Goniotomy for Juvenile Open-Angle Glaucoma

Yeung, Helen H. BS*; Walton, David S. MD

doi: 10.1097/IJG.0b013e3181a2fa31
Original Study

Purpose: To report the surgical results of goniotomy surgery for patients with acquired juvenile open-angle glaucoma (JOAG).

Patients and Methods: The medical records of 10 patients with bilateral acquired JOAG were reviewed to learn the results of goniotomy for their glaucoma. Twenty goniotomy procedures were performed for 17 eyes. Sex, ethnicity, family history, refraction, preoperative gonioscopic findings, surgical outcome, age at initial goniotomy, duration of postoperative observation, preoperative and postoperative intraocular pressures (IOP), and glaucoma medication used were reviewed. Complete success was defined as an IOP ≤21 mm Hg, qualified success as IOP ≤21 mm Hg with use of glaucoma medications, and failure as IOP >21 mm Hg despite medical therapy. The surgical technique used to perform the goniotomy procedures was reviewed and the absence of significant complications noted.

Results: Overall surgical success was achieved in 77% (13 out of 17) of the eyes. Average IOP for complete success (9 eyes) was 14.7±2.1 mm Hg (range: 12 to 18 mm Hg), qualified success (4 eyes) 16.5±2.4 mm Hg (range: 14 to 19 mm Hg), and failure (4 eyes) 33.5±5.7 mm Hg (range: 30 to 42 mm Hg). The mean age at surgery was 16.3±8.1 years (range: 7.3 to 32 y). Mean follow-up interval was 7.8±6.2 years (range: 0.1 to 16.3 y). Gonioscopy demonstrated normal appearing filtration angles in all eyes. No significant surgical complications occurred. Mean refractive error was −3.3±2.8 (range: 0.0 to −7.8). Sixty percent of patients possessed a family history of JOAG.

Conclusions: Goniotomy is a potentially effective initial surgical treatment of JOAG. Goniotomy for JOAG can be successfully performed using a standard goniotomy technique.

*Tufts University School of Medicine

Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA

Reprints: David S. Walton, MD, Massachusetts Eye and Ear Infirmary, Harvard Medical School, 2 Longfellow Place, Suite 201, Boston, MA 02114 (e-mail: blacki@att.net).

Received for publication January 7, 2008; accepted February 24, 2009

The authors have no financial interest in and received no financial support of any kind for this report.

Article Outline

Juvenile open-angle glaucoma (JOAG) is an acquired autosomal dominantly inherited childhood glaucoma. Its onset is asymptomatic and subtle causing no photophobia and only occasional mild aching eyes or headache. Most occurrences of JOAG are first recognized between the ages of 5 and 18 years.1 It does not cause enlargement of the cornea or breaks in Descemet membrane as seen in more common primary congenital glaucoma. JOAG is always bilateral, but may be worse in one eye than the other.2

JOAG was initially linked to the GLC1A gene on chromosome 1q23-q31.3 The trabecular meshwork inducible glucocorticoid response TIGR) gene (also called MYOC) was reported as the apparent cause of GLC1A glaucoma based on identification of mutations found in patients with JOAG.3,4 Clinically, examination of the anterior segments of patients with JOAG reveals no abnormalities. JOAG is not typically associated with filtration angle anomalies.2 Early recognition depends on tonometry or a positive family history.

Trabeculectomy with and without mitomycin-C has been recommended and reported to be successful surgery for JOAG, however, there is a risk of complications such as hypotony maculopathy, endophthalmitis, and bleb leakage.5 Goniotomy, a minimally invasive glaucoma procedure, has been shown to be an effective treatment for many forms of childhood glaucoma. The purpose of this study is to report the outcomes of the goniotomy procedure for patients with acquired JOAG.

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This study (protocol no. 09-014-0010) was granted approval by the New England Institutional Review Board who determined that this research study of existing data was exempt from New England Institutional Review Board review.

The medical records of all patients (10) with acquired JOAG with a history of goniotomy and cared for by the authors from 1970 to 2006 were reviewed. Glaucoma was diagnosed by the presence of a persistent elevation of the intraocular pressure (IOP) greater than 25 mm Hg. Seventeen of 20 eyes belonging to these 10 patients were treated with the reviewed goniotomy procedures (Table 1). Gonioscopic examination revealed normal appearing filtration angles in all patients except for abnormalities related to previous goniosurgery (Fig. 1). All patients were referred to the authors because of a family history of glaucoma or for management of their acquired glaucoma. No simultaneous bilateral goniotomy procedures were performed. Seven patients had bilateral surgery and 3 eyes had 2 goniotomy procedures. No other initial glaucoma procedure was performed by the authors for any of the 10 patients and no patients were excluded. For 8 out of 10 patients, goniotomy was their initial glaucoma surgery. Prior goniotomy was performed for 3 eyes of 2 patients and prior trabeculectomy was performed for both eyes for 1 patient.

