Surgical Management in Primary Congenital are present.
PCG is typically autosomal
Glaucoma: Four DebatesTa C. Chang and Kara recessive, ranging in incidence from 1:1250 M. CavuotoDepartment of Ophthalmology, in consanguineous Slovakian Roms [1] to Bascom Palmer Eye Institute, University of 1:30,000 in Western countries [2]. The Miami Miller School of Medicine, Miami, FL differential diagnoses for PCG is broad and 33136, USAReceived 28 February 2013; would include congenital glaucoma associated Accepted 21 April 2013Academic Editor: with nonacquired ocular anomalies or Huseyin Gursoy Copyright 2013 Ta C. Chang systemic disease/syndromes. Hence, careful and Kara M. Cavuoto. This is an open access surveillance of any systemic comorbidity is article distributed under the Creative crucial in the well-being of the patient, as Commons Attribution License, which permits some systemic syndromes may carry life- unrestricted use, distribution, and threatening abnormalities that require reproduction in any medium, provided the prompt attention (e.g., cardiac outflow tract original work is properly abnormalities in Axenfeld-Rieger syndrome) cited.AbstractPrimary congenital glaucoma is [3]. Other types of childhood glaucoma, such a worldwide diagnostic and therapeutic as traumatic, uveitic, infectious, and steroid- challenge. Although medical management is induced should be ruled out with careful often a temporizing measure, early surgical history and examination.Appropriate and intervention is the definitive treatment. As timely diagnosis and treatment can the abundance of surgical treatment options dramatically alter the course of disease and continues to expand, the authors will restore visual development. In assessing the compare and contrast the available options risk/benefit profile of medical versus surgical and attempt to provide a consensus on treatment, angle surgery offers a high chance surgical management.1. Background and of success with low complication rates. Hence, Outcome of Primary Congenital surgery is often considered first-line therapy. GlaucomaOphthalmologists throughout the However, medical therapy provides a world are faced with the diagnostic and temporizing measure to clear the cornea to therapeutic challenge of primary congenital facilitate examination and surgical glaucoma (PCG). Often, infants present with intervention. Alpha-agonists are the classic triad of blepharospasm, epiphora, contraindicated in infants due to high risk of and photophobia. Characteristically, the central depression [4]. Topical beta-blockers patients are male (65%) with bilateral have been studied extensively and show involvement (70%) and diagnosed within the improvement in intraocular pressure in first year of life (80%) [1]. The natural course approximately one-third of patients in of congenital glaucoma results in pan-ocular multiple studies [57]. Cautious use of this dysfunctions, with vision loss resulting from class of medication is advised as beta-blockers Descemet breaks, corneal edema, and optic may lead to respiratory complications in neuropathy, and eventually buphthalmos, and predisposed young children. For this reason, amblyopia. Prior to the advent of surgical and selective beta-blocker (e.g., betaxolol) is medical treatments, most of these children preferred over nonselective ones, and the develop little or no useful vision in the lower concentrations than typical adult affected eyes. Primary congenital glaucoma formulations are used. The ophthalmologist accounts for 0.01%0.04% of total blindness should communicate with the pediatrician to [1]. The incidence varies with the founder assure there are no cardiac or pulmonary effect and when high rates of consanguinity contraindications. Carbonic anhydrase inhibitors are generally safe and well is also possible to remove the corneal tolerated. To avoid severe metabolic acidosis, epithelium to improve visibility. The success topical drops are preferred to systemic of goniotomy is thought to be approximately suspensions. Prostaglandin analogues have 80% with a single procedure, especially if been tried however showing mostly recognized and treated within the first year of nonresponders in the PCG group [8].2. life [1]. Benefits of goniotomy are that it is Surgical ApproachHistorically, Hippocrates minimally traumatic to the surrounding described a child with likely congenital tissues, does not result in conjunctival glaucoma as early as the 5th century BCE. scarring, and has a shorter operating time. Nearly two thousand years later, PCG retained However, multiple incisions are to treat the a poor prognosis despite recognition of the entire angle. Complications of goniotomy condition and various attempts at surgical include hyphema, iridodialysis, peripheral control. It is thought that goniotomy was first