Secondary Congenital Glaucoma



This article discusses developmental glaucomas with associated ocular or systemic anomalies and the most identifiable causes. Aniridia and Peters Anomaly are discussed in other articles.

Glaucoma associated with congenital ocular abnormalities includes the following:

Glaucomas associated with systemic congenital abnormalities include the following:


The main pathology is malformation of the trabecular meshwork and iris (iridotrabeculodysgenesis) or iridocorneal dysgenesis. Numerous iris processes and iridocorneal adhesions could be seen in these diseases. Neovascular glaucoma has been reported in Stickler syndrome. Isolated trabeculodysgenesis is the usual finding in primary congenital glaucoma.



United States

Aniridia is rare, occurring in 1.8 per 100,000 live births; 50% of these patients develop glaucoma. Axenfeld-Rieger syndrome is autosomal dominant and rare; 50% of patients develop glaucoma.[1] Glaucoma occurs in 15% of patients with posterior polymorphous dystrophy. The prevalence of neurofibromatosis-1 (NF-1) is 1 in 3000-5000 people; glaucoma occurs in 1-2% of these patients. Glaucoma occurs in one half of patients with Sturge-Weber syndrome.[2] von Hippel-Lindau occurs in 1 in 22,500 people.


Medical treatment usually fails in secondary congenital glaucoma, and surgery is necessary in most cases.

Associated disorders (eg, corneal opacity, cataract, strabismus) increase the likelihood of amblyopia, unless intervention occurs at an early age.


No racial predilection exists.


No sex predilection exists in aniridia, Axenfeld-Rieger syndrome, Peters anomaly, or phakomatoses.

Lowe syndrome, one of the causes of secondary congenital glaucoma, has X-linked transmission and appears in males.


Glaucoma can appear at any age depending on the underlying condition. For instance, in Peters anomaly, glaucoma is usually present at birth; on the other hand, in Axenfeld-Rieger syndrome, glaucoma may not occur until young adulthood.


Prognosis in secondary congenital glaucoma is guarded.

Earlier age at onset of glaucoma usually is more difficult to manage. Patients need multiple procedures, each of which has its own risks.

Associated ocular problems (eg, strabismus, cataract, microphthalmia, amblyopia) also worsen the prognosis.

In the study by Kargi et al, visual function was evaluated retrospectively with an average follow-up of 11.6 years in 204 eyes of 126 patients who had childhood glaucoma including congenital glaucoma and secondary glaucoma with or without syndrome association.[3]  They found that decreased final visual acuity (less than 20/40 is considered as decreased vision) is strongly correlated with amblyopia and optic nerve damage. Anisometric or strabismic amblyopia was seen, but deprivation amblyopia was the most common type in syndrome-associated glaucoma. Cornea- and lens-associated problems were more common on syndrome-associated glaucoma; therefore, their final visual acuity was worse than other groups at the end of the follow-up period.

In a series by Yang et al of 34 eyes of 19 children with Peters anomaly, IOP control with or without antiglaucoma medicine was achieved in 11 eyes (32%) after 1 or more surgical procedures.[4]  The visual outcome was poor due to glaucomatous optic neuropathy, amblyopia, and other associated anomalies.

Agarwal et al studied 18 eyes of patients with Sturge-Weber syndrome who underwent the combined trabeculotomy-trabeculectomy procedure. The follow-up (mean, 42 mo) results are as follows: IOP was controlled in 11 eyes (61.1%), and visual acuity was better than 6/60 (20/200) in 8 patients.[5]

Patients with Lowe syndrome have a poor life expectancy.

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Axenfeld-Rieger syndrome

Distinct iris and pupil abnormalities are noticed by parents at an early age.

Peters anomaly

Most cases are clinically recognizable in infancy with the loss of corneal clarity due to edema or scar.


Neurofibromatosis (von Recklinghausen disease): Patients seek medical attention for skin or eyelid lesions or eye enlargement.

