What's your diagnosis? Congenital iris ectropion syndrome associated with glaucoma.
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Biomedical subjects
Publications and source records attributed to David S Walton.
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BACKGROUND: Newborn primary congenital glaucoma is an unusual and important subtype of primary congenital glaucoma. Affected patients typically manifest specific signs that facilitate its recognition at birth and become important in the assessment and determination of appropriate treatment for this severe expression of primary congenital glaucoma. PATIENTS AND METHODS: The medical records of 35 patients with newborn primary congenital glaucoma were reviewed to determine its clinical manifestations and response to therapy. The published literature related to newborn primary congenital glaucoma was reviewed and referenced. RESULTS: Newborn primary congenital glaucoma is recognized at birth because of the associated corneal opacification. Ocular examination reveals anterior segment abnormalities of the cornea, iris, and filtration angle as well as related elevated intraocular pressure. Genetic analysis of a subset of patients with newborn primary congenital glaucoma confirmed its relationship with the less severe infantile form of primary congenital glaucoma, which is characterized by favorable results after goniosurgery. In contrast, goniosurgery was found to have unfavorable results and be less successful compared with trabeculectomy and glaucoma drainage tube shunts as initial therapy for newborn primary congenital glaucoma. CONCLUSIONS: Newborn primary congenital glaucoma is an important subtype of primary congenital glaucoma. It can be differentiated from the more frequent and familiar infantile type by history and careful anterior segment examinations to enable and support appropriate choices for its successful surgical treatment.
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We report the second case of infantile glaucoma associated with cranio-cerebello-cardiac (3C) syndrome. An infant with systemic features consistent with 3C syndrome presented with glaucoma and underwent a goniotomy for each eye. Patients with 3C syndrome should be evaluated for glaucoma.
BACKGROUND: It is well-known that lensectomy surgery during the first year of life increases one's risk of aphakic glaucoma. However, it is controversial whether there is a specific time period during the first year of life after which performing lensectomy surgery has a lower risk of aphakic glaucoma development. PATIENTS AND METHODS: A retrospective chart review was performed of all patients seen by a pediatric glaucoma specialist from 1970 to 2003. Patients were included in the study if they underwent congenital cataract surgery. Cataracts were defined as congenital if they were identified within the first 6 months of life, were dominantly inherited, or were of the lamellar type. Aphakic glaucoma was defined as having repeated intraocular pressures > 25 mm Hg after congenital cataract surgery. Patients were excluded if they had any conditions that were independently associated with glaucoma. RESULTS: A total of 368 eyes of 258 patients were included in the study. Of these, 216 (58.7%) eyes of 150 patients developed aphakic glaucoma. Risk factors of greatest significance (P < .0001) included having lensectomy within the first year of life and the development of postoperative complications. CONCLUSIONS: No specific age for lensectomy during the first year of life was associated with a decreased risk for development of aphakic glaucoma. Surgery for congenital cataracts should not be delayed if the only reason for delay is to prevent the development of aphakic glaucoma.
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BACKGROUND AND OBJECTIVE: To better define late complications and long-term visual outcomes associated with lensectomy surgery for pediatric cataracts. PATIENTS AND METHODS: Retrospective review of all patients seen by a pediatric ophthalmologist from 1970 to 2002. Patients were seen at a university-based clinical practice setting. This study defines the incidence of late post-lensectomy complications. Final long-term visual acuities were correlated with specific surgical complications and ocular and systemic anomalies. RESULTS: There were 193 eyes of 138 patients who had lensectomy for pediatric cataracts. The average follow-up time was 10.8 +/- 6.7 years (range, 3 months to 31.3 years). The most common postoperative complication was aphakic glaucoma (20.2%) at an average of 3.4 +/- 3.7 years after lensectomy surgery. At last follow-up, the median visual acuity was 20/40 in eyes without complications and 20/80 in eyes with complications. CONCLUSIONS: Aphakic glaucoma was the most common postoperative complication (20.2%). Most eyes with associated ocular and systemic anomalies and postoperative complications have poorer visual outcomes.