UNILATERAL CONGENITAL GLAUCOMA WITH BUPHTHALMOS IN TWO SISTERS - CASE REPORTS WITH LONG TERM FOLLOW-UP.

We report on two siblings with unilateral ipsilateral congenital glaucoma. Both sisters presented with corneal clouding at the time of diagnosis, and one sister presented with Haab lines. In both, the trabeculectomy procedure was performed less than 2 months after diagnosis and within 6 months of life. 

A 5-month-old girl and her parents presented to the Children's Ophthalmology Outpatient Clinic because of a slight corneal clouding noticed by the pediatrician. The family history of ophthalmic diseases was not significant. Anterior fundus examination revealed a single Haab line and corneal clouding in the left eye due to corneal oedema, and the fundus was within normal range for her age. The intraocular pressure (IOP) was 14 mmHg for the right eye and 34 mmHg for the left eye, respectively. The corneal diameter of the left eye was 13.5 mm. Local adjuvant treatment was instituted and a trabeculectomy with iridectomy was performed in the sixth month of the child's life, which resulted in normalization of IOP. The same situation was observed in the patient's sister, who three years later, at the age of 4 months, also came to our outpatient clinic with corneal haze, but without Haab lines on left anterior segment examination. IOP was 13 mmHg and 22 mmHg, respectively. Since attempts to stabilize the IOP with topical medications were unsuccessful, it was decided to perform a trabeculectomy with iridectomy of the left eye in the second patient likewise. In both patients, during the period of almost 15 years of follow-up, there was no recurrence of intraocular pressure elevation, no morphological changes of the retina of the affected eye, which could suggest the occurrence of glaucoma or the progression of the disease, were ever observed.