Sturge-Weber syndrome (encephalofacial angiomatosis): The characteristic presentation is a red facial lesion since birth and is shown in the image below.

View Image

Female infant with Sturge-Weber syndrome. Facial port-wine nevus involves the left eyelid, associated with ipsilateral buphthalmos.

von Hippel-Lindau (retinal angiomatosis)

Patients usually present in their 20s or 30s with benign and malignant tumors in multiple organs.


Most patients present a few months after birth with pupillary abnormalities.

Obtain a full family history, and perform an ocular examination of parents and other relatives. Most cases of aniridia are familial, and it has an autosomal dominant transmission.

Decreased vision, photophobia, nystagmus, and strabismus are the most common clinical manifestations. Because glaucoma develops later in life, enlargement of the cornea is not part of the presentation.


Nanophthalmos is a developmental retardation of the globe after the closure of embryonic fissure. It is usually bilateral. It may be sporadic or transmitted in an autosomal dominant or recessive pattern. The eye is normal in shape but reduced in volume, and the sclera is remarkably thick. Young patients have high hyperopic refraction.


Ocular examination findings for associated conditions are described below.


Decreased visual acuity, pendular nystagmus, corneal pannus, microcornea, focal lens opacity, or lens subluxation may be noticeable.

Intraocular pressure (IOP) is elevated.

Foveal and optic nerve hypoplasia are frequent findings and partially the cause of poor visual acuity.

Gonioscopic examination reveals rudimentary iris, even if there is no visible iris during regular slit lamp examination.

The angle may be closed with peripheral anterior synechiae.

Axenfeld-Rieger syndrome

A prominent, anteriorly displaced Schwalbe line is seen as a white line with slit lamp microscopy.

Microcornea, macrocornea, and corneal opacity may be observed in certain cases.

Iris examination reveals polycoria, corectopia (shown in the image below), and ectropion uvea.

View Image

Axenfeld-Rieger syndrome with iris atrophy, corectopia, and pseudopolycoria.

Iris strands and high insertion of iris (especially in patients with glaucoma) are prominent in gonioscopy.

IOP is elevated when glaucoma develops. In association, strabismus, cataract, macular degeneration, and coloboma may be found.

Peters anomaly

Corneal findings vary from minimal corneal edema to dense corneal leukoma. In some cases, corneal edema may regress with leaving residual scar behind.

Iridocorneal adhesions more commonly are located temporally.

Fifty percent of patients have glaucoma.

Associated anomalies may be present, as follows: anterior polar cataract, microphthalmia, microcornea, sclerocornea, or Axenfeld anomaly.

Neurofibromatosis (von Recklinghausen disease)

Patients have characteristic café au lait spots, plexiform neurofibroma of the upper eyelid, and axillary freckles. If plexiform neurofibroma is present, a 50% chance of developing glaucoma exists.

Glaucoma is always unilateral, usually exists at birth or shortly after, and presents as buphthalmos with or without corneal edema.

Buphthalmos may occur in the absence of elevated pressure due to regional hypertrophy. Buphthalmos is shown in the image below.

View Image

Female patient with plexiform neurofibroma (NF-1). Upper right eyelid involvement, associated with ipsilateral buphthalmos. In Image A (left), patient....

Congenital ectropion uvea sometimes occurs with neurofibromatosis, and it is more likely to be associated with glaucoma.

Lisch nodules (bilateral, yellow greenish, dome-shaped iris elevations) usually appear after patients are 3 years and more frequently as the patient ages.

Pulsating proptosis, choroidal lesions, optic nerve glioma, and optic nerve sheath meningioma are other ocular findings.

Sturge-Weber syndrome (encephalofacial angiomatosis)

Corneal enlargement with or without glaucoma is seen in about two thirds of cases.

IOP is elevated in glaucoma cases.

Conjunctival vessels are dilated.

Retinal vessels appear tortuous, and choroidal hemangioma presents with a tomato catsup appearance at the posterior pole.


