Characteristics of Children With Primary Congenital Glaucoma Receiving Trabeculotomy and Goniotomy

Size: px

Start display at page:

Download "Characteristics of Children With Primary Congenital Glaucoma Receiving Trabeculotomy and Goniotomy"

Pierce Hardy
6 years ago
Views:

1 Characteristics of Children With Primary Congenital Glaucoma Receiving Trabeculotomy and Goniotomy Lekha Mukkamala, MD; Robert Fechtner, MD; Bart Holland, MPH, PhD; Albert S. Khouri, MD ABSTRACT Purpose: To describe the groups of patients who received trabeculotomy or goniotomy for the treatment of primary congenital glaucoma (PCG) regarding age at treatment, intraocular pressure (IOP) outcome, and medication burden. Methods: A retrospective chart review of patients with PCG seen at Rutgers New Jersey Medical School, Newark, New Jersey, from 1998 to 2012 was conducted. Inclusion criteria were patients who received trabeculotomy or goniotomy with at least 9 months of follow-up. Presenting examination, surgical intervention, IOP, and number of medications at 1 and 2 years postoperatively were recorded. Absolute and qualified success, defined as IOP greater than 5 and less than 21 mm Hg without and with medications, respectively, was determined. Results: Fifty eyes of 29 patients were diagnosed as having PCG. Of those, 25 eyes received trabeculotomy or goniotomy, with 19 fulfilling inclusion criteria. Average age at the time of trabeculotomy was 8 months versus 21 months for patients undergoing goniotomy. Mean IOP was significantly reduced (P <.001) for both trabeculotomy and goniotomy by 29.5% at 1 year and 33.3% at 2 years. There was no significant difference in IOP control between trabeculotomy and goniotomy groups. Patients in the goniotomy group were treated with significantly more medications before and after surgery compared to patients receiving trabeculotomy (P <.01), resulting in a greater rate of absolute success in trabeculotomy at 1 and 2 years. Conclusions: Patients with PCG who underwent trabeculotomy had higher IOP and were treated at an earlier age than those who had goniotomy. Both effectively lowered IOP up to 2 years with greater medication burden in patients receiving goniotomy. [J Pediatr Ophthalmol Strabismus. 2015;52(6): ] INTRODUCTION Primary congenital glaucoma (PCG) is a rare disorder of isolated trabecular dysgenesis unrelated to ocular or systemic disorders that results in increased intraocular pressure (IOP) from birth to before age 16 years. 1 The dysgenesis is present at birth, with most patients presenting for medical attention within 1 year, although PCG may not manifest until several years later. 1,2 Presenting signs and symptoms of PCG vary from the classic triad of epiphora, photophobia, and blepharospasm, thought to be due to corneal epithelial edema from increased IOP, 2 to From the Institute of Ophthalmology and Visual Science (LM, RF, ASK) and the Department of Preventative Medicine (BH), Rutgers New Jersey Medical School, Newark, New Jersey. Submitted: July 2, 2015; Accepted: August 13, 2015 The authors have no financial or proprietary interest in the materials presented herein. The authors thank The Lions Eye Research Foundation of New Jersey and The Eye Institute of New Jersey. Correspondence: Albert S. Khouri, MD, Institute of Ophthalmology and Visual Science, Rutgers New Jersey Medical School, 90 Bergen Street, Suite 6100, Newark, NJ albert.khouri@rutgers.edu doi: / Journal of Pediatric Ophthalmology & Strabismus Vol. 52, No. 6,

