Newborn primary congenital glaucoma: 2005 update

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.