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Yeung HH, Walton DS. Clinical Classification of Childhood Glaucomas. Arch Ophthalmol. 2010;128(6):680–684. doi:10.1001/archophthalmol.2010.96
An updated classification of the primary and secondary childhood glaucomas is offered for clinical use, and associated systemic diseases are included to enable their early recognition in children with known glaucoma.
Approximately 650 clinical records of patients with pediatric glaucoma were reviewed for type of glaucoma and associated systemic disease. A literature search was done for additional reported causes of childhood glaucoma. Previous classifications of pediatric glaucomas were also reviewed. Pertinent references to support inclusion of each clinical entity in the updated classification are included.
A comprehensive and referenced classification of the pediatric glaucomas was enabled by this review.
A comprehensive, etiologically based classification of the pediatric glaucomas is now available to assist with the recognition of the many causes of primary and secondary glaucoma in childhood and to support the selection of specific treatment choices.
The childhood glaucomas have been classified by the age of onset, inheritance, associated systemic findings, and anatomy, according to the associated and responsible anterior segment anomalies.1,2 In this article, we offer a comprehensive classification of the childhood glaucomas to assist in the recognition and differential diagnosis of the reported clinically recognizable causes of primary and secondary pediatric glaucomas (Table).
Historically, the childhood glaucomas have been labeled developmental glaucomas based on the associated presence of developmental defects of the eye.1Primary childhood glaucomas will be classified as those caused by anomalies of the filtration angle. These glaucomas are often of genetic origin and may be associated with systemic diseases and other ocular defects. We have identified the systemic diseases that have been described in association with childhood glaucoma.
Congenital glaucoma denotes a glaucoma that occurs early in life related to a congenital anomaly. Newborn primary congenital glaucoma defines a glaucoma entity that is recognized immediately at birth with the presence of profound defects of the anterior segment. Infantile primary congenital glaucoma includes patients with evidence of glaucoma most often recognized in the first year of life. Late recognized primary congenital glaucoma indicates an entity diagnosed significantly after an age when ophthalmologic examination of the patient would have recognized the presence of abnormalities related to glaucoma.
Juvenile glaucoma has been used to describe glaucoma in childhood. We have continued its use specifically with juvenile open-angle glaucoma that characteristically develops during childhood.
The secondary childhood glaucomas are those that occur as the result of independent disease mechanisms that secondarily impair the function of the filtration angle.
All classifications of the childhood glaucomas have revealed the impressive number of clinical entities that may feature or be complicated by childhood glaucoma. Previously these diseases have been variably identified and classified.2 The term association in reference to glaucoma with systemic diseases does not require that the glaucoma is an essential aspect of the disease. In our classification, we list these entities together; however, the strength of the glaucoma relationship might be different. Glaucoma may be coincidental or strongly genetically related to the systemic disease. In the future, additional clinical experience and genetic testing may establish the relative importance of these relationships.
The use of this classification as a clinical aid can facilitate early recognition of glaucoma and identification of the specific glaucoma diagnosis as well as meaningfully influence the choice of treatment. Without recognition of specific types of childhood glaucoma, it is more difficult for the clinician to appropriately select treatment that has been found to be most beneficial. The secondary glaucomas are an important and large group of childhood glaucomas. When confronted with a child with glaucoma and atypical clinical findings, the clinician may be assisted in making an accurate etiologic diagnosis by reviewing the tabulated causes of both the primary and secondary glaucomas. Finally, this childhood glaucoma classification can enable improved communication between those who care for these particular patients.
Correspondence: David S. Walton, MD, 2 Longfellow Place, Ste 201, Boston, MA 02114 (firstname.lastname@example.org).
Submitted for Publication: August 21, 2009; final revision received August 21, 2009; accepted November 12, 2009.
Author Contributions: The authors had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the analysis and presentation.
Financial Disclosure: None reported.