The outcome result was decided by the IOP measurement from at least the last 2 follow-up examinations after surgery or by failure indicated by a persistent IOP elevation. Complete success was defined as an IOP ≤21 mm Hg, qualified success as IOP ≤21 mm Hg with use of glaucoma medications, and failure as IOP >21 mm Hg despite medical therapy. Overall success includes all patients with complete or qualified success. Patients who qualified as successful were followed for an interval of at least 6 months. Office preoperative and postoperative IOP were measured with a Perkins applanation tonometer without use of sedation. Preoperative gonioscopic observations were performed in all eyes using Koeppe lenses. All of the goniotomy procedures were performed by an author (D.S.W.), and general anesthesia was used for each operation.

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Goniotomy Technique

Topical glaucoma medications were discontinued 24 hours before surgery. If acetazolamide was previously prescribed it was stopped 18 hours before surgery. Topical bacitracin/polymyxin ointment was administered to both eyes at bedtime before surgery. Before each procedure, an examination under anesthesia (EUA) of both eyes was performed. The EUA included tonometry, gonioscopy, hand slit-lamp examination, and fundoscopy.

After the EUA, limited apraclonidine HCl 0.5% was administered on a microsurgical sponge to the limbal region of the planned goniotomy, to lessen the immediate postoperative reflux of blood. A standard goniotomy procedure was used with very little modification.6 For all procedures a 2.3× magnification loupe, headlight illuminator, and a nonirrigating needle knife was used. All procedures were performed under direct observation using operating goniotomy lenses.

After preparation of the surgical field, the lashes were secured with tape to prevent contact with the goniotomy knife and to facilitate unimpeded suturing of the cornea. Locking-toothed forceps were placed on the vertical rectus muscle insertions to avoid distortion of the cornea and to enable slight elevation of the eye and cornea away from the lid margins to facilitate entry of the goniotomy knife through clear cornea. Because the corneas were not enlarged a small operating lens was found essential to allow comfortable entry of the knife away from the operating lens to avoid the risk of elevating the lens during the procedure. Initial surgery was always performed in the nasal angle. Passage of the knife across the anterior chamber was visually monitored and its tip engaged into the mid-to-posterior trabecular meshwork followed by a circumferential incision for approximately 5 clock hours. After removal of the operating knife, deepening of the anterior chamber was performed with balanced saline. When the reflux of blood was excessive, epinephrine 1:16,000 was irrigated into the anterior chamber. When necessary the wound was secured with a 10-0 Vicryl (Ethicon, Inc, Somerville, NJ) suture.

Eyes were dressed after administration of topical prednisolone acetate 1%, apraclonidine HCl 0.5%, and an antibiotic ointment. Postoperatively, topical prednisolone acetate 1% was administered every 12 hours for 5 days. Apraclonidine HCl 0.5% was given twice daily when continued postoperative reflux of blood was observed. Patients were asked to sleep with their heads elevated to further discourage blood reflux.

Data is shown as mean±SD unless otherwise noted. Owing to the small number of patients, statistical analysis was not used.

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The medical records of 10 patients who possessed 17 eyes treated with 20 goniotomy procedures were studied (Table 2). The mean age at surgery was 16.3±8.1 years (range: 7.3 to 32 y). The mean preoperative IOP was 32.3±6.7 mm Hg (range: 21.5 to 45 mm Hg) and the mean postoperative IOP was 19.5±8.6 mm Hg (range: 12 to 42 mm Hg). Nine eyes (53%) achieved complete success, 4 eyes (23.5%) qualified success, and 4 eyes (23.5%) experienced failure. Failure occurred in 4 eyes of 2 patients. In 1 patient, failure was associated with ocular trauma 2 months following goniotomy surgery and after 1 goniotomy in the other eye. In the other patient, failure occurred after 2 goniotomies for 1 eye and 1 goniotomy for the other. Goniotomy was not recommended again to those 2 patients who both had been lost to follow up. Fourteen eyes (82%) had 1 procedure, and 3 eyes had 2 procedures, including 2 eyes with complete success and 1 eye with failure. The mean postoperative follow-up was 7.8±6.2 years (range: 0.1 to 16.3 y). Preoperative gonioscopy of the 17 eyes revealed normal filtration angles with identifiable trabecular meshwork, ciliary body band, and scleral spur landmarks.