Patients have deeply set eyeballs in addition to narrowing of the palpebral fissure. Narrowing of the anterior chamber angles occurs between the fourth and sixth decades due to the short axial length, small cornea, and forward rotation of the lens/iris diaphragm and ciliary body.

von Hippel-Lindau (retinal angiomatosis)

On retinal examination, an elevated globular mass and enlarged feeding artery and vein are seen next to the lesion.

Exudation from the mass causes retinal detachment. Intravitreal and intraretinal hemorrhage causes loss of vision.

Neovascular glaucoma is observed in long-standing cases.

Lowe syndrome (oculocerebrorenal syndrome)

Findings include microphthalmos, strabismus, nystagmus, miosis, and iris atrophy.

Persistent hyperplastic primary vitreous

Persistent hyperplastic primary vitreous (persistent fetal vasculature) consists of microphthalmos, cataract, glaucoma, and retinal detachment that results from persistence and growth of fibrovascular structure anteriorly.

Retinopathy of prematurity

Bilateral glaucoma is caused by a shallow anterior chamber due to contraction of retrolental mass and peripheral anterior synechiae.

Anterior chamber abnormalities, such as a prominent Schwalbe line, have been described.

Glaucoma occurs in late stages, usually after age 2 years.



A defect in the PAX6 gene on chromosome 11 has been identified as the cause of aniridia. It can be sporadic and familial. The sporadic type is associated with Wilms tumor.

Although the name aniridia indicates a total absence of the iris, some amount of the iris stump is commonly found.

The anterior chamber angle closes slowly as the iris strands from the stump progresses toward the angle, which causes clinical manifestations of glaucoma in late childhood or adolescence.

Axenfeld-Rieger syndrome

On postulated pathophysiologic mechanisms that have been abandoned, Axenfeld-Rieger syndrome also was known as anterior chamber cleavage syndrome and mesodermal dysgenesis of the cornea and iris. The basic pathology is the developmental arrest of the neural crest cells during gestation. Because these cells are the origin of facial bones and teeth, abnormalities of these structures are associated with ocular abnormalities.

In Axenfeld anomaly, a prominent, anteriorly (centrally) displaced Schwalbe line (posterior embryotoxon) and iris strands are present that reach to the angle.

When Axenfeld anomaly is associated with glaucoma, it is termed Axenfeld syndrome.

In Rieger anomaly, with or without posterior embryotoxon or iris strands, corectopia (displacement of pupil) is present due to iridocorneal adhesions that are associated with membrane covering angle; polycoria (multiple pupils) due to iris atrophy and hole formation; and ectropion uvea.

Small-sized teeth (microdontia) and decreased number of teeth (hypodontia) of anterior maxillary incisors are observed.

When the whole anomaly is associated with glaucoma, it is termed Rieger syndrome.

Lately, Axenfeld-Rieger syndrome has been used for all types of clinical presentations.

Peters anomaly

Peters anomaly is characterized by bilateral congenital central corneal opacity, which is associated with iridocorneal adhesions toward the defective area.

The peripheral cornea is clear, and the total cornea is of normal size in most cases.

Peters anomaly results from the absence or thinning of endothelium, Descemet membrane, posterior corneal stroma, and sometimes Bowman layer.

Large fibroblastic cells fill into this space and adhere to the counterpart iris section.

Several mechanisms have been proposed, as follows: anoxia, infection (eg, rubella), and a mechanism that is similar to Axenfeld-Rieger anomaly.

Most cases of Peters anomaly have a sporadic origin. A mutation in the PAX6 gene on chromosome 11 has been identified.

The mechanism of glaucoma is reported as a problem in differentiation from neural crest cells, causing trabecular meshwork anomalies, such as absence of the Schlemm canal.

Neurofibromatosis (von Recklinghausen disease)

This is an autosomal disorder that is characterized by skin lesions in peripheral neurofibromatosis (NF-1) or by multiple nervous system tumors (eg, acoustic neurinoma, meningioma) in central neurofibromatosis (NF-2). Sporadic cases have been reported, especially in NF-1. Mutation of chromosome 17 in NF-1 and chromosome 22 in NF-2 has been described.