anterior synechiae, cyclodialysis, and described in 1893 by Carlo de Vincentiis; lenticular injury.Trabeculotomy involves however it was performed without disrupting the tissue between Schlemms visualization of the angle and resulted in poor canal and the anterior chamber using an ab outcomes [9]. It was not until 1938 when Otto externo approach to create direct Barkan performed the first goniotomy utilizing communication. Currently, there are at least gonioscopy and a knife to incise the three different approaches: rigid probe, trabecular tissue [1]. Although goniotomy was suture, and illuminated microcatheter. difficult or impossible in eyes with significant Pilocarpine is used for pupillary constriction corneal edema, it became the standard of and a peritomy is typically performed. A treatment for PCG. Trabeculotomy, which partial-thickness scleral flap is created and could be performed in the presence of corneal Schlemms canal is identified, incised, and opacity, was later developed by Smith in 1960 cannulated for 360 degrees. High success [10]. Both of these procedures have excellent rates ranging from 87% to 92% in cases of PCG success rates and continue to remain part of presenting before one year of age make every pediatric glaucoma surgeons arsenal of trabeculotomy an excellent procedure [9]. techniques to manage intraocular pressure in Other benefits of trabeculotomy are that it PCG. Debate 1: Reinvestigating the Basics: can be performed with a cloudy cornea and Goniotomy versus Trabeculotomy Which Is one incision can be used to treat the entire Better?. Goniotomy is designed to create a 360 degrees. Like goniotomy, a detailed route for aqueous drainage through understanding of angle anatomy is required to Schlemms canal by incision of the trabecular perform the procedure. Complications include meshwork under direct visualization. hyphema, unintentional filtering blebs, Pilocarpine is often used to constrict the pupil choroidal detachment or hemorrhage, and and a goniolens is used for visualization of false passages into the eye. Compared to angle structures. The trabecular meshwork is goniotomy, trabeculotomy takes more time incised with a goniotomy knife or small gauge and causes conjunctival scarring which may needle for 4-5 clock hours with a single compromise the future options of placing incision. To perform the procedure, an glaucoma drainage implants or perform a adequate view of the angle and knowledge of trabeculectomy. However, if the procedure is angle anatomy are required. If corneal edema done temporally it does not compromise is present, it is sometimes possible to treat it subsequent filtration or implant surgery. Due medically for a short time to reduce edema. It to the history of success with both procedures with well-defined patient parameters, this is nylon filament or the later-adapted the most straightforward of the debates. polypropylene filament as described by Beck Goniotomy is preferred in children less than and Lynch [15], the rigid probe offers better one-year old with good visibility of angle directional control once it enters the structures. Trabeculotomy is the procedure of Schlemms canal, and the later double choice in children with poor visualization of parallel-pronged design (as in the Harms angle structures [9, 12], although some trabeculotomy probes, Figure 3) helps the surgeons advocate filament-assisted surgeon determine the probes position in the circumferential trabeculotomy over canal and anterior chamber. A single incision goniotomy as a primary procedure even in allows opening of approximately 120 degrees PCG patients with clear corneas due to of angle. In the case of treatment failure, greater success when compared to goniotomy subsequent angle surgery is usually offered [13].Debate 2: The Evolution of the Basics: Is prior to considering glaucoma drainage device Rigid Probe Trabeculotomy Superior to implantation. fig2Figure 2: Two varieties of Filament-Assisted Trabeculotomy? Smiths rigid probes as described by Allen and Burian initial description of trabeculotomy ab and used in sector trabeculotomy. The probe externo in 1960 involves passing a nylon on the left has a blunt tip, while the one on filament around Schlemms canal via an the right is fitted with a drawknife edge external radial incision and rupturing the [11].612708.fig.003Figure 3: Harms trabecular tissue in a drawstring fashion [10]. trabeculotomy probe with double parallel This approach has the advantage of incising all prong design.There are no well-designed, 360 degrees of angle, thus avoiding multiple large prospective double-masked controlled treatments. However, potential for false trials comparing the outcomes of passages and the inability to visualize or trabeculotomy performed with a filament to control the filaments position can be