Management of Congenital Glaucoma with Low Vision Rehabilitation: A Case Study

Abstract:

Background: Congenital glaucoma is a rare but serious ocular condition that can lead to irreversible vision loss if not managed effectively. It frequently manifests in infancy or early childhood and requires a comprehensive strategy combining medical, surgical, and rehabilitation therapies. Early diagnosis, control of intraocular pressure (IOP), and visual rehabilitation are essential for maintaining functional vision and improving the patient's quality of life.

Case Report: This case report covers the therapy of a 10-year-old female diagnosed with congenital glaucoma, who presented severe visual impairment. The patient had a history of several surgical procedures, including goniotomy, trabeculotomy, and peripheral iridectomy, but still experienced increased intraocular pressure and progressive glaucomatous damage. Her right eye exhibited restricted functional vision (BCVA 20/125), whilst her left eye was largely non-functional. Despite regular administration of medicine (Xalatan and Cosopt), her intraocular pressure remained elevated, especially in the left eye. A thorough low vision rehabilitation strategy was executed, integrating optical aids including a 4X spectacle-mounted telescope and a 12.5X stand magnifier, as well as non-optical aids, educational assistance, and social adaptation tactics.

Conclusion: The management of congenital glaucoma comprises not only intraocular pressure control but also vision rehabilitation to maximize functional capabilities. This case highlights the importance of early intervention, continual evaluation, and adaptable strategies to improve the patient's independence and quality of life. Despite a poor visual prognosis, suitable assistive technologies and educational modifications might facilitate young individuals with congenital glaucoma to participate effectively in academic and social activities.

Keywords: Congenital glaucoma, IOP, Low vision rehabilitation, Quality of life.

Introduction:

Congenital glaucoma being a disease with low incidence rate is a rare but serious condition that can lead to significant visual impairment if not managed promptly and effectively (Yu Chan et al., 2015). This case study presents the management of a 10-year-old female patient with congenital glaucoma, highlighting the diagnostic procedures, treatment plan, and rehabilitation strategies employed to improve her quality of life. The case underscores the importance of early intervention, regular follow-up, and the use of optical and non-optical aids in managing congenital glaucoma as stated by (Haddad et al., 2009).

Case Report:

A 10-year-old girl who is enrolled in the sixth grade, came in with her mother and older brother to get a second opinion about how to manage her congenital glaucoma, which she has had for the previous ten years. Given that her grandmother was diagnosed with glaucoma and that her older brother also had congenital glaucoma, her family history is worth noting for glaucoma and may indicate a genetic predisposition. She has been wearing corrective glasses for the past 1.5 months to treat her refractive problems, and her intraocular pressure (IOP) in the right eye (OD) was just measured at 30 mmHg, which is noticeably increased. Concerning visual field abnormalities in the OD, such as complete superior and incomplete inferior arcuate scotomas, were found by a Humphrey Visual Field (HVF) test, suggesting increasing glaucomatous damage. The chronic and complicated nature of her illness is reflected in her surgical history, which includes a goniotomy with peripheral iridectomy (PI) in the left eye (OS) at 6 months of age, followed by trabeculotomy twice in the OS (2012) and once in the OD (2017). To control her IOP, she is currently using Cosopt (Dorzolamide-Timolol) eye drops twice a day and Xalatan (Latanoprost) eye drops before bed. She utilizes a clear balance lens of -5.50 DS in the OS and single-vision CR-39 lenses with a prescription of -10.00/-4.00 x 180 in the OD for vision correction. In order to maintain her remaining eyesight and quality of life, this case highlights the difficulties in managing congenital glaucoma in a young patient, stressing the necessity of careful follow-up, rigorous IOP management, and specialized therapies.

Diagnostic Procedure:

Clinical Findings

The diagnostic evaluation involved multiple assessments to determine the extent of visual impairment.

1) The visual acuity assessment revealed significant impairment in the right eye (OD), with uncorrected vision of 20/760 improving to 20/125 with correction, and near vision recorded at N8 at 8 cm, while the left eye (OS) exhibited only light perception with incorrect projection.

