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Long-term structural and functional outcomes of primary congenital glaucoma

  • Glaucoma
  • Published:
Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

Abstract

Purpose

To investigate the clinical characteristics and long-term outcomes of primary congenital glaucoma (PCG) patients.

Methods

In this retrospective, longitudinal, cohort study, PCG patients with reliable visual field (VF) tests and optical coherence tomography (OCT) were included. Disease progression was detected using guided progression analysis with OCT and the change analysis of mean deviation (MD) slope with VF tests. Factors associated with the disease progression and visual prognosis were analyzed.

Results

Twenty-nine eyes from 11 bilateral and 7 unilateral PCG patients were enrolled. LogMAR visual acuity declined (0.15 vs. 0.40, P < 0.001). The change rate of the average retinal nerve fiber layer thickness was − 0.83 ± 1.45 µm/year, and 28% of eyes showed glaucoma progression on OCT. The median of the MD slope was 0.16 (− 1.19 to 1.07) dB/year, and 14% of eyes showed glaucoma progression on the VF test. Higher average intraocular pressure (IOP) (P = 0.046) and IOP fluctuation (P = 0.031) predicted disease progression. None of the fellow eyes of unilateral PCG patients developed glaucoma during the follow-up. At last, 59% of eyes had visual acuity > 20/70, and 31% had MD >  − 6 dB. Patients with worse baseline visual acuity (P = 0.027), worse baseline MD (P < 0.001), and smaller neuroretinal rim area (P < 0.001) showed worse final MD values.

Conclusions

Aggressive IOP control is necessary to prevent structural and functional decline in PCG patients. Their fellow eyes are not at risk of glaucoma. Baseline neuroretinal rim area can predict the functional outcome.

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Data availability

Data is available upon reasonable request.

Code availability

Not applicable.

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Acknowledgement

Statistical and writing consultation was provided by the Department of Medical Research at National Taiwan University Hospital.

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Authors and Affiliations

  1. Department of Ophthalmology, National Taiwan University Hospital, No 7, Chung-Shan S. Rd., Taipei, 100, Taiwan

    Yun Hsia, Tso-Ting Lai, Chien-Chia Su, Tsing-Hong Wang & Jehn-Yu Huang

  2. National Taiwan University Hospital Jin-Shan Branch, New Taipei City, Taiwan

    Yun Hsia

  3. Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan

    Tso-Ting Lai

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  1. Yun Hsia
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Correspondence to Jehn-Yu Huang.

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This study was approved by the Institutional Review Board of National Taiwan University Hospital and adhered to the tenets of the Declaration of Helsinki.

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Hsia, Y., Lai, TT., Su, CC. et al. Long-term structural and functional outcomes of primary congenital glaucoma. Graefes Arch Clin Exp Ophthalmol 259, 2317–2326 (2021). https://doi.org/10.1007/s00417-021-05185-1

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  • DOI: https://doi.org/10.1007/s00417-021-05185-1

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