the outcomes performed with a rigid probe, problematic. Smiths follow-up publication in although several smaller retrospective reports 1962 described a complication in which the lend insight into the efficacy of each distal end of nylon filament was not recovered technique. The definition of success varies from the opposing Schlemms canal cut-down widely between studies, although most opening [14]. Filament entering the anterior criteria indicated clinically stable result. Using chamber, suprachoroidal or subretinal spaces the filament technique, Beck and Lynch can cause serious complications, including reported 15 PCG patients (mean age 8.5 damage to the cornea and lens, hyphema, and months), 87% of whom achieved treatment chorioretinal scarring (Figure success (IOP 1).612708.fig.001Figure 1: Color fundus photography demonstrating circumferential chorioretinal scarring after misdirection of blunted 6-0 polypropylene suture used in filament-assisted circumferential trabeculotomy (photograph courtesy of Ms. Ditte Hess).Allen and Burian described an alternative technique of trabeculotomy using specifically designed rigid probes (Figure 2) that achieved sector opening of the trabecular tissue in 1962 [11]. Compared to the flexible 22mmHg without medication, stabilization or improvement of disc appearance, absence of progressive corneal enlargement, or axial 18mmHg under general anesthesia or length increase) after mean followup of 12 months [15]. Mendicino et al. in comparing filament-assisted trabeculotomy with goniotomy reports on 24 eyes in PCG patients (mean age 4.5 months) that had undergone circumferential trabeculotomy with a filament, 92% of which with favorable treatment outcome (IOP less than 22mmHg with or without medications, no progressive disc changes, no additional surgeries) after mean followup of four years [13]. More 21mmHg while awake, stable axial length recently, Sarkisian reports a series of 16 eyes and optic nerve appearance, clear cornea) of in which filament trabeculotomy was 77% at 6 months and 59% at 2 years. successfully performed using an illuminated Treatment success extrapolated from the microcatheter in children under 3 years of age published life table at 1 year is approximately diagnosed with PCG. Circumferential 60%. In a consecutive series of 46 patients canulation was achieved in 75% of these eyes, followed for an average of 38 months, Filou and the cohorts mean intraocular pressure and Brunov [18] reports a cumulative success averaged under 21mmHg after 6 months of 87% (success defined by IOP less than 21 followup although criteria for treatment without progressive corneal or optic disc success was not defined nor was the changes), 22% of whom required additional population of sufficient size to allow statistical angle surgery after the initial rigid-probe analysis. The illuminated microcatheter allows trabeculotomy. In summary, rigid-probe continued visualized of the catheter tip during trabeculotomy has an overall success of canulation, hence reducing the risk of approximately 60%87% after a mean undetected catheter misdirection followup of 13 years, with a subset of the [16].Dietlein et al. [17] compared rigid-probe patients requiring additional angle surgery. A trabeculotomy to combined 360 filament trabeculotomy, on the other trabeculotomy/trabeculectomy and to hand, achieved surgical success in 87%92% trabeculectomy alone in children. In their of patients after 14 years of followup. No trabeculotomy arm (17 of the 61 trial significant complications were noted in any of patients), they reported treatment success the trials. The illuminated microcatheter (IOP improves the safety of filament trabeculotomy by allowing continuous visualization of filament tip and allows rapid detection of misdirection. Given the available outcome data and the improved safety profile of illuminated microcatheter, the authors recommend filament trabeculotomy with an illuminated microcatheter over rigid-probe trabeculotomy as an initial procedure in PGC. Debate 3: Trabeculectomy versus Tube Shunt in the Pediatric Congenital Glaucoma Group: perform data analysis based on glaucoma Are the Results the Same? After failing subtypes, which lends insight into our clinical treatment of 360 degrees of angle (whether debate. Initial studies argued against the one-time filament trabeculotomy or multiple- consideration of trabeculectomy in the session with goniotomy/rigid-probe treatment of PCG. In 1979, Beauchamp and trabeculotomy) and maximizing medical Parks reported 50% success in children who treatment, the next procedure of choice often had undergone trabeculectomy after 1039 depends on surgeons training and practice months of followup, with numerous style. Most surgeons favor either a shunting complications including vitreous loss, retinal