Pathology: Generally, abnormal neural crest cell proliferation is present. Glaucoma more likely occurs when the ipsilateral upper eyelid is involved with plexiform neurofibroma. It always is unilateral.

The exact mechanism of glaucoma is unknown, but developmental malformation of the angle, peripheral anterior synechiae, infiltration of angle with neurofibromatosis tissue, thickened choroid, and anteriorly extended ciliary processes are pathological findings.

Sturge-Weber syndrome (encephalofacial angiomatosis)

This nonhereditary neurocutaneous syndrome is characterized by facial cutaneous hemangioma (nevus flammeus or port wine stain) that affects first and second divisions of the trigeminal nerve and can result in seizures and mental deficiency.

Hemangiomas may occur bilaterally in 10-30% of cases.

Glaucoma may appear on both eyes, regardless of whether facial hemangioma is unilateral or bilateral.

Choroidal hemangioma (which grows slowly) occurs in 40% of cases and is a cavernous type of hemangioma. Calcification of cortex, especially of occipital lobe, is seen on CT scan of the head.

Mental deficits are observed in 60% of patients.

Hemianopsia and hemiparesis also are common.

The combination of elevated episcleral pressure secondary to small arteriovenous fistulas and developmental angle abnormality causes glaucoma.

von Hippel-Lindau (retinal angiomatosis)

This neurocutaneous disorder is autosomal dominant and is associated with hemangioblastoma of the retina and cerebellum. Renal cell carcinoma also develops later in life.

Average age of onset of the disease is 20-25 years.

Retinal angiomas consist of capillary proliferation, which leaks on fluorescent angiography. Iridocyclitis and neovascularization of the iris cause neovascular glaucoma.

Lowe syndrome (oculocerebrorenal syndrome)

This disorder is X-linked and is characterized by ocular and renal abnormalities and mental retardation.

Ocular disorders include congenital cataract and glaucoma, which are the earliest signs of the syndrome.

Pathology: Glaucoma is secondary to the microphakia and angle abnormalities.

Persistent hyperplastic primary vitreous (persistent fetal vasculature)

Glaucoma is secondary to shallow anterior chamber, which results from cataract formation or pulling of fibrovascular membrane lens-iris diaphragm forward.

Ciliary processes also are pulled forward.

Stickler syndrome

Also called hereditary arthro-ophthalmology, Stickler syndrome is transmitted in an autosomal dominant pattern. Besides glaucoma, other ocular findings include strabismus, amblyopia, high myopia, cataract, vitreoretinal degeneration, and retinal detachment. Glaucoma associated with Stickler syndrome usually responds well to medical treatment. Miotics should be avoided because of the risk of retinal detachment.


Complications include intractable glaucoma despite numerous interventions. Open-angle glaucoma can develop into angle-closure glaucoma or neovascular glaucoma. Eyes eventually can be phthisical.

Sturge-Weber syndrome (encephalofacial angiomatosis)

These eyes carry high risk for expulsive choroidal hemorrhage upon entering the eye causing sudden pressure change.

They are more likely to develop choroidal effusion from choroidal cavernous hemangioma.

Reducing the IOP as low as possible and performing posterior sclerotomies prior to entering the eye may reduce complications.

In a study by Iwach et al, intraoperative choroidal expansion was detected in 24% of patients who underwent trabeculectomy.[6]

In Agarwal's series of 18 patients with Sturge-Weber syndrome who underwent the trabeculotomy-trabeculectomy procedure, the following complications were noted: intraoperative hyphema (22.2%), vitreous loss (16.7%), and vitreous hemorrhage (5.6%).[5]

von Hippel-Lindau (retinal angiomatosis)

This condition consists of capillary proliferation that leaks on fluorescent angiography.