2 more subtle signs such as the inability to track objects. Without treatment, glaucomatous damage can progress, leading to the development of buphthalmos, Haab s striae, stromal edema, and eventually amblyopia and blindness. 3 Surgical intervention is considered the most effective and definitive treatment modality for patients diagnosed as having PCG, specifically the angle surgeries of trabeculotomy and goniotomy. 4-6 Goniotomy has traditionally been preferred when the cornea is clear enough to allow sufficient visualization of the angle structures, whereas trabeculotomy is selected when the cornea is hazy. Improved outcomes for both procedures have been noted when surgery is performed within 1 year of diagnosis. 2 Both trabeculotomy and goniotomy have been shown to effectively lower IOP, with most studies reporting similar efficacy between the two procedures. 2,3,7 Medications tend to be used as temporizing or adjunctive treatment to assist with IOP control, but yield a lower success rate and may cause adverse effects in children. 2,6 Few studies have examined patient characteristics and long-term outcomes of these procedures or the concurrent medication burden. In this study, we aim to characterize the group of patients who received trabeculotomy and goniotomy with respect to age at treatment and the longterm IOP control and medication burden. PATIENTS AND METHODS A retrospective review of medical records of children who presented to Rutgers New Jersey Medical School, Newark, New Jersey, from 1998 to 2012 with PCG who received trabeculotomy or goniotomy was conducted. PCG was classified as being congenital, infantile, or late-recognized, based on the age of diagnosis: less than 1 month, 1 month to 2 years, or 2 to 16 years, respectively. The treatment for PCG was angle surgery, either trabeculotomy or goniotomy. Inclusion criteria were any patient with PCG who had either trabeculotomy or goniotomy with at least 9 months of follow-up. Excluded were children with other forms of childhood glaucoma (secondary to trauma, trabecular dysgenesis syndromes, postcataract, etc.), surgery other than trabeculotomy or goniotomy, or follow-up of less than 9 months. Subsequent surgeries performed on the same eye but in different portions of the angle were also included in the analysis and considered separate procedures. Demographics, presenting symptoms, type of surgery, age at time of procedure, IOP, and number of glaucoma medications before and at 1 and 2 years postoperatively were recorded. The number of eyes achieving different levels of IOP control (> 5 and < 18, < 21, or < 24 mm Hg) was calculated. Absolute success (defined as IOP > 5 and < 21 mm Hg with no medications) and qualified success (IOP > 5 and < 21 mm Hg with or without medication) were measured. Means, standard deviations, t test, and Fisher exact test were performed. A P value less than.05 was considered significant, with power more than 80% for t tests. Surgical Technique Trabeculotomy was performed in the standard technique, which entailed performing a conjunctival peritomy followed by wet field bipolar cautery. A triangular partial-thickness scleral flap was then dissected. A sharp blade was used to perform a graded cut overlying the gray limbus until Schlemm s canal was identified. At that point, a 6-0 polypropylene suture was used to confirm the anatomic location by cannulating Schlemm s canal both nasally and temporally. The Harms trabeculotomes were then introduced nasally and temporally and rotated into the anterior chamber to complete the trabeculotomy. A single 10-0 nylon suture was used to close the scleral flap and the conjunctiva was closed with 8-0 polyglactin 910 suture. Trabeculotomy was first performed on the superior 180 of the angle, with some patients undergoing surgery on the inferior 180 at a later time. For goniotomy the head of the patient was rotated and the surgical microscope tilted to allow optimal visualization of the angle during intraoperative gonioscopy with a Swan Jacob gonioscopy lens. A corneal limbal incision was performed with a microvitrectomy blade followed by injection of acetylcholine chloride to constrict the pupil. Viscoelastic was injected in the anterior chamber and the blade was introduced under direct gonioscopic visualization with an incision being performed in the angle through the trabecular meshwork. The extent of the goniotomy (approximately 5 hours) was determined by the ability to directly visualize angle structures with the gonioscopy lens. The viscoelastic was then washed out of the anterior chamber and a single 10-0 polyglactin 910 suture was used to close the corneal wound. Goniotomy was preferred first 378 Copyright SLACK Incorporated