procedure, that is, a glaucoma drainage detachment, and endophthalmitis [19]. device (GDD), or a filtering procedure, usually Subsequent studies suggested that there was a trabeculectomy (with or without some utility in performing trabeculectomies in augmentation with antifibrotic agents). The children with PCG. Fulcher et al. [20] two choices have different risk/benefit retrospectively reviewed 13 eyes with PCG profiles. Trabeculectomy does not involve a that had undergone trabeculectomy without foreign-body implant and the associated MMC with 514 years of followup and complications including corneal trauma, reported an overall success of 92.3% after 1 motility disturbances, and exposure. single trabeculectomy and 100% success with However, the exuberant cicatricial response in two trabeculectomies at the last follow-up these patients often results in low success in visit. The authors reported no serious intraocular pressure control. Antifibrotic complications in any patients. Due to the agents may improve IOP control, but their use exuberant fibrotic response to surgery in increases the chance of serious bleb-related children, several authors have investigated complications. On the other hand, glaucoma the use of antifibrotic agents to increase the drainage devices may offer greater IOP chances of surgical success. Al-Hazmi et al. control over long-term followup, but the risk [21] reviewed 254 eyes (including 98% PCG) of implant-related complications may increase that had undergone trabeculectomy over time. Some argue that initial filtering augmented with mitomycin-C (MMC). They procedures might keep more options open report age-related success rates of between as there is an inherent bias toward implanting 32% (age less than 6 months at time of a second GDD after the first one fails instead surgery) to 85% (aged 4985 months of age) of performing a trabeculectomy, while a failed after at least one year of follow-up. However, trabeculectomy does not preclude the authors also reported age-related rates of subsequent GDD procedures. In the complications between 0% (age less than 6 evaluation of trabeculectomy versus months) and 50% (age 4984 months) after at glaucoma drainage device implantation after least 1 year of follow up. The most frequent failure of angle surgery, we must consider complication was the development of cystic both long-term outcome and the risk of bleb, followed by hypotony and bleb leak. complications. There is a paucity of clinical Sidoti et al. [22] reviewed a cohort of 15 eyes data regarding the efficacy and safety of with PCG which had undergone trabeculectomy versus glaucoma drainage trabeculectomy with MMC and reported devices in PCG. Most retrospective reviews on treatment success of 87% of patients at 12 this subject included patients of various months, 78% at 24 months, with a cumulative subtypes of childhood glaucoma or combined bleb-related infection in 27% of this cohort at surgical techniques. Some of these studies did the last follow-up visit. Rodrigues et al. [23] retrospectively reviewed 91 eyes with PCG tube implantation.The efficacy and that had undergone trabeculectomy (61 complication rates of trabeculectomy with without MMC and 30 with MMC) and found MMC and the valved Ahmed glaucoma that, with a minimum followup of 2 years, implant augmented with MMC were long-term surgical outcome did not differ compared in a randomized clinical trial by between the MMC versus non-MMC groups, Pakravan et al. [27] in a study involving 30 though the MMC group had higher incidents aphakic eyes with glaucoma. Although the of complication. Agarwal et al. [24] compared study focused on aphakic eyes with glaucoma, the effects of 0.2mg/mL and 0.4mg/mL MMC an entity quite distinct from PCG in in trabeculectomy and included 17 patients pathophysiology, onset, presentation, and with PCG. They noted no difference in success outcome, the authors prospective rate between the two concentrations, randomized method allows a head-to-head although the 0.4mg/mL group had a higher comparison between the two surgical complication rate (33.3% in 0.2mg/mL group approaches, which lend insight into the versus 66.6% in 0.4mg/mL group over 18 efficacy and safety of each. The authors months). The overall success rate at 18 surgical technique in tube implantation does months was approximately 60%86.7%. not include concurrent pars plana vitrectomy Overall, the success rate of trabeculectomy is to minimize the risk of vitreous prolapse and difficult to interpret based on the data tube obstruction. The authors defined success presented, but from the life table as IOP between 5 and 21mmHg. If an eye did extrapolation, approximately 50% of the not require medication for IOP control, it was surgery fail within the first 510 years.Netland called a complete success, and