Primary juvenile glaucoma is glaucoma that develops due to ocular hypertension and is evident either at birth or within the first few years of life. It is caused due to abnormalities in the anterior chamber angle development that obstruct aqueous outflow in the absence of systemic anomalies or other ocular malformation. Presentation
The typical infant who has congenital glaucoma usually is initially referred to an ophthalmologist because of apparent corneal edema. The commonly described triad of epiphora (excessive tearing), blepharospasm and photophobia may be missed until the corneal edema becomes apparent. Systemic associations
Two of the more commonly encountered disorders that may be associated with congenital glaucoma are Aniridia and Sturge-Weber syndrome. Genetics
JOAG is an autosomal dominant condition. The primary cause is the myocilin protein dysfunction. Myocilin gene mutations are identified in approximately 10% of patients affected by juvenile glaucoma. Diagnosis
The diagnosis is clinical. The intraocular pressure (IOP) can be measured in the office in a conscious swaddled infant using a Tonopen or hand-held Goldmann tonometer. Usually, the IOP in normal infants is in the range of 11-14 mmHg. Buphthalmos and Haabs striae can often be seen in case of congenital glaucoma. Differential diagnosis
Corneal cloudiness may have myriad of causes. Corneal opacity that results from hereditary dystrophies is usually symmetric. Corneal enlargement may result from megalocornea, a condition in which the diameter of the cornea is larger than usual and the eye is otherwise normal. Treatment
The preferred treatment of congenital glaucoma is surgical, not medical.
Also, it may be associated with hyponatremia (hypervolemic hyponatremia). Causes
Excessive sodium and/or fluid intake:

IV therapy containing sodium
As a transfusion reaction to a rapid blood transfusion. High intake of sodiumSodium and water retention:

Heart failure
Liver cirrhosis
Nephrotic syndrome
Corticosteroid therapy
Hyperaldosteronism
Low protein intakeFluid shift into the intravascular space:

Fluid remobilization after burn treatment
Administration of hypertonic fluids, e.g. mannitol or hypertonic saline solution
Administration of plasma proteins, such as albumin

Treatment
Treatment includes diuretics, particularly loop diuretics. See also
Volume status
Volume overload
Fluid balance
Edema
Anasarca (swelling of skin)
Pleural effusion (excess fluid in the pleural cavity)
Diuretics
Aquapheresis
Low pressure receptor zones
Apsia

References


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