3 TABLE 1 Characteristics of the Groups at Baseline Group No. of Patients No. of Eyes No. of Surgeries Trabeculotomy Goniotomy Total on the nasal angle and then on the temporal angle when needed. RESULTS In this cohort, 29 patients (50 eyes) were found to have PCG. Seventeen patients were boys and 12 were girls. Of the 50 eyes with PCG, 25 met the inclusion criteria and received trabeculotomy or goniotomy. Of those, 6 were excluded from the analysis for inadequate follow-up time, resulting in 19 eyes that met the inclusion criteria. Characteristics of the trabeculotomy and goniotomy cohorts are shown in Table 1. All patients who received trabeculotomy were bilaterally affected, but only five patients underwent surgery in both eyes, with the other two eyes being controlled medically. In the goniotomy cohort, three of the five patients were affected bilaterally, with two of those patients receiving surgery in both eyes and one being treated with medication. In all but one patient, the fellow eye was treated with the same type of surgery as the original eye. The mean age at surgery for all patients was 12 months, with patients receiving trabeculotomy being younger (8 months) than patients receiving Figure 1. Baseline and postoperative intraocular pressure (IOP) in patients in the trabeculotomy (TR), goniotomy (GO), and combined groups (total). All groups showed a significant reduction of approximately 30% from baseline at 1 and 2 years postoperatively (P <.001). Bars show 95% confidence interval. * = significant difference from baseline. goniotomy (21 months). All patients had 1 year of follow-up with a follow-up rate of approximately 60% at 2 years after surgery. Overall mean IOP reduction from baseline for both groups was 29.5% at 1 year and 33.3% at 2 years, which was a significant decrease from baseline (P <.001) (Figure 1, Table 2). IOP for both the trabeculotomy and goniotomy groups independently was also significantly reduced from baseline to 1 and 2 years (P <.001 and <.05, respectively) (Figure 1, Table 2). When comparing IOP between patients who underwent trabeculotomy versus goniotomy, trabeculotomy had higher IOP than goniotomy at baseline and at 1 and 2 years postoperatively (Table 2), but the difference was not significant at any of the time points (P >.05). When also comparing categories of IOP control (> 5 and < 18, < 21, or < 24 mm Hg) at baseline or 1 Group TABLE 2 Baseline and Postoperative IOP and Percent Reduction in the Groups Baseline 1 Year 2 Years IOP ± SD (mm Hg) All ± 6.1 (n = 27) Trabeculotomy ± 5.7 (n = 16) Goniotomy ± 5.7 (n = 11) IOP ± SD IOP ± SD (mm Hg) Reduction P a (mm Hg) Reduction P a ± 7.6 (n = 27) ± 6.6 (n = 16) ± 8.7 (n = 11) IOP = intraocular pressure; SD = standard deviation a P values represent difference between baseline and postoperative IOP. 30% < ± 7.6 (n = 23) 29% < ± 4.2 (n = 13) 28% < ± 10.7 (n = 10) 33% < % < % <.05 Journal of Pediatric Ophthalmology & Strabismus Vol. 52, No. 6,

Group TABLE 3 Mean No. of Medications at Baseline and 1 and 2 Years After Surgery No. of Meds Baseline 1 Year 2 Years % of Cases (No.) No. of Meds % of Cases (No.) No. of Meds % of Cases (No.) All 0.

