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Glaucoma Physician

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Update on Current Surgical Management of Pediatric Glaucoma

Optimal management must consider features specific to each child.

By: Rebecca F. Neustein, MD, MARLENE R. MOSTER, M.D.
Glaucoma Physician June 1, 2022 Vol Ophthalmology Management 26, Issue June 2022 Page(s): 30-33

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Glaucoma is an uncommon disease in children, and its pathology is varied. Elevated intraocular pressure (IOP) in childhood glaucoma may result from primary developmental abnormality of the anterior chamber, as in primary congenital glaucoma (PCG), or glaucoma associated with nonacquired ocular anomalies. Intraocular pressure may also be elevated from acquired obstruction to aqueous outflow from an ocular or systemic disease process, as in secondary pediatric glaucomas.1,2 Clinically, infants with glaucoma often present with photophobia, epiphora, and blepharospasm. Examination findings may include corneal clouding and enlargement, Haab’s striae, increased cup-to-disc ratio, an abnormally deep anterior chamber, and astigmatism or myopia.3 In children with secondary pediatric glaucoma, exam findings may include clinical signs of ocular or systemic disorders leading to the glaucomatous process, such as aniridia, aphakia with a history of congenital cataract, iridolenticular touch, posterior embryotoxin, intraocular inflammation, or sequelae of trauma. Without medical or surgical IOP reduction, irreversible optic nerve axonal loss — and in the case of children with PCG or other glaucomas with infant-onset disease, corneal opacification — leads to severely reduced vision or complete blindness.4-6

Unlike adult variants of glaucoma, pediatric glaucoma often requires surgical intervention as first-line therapy to adequately control IOP and preserve vision for the long term in this vulnerable population.7 Surgical options can be grouped into 3 broad categories: (1) procedures that restore flow through the eye’s normal aqueous outflow pathway (angle surgery); (2) procedures which bypass the drainage angle and create an external pathway for aqueous outflow (trabeculectomy and the implantation of glaucoma drainage devices [GDDs]); and (3) procedures that reduce aqueous production of the eye (cyclodestructive procedures).

Angle Surgery

Goniotomy aims to improve aqueous outflow into Schlemm’s canal by removing abnormal or malfunctioning trabecular meshwork tissue. Together with ab externo trabeculotomy procedures introduced in the 1960s,8-10 these techniques revolutionized treatment and vastly improved prognoses for children with PCG. Circumferential trabeculotomy with a blunted 6-0 polypropylene suture, and later an illuminated microcatheter, has gained popularity.11,12 Goniotomy and trabeculotomy have demonstrated good success in longitudinal studies, particularly in eyes with PCG, with circumferential (360°) trabeculotomy providing improved long-term IOP control and visual outcomes compared to conventional angle surgery in these children.13-15

Angle surgery has become more frequently employed in glaucoma following congenital cataract surgery (GFCS).1 El Sayed et al reported 1-year success after conventional angle surgery of 89.6%, and Lim et al noted 3-year success of 72% after circumferential trabeculotomy for GFCS.16,17 Angle surgery as first-line surgery for refractory secondary childhood glaucoma may control IOP for several years or more, delaying or obviating the need for more invasive procedures like GDDs, and therefore hopefully improving long-term visual prognosis for these children.

For some children, initial angle procedures may not adequately control IOP long term, and further IOP-lowering surgery may be required. For these children, potential surgical options include trabeculectomy, GDDs, and cycloablative therapy.

Trabeculectomy

Trabeculectomy in the pediatric population has historically been technically challenging, with varied but limited long-term success.18 The higher rate of failure initially described for trabeculectomy in the very young has been ascribed to children’s robust healing response,18,19 which antiscarring agents such as mitomycin C have improved. However, mitomycin C use in pediatric eyes engenders an increased risk of hypotony and thin, cystic blebs, which predispose to late leaks and bleb-related infections, including endophthalmitis — nontrivial complications in young children. Also, reported outcomes in children younger than 12 months at time of surgery remain suboptimal compared to those of older phakic children and adults.20-23 Success of trabeculectomy in pediatric eyes has been reported to be 59% to 95% with follow-up of 2 years or less, and to drop to 19% to 51% at 6 years postoperatively.24,25 The procedure is thought to be more prone to failure in aphakic eyes.26 When trabeculectomy was compared to GDDs with regard to glaucoma control achieved in children 2 years and younger by Beck et al, GDDs were found to provide greater chance of IOP control in this age group, but more often required a return to the operating room than eyes in the trabeculectomy group.25

More recently, Jayaram et al achieved improved long-term success rates among children under 2 years old, reporting 78% success at 1 year and 60% success at 7 years in phakic children with PCG who underwent fornix-based trabeculectomy with 3 early postoperative examinations under anesthesia (EUA) for suture removal and bleb modulation with 5-FU or subconjunctival steroid.27 The authors note that their long-term success rates are comparable to those published for GDDs, and that while their intensive postoperative care algorithm included serial EUAs, these may be balanced with less frequent need for later return to the operating room than may occur after a GDD.