Iridocyclitis and neovascularization of the iris cause neovascular glaucoma.

Laboratory Studies

Aniridia: Chromosome analysis and genetic counseling are important parts of the workup.

Axenfeld-Rieger syndrome: Patients may need workup for associated systemic abnormalities.

Many other systemic anomaly – associated glaucoma conditions require appropriate workup including genetic analysis, laboratory studies, and imaging to diagnose and manage the patient's systemic diseases.

Imaging Studies


The most serious complication of neurofibromatosis type 1 (NF-1) is optic nerve and/or chiasm glioma (in 15% of patients).

An MRI of the orbit and brain is needed to screen for the tumor.

Sturge-Weber syndrome

In Sturge-Weber syndrome, calcium deposits located predominantly in the occipital lobe of the brain parenchyma can be detected by a CT scan. These deposits follow the cerebral convolutions and give the appearance of a railroad track.

Angiomatous malformations, decreased cerebral volume, and increased choroidal plexus volume are the other findings of Sturge-Weber syndrome. An MRI can delineate these findings better than a CT scan.

Other Tests

Peters anomaly

B-scan is needed to evaluate intraocular structures that are obstructed by the corneal opacity.

Electrophysiologic tests occasionally are needed to evaluate the visual potential of the eye prior to making decision on intervention.


Pachymetry, A- and B-scan ultrasonography, and ultrasound biomicroscopy (UBM) are useful in helping to establish a diagnosis.

All glaucoma types

Pachymetry readings are important in all types of glaucoma, including childhood glaucoma, to adjust for IOP readings.

In a small study, the mean central corneal thickness of children with different types of childhood glaucoma was measured. According to this study, in 34 children with glaucoma, IOP was overestimated by 3 mm Hg or more in 41.2% of them. In children with Sturge-Weber syndrome, the mean central corneal thickness was 591.9 +/- 23.1 µm, and, in children with aniridia, the mean central corneal thickness was 754.5 +/- 92.6 µm.[7]

Medical Care

In managing secondary congenital glaucoma, medical therapy is the first-line treatment.

Topical beta-blockers, carbonic anhydrase inhibitors (CAI), and prostaglandin analogs can be used. Alpha-2-adrenergic agonists should be avoided in children younger than 3 years due to possible apnea and other central nervous system adverse effects. Topical beta-blockers should be used very carefully in neonates and should be avoided in premature babies. Timolol should be started low dose as 0.25% initially. Bradycardia and bronchospasm can be a problem in some children.

When beta-blockers are contraindicated, topical CAIs (dorzolamide or brinzolamide bid or tid) can be used. They can still be helpful to add even if the child is taking oral CAIs (oral acetazolamide or methazolamide). Acetazolamide is used with food in divided doses at the dose of 10-20 mg/kg/d. Adverse effects of topical CAIs on graft survival have been questioned.

Miotics are not used as often as they used to be. Pilocarpine is only used before and after goniotomy or trabeculectomy. Echothiophate iodide can still be used successfully in aphakic glaucoma in some cases.

Prostaglandin analogs have not been extensively studied in children. However, they appear to be safe. Lengthening of eyelashes with drops is more excessive in children.

Depending on whether the glaucoma is early or late onset or depending on its severity, patients may respond to treatment, but surgery is necessary in most cases.

Surgical Care

Patients may need multiple procedures for adequate IOP control.

If the cornea is clear, goniotomy or trabeculotomy may be performed first.

Goniotomy works best in primary congenital glaucoma. Children with enlarged cornea and more severe angle deformation and with associated systemic abnormalities do not do as well. Trabeculotomy is advantageous for eyes when the cornea is cloudy and goniotomy cannot be performed.

Trabeculotomy ab interno with the Trabectome (Neomedix) and goniotomy-assisted transluminal trabeculotomy (GATT) are newer options for pediatric glaucomas. In these techniques, a goniolens over the cornea is used to identify the angle structures, requiring the cornea to be clear.

