Congenital Glaucoma Congenital Glaucoma is present at birth and may not be diagnosed until infancy or early childhood. It is characterized by improper development of the eye's aqueous outflow system, leading to increased intraocular pressure (IOP), with consequent damage to ocular structures, resulting in loss of vision. Although the disease is relatively rare, the impact on visual development can be extreme. Early recognition and appropriate therapy of the glaucoma can significantly improve the child's visual future. The cause of this disorder is not completely understood. Most cases of congenital glaucoma are inherited and others are not. The problem of improper drainage of fluid is the result of the lack of full or proper development before birth of the network of cells and tissue that make up the drain. Congenital glaucoma is often diagnosed among babies. It often runs in families because it is inherited. It is quite difficult to predict which babies will be born with this disorder. One risk factor is a family history of congenital glaucoma. If the first and second child have the disease, the risk increases significantly for later children. About twice as many boys as girls are born with congenital glaucoma. It often affects only one eye. Although in three out of every four cases both eyes are may be affected. The diagnosis of primary congenital glaucoma can often be made clinically, even without an accurate measurement of intraocular pressure. The hallmark of the disease, however, is an elevated intraocular pressure in the absence of other conditions that can cause glaucoma, such as Axenfeld-Reiger syndrome or aphakia. The classic clinical triad is epiphora, photophobia, and blepharospasm in the absence of the discharge that typically accompanies a nasolacrimal duct obstruction or conjunctivitis. A positive family history is helpful but often is not present since most cases are sporadic. Although glaucoma cannot be cured, it can be controlled. People with glaucoma need to have regular eye examinations and usually need to continue treatment for the rest of their lives. Untreated acute glaucoma results in permanent vision loss. Untreated chronic glaucoma can progress to blindness within several years. Early diagnosis and treatment usually results in excellent success and vision is saved. The outcome for congenital glaucoma varies depending on the age that symptoms are detected and the child’s response to therapy. The first choice for treating primary congenital glaucoma is almost always surgery. Due to the risk for young children associated with anesthesia, the surgery is often performed at the