While the results of this recent paper paint trabeculectomy in pediatric patients in a more positive light, no consensus exists regarding the optimum next procedure after failed angle surgery in children with refractory glaucoma, and children with trabeculectomies require close follow-up throughout childhood and adolescence to monitor for late bleb-related, visually threatening complications.27

Glaucoma Drainage Devices

Glaucoma drainage devices have historically had moderate success in pediatric glaucoma, with a wide range of reported visual outcomes and a lower tendency toward serious complications.28-38 They often emerge as a logical next surgical step for young children with refractory glaucoma and occasionally as a first-choice surgical intervention for complex secondary glaucomas in which angle surgery is not feasible.32,39-41

Since the introduction of the Molteno GDD (IOP Inc), this nonvalved GDD has largely been replaced by the non-valved Baerveldt GDD (AMO Inc) and the valved Ahmed GDD (New World Medical), which have flexible silicone plates with larger surface areas than the single-plate Molteno. The Baerveldt GDD was introduced in the 1990s, and involves a non-valved tube attached to a silicone plate, available in 250 mm2 and 350 mm2 sizes. Ligation of the tube with an absorbable suture is necessary to delay flow through the nonvalved tube until reservoir encapsulation has occurred. Rates of successful IOP control with the Baerveldt GDD have been reported in the literature to be excellent short-term — 84% to 90% at 1 year42-44 — with modest attrition over time. Jacobson et al recently reported 60% success for the Baerveldt GDD at 5 years, dropping to 32% at 8 years postoperatively.45 Budenz et al similarly found 60% probability of success out to 106 months.31

The Ahmed GDD, with a unilateral flow-restriction mechanism to limit aqueous humor flow below an IOP <8 mmHg, provides similar success rates to the Baerveldt GDD: 1-year and 5-year success ranges from 81% to 92% and 55% to 64%, respectively, and as with the Baerveldt GDD, 10-year success probabilities drop to 36%.33,46,47 Several authors have reported improved surgical success with the Ahmed GDD in secondary pediatric glaucoma compared to PCG.34,47

While GDD options carry a significant risk of postoperative complications — reported incidence in recent literature ranges from 25% to 50% — and tube revisions are not uncommonly required in these children,29,37,46 incidence of devastating complications such as endophthalmitis and blindness are rare with GDDs. The choice of GDD is generally surgeon- and patient-dependent: the Baerveldt may provide slightly lower IOPs long-term, while the Ahmed is associated with fewer short-term complications, and all GDDs generally demonstrate reduction in success over time and the need for continued adjunctive medication use.26,48

Cycloablative Therapy

Cycloablative therapy has historically been considered a last resort in children due to low long-term success rates and the potential for chronic inflammation, vision loss, phthisis, and sympathetic ophthalmia.49-51 Studies of transscleral diode cyclophotocoagulation (CPC) in children have reported success rates of 37% to 75% — suggested to be higher in aphakic eyes — with ongoing use of glaucoma medications and with frequent need for repeat treatment.50,52,53 Endoscopic CPC has demonstrated limited success in children with refractory glaucoma, and lower success rates than transscleral CPC in adults.54,55 As technology has improved, the procedure has become a safer option50; however, with an eye toward the long potential lifespan of the pediatric patient, pursuing other surgical options and holding CPC in reserve for those in whom incisional surgery is contraindicated or high-risk may remain prudent.

Conclusion

The surgical management of childhood glaucoma remains challenging. The surgeon must contend not only with operating on eyes with often distorted anatomy, but also with a vigorous healing response, in patients whose limited ability to cooperate with clinic visits and postoperative medication regimens endangers the long-term IOP control — and by extension, visual preservation — surgeons strive so hard to achieve. Optimal surgical management must consider features specific to the child’s eye (eg, glaucoma type and severity, ocular features, prior surgeries, visual potential), the child’s age and general health, and the specific family support system (regarding ability to adhere to postoperative medications, care, and return visits). Hopefully the future holds novel surgical techniques that can boost the surgical armamentarium of the pediatric glaucoma specialist, and thereby improve long-term outcomes and decrease complication rates for children with glaucoma and their families. GP

Rebecca F. Neustein, MD, is a glaucoma fellow at Wills Eye Hospital in Philadelphia. She reports no relevant disclosures. Reach Dr. Neustein at rebecca.neustein@gmail.com.

Marlene R. Moster, MD, is a professor of ophthalmology at Sidney Kimmel Medical College of Thomas Jefferson University in Philadelphia and attending surgeon at Wills Eye Hospital. She reports no relevant disclosures.

References

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