4 Group TABLE 3 Mean No. of Medications at Baseline and 1 and 2 Years After Surgery No. of Meds Baseline 1 Year 2 Years % of Cases (No.) No. of Meds % of Cases (No.) No. of Meds % of Cases (No.) All % (6/27) % (9/27) % (9/23) Trabeculotomy 0 0% (0/16) % (2/16) % (2/13) Goniotomy % (6/11) % (7/11) % (7/10) P a < Meds = medications a P values represent difference between trabeculotomy and goniotomy cohorts. Figure 2. Number of cases of postoperative qualified versus absolute success, with qualified success defined as intraocular pressure (IOP) greater than 5 and less than 21 mm Hg with or without medications and absolute success being IOP greater than 5 and less than 21 mm Hg without medications. No significant difference was found between trabeculotomy (TR) and goniotomy (GO) by Fisher exact test at 1 or 2 years postoperatively (P >.05). or 2 years postoperatively, there was no significant difference between the trabeculotomy and goniotomy groups by Fisher exact test (P >.05). The mean number of medications before and after each procedure is shown in Table 3. There was an increasing trend in the number of medications required for a period of time. Most patients were not concurrently treated with medications prior to or after surgical intervention, but the majority of those patients who received medical treatment were in the goniotomy group. Patients who received goniotomy were treated with significantly more medications than trabeculotomy both prior to and after surgery (Table 3) (P <.01 at baseline, P <.001 at 1 year, and P <.001 at 2 years). The rate of absolute success (IOP > 5 and < 21 mm Hg without medication) 1 and 2 years after trabeculotomy was 44% and 62%, respectively. The goniotomy group had absolute success of 37% and 30% at 1 and 2 years of follow-up, respectively. The rate of qualified success (IOP > 5 and < 21 mm Hg with or without medication) at 1 and 2 years after trabeculotomy was 50% and 62%, respectively, whereas goniotomy had rates of 63% and 60%. Therefore, both procedures had comparable rates of qualified success, but trabeculotomy had a higher percentage of patients with absolute success at both 1 and 2 years postoperatively compared to goniotomy (Figure 2). However, this difference was not statistically significant (P >.05, Fisher exact test). DISCUSSION Primary congenital glaucoma can lead to devastating visual consequences for children and inflict significant burden for families and caregivers. Surgical intervention is the primary modality of treatment, with medications being used perioperatively or postoperatively to improve IOP control and slow progressive optic nerve damage and visual loss. 1-7 Only half of our cohort of eyes with PCG received trabeculotomy or goniotomy based on clinical determination by the treating physician that incisional surgery was not recommended due to end-stage disease at the time of presentation to our facility. Few studies have examined the characteristics of patients who received traditional trabeculotomy compared to goniotomy. In our cohort, patients who received trabeculotomy were treated at a younger age (8 months) relative to patients who underwent goniotomy (21 months). We hypothesize that patients with corneal haze are more likely to be noticed by parents and may thus present for treatment at an earlier age. These patients are then more likely to receive trabeculotomy, given that visualization of angle structures is limited by the corneal haze, par- 380 Copyright SLACK Incorporated

5 ticularly when stromal edema is present. Additionally, baseline IOP was higher among patients who underwent trabeculotomy versus goniotomy, which may also have contributed to corneal signs and earlier disease recognition. In our study, there was an increasing medication burden during 2 years in both the trabeculotomy and goniotomy groups. We hypothesize that this was the clinician s attempt to further reduce IOP to a more desirable target without additional surgical intervention. Although most patients did not require medications, those who did had received goniotomy. Given that patients with clearer corneas were more likely to have goniotomy, there was less of a threat of vision loss from deprivation amblyopia, and therefore it may have been more likely that medical management was pursued over surgical intervention. Thus, the rate of absolute success (defined as IOP > 5 and < 21 mm Hg without medications), albeit not statistically significant, was higher in the trabeculotomy group than in the goniotomy group (62% vs 30%, respectively) at 2 years. One may argue that trabeculotomy was a superior procedure in this cohort of patients given that a similar outcome was achieved with goniotomy, but in patients with more severe disease and with less concurrent medical treatment. As reported in the literature and confirmed in our study, trabeculotomy and goniotomy have a similar efficacy in decreasing IOP in patients with PCG. Success rates as high as 87% to 92% for trabeculotomy 3 and 80% for goniotomy 7 when treated within the first year have been reported. These values are compiled from multiple studies that used different definitions of success and variable follow-up durations. Studies comparing trabeculotomy and goniotomy have shown that they are similarly effective at lowering IOP. 1-3,7 Chang and Cavuoto summarized that goniotomy is preferred for patients with good visibility of the structures of the angle. 7 Recent studies have reported improved success with circumferential trabeculotomy with either suture or illuminated tip microcatheter. 8,9 Mendicino et al. showed that trabeculotomy had a higher success rate (34%) compared to goniotomy (92% vs 58%, respectively), with success defined as IOP less than 22 mm Hg with or without medications in a longer term study. 8 Girkin et al. similarly found a greater than 30% higher qualified and unqualified success rate (defined as IOP < 21 mm Hg with approximately 30% reduction in IOP with and without medications, respectively) with microcatheter-assisted circumferential trabeculotomy vs conventional goniotomy (92% vs 54% qualified success, respectively) at 1 year of follow-up. 9 However, it remains challenging to compare outcomes from various studies due to different definitions of success, patient populations, and follow-up. To date, goniotomy and trabeculotomy remain the standard of care procedures for PCG, with few studies on long-term IOP control with newer interventions. In our study, the rates of qualified success at 2 years of follow-up were 62% for the trabeculotomy group and 60% for the goniotomy group. We hypothesize that our qualified success rate was lower than previous reports, likely due to a selection bias of patients with more severe disease being referred to a tertiary care center, as well as loss of patients with better control to their local referring physicians. There are limitations to our study. It is a retrospective analysis and patients were excluded if adequate follow-up data were not available. The small sample size for a rare condition and the goal for long-term follow-up made it difficult to draw additional conclusions. There is a possibility that the success of procedures is underestimated if patients who maintain follow-up are those with more severe disease. A larger multicenter prospective study would be valuable in addressing some of these limitations. Patients who were treated with trabeculotomy had higher IOP and corneal haze and were treated at a younger age than patients in the goniotomy group, who had lower IOPs and were treated at an older age. Increased awareness by ophthalmologists and pediatricians can hopefully promote earlier identification and treatment of PCG. Both procedures yielded a significant reduction in IOP at 1 and 2 years with no significant difference in IOP control between the two surgeries; however, the medication burden increased for a period of time and was greater for patients who received goniotomy than trabeculotomy. It is essential for physicians and caregivers of patients with PCG to recognize the importance of regular follow-up to monitor progression of disease, detect potential side effects of medications, and optimize treatment in hopes of minimizing visual loss. Journal of Pediatric Ophthalmology & Strabismus Vol. 52, No. 6,