if medication and Walton [25] first introduced glaucoma was needed it was designated a qualified drainage devices to be used in pediatric success. With 15 eyes in each arm, the glaucoma. Since that time, various GDDs have trabeculectomy group, after a mean followup been utilized for intraocular pressure control of 14.8 months, had an overall success of in these children, including Baerveldt, 73.3% (including 33.3% complete and 40% Molteno, and Ahmed devices. In a qualified success), while the Ahmed group, retrospective case-control study, Beck et al. after mean followup of 13.1 months, enjoyed [26] identified 46 pediatric eyes with various an overall success of 86.7% (20% complete glaucoma subtypes that had received aqueous and 66.7% qualified success). There were no shunt device prior to age of 24 months and significant differences between the visual compared the outcome to age-matched eyes acuity outcomes between the two groups, that received MMC-augmented while there were 40% complications in the trabeculectomy. At the last followup, 20.8% trabeculectomy group and 26.7% in the were considered successful in the Ahmed group. In the comprehensive review trabeculectomy group (mean followup 11.5 by Ishida et al. [28], the authors collimated months) while 71.7% were considered the outcomes of twenty-one studies of successful in the aqueous shunt devices group glaucoma drainage implants in pediatric (mean followup 31.5 months). The patients between 1984 and 2004. The overall trabeculectomy group had an 8.3% success ranged from 54% to 95% (mean cumulative incidence of endophthalmitis, approximately 75%), with success criteria while 45.7% of aqueous shunt device group generally based on IOP less than 22 mmHg required more surgical procedures, such as (with or without medical) and after variable tube repositioning, due to complications of follow up lengths ranging from 12 to 124 months. The long-term effectiveness of intraocular pressure control has also been noted by several other groups; however the effectiveness must be balanced with the complications related to hypotony and to the tube itself [2931].In summary, after various 21mmHg). Within the PCG subgroup, the durations of followup, trabeculectomy has a 78% of patients had surgical success over 10 success rate at one year ranging between 32% months, which compares well with other and 100% with most studies reporting 50% series with rigid-probe trabeculotomy alone. 87%. The frequency of complications vary, This suggests that the addition of although at the high end it is approximately trabeculectomy with MMC to the rigid-probe 66.6% when MMC was utilized in conjunction trabeculotomy offers no additional advantage with the surgical procedure. Surgical efficacy and presumably would increase the risk of decreases over time, young patients bleb-associated complications. (specifically those under 6 months of age) Nonpenetrating procedures are of particular tend to do poorly, and augmentation with interest in pediatric patients because they are MMC makes little or no differences in success assumed to have a decreased risk of rate but may increase the risk of complication. hypotony, infection, lens injury, Descemet Tube shunt, on the other hand, has an overall detachment, and other intraocular trauma. success of approximately 75%86.7% after 1-2 Deep sclerectomy involves the unroofing of years and a rate of severe complication of Schlemms canal under a sclera flap, with between 26.7% and 45.7% in the same time concurrent removal of the juxtacanalicular period. With comparable efficacy and a lower trabecular tissues without entering into the complication rate, tube shunt surgery seems eye (Figure 4). The procedure leaves behind a to be the favored procedure in children with trabeculodescemetic window that provides PCG who have failed angle surgery.Debate 4: the resistance to aqueous drainage to prevent On the Horizon: Looking at the Alternatives in hypotony. Al-Obeidan et al. [33] prospectively Pediatric Congenital Glaucoma Surgery. Some followed 143 eyes of 120 patients with surgeons advocate combined procedures or congenital glaucoma without concurrent nonpenetrating surgeries as alternatives to anterior segment anomalies (90.9% diagnosed traditional angle surgeries as primary with PCG). Non-penetrating deep sclerectomy procedures. Mullaney et al. [32] was technically successfully in 74 eyes retrospectively reviewed 100 consecutive (52.4%), with the remainder converted to eyes with congenital glaucoma, 63% of which penetrating procedures with either were PCG. These patients underwent involuntary perforation of the combined rigid-probe trabeculotomy and trabeculodescemetic window or an trabeculectomy augmented with MMC. The intentional perforation due to inadequate authors reported success in 67% of patients aqueous