Secondary Congenital Glaucoma
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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.
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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.
For excellent patient education resources, visit eMedicineHealth’s Eye and Vision Center. Also, see eMedicineHealth’s patient education articles Glaucoma Overview, Glaucoma FAQs, and Glaucoma Medications.
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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
Inci Irak Dersu, MD, MPH is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society
Disclosure: Nothing to disclose.
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
Martin B Wax, MD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, Society for Neuroscience
Disclosure: Nothing to disclose.
Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.
Andrew I Rabinowitz, MD Director of Glaucoma Service, Barnet Dulaney Perkins Eye Center
Andrew I Rabinowitz, MD is a member of the following medical societies: Aerospace Medical Association, American Academy of Ophthalmology, American Society for Laser Medicine and Surgery, American Academy of Ophthalmology, American Medical Association
Disclosure: Nothing to disclose.
Secondary Congenital Glaucoma
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