2) Retinoscopy indicated high myopic astigmatism in the right eye, showing a prescription of -11.00/-4.75 x 180, but no reflex was detected; the final correction of -10.00/-4.00 x 180 resulted in a best-corrected acuity of 20/125 and near vision of N8 at 8 cm.

3) External examination noted jerky nystagmus in both eyes, though extraocular movements were intact.

4) Slit lamp examination of the right eye showed a clean cornea, a deep anterior chamber.

5) Intraocular pressure (IOP) measurement recorded 14 mmHg in the right eye and 24 mmHg in the left eye, indicating better pressure control in the right eye.

6) Fundus examination of the right eye revealed complete cupping with 2+ pallor, confirming end-stage glaucomatous damage, while the left eye's fundus was obscured due to media opacity.

7) B-scan ultrasound of the left eye demonstrated low-reflective vitreous opacities and a cataractous, calcified lens, confirming advanced structural degeneration.              These findings collectively illustrate the severe and asymmetric nature of the patient's congenital glaucoma, with the right eye maintaining some functional vision despite extensive damage, while the left eye remains largely non-functional.

Prognosis:

The patient has severe visual impairment due to congenital glaucoma, resulting in significant optic nerve damage. While the prognosis of an individual with congenital glaucoma for substantial vision improvement is poor due to structural abnormalities (Zagora et al., 2015), rehabilitation and the use of low vision aids can help optimize functional vision and enhance daily activities (Deemer et al., 2023).

Treatment Plan:

The treatment plan consists of two key components:

1.     Medication management:

For medication management, the patient will continue using Xalatan (Latanoprost) once at bedtime and Cosopt (Dorzolamide-Timolol) twice daily to regulate intraocular pressure (IOP), despite persistent challenges such as consistently elevated IOP in the left eye.

2.     Low vision rehabilitation: will focus on optimizing functional vision and adaptation strategies.

(a) Optical aids will include a 4X Spec well telescope for distance tasks, a 6X pocket magnifier for mobility, and a 12.5X stand magnifier for detailed near work.

(b) Non-optical aids such as felt-tip pens, a reading stand, tactile maps, and bold-line notebooks will be integrated to enhance daily activities and academic performance.

(c) Educational support will involve counselling for both the patient and her family, teacher training to improve instructional methods, and the use of audiobooks to supplement her studies.

(d) Social adaptation strategies will focus on encouraging telescope use in school, fostering peer support, and creating an inclusive environment to ease her transition and boost confidence in utilizing assistive devices. This comprehensive approach aims to enhance her independence, academic success, and overall quality of life despite her significant visual impairment.

Discussion:

The management of congenital glaucoma requires a multidisciplinary approach, including medical, surgical, and rehabilitative interventions (Neves et al., 2005) . It is a rare but severe form of glaucoma that typically manifests early in life. In this case, despite multiple surgeries, the patient's IOP remained elevated, necessitating the use of multiple glaucoma medications. The use of optical aids, such as telescopes and magnifiers, significantly improved her functional vision, allowing her to continue her education and perform daily activities independently. The presence of a strong family history suggests a potential genetic predisposition. The repeated need for surgical interventions highlights the aggressive nature of the disease (Gogate et al., 2011). Despite surgical and medical management, persistent high IOP and visual field defects indicate progressive glaucomatous damage, necessitating ongoing monitoring and potential adjustments in treatment strategy.

Further management should focus on rigorous IOP control, continued visual field monitoring, and potential consideration of additional surgical or pharmacologic interventions to preserve visual function. Genetic counselling may also be recommended for the family to assess hereditary risk factors (Stone et al., 2012). The patient's reluctance to use the telescope in school due to embarrassment highlights the psychosocial challenges faced by children with visual impairments (White, 2010). Counselling and support from teachers and peers are crucial in overcoming these barriers (Ampadu, 2017). The introduction of tactile maps and talking books further aided her learning process, demonstrating the importance of adaptive technologies in rehabilitation

Conclusion:

This case highlights the importance of early intervention, continuous monitoring, and a multidisciplinary rehabilitation approach in managing congenital glaucoma with low vision(Mandal & Chakrabarti, 2011). Despite a poor visual prognosis, appropriate assistive devices and educational accommodations can significantly improve an individual’s functional abilities and quality of life. 

References: 

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