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Year : 2020  |  Volume : 58  |  Issue : 2  |  Page : 106-108

A case report of late presentation of asymptomatic primary congenital glaucoma

1 Department of Glaucoma, Aravind Eye Hospital, Madurai, Tamil Nadu, India
2 Department of Pediatric Ophthalmology and Strabismus, Aravind Eye Hospital, Madurai, Tamil Nadu, India

Date of Submission21-Feb-2020
Date of Decision21-Apr-2020
Date of Acceptance01-May-2020
Date of Web Publication17-Jun-2020

Correspondence Address:
Dr. Raja Vidya
Department of Glaucoma, Aravind Eye Hospital, No 1 Anna Nagar, Madurai - 625 020, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_10_20

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An asymptomatic 3-year-old child presented with megalocornea, mild corneal haze, high intraocular pressure, and advanced optic disc damage in both eyes. The child was taken up for trabeculectomy and trabeculotomy in the right eye and trabeculectomy alone in the left eye. Due to the failure of the procedure in the right eye, Aurolab aqueous drainage implant was placed. The asymptomatic nature of the child led to the late presentation with advanced disc damage. Therefore, awareness of subtle signs of congenital glaucoma is crucial among the pediatricians for proper screening and referral.

Keywords: Advanced childhood glaucoma, late-onset childhood glaucoma, primary congenital glaucoma

How to cite this article:
Vidya R, Pallamparthy S, Anjanamoorthy R, Sharmila R. A case report of late presentation of asymptomatic primary congenital glaucoma. TNOA J Ophthalmic Sci Res 2020;58:106-8

How to cite this URL:
Vidya R, Pallamparthy S, Anjanamoorthy R, Sharmila R. A case report of late presentation of asymptomatic primary congenital glaucoma. TNOA J Ophthalmic Sci Res [serial online] 2020 [cited 2023 Feb 3];58:106-8. Available from: https://www.tnoajosr.com/text.asp?2020/58/2/106/286922

  Introduction Top

Primary congenital glaucoma is a rare disorder affecting infants and younger children. Its prevalence is high among certain populations of the world, especially where inbreeding and consanguinity are common.[1] It is comparatively less in the Western populations. In India, the presentation is usually severe with near-total to total corneal edema.[2]

  Case Report Top

A 3-year-old female child from Tamil Nadu, India, was brought by her parents to the outpatient department of our tertiary care facility with complaints of discoloration and an increase in the size of both eyes. The child was comfortable with no ocular symptoms. She was born out of nonconsanguineous marriage, full-term spontaneous normal delivery, normal birth weight, and normal milestones. There was no history of medical or surgical intervention. On examination, the child was conscious, nonirritable, and cooperative. There were no signs of photophobia, tearing, or blepharospasm. The vision was not elicited at the visit, but the refraction was done which showed the child to be myopic. Extraocular movements were full and free, and there was no deviation noted in either eye. Torchlight examination showed fairly clear corneas with the visibility of anterior segment structures [Figure 1]. Slit-lamp examination showed megalocornea in both eyes with mild corneal haze and few areas of bluish discoloration of sclera suggestive of areas of scleral thinning. Pupillary examination showed a relative afferent pupillary defect in the right eye. Applanation tonometry revealed an intraocular pressure (IOP) of 58 mmHg in the right eye and 54 mmHg in the left eye with the central corneal thickness of 580 μ and 610 μ in the right and left eyes, respectively. Four-mirror gonioscopic examination showed patchy pigmentation and anterior insertion of the iris in both eyes. Dilated fundus examination showed an optic nerve head with vertical cup disc ratio of 0.9 in the right eye with mild pallor and 0.85 in the left eye. The axial length measured was 26.31 mm in the right eye and 25.29 mm in the left eye. The child was diagnosed with late presentation of primary congenital glaucoma. Topical antiglaucoma medication (2% dorzolamide and 0.5% timolol) was started and was posted for combined trabeculotomy and trabeculectomy in the right eye first [Figure 2]. The complications of the use of Mitomycin C (MMC) are flat anterior chambers, hypotony, choroidal detachments, and endophthalmitis in patients with congenital glaucoma, and its use is probably not justified in primary trabeculectomy.[3] The corneal diameter was measured under anesthesia before the surgery showed vertical and horizontal diameters 15 mm and 15 mm, respectively, in the right eye and 15.5 mm and 15 mm, respectively, in the left eye. The surgery was uneventful, and postoperative IOP by applanation tonometry in the right eye on the 30th postoperative day was 34 mmHg. As there was failure of trabeculotomy in the right eye, trabeculecomy alone was planned in the right eye, 20 days after the procedure in the right eye. Failure of procedure was noted in the right eye with IOP over 30 mmHg in the consequent visits. Hence, Aurolab aqueous drainage implant was placed after 3-month gap of the primary position in the right eye. We could not risk high IOP in the right eye for a prolonged time as there was a threat to vision due to glaucomatous optic atrophy. Milestones of surgical interventions are depicted in [Figure 3]. At present, the child is fixing and following light bilaterally with IOP of 16 mmHg in the right eye and 18 mmHg in the left eye. There was no change in corneal diameter, axial length, and cup–disc ratio.
Figure 1: The corneas appear clear with proper visibility of anterior chamber structures. The pupil was pharmacologically dilated in the photograph

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Figure 2: Photograph showing the right eye after combined trabeculotomy and trabeculectomy

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Figure 3: Flowchart showing surgical interventions done in both eyes

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  Discussion Top

We are reporting the case as it is a rare presentation of primary congenital glaucoma with no obvious signs or symptoms except for the enlargement of the globe. These subtle signs were ignored by the parents which led to the presentation of the child at the advanced stage. Would there have been proper awareness regarding the possibility of glaucoma with such minimal signs, these consequences could have been avoided.

