An Unusual Presentation Of Juvenile Open Angle Glaucoma In A Chinese Girl: A Case Report

1. Siti Hajar Ayub¹
2. Norlina Ramli¹
3. Fong Choong Sian¹
4. Liza-Sharmini AT²
5. Mimiwati Zahari¹

¹University Malaya Medical Centre, Kuala Lumpur, Malaysia
²School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Malaysia

Purpose:
To report a case of Juvenile Open Angle Glaucoma (JOAG) with an unusual presentation.

Methods: Case report

Results:
A healthy 19-year-old Chinese girl with high myopia presented with blurring of both vision and a mild headache for 4 months. Best corrected visual acuities (BCVA) at presentation were HM OD and 6/60 OS. IOP was 44mmHg OD and 42mmHg OS. Both angles were open (Shaffers Grade IV). Both optic discs were fully cupped, with collateral vessels on the left disc. Perivascular sheathing of retinal vesels were seen in both eyes. No signs of uveitis were detected in either eye. There was advanced bilateral visual field loss with markedly reduced RNFL thickness on OCT. The optic disc vessels did not leak on Fundus Fluorescein Angiography. Myocillin gene screening failed to detect any possible mutation. However, substitution of G to A at IVS2 730+35 was found. The role of this polymorphism in susceptibility of JOAG is not known. There was no evidence of any systemic disease. After the IOP was controlled with augmented trabeculectomy, both BCVA improved to 6/12OD, 6/9OS.

Conclusions:
Optic disc collaterals and retinal vessel sheathing are rarely documented in cases of primary glaucoma. Their presence in this case of JOAG may be due to chronic retinal ischemia from long standing high intraocular pressure.