BACKGROUND
Primary congenital glaucoma is the most frequent childhood glaucoma and an important cause of blindness. We describe the current understanding regarding this disease, the evaluation of children with it, and its treatment.
PATIENTS AND METHODS
We accessed information derived from a review of 287 patients with primary congenital glaucoma and current published data related to primary congenital glaucoma.
RESULTS
The nomenclature for childhood glaucoma has been inconsistent, but is clarified for children with primary congenital glaucoma. The epidemiology of primary congenital glaucoma notes its variable incidence worldwide. Familial occurrence supports autosomal recessive transmission; chromosomal loci have been identified, and the CYP1B1 gene has been identified and clinically correlated. The histopathology of eyes with primary congenital glaucoma confirms the presence of a variable trabecular meshwork anomaly and the absence of an imperforate membrane. Children with primary congenital glaucoma are diagnosed after recognition of corneal signs and symptoms of glaucoma.
CONCLUSIONS
The examinations of patients with primary congenital glaucoma must be thorough to distinguish this glaucoma from other types of childhood glaucoma, to prepare for surgery, and to follow progress with treatment. Medical treatment must be tailored to the pediatric patient. Goniosurgery is the definitive procedure of choice for most children with primary congenital glaucoma, but other procedures are also used successfully after goniosurgery fails or is determined to be inappropriate. Future success will be determined by physicians who sustain continued progress for children with glaucoma.
J Pediatr Ophthalmol Strabismus 2004;41:271-288.
AUTHORS
Dr. Ho is from the Singapore National Eye Centre, Singapore, Republic of Singapore. Dr. Walton is from the Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts.
Originally submitted May 5, 2004.
Accepted for publication June 3, 2004.
Address reprint requests to David S. Walton, MD, 2 Longfellow Place, Suite 201, Boston, MA 02114-2224.
The authors have no industry relationships to disclose.
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