Trabeculectomy, shunt devices for patients with useful vision, and cyclocryotherapy and diode laser cyclophotocoagulation for patients with poor vision can be used.[8]

Trabeculectomy can be used adjunct with antifibrotic agents (5-fluoruracil and mitomycin-c).[9, 10] Antifibrotic agents increase the success rate; however, bleb-related infections are also a risk. Therefore, children with avascular bleb must be closely observed.

Shunt devices are usually used when trabeculectomy fails in adults. In secondary congenital glaucoma, however, particularly in those younger than age 2 years, shunt devices may work better as first-line surgical treatment in comparison to trabeculectomy.

Cycloablation, which includes cyclocryotherapy, transscleral diode laser, or endoscopic diode laser, can be used when medical and other surgical treatments are exhausted.

Repeated cyclodiode treatments may be necessary to control IOP. Compared with adults, the success rate of cyclodiode treatments is lower in children because of their faster recovery of ciliary body function; however, it is still a viable option. In patients with Peters anomaly, performing penetrating keratoplasty early to prevent amblyopia if the corneal opacities are dense is suggested. The result of unilateral keratoplasty generally is poor.

Patients with nanophthalmos may need laser peripheral iridectomy and laser peripheral iridoplasty.

The need for posterior sclerectomies during filtering surgery in patients with Sturge-Weber syndrome has been questioned. Instead, the application of viscoelastic material seemed to reduce the complication rate after decompression of the eye.


Genetic consultation helps to subclassify the diseases and sometimes to predict the prognosis.

Consult with other specialties regarding the existing systemic abnormalities.

Axenfeld-Rieger syndrome: Patients may need workup for associated systemic abnormalities, so referring to a pediatrician or an internist is important.

Phakomatoses have typical ocular findings. Because of their systemic nature, evaluation by the proper specialty is necessary.

For the sporadic type of aniridia, consultation with nephrology is necessary to evaluate the possibility of Wilms tumor.


Obtaining IOP under 19 mm Hg in the pediatric population with glaucoma may prevent optic nerve damage. Treating amblyopia early can achieve good visual outcome in the long term.

Long-Term Monitoring

Closely monitor patients with Axenfeld-Rieger anomaly to detect early signs of developing glaucoma.

Closely monitor patients with neurofibromatosis in childhood. Occurrence of new ocular complications (eg, glaucoma) decreases in adulthood.

Periodically examine patients with neurofibromatosis using gonioscopy to assess for the risk of developing angle-closure component over presence of open angles.

Children with Sturge-Weber syndrome should periodically have complete ocular examinations to detect early signs of ocular complications.

Starting from an early age, periodically monitor children with von Hippel-Lindau disease.

Medication Summary

Medications that decrease the aqueous production or increase the outflow are used as initial treatment in adult-onset secondary congenital glaucoma.

Beta-blockers, parasympathomimetics, alpha-adrenergic agonists, prostaglandin analogs, and carbonic anhydrase inhibitors can be used.

Timolol ophthalmic (Timoptic, Timoptic XE, Betimol)

Clinical Context:  May reduce elevated and normal IOP, with or without glaucoma, by reducing production of aqueous humor or by outflow.

Levobunolol (AKBeta, Betagan)

Clinical Context:  Nonselective beta-adrenergic blocking agent that lowers intraocular pressure by reducing aqueous humor production and possibly increases outflow of aqueous humor.

Class Summary

These agents decrease aqueous production and IOP.

Brimonidine (Alphagan, Alphagan-P 0.15% and 0.10 %)

Clinical Context:  Lowers IOP by decreasing aqueous production and increasing uveoscleral outflow.

Class Summary

These agents decrease IOP.

Latanoprost (Xalatan, Xelpros)

Clinical Context:  May decrease IOP by increasing outflow of aqueous humor.

Bimatoprost ophthalmic solution (Lumigan)

Clinical Context:  A prostamide analogue with ocular hypotensive activity. Mimics the IOP-lowering activity of prostamides via the prostamide pathway. Used to reduce IOP in open-angle glaucoma or ocular hypertension.

