dissected from a donor eye at the same sitting), banked dura or pericardium. If this type of tissue is not available, the entry into the anterior chamber may be created under a partial thickness, limbus-based scleral flap similar to that used for a trabeculectomy or trabeculotomy. Alternatively, a partial thickness scleral patch can be dissected from an area adjacent to the tube placement and used to cover the tube. When sclera in the host eye is still attached at the limbus, the tube entry should be directed somewhat posteriorly as it will be canted anteriorly when the flap is sewn back into place. Pars plana tubes are an option for aphakic children but the formed nature of the pediatric vitreous will lower success rates unless a total vitrectomy has been performed previously or will be done at the same time as the tube is placed.
After instilling atropine, antibiotic, and steroid ointments the eye is patched and shielded. Daily postoperative follow-up is recommended for at least the first several days as hypotony due to overflow or leak around the tube or even high IOP may occur. Early complications may include iris or vitreous in the tube, corneal touch by the tube, iritis, hyphema, conjunctival or scleral patch retraction, flat anterior chamber, or serous retinal detachment and choroidal effusion. The surgeon must be prepared to deal with these complications medically and/or surgically if they wish to undertake this procedure. Hypotony can often be managed by aggressive use of cycloplegics and simply waiting. Reformation of the AC using air or viscoelastic is sometimes needed. Removal of iris or vitreous from the tube can be delayed for 1-2 weeks while the tissues have a chance to heal.
Whether using cryo or diode laser modes this is a choice of last resort. Success is quoted as being 30-45% per application but along with hypotony or poor control, longterm complications may be band keratopathy, corneal decompensation, cataract formation, and phthisis. Diode laser cycloablation seems to offer fewer complications than cryo.
Peribulbar anaesthesia is used here for postoperative pain. In some cases alcohol may be used to provide longer acting pain relief.
This type of procedure is usually done only on ½ to ¾ of the circumference of the limbus so as to decrease the chances of hypotony. In the case of cryotherapy the ice ball should be completely free of the probe tip before moving the tip. Only a small area of ice should go onto the cornea at the time of application. The center of the probe should be 1 ½ mm posterior to the limbus while in diode cycloablation, the probe edge if up against the limbus. The difference in locations is dictated by the radial application of the cryoprobe and the semi-tangential direction of the G-probe.
Cycloblative procedures - A cryotherapy; B external diode laser (using G-probe)