No significant surgical complications occurred. Minimal reflux hyphema occurred following removal of the goniotomy knife after 13 goniotomies (76%). This was transient with complete spontaneous clearing within a week in all affected eyes.

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The term juvenile related to JOAG has been variably and loosely applied to glaucoma patients recognized between 3 and 50 years of age. Scheie in 19617 refers to JOAG as being a type of primary glaucoma occurring in older children and young adults of 30 years or less of age without signs of infantile glaucoma (buphthalmos). Chandler and Grant8 described JOAG as “primary open-angle glaucoma” in children and “very young adults” and not associated with gonioscopic abnormalities of the angle. In this study, we focused on that JOAG with typical onset in the first 15 years of life, often associated with myopia, and demonstrating normal appearing filtration angles. When familial, our cases demonstrated autosomal dominant inheritance consistent with pedigrees reported associated with the trabecular meshwork inducible glucocorticoid response (TIGR, MYOC) myocilin gene.4

In 1961, Scheie7 described the results of goniopuncture for JOAG in 35 eyes of 23 patients. IOP was normalized in 57% (19 of 35 eyes) by one or more goniopuncture procedures. In 1963, Scheie9 then reported peripheral iridectomy with scleral cautery for treatment of JOAG after failed goniopuncture with controlled IOP in 87% (13 of 15 eyes) with JOAG.

The use of neodymium: yttrium-aluminium-garnet (Nd: YAG) laser for trabeculopuncture in JOAG was reported in 1985 to be effective in controlling IOP and was recommended for use before invasive procedures such as goniosurgery or filtration glaucoma operations.10,11 In 1993, Kjer and Kessing12 reported external trabeculotomy for JOAG in 16 eyes of 11 patients ranging in age of 10 to 45 years. All 13 eyes of patients under 40 years of age maintained a postoperative IOP of 21 mm Hg or less with a mean postoperative follow-up of 7 years. In 2000, Groh et al13 studied the success rate of trabeculotomy in patients with JOAG, in a series consisting of 46 eyes of 41 juvenile glaucoma patients with ages ranging between 11 and 49 years with a mean follow-up of 35 to 46 months. Postoperatively, 84% of the patients had an IOP of less than 23 mm Hg with or without glaucoma medication. Trabeculotomy combined with sinusotomy has also been studied and found to show no additional benefit compared with trabeculotomy alone.14

Trabeculoctomy with and without the use of antimetabolites has been used for JOAG and shown to be effective as an initial surgical treatment.5,15 However, trabeculectomy can be associated with intraoperative and postoperative complications such as flat anterior-chamber, hyphema, bleb-related endophthalmitis, hypotony maculopathy, and the formation of cataracts.5,15–17 In cases when the JOAG has been refractory to previous glaucoma surgeries, drainage implants have been used to control IOP.18,19

More recently, Stangos et al20 showed that primary viscocanalostomy is effective in lowering IOP in cases of medically uncontrolled JOAG. They studied 20 eyes of 20 patients with medical refractory JOAG and found an 80% of overall surgical success associated with no serious postoperative complications.

Goniotomy is most commonly performed for children with infantile primary congenital glaucoma. It has also been found efficacious, however, for other childhood glaucomas, including glaucoma complicating chronic anterior uveitis,21–23 congenital aniridic glaucoma,24 infantile glaucoma secondary to maternal rubella,25 and for prophylaxis against the development of acquired aniridic glaucoma.26 This pilot study reports favorable results of goniotomy for JOAG. Overall success was achieved in 76.5% of eyes and complete success in 53% of eyes at the mean follow-up of 9.1±5.2 years (range: 2.1 to 16 y) unassociated with significant surgical complications.

Although goniotomy is widely recognized as an effective first-line surgery for primary congenital glaucoma, we recommend that it also be considered for initial surgical treatment for uncontrolled JOAG. Goniotomy is less traumatic, has fewer potential surgical complications, and improves aqueous outflow through the drainage system of the eye compared with alternative glaucoma procedures that create an artificial pathway that bypasses this physiologic route.

In conclusion, we report that simple goniotomy is a safe and effective surgical treatment for acquired JOAG. It should be considered the initial procedure of choice because it can restore function to the trabecular meshwork without bypassing it, is the least invasive of the glaucoma surgeries, and does not require manipulation of the conjunctiva which could complicate future trabeculectomy or glaucoma implant surgery if needed for these young patients.

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goniotomy; juvenile open-angle glaucoma; JOAG

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