Travoprost ophthalmic solution 0.004% (Travatan, Travatan Z)

Clinical Context:  Prostaglandin F2-alpha analog. Selective FP prostanoid receptor agonist believed to reduce IOP by increasing uveoscleral outflow. Used to treat open-angle glaucoma or ocular hypertension.

Unoprostone (Rescula)

Clinical Context:  Prostaglandin F2-alpha analog and selective FP prostanoid receptor agonist. Exact mechanism of action unknown but believed to reduce IOP by increasing uveoscleral outflow.

Class Summary

These agents decrease IOP by increasing uveoscleral outflow.

Pilocarpine ophthalmic (Adsorbocarpine, Akarpine, Isopto Carpine, Pilocar, Pilostat)

Clinical Context:  Increase outflow by pulling the longitudinal part of the ciliary muscle. Indirect-acting miotics are used less commonly.

Class Summary

These agents decrease IOP by increasing aqueous humor outflow.

Acetazolamide (Diamox, Diamox Sequels)

Clinical Context:  Inhibits enzyme carbonic anhydrase, reducing rate of aqueous humor formation, which, in turn, reduces IOP. Acetazolamide increases sickling in patients with sickle cell trait or disease; in these patients, methazolamide may be safer.

Methazolamide (Neptazane)

Clinical Context:  Reduces aqueous humor formation by inhibiting enzyme carbonic anhydrase, which results in decreased IOP.

Brinzolamide (Azopt)

Clinical Context:  Inhibits carbonic anhydrase, which, in turn, leads to a decrease in aqueous humor secretion. May use concomitantly with other topical ophthalmic drug products to lower IOP. If more than one topical ophthalmic drug is being used, administer drugs at least 10 min apart.

Dorzolamide (Trusopt)

Clinical Context:  Used concomitantly with other topical ophthalmic drug products to lower IOP. If more than one ophthalmic drug is being used, administer the drugs at least 10 min apart. Inhibits carbonic anhydrase, which, in turn, leads to a decrease in aqueous humor secretion.

Class Summary

Decrease aqueous production and IOP. May use temporarily before surgery or longer, if patient tolerates it. Concomitant use of topical and systemic carbonic anhydrase inhibitors is not recommended.


Inci Irak Dersu, MD, MPH, Associate Professor of Clinical Ophthalmology, State University of New York Downstate College of Medicine; Attending Physician, SUNY Downstate Medical Center, Kings County Hospital, and VA Harbor Health Care System

Disclosure: Nothing to disclose.

Specialty Editors

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Martin B Wax, MD, Professor, Department of Ophthalmology, University of Texas Southwestern Medical School; Vice President, Research and Development, Head, Ophthalmology Discovery Research and Preclinical Sciences, Alcon Laboratories, Inc

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy, Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Andrew I Rabinowitz, MD, Director of Glaucoma Service, Barnet Dulaney Perkins Eye Center

Disclosure: Nothing to disclose.


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Female infant with Sturge-Weber syndrome. Facial port-wine nevus involves the left eyelid, associated with ipsilateral buphthalmos.

Axenfeld-Rieger syndrome with iris atrophy, corectopia, and pseudopolycoria.

Female patient with plexiform neurofibroma (NF-1). Upper right eyelid involvement, associated with ipsilateral buphthalmos. In Image A (left), patient is aged 8 months; in Image B (right), patient is aged 8 years.

Axenfeld-Rieger syndrome with iris atrophy, corectopia, and pseudopolycoria.

Female patient with plexiform neurofibroma (NF-1). Upper right eyelid involvement, associated with ipsilateral buphthalmos. In Image A (left), patient is aged 8 months; in Image B (right), patient is aged 8 years.

Female infant with Sturge-Weber syndrome. Facial port-wine nevus involves the left eyelid, associated with ipsilateral buphthalmos.