eMedicine Specialties > Ophthalmology > Intraocular Pressure
Glaucoma, Secondary Congenital: Treatment & Medication
Updated: Feb 15, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
In managing secondary congenital glaucoma, medical therapy is the first-line treatment.
Beta-blockers, carbonic anhydrase inhibitors, 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.
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.
- Trabeculectomy, shunt devices for patients with useful vision, and cyclocryotherapy and diode laser cyclophotocoagulation for patients with poor vision can be used.
- Repeated cyclodiode treatments may be necessary to control IOP.
- Compared to adults, the success rate of cyclodiode treatments is lower in children because of their faster recovery from the surgery; however, it is still a viable option, as it has fewer complications in comparison to other surgeries.2
- 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 recently been questioned. Instead, the application of viscoelastic material seemed to reduce the complication rate after decompression of the eye.
Consultations
- 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.
Medication
Medications that decrease the aqueous production or increase the outflow are used as initial treatment in adult-onset secondary congenital glaucoma.
Beta-adrenergic blockers
These agents decrease aqueous production and IOP.
Timolol (Timoptic, Timoptic XE, Betimol)
May reduce elevated and normal IOP, with or without glaucoma, by reducing production of aqueous humor or by outflow.
Adult
Solution: 1 gtt in affected eye(s) bid
Gel: 1 gtt in affected eye(s) qd
Pediatric
Not established; recommended dose is as in adults
May cause bradycardia and asystole when used in combination with systemic beta-blockers (may cause additive effects)
Documented hypersensitivity; bronchial asthma; sinus bradycardia; second- and third-degree AV block; severe chronic obstructive pulmonary disease; overt cardiac failure; cardiogenic shock
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Product may have sulfites, which may cause allergic-type reactions in susceptible patients
Levobunolol (Betagan)
Nonselective beta-adrenergic blocking agent that lowers intraocular pressure by reducing aqueous humor production and possibly increases outflow of aqueous humor.
Adult
0.25% solution: 1-2 gtt in affected eye(s) bid
0.5% solution: 1-2 gtt in affected eye(s) qd; >1 gtt in affected eye(s) bid not shown to be more effective; if IOP not at satisfactory level on this regimen, concomitant therapy can be instituted; do not administer 2 or more topical ophthalmic beta-adrenergic blocking agents simultaneously
Severe or uncontrolled glaucoma: 0.5% solution bid; closely monitor patient
Pediatric
Not established
May cause bradycardia and asystole when used in combination with systemic beta-blockers (may cause additive effects)
Documented hypersensitivity; bronchial asthma; severe chronic obstructive pulmonary disease; sinus bradycardia; second- and third-degree AV block; overt cardiac failure; cardiogenic shock
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Beta-blockade may potentiate muscle weakness that is consistent with certain myasthenic symptoms (eg, diplopia, ptosis, generalized weakness); product may have sulfites, which may cause allergic-type reactions in certain susceptible persons
Alpha2-adrenergic agonists
These agents decrease IOP.
Brimonidine tartrate 0.2% (Alphagan, Alphagan-P 0.15% and 0.10 %)
Lowers IOP by decreasing aqueous production and increasing uveoscleral outflow.
Adult
1 gtt in affected eye(s) bid/tid
Pediatric
Not established; recommended dose is as in adults
Brimonidine increases effect of CNS depressants (eg, alcohol, barbiturates, sedatives), beta-blockers, antihypertensives, and cardiac glycosides; not preferred in very young patients because of CNS toxicity
Documented hypersensitivity; concomitant use of MAOIs
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in severe cardiovascular disease, depression, cerebral and coronary insufficiency, Raynaud phenomenon, and orthostatic hypotension; may lose effectiveness during course of treatment; instruct patients who wear soft contact lenses to wait at least 15 min after instilling to insert contact lenses
Prostaglandin F2-alpha analogs
These agents decrease IOP by increasing uveoscleral outflow.
Latanoprost 0.005% (Xalatan)
May decrease IOP by increasing outflow of aqueous humor.
Adult
1 gtt in affected eye(s) once hs
Pediatric
Not established; recommended dose is as in adults
Coadministration with eye drops containing the preservative thimerosal may reduce effects (administer at intervals of 5 min between applications)
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Instruct patients who wear soft contact lenses to wait at least 15 min after instilling to insert contact lenses; increased inflammation and granulomatous uveitis may occur; do not use in patients with active inflammation; may change eye color by increasing the number of melanosomes in melanocytes (long-term effects of this change are unknown)
Bimatoprost ophthalmic solution (Lumigan)
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.