With adequate and timely treatment, primary congenital glaucoma has a good visual prognosis when compared to other pediatric glaucomas. High IOP for a long time can lead to irreversible optic nerve damage, corneal opacity, refractive error, staphyloma, amblyopia, and may lead to loss of vision. It is, therefore, of utmost priority to diagnose congenital glaucoma early and provide adequate treatment. The early detection of the abnormality was not possible in this case, most likely due to asymptomatic nature.

The close differentials of the case were juvenile open-angle glaucoma and megalocornea. Juvenile open-angle glaucoma usually affects the population of ages 5–35 years.[4] It is distinct from primary congenital glaucoma as there is no associated enlargement of the globe or cornea.[5] Another close differential for primary congenital glaucoma is megalocornea. It is an inherited disorder with bilateral symmetrically enlarged clear corneas. The children with megalocornea have normal IOP and clear cornea.

Because of the high presenting IOPs and large cup–disc ratio, combined trabeculotomy and trabeculectomy was advised. This procedure has found to have reasonable success rates when done as primary surgery in congenital glaucoma, especially in severe cases.[6] Combined trabeculotomy and trabeculectomy is less dependent on corneal clarity and deals with the increased trabecular meshwork resistance and makes an additional pathway through the trabeculectomy bleb site.[7] Trabeculectomy, though a popular surgery for adults, is not preferred as primary surgery in younger age groups. Nevertheless, it is preferred by most surgeons as they are familiar with the procedure, and it is technically easier than goniotomy or trabeculotomy. Aurolab aqueous drainage implant (AADI) being a cost-effective device has shown promising results in pediatric glaucoma with control of IOP in the intermediate term and is comparable with Ahmed glaucoma valve in effectiveness and safety profile.[8],[9],[10] Complete success ranges from 75.4 to 89.9 at 1 year and 67.8 to 84.2% at 2 years.[3] Subconjunctival fibrosis as a cause of failure in trabeculectomy is not frequently seen with the tube surgery.

  Conclusion Top

It is important to recognize glaucoma in pediatric patients at an early stage to avoid visual loss and amblyopia. This case highlights the importance of corneal enlargement which can be the only presenting sign of glaucoma in this population. Awareness of such subtle signs is crucial, especially among treating pediatricians to help in early identification and referral.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Elder MJ. Congenital glaucoma in the West Bank and Gaza Strip. Br J Ophthalmol 1993;77:413-6.  Back to cited text no. 1
Dandona L, Williams JD, Williams BC, Rao GN. Population-based assessment of childhood blindness in Southern India. Arch Ophthalmol 1998;116:545-6.  Back to cited text no. 2
Susanna R Jr., Oltrogge EW, Carani JC, Nicolela MT. Mitomycin as adjunct chemotherapy with trabeculectomy in congenital and developmental glaucomas. J Glaucoma 1995;4:151-7.  Back to cited text no. 3
Turalba AV, Chen TC. Clinical and genetic characteristics of primary juvenile-onset open-angle glaucoma (JOAG). Semin Ophthalmol 2008;23:19-25.  Back to cited text no. 4
Kwun Y, Lee EJ, Han JC, Kee C. Clinical characteristics of juvenile-onset open angle glaucoma. Korean J Ophthalmol 2016;30:127-33.  Back to cited text no. 5
Al-Hazmi A, Awad A, Zwaan J, Al-Mesfer SA, Al-Jadaan I, Al-Mohammed A. Correlation between surgical success rate and severity of congenital glaucoma. Br J Ophthalmol 2005;89:449-53.  Back to cited text no. 6
Elder MJ. Combined trabeculotomy-trabeculectomy compared with primary trabeculectomy for congenital glaucoma. Br J Ophthalmol 1994;78:745-8.  Back to cited text no. 7
Puthuran GV, Palmberg PF, Wijesinghe HK, Pallamparthy S, Krishnadas SR, Robin AL. Intermediate-term outcomes of Aurolab aqueous drainage implant in refractory paediatric glaucoma. Br J Ophthalmol 2019. pii: bjophthalmol-2019-314399.  Back to cited text no. 8
Kaushik S, Kataria P, Raj S, Pandav SS, Ram J. Safety and efficacy of a low-cost glaucoma drainage device for refractory childhood glaucoma. Br J Ophthalmol 2017;101:1623-7.  Back to cited text no. 9
Rateb MF, Abdel Motaal H, Shehata M, Anwar M, Tohamy D, Saleh MG. Outcome of a low-cost glaucoma implant versus the baerveldt glaucoma implant for paediatric glaucoma in a tertiary hospital in Egypt. J Ophthalmol 2019;2019:5134190.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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