percolation. Within the group in after average followup of 10 months (success which non-penetrating deep sclerectomy was defined as IOP performed, success (IOP under 21mmHg) after mean followup of 35.8 months was 82.4% (79.9% complete success, 2.7% qualified success), while the cohort with penetrated deep sclerectomy had complete and overall success in 84.1% and 89.9% of patients, respectively. The surgical technique adjacent to the ostia, hence converting this of deep sclerectomy proved to be difficult, as procedure into a penetrating procedure. After in the authors experienced hands only 51.7% mean followup of 20 months, the overall of eyes had successful completion for the success was 89% without serious non-penetrating procedure. Furthermore, the intraoperative or postoperative complications authors data seem to suggest that noted. In summary, based on the literature perforation does not seem to worsen reviewed, the addition of trabeculectomy to outcome or increase complication rates, trabeculotomy does not seem to offer which challenges the notion that non- significant advantage in success rate and may penetrating procedures offer greater safety increase the risk of complications. Non- profile in this patient population. penetrating deep sclerectomy may hold Intraoperative use of mitomycin-C and promise given its theoretical benefits over nonabsorbable space-maintaining material penetrating surgeries but may be technically may negate the benefits of this minimally difficult to perform in inexperienced hands. traumatic approach by increasing the future Viscocanalostomy may offer an alternative to (and life-long) risk of hardware exposure and classic angle surgery if it is demonstrated to scleral ectasia. 612708.fig.004Figure 4: be safe and noninferior.3. Schematic drawing demonstrating techniques Summaries(1)Goniotomy and rigid-probe of deep sclerectomy.Viscocanalostomy is a trabeculotomy are both successful in treating non-penetrating procedure first described by PCG. Goniotomy has a shorter operating time Stegmann et al. [34]. This procedure combines and does not cause conjunctival scarring but is both deep sclerectomy with unroofing of limited to cases with a clear cornea. While Schlemms canal and dilation of the goniotomy is traditionally offered as primary Schlemms canal with injection of sodium procedure in PCG patients with clear cornea, hyaluronate into the canal to provide a the improved success rate and safety profile physical barrier to fibrinogen migration. The of circumferential trabeculotomy may offer results show promising IOP reduction in adult greater advantage over patients who had previously been goniotomy.(2)Filament-assisted uncontrolled on medical therapy, with a trabeculotomy has superior success rates in postoperative IOP of 22mmHg or less the literature when compared to rigid probe without medical therapy in 82.7% of eyes with trabeculotomy. Illuminated microcatheters an average followup of 35 months. Noureddin allow improved visualization of the catheter et al. [35] conducted a pilot study of eight tip during canulation, reducing the risk of consecutive infants diagnosed with bilateral misdirection. Filament trabeculotomy with an PCG, with the more severe eye undergoing illuminated microcatheter is superior to rigid- randomization to either trabeculotomy or to probe trabeculotomy as an initial procedure. viscocanalostomy. The observation was (3)After angle surgery, trabeculectomy may similarly well-controlled mean IOP between be useful in children to avoid the implantation the two groups with a followup of twelve of a glaucoma drainage device; however months. Kay et al. [36] reported a series approximately 50% fail in the initial 510 including 19 surgically nave PCG eyes that years. Augmentation of trabeculectomy with underwent modified viscocanalostomy. In mitomycin-C does not appear to improve addition to classically described surgical outcomes however does increase viscocanalostomy, the author performed stab complication rates, especially in high incisions that entered the anterior chamber concentrations. The addition of trabeculectomy with MMC to the rigid-probe trabeculotomy seems to offer no additional advantage and presumably would increase the risk of bleb-associated complications. Glaucoma drainage devices seem to have a higher success rate and a lower complication rate when compared with trabeculectomy.(4)New techniques, including deep sclerectomy and viscocanalostomy, appear to offer excellent success rates while minimizing the risk of glaucoma surgery. Non- penetrating surgery offers both the efficacy of lowering IOP and guards against hypotony but is technically difficult to achieve. Viscocanalostomy may offer similar efficacy to traditional trabeculotomy, although more studies are needed for conclusive evidence.