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Kim, Kim, Cheong, and Yu: Clinical Features and Surgical Outcomes of Congenital Cataracts in Patients With Oculocerebrorenal Syndrome
Original Article

J Korean Ophthalmol Soc 2009;50(5):698-703.

Published online: 7 September 2009

DOI: https://doi.org/10.3341/jkos.2009.50.5.698

Clinical Features and Surgical Outcomes of Congenital Cataracts in Patients With Oculocerebrorenal Syndrome

Jongshin Kim, MD1, Jeong Hun Kim, MD1, Hae Il Cheong, MD, PhD2, Young Suk Yu, MD, PhD1

1Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea

2Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea

Address reprint requests to Young Suk Yu, MD Department of Ophthalmology, Seoul National University Hospital #28 Yeongeon-dong, Jongno-gu, Seoul 110-744, Korea Tel: 82-2-2072-2438, Fax: 82-2-741-3187, E-mail: ysyu@snu.ac.kr

Received 1 September 2008       Accepted 8 January 2009

Copyright © 2009 Korean Ophthalmological Society

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

To describe the clinical features and surgical outcomes of congenital cataracts in patients with oculocerebrorenal syndrome.

Methods

We retrospectively analyzed the age, sex, types of cataracts, operation method and time, perioperative visual acuity, postoperative intraocular pressure, and associated ocular disease in 10 patients (20 eyes) that had been diagnosed with congenital cataracts associated with oculocerebrorenal syndrome and undergone cataract surgery with a minimum follow-up period of 1 year.

Results

All 10 patients were male and had bilateral cataracts detected on their first full ophthalmic examination. The mean age at diagnosis was 2.5 (2.5±1.4) months, and the mean duration of the follow-up period was 4.9 (4.9±4.2) years. The types of cataracts were nuclear sclerosis in 18 eyes of nine patients and cortical opacity in two eyes of one patient. All patients underwent irrigation and aspiration of the lenses, posterior capsulectomy and anterior vitrectomy in both eyes before the age of 1 year. Nine patients (18 eyes) underwent cataract extraction before the age of 6 months, and three patients (6 eyes) underwent the surgery before the age of 2 months. Secondary intraocular lens implantation was done in six eyes of three patients at the age of 34 (34±13.2) months. Postoperative best corrected visual acuity ranged from light perception to 0.15. Out of the nine patients who underwent cataract extraction before the age of 6 months, seven patients could fix and follow objects moderately. The visual acuity of patient who underwent cataract extraction of each eye at the age of 9 months and 12 months was hand motion. In addition to cataracts, glaucoma occurred in three eyes of two patients, corneal opacity in two eyes of one patient and strabismus in three patients.

Conclusions

Visual acuity after cataract surgery was poor in patients with oculocerebrorenal syndrome, especially when the diagnosis and surgery was delayed. Earlier identification and surgical removal of cataracts is recommended. Patients should be monitored regularly for other ocular diseases such as glaucoma, corneal opacity and strabismus.

Keywords: Congenital cataract, Lowe's, syndrome, Oculocerebrerenal syndrome

References

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Table 1.
Overview of cataracts and glaucoma in patients with oculocerebrorenal syndrome
Patient Eye Cataract Diagnosis (mo) I&A‡(mo) 2′ IOL§(mo) BCVA Nystagmus (+/‐) IOP (mmHg) GD∏/ Latest＃ Other surgery or treatment Follow‐up (yr)
1 OD N * 1 2 Mod F&F + 6/9 4
OS N 2 Mod F&F + 6/8
2 OD N 2 2 Mod F&F + 13/10 1
OS N 2 Mod F&F + 13/11
3 OD N 1 2 32 Mod F&F ‐ 6/21 3
OS N 2 27 Mod F&F ‐ 6/12
4 OD N 3 3 Mod F&F + 18/18 7
OS N 3 Mod F&F + 16/17
5 OD N 2 3 0.15 + 6/18 BUL) Frontalis 4
OS N 3 0.2‐1 + 8/11 sling operation
6 OD N 2 3 LP + 6/8 Antiepileptics 3
OS N 3 LP + 6/12
7 OD N 3 3 24 Mod F&F + 8/13 3
OS N 3 25 Mod F&F + 8/12
8 OD N 3 4 50 Mod F&F + 30/13 4
OS N 4 49 Mod F&F + 17/15
9 OD N 1 6 Mod F&F + 6/7 4
OS N 6 Mod F&F + 7/8
10 OD C† 6 12 HM + 32/19 PKPx3, Ahmed 16
OS C 9 HM + 24/91

* N=nuclear sclerosis

† C=cortical opacity

‡ I&A=irrigation & aspiration of cataract

§ 2′ IOL=secondary IOL insertion

∏ GD=mean IOP at glaucoma diagnosis (if not glaucoma, postoperative first mean IOP)

＃ Latest=postoperative latest mean IOP.

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