Adult
1 gtt of 0.03% solution in affected eye(s) hs; not to exceed 1 dose/d
Pediatric
Not established
None reported
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May cause permanent increase in pigment to iris (ie, increases brown pigment) and eyelid; may increase eyelash growth; caution in uveitis or macular edema; do not instill if wearing contact lenses; safety has not been tested in pediatric patients
Travoprost ophthalmic solution 0.004% (Travatan)
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.
Adult
1 gtt in affected eye(s) hs; not to exceed 1 dose/d
Pediatric
Not established
None reported
Documented hypersensitivity; pregnancy
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Commonly causes ocular hyperemia; may cause permanent increase in pigment to iris (ie, increases brown pigment) and eyelid; may increase eyelash growth; caution in uveitis or macular edema; do not instill if wearing contact lenses; safety has not been tested in pediatric patients
Unoprostone (Rescula)
Prostaglandin F2-alpha analog and selective FP prostanoid receptor agonist. Exact mechanism of action unknown but believed to reduce IOP by increasing uveoscleral outflow.
Adult
1 gtt in affected eye(s) bid
Pediatric
Not established
None reported
Documented hypersensitivity; signs of inflammation
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Commonly causes ocular hyperemia; may cause permanent increase in pigment to iris (ie, increases brown pigment) and eyelid; eyelash growth may increase; bacterial keratitis may occur; caution in uveitis or macular edema; do not instill if wearing contact lenses
Miotics
These agents decrease IOP by increasing aqueous humor outflow.
Pilocarpine (Adsorbocarpine, Akarpine, Isopto Carpine, Pilocar, Pilostat)
Increase outflow by pulling the longitudinal part of the ciliary muscle. Indirect-acting miotics are used less commonly.
Adult
1 gtt in affected eye(s) qid
Pediatric
Not established; recommended dose is as in adults
May be ineffective when used in patients who have been treated with topical nonsteroidal anti-inflammatory agents
Documented hypersensitivity; acute inflammatory disease
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in acute cardiac failure, asthma, peptic ulcer, GI spasm, urinary tract obstruction, Parkinson disease, recent MI, and hypotension or hypertension; miotics can cause retinal detachment in susceptible individuals; caution in patients who are disposed to retinal tears; keep signs of toxicity (eg, salivation, lacrimation, sweating, nausea, vomiting, diarrhea) in mind
Carbonic anhydrase inhibitors
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.
Acetazolamide (Diamox, Diamox Sequels)
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.
Adult
500 mg IV q6h initially, followed by 250 mg tab PO qid
Alternatively, 5-10 mg/kg IV q6h
Pediatric
10-15 mg/kg/d PO in divided doses q6-8h
Can decrease therapeutic levels of lithium and alter excretion of drugs (amphetamines, quinidine, phenobarbital, salicylates) by alkalinizing urine
Documented hypersensitivity; hepatic disease; severe renal disease; adrenocortical insufficiency; severe pulmonary obstruction
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Patients with impaired hepatic function may go into coma; may cause substantial increase in blood glucose in some diabetic patients
Methazolamide (Neptazane)
Reduces aqueous humor formation by inhibiting enzyme carbonic anhydrase, which results in decreased IOP.
Adult
25-50 mg PO bid/tid
Pediatric
Not established
May increase toxicity of salicylate, digoxin; coadministration with other diuretics may induce hypokalemia; decreases effects of lithium and alters excretion of other drugs by alkalinizing urine
Documented hypersensitivity; renal impairment
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in respiratory acidosis and diabetes mellitus; impairs mental alertness and/or physical coordination; hematuria, glycosuria, polyuria, hepatic insufficiency, bone marrow suppression, thrombocytopenia/purpura, agranulocytosis, urticaria, pruritus, and rash may occur
Dichlorphenamide (Daranide)
Inhibits enzyme carbonic anhydrase, reducing rate of aqueous humor formation, which, in turn, reduces IOP.
Adult
Initial: 100 mg PO q12h
Maintenance: 25-50 mg PO qd/tid
Pediatric
Not established
Can decrease therapeutic levels of lithium and alter excretion of drugs (amphetamines, quinidine, phenobarbital, salicylates) by alkalinizing urine
Documented hypersensitivity; hepatic disease; severe renal disease; adrenocortical insufficiency; severe pulmonary obstruction
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Patients with impaired hepatic function may go into coma; may cause substantial increase in blood glucose in some diabetic patients
Brinzolamide (Azopt)
Catalyzes reversible reaction involving hydration of carbon dioxide and dehydration of carbonic acid. 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.