6 REFERENCES 1. Papadopoulos M, Cable N, Rahi J, Khaw PT, BIG Eye Study Investigators. The British infantile and childhood glaucoma (BIG) eye study. Invest Ophthalmol Vis Sci. 2007;48: Mandal AK, Chakrabarti D. Update on congenital glaucoma. Indian J Ophthalmol. 2011;59:S148-S Pickering T, Wong P, Dickens C, Hoskins HD. The developmental glaucomas. In: Tasnam W, Jaeger EA, eds. Duane s Ophthalmology. Philadelphia: Lippincott Williams & Wilkins; 2013: Vol 3(51). Accessed online June 3, Brandt JD, Suhr AW. Surgery for pediatric glaucoma. In: Tasnam W, Jaeger EA, eds. Duane s Ophthalmology. Philadelphia: Lippincott Williams & Wilkins; 2013: Vol 6(23). Accessed online June 3, Papadopoulos M, Edmunds B, Fenerty C, Khaw PT. Childhood glaucoma surgery in the 21st century. Eye (Lond). 2014;28: Weinreb RN, Grajewski AL, Papadopoulos M, Grigg J, Freedman S, eds. Childhood Glaucoma. World Glaucoma Association Consensus Series-9. Amsterdam; Kugler Publications; Available at: Accessed online May 22, Chang TC, Cavuoto KM. Surgical management in primary congenital glaucoma: four Debates. J Ophthalmol. 2013; Mendicino ME, Lynch MG, Drack A, et al. Long-term surgical and visual outcomes in primary congenital glaucoma: 3600 trabeculotomy versus goniotomy. J AAPOS. 2000;4: Girkin CA, Rhodes L, McGwin G, Marchase N, Cogen MS. Goniotomy versus circumferential trabeculotomy with an illuminated microcatheter in congenital glaucoma. J AAPOS. 2012;16: Copyright SLACK Incorporated

Institution: Glick Eye Institute, Department of Ophthalmology, Indiana University School of Medicine

Institution: Glick Eye Institute, Department of Ophthalmology, Indiana University School of Medicine Comparison of Circumferential and Traditional Trabeculotomy in Pediatric Glaucoma Maria E. Lim MD, Daniel E. Neely MD, Jingyun Wang, PhD, Kathryn M. Haider MD, Heather A. Smith MD, David A. Plager MD Glick