Adult
1 gtt in affected eye(s) tid
Pediatric
Not established; recommended dose is as in adults
May have additive systemic effects if patient is already on oral carbonic anhydrase inhibitors
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Local ocular adverse effects, primarily conjunctivitis and lid reactions, may occur with long-term administration (discontinue therapy and evaluate patient before restarting therapy)
Dorzolamide (Trusopt) 2%
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. Reversibly inhibits carbonic anhydrase, reducing hydrogen ion secretion at renal tubule and increasing renal excretion of sodium, potassium bicarbonate, and water to decrease production of aqueous humor.
Adult
1 gtt in affected eye(s) tid
Pediatric
Not established; recommended dose is as in adults
Coadministration with high-dose salicylate therapy may increase toxicity; may have additive systemic effects if patient is already on oral carbonic anhydrase inhibitors
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Local ocular adverse effects, primarily conjunctivitis and lid reactions, may occur with long-term administration of dorzolamide (discontinue therapy and evaluate patient before restarting therapy)
More on Glaucoma, Secondary Congenital |
| Overview: Glaucoma, Secondary Congenital |
| Differential Diagnoses & Workup: Glaucoma, Secondary Congenital |
 Treatment & Medication: Glaucoma, Secondary Congenital |
| Follow-up: Glaucoma, Secondary Congenital |
| Multimedia: Glaucoma, Secondary Congenital |
| References |
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References
Lopes JE, Wilson RR, Alvim HS, Shields CL, Shields JA, Calhoun J, et al. Central corneal thickness in pediatric glaucoma. J Pediatr Ophthalmol Strabismus. Mar-Apr 2007;44(2):112-7. [Medline].
Kirwan JF, Shah P, Khaw PT. Diode laser cyclophotocoagulation: role in the management of refractory pediatric glaucomas. Ophthalmology. Feb 2002;109(2):316-23. [Medline].
Iwach AG, Hoskins HD Jr, Hetherington J Jr, Shaffer RN. Analysis of surgical and medical management of glaucoma in Sturge-Weber syndrome. Ophthalmology. Jul 1990;97(7):904-9. [Medline].
Agarwal HC, Sandramouli S, Sihota R, Sood NN. Sturge-Weber syndrome: management of glaucoma with combined trabeculotomy-trabeculectomy. Ophthalmic Surg. Jun 1993;24(6):399-402. [Medline].
Yang LL, Lambert SR, Lynn MJ, Stulting RD. Surgical management of glaucoma in infants and children with Peters' anomaly: long-term structural and functional outcome. Ophthalmology. Jan 2004;111(1):112-7. [Medline].
Cantor LB. Glaucoma associated with congenital disorders. In: Ritch R, ed. The Glaucomas. Vol 2. St Louis: Mosby; 1989:931-960.
Eibschitz-Tsimhoni M, Lichter PR, Del Monte MA, Archer SM, Musch DC, Schertzer RM, et al. Assessing the need for posterior sclerotomy at the time of filtering surgery in patients with Sturge-Weber syndrome. Ophthalmology. Jul 2003;110(7):1361-3. [Medline].
Facts and Comparisons. Drug Facts and Comparisons. St Louis; 1999.
Hittner HM. Aniridia. In: Ritch R, ed. The Glaucomas. Vol 2. St Louis: Mosby; 1989:869-884.
Schottenstein EM. Peter's anomaly. In: Ritch R, ed. The Glaucomas. Vol 2. St Louis: Mosby; 1989:897-903.
Shields MB. Textbook of Glaucoma. 3rd ed. Baltimore: Williams & Wilkins; 1992:235-257, 348-351.
Shields MB. Axenfeld-Rieger syndrome. In: Ritch R, ed. The Glaucomas. Vol 2. St Louis, Mo: Mosby; 1989:885-95.
Singh OS. Nanophthalmos guidelines for diagnosis and therapy. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. 4. 2000:2846-2859.
Walsh J, Muldoon T. Glaucoma associated with retinal vitreoretinal disorders. In: Ritch R, Shield MB, Krupin T. The Glaucomas. 2. 1996:1055-1071.
Weiss JS, Ritch R. Glaucoma in the phakomatoses. In: Ritch R, ed. The Glaucomas. Vol 2. St Louis: Mosby; 1989:905-29.
Wilson ME, Buckley EG, Kivlin JD. Pediatric Ophthalmology and Strabismus. AAO, Basic and Clinical Science Course. 1998;6:330-345.
Further Reading
Keywords
secondary congenital glaucoma, developmental glaucoma, open angle, closed angle, vision loss, visual deficit, congenital ocular anomalies, congenital ocular abnormalities, systemic anomalies
