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Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 515-516

A curious case of unilateral buphthalmos: Angle-closure glaucoma secondary to uveitis in a newborn

Glaucoma Department, Narayana Nethralaya-2, Bangalore, Karnataka, India

Date of Submission17-Sep-2020
Date of Acceptance01-Mar-2021
Date of Web Publication02-Jul-2021

Correspondence Address:
Dr. Sushma Tejwani
Glaucoma Services, Narayana Nethralaya-2, Narayana Hrudalaya Complex, #258/A, Bommasandra, Hosur Road, Bangalore, Karnataka - 560 099
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2990_20

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Unilateral secondary angle-closure glaucoma due to uveitis is a rare presentation in a neonate. While the congenital pupillary membranes present predominantly with iris and pupillary changes, glaucoma being a late presentation in a milder form. We report an unusual presentation of a few weeks old baby, having unilateral buphthalmos, normal iris, flat anterior chamber, and elevated intraocular pressure with a pupillary membrane, causing secondary angle-closure glaucoma. There was indirect evidence of maternal infection during the antenatal period with raised IgG titers. The case was managed with anterior chamber reformation, iridectomy along with membranectomy, and did not require trabeculectomy.

Keywords: Angle-closure glaucoma, congenital pupillary-iris membrane, glaucoma in neonate, uveitic glaucoma in children

How to cite this article:
Tejwani S, Kamath V, Sharma N. A curious case of unilateral buphthalmos: Angle-closure glaucoma secondary to uveitis in a newborn. Indian J Ophthalmol Case Rep 2021;1:515-6

How to cite this URL:
Tejwani S, Kamath V, Sharma N. A curious case of unilateral buphthalmos: Angle-closure glaucoma secondary to uveitis in a newborn. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 26];1:515-6. Available from: https://www.ijoreports.in/text.asp?2021/1/3/515/320139

Angle-closure glaucoma in children is an uncommon entity. Iridociliary cysts, retinopathy of prematurity, uveitis, isolated nanophthalmos, relative pupillary block,  Weill-Marchesani syndrome More Details, persistent hyperplastic primary vitreous are some of the entities reported having caused secondary angle closure in young patients.[1] Neonatal uveitis or congenital pupillary membranes usually present with milder forms of glaucoma and the age of presentation is usually after the first year of life.[2],[3] We report an unusual case of secondary angle-closure, causing unilateral buphthalmos of severe grade at 7 weeks of age with normal iris and positive IgG titers.

  Case Report Top

A young non-consanguineous couple brought their 7-week old female infant to our facility with the history of the left eye (LE) prominence since birth. The mother had an uneventful antenatal period with normal vaginal delivery. On examination, baby was fixing and following the light from the right eye (RE) and resisted its occlusion. LE was enlarged with a larger corneal diameter, small pupil, and corneal haze. LE pupil failed to dilate with topical mydriatics, and the pupillary area had a whitish opacity. On examination under anesthesia, the Intraocular pressure (IOP) measured with Perkins tonometer was 8 and 18 mmHg in RE and LE respectively. The horizontal corneal diameter measured 11 mm in RE and 13.5 mm in LE. RE was unremarkable with the normal anterior segment, open angles, clear lens, and normal fundus with CD ratio of 0.3. LE showed 360-degree iridocorneal touch in the periphery with a very shallow anterior chamber (AC) centrally [Figure 1]a and [Figure 1]b. The iris was normal; there was no iris atrophy, membranes or pupillary distortion. LE pupil was 2 mm and there was a white membrane of variable intensity, which appeared like a partially absorbed lens causing occlusio-pupillae. B scan LE showed an echo-free vitreous cavity. On A scan, axial length was 18.1 and 22.92 mm, anterior chamber depth (ACD) was 2.25 and 3.98 mm in RE and LE respectively. The normal ACD on A scan suggested that the white membrane was not the lens. Ultrasound biomicroscopy showed a considerable gap between the posterior corneal surface and the crystalline lens [Supplementary [Figure][Additional file 1] with a highly reflective structure at the pupillary area close to cornea indicating, a membrane causing the pupillary block. Thus, the diagnosis of left eye buphthalmos secondary to pupillary block by inflammatory membrane was made.
Figure 1: (a) Preoperative picture of the child showing the buphthalmic left eye, corneal haze, enlarged corneal diameter, and small pupil with a whitish reflex (arrow). (b) Shows the preoperative slit lamp view of the left eye using a hand-held slit lamp, demonstrating a very shallow anterior chamber throughout

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In view of secondary angle closure in the LE, AC reformation, peripheral iridectomy, and membranectomy were performed. Surgically, the AC was formed with viscoelastics using a side port incision. A 360° synechiolysis was performed with iris repositor and the peripheral iridectomy was done using a vitrectomy cutter. Once the pupillary membrane was removed using pediatric capsulorrhexis forceps and horizontal scissors, a clear crystalline lens and a good fundal glow was observed.

On the first postoperative day, LE had an IOP of 4 mmHg with clear cornea, well-formed AC, clear lens [Figure 2]a and [Figure 2]b and a normal fundus with a cup-disc ratio of 0.4. Both the mother and baby were evaluated systemically and for TORCH group of infections to understand the cause of uveitis. Both were systemically unremarkable, however, the titers of IgG to Rubella, Herpes simplex virus (HSV) I and Cytomegalovirus were raised for both. Six months postoperatively, the child had best corrected visual acuity (BCVA) of 20/260 (2.40 cycles/deg) in RE and 20/470 (1.30 cycles/deg) in LE (by Teller Acuity cards-II at 55 cm) with refraction of + 1.25 DS in the RE and -11.0 DS in the LE. The left eye had a well-formed AC, patent iridectomy and well-controlled IOP of 12 mm Hg. The parents were advised to start glasses and amblyopia therapy with part-time occlusion of the RE for the child. At the last follow-up of 18 months, BCVA improved to 20/380 (1.6 cycles/deg) in the LE with clear visual axis, deep AC, and IOP of 14 mmHg.
Figure 2: (a) Postoperative picture of the left eye showing clear cornea, mid-dilated pupil (arrow) with no white reflex, and a patent peripheral iridectomy (arrow). (b) Shows the postoperative slit lamp view of the left eye using a hand-held slit lamp, demonstrating the deep and formed anterior chamber

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  Discussion Top

Uveitis in neonatal life can be due to intrauterine Toxoplasma, Rubella, Cytomegalovirus, Herpes simplex virus, Treponema pallidum, Varicella-zoster virus, and Epstein-barr virus.[3],[4] Since these infections cause very mild illness in the mother, the symptoms are often unnoticed, but the significant impact gets detected in child at birth. In a baby, the production of IgG does not start until 6 months age; therefore, the raised IgG levels in a child less than that, are from trans-placental transfer from the mother.[3] In our case, Herpes and cytomegalovirus were positive suggestive of subclinical infection in the mother during pregnancy with the possibility of uveitis in the fetus.

Uveitis in utero or in infancy is a known cause for damage to the outflow mechanism leading to rise in the IOP which in turn causes a gradual increase in the size of the eyeball.[5] The occlusio-pupillae caused by the membrane causes a rise in the pressure in the posterior chamber pushing the iris diaphragm anteriorly thus making the AC shallow. Though this presentation may be seen in adults, it is rare in a newborn.[4]

Further, the cause of angle closure in the younger population may be typically associated with structural or developmental ocular anomalies rather than relative pupillary block.[6] The entity congenital pupillary-iris-lens membrane has been described by Cibis et al.[7] and also reported by Richard Robb[2] with no maternal or systemic issues and unilateral presentation. However those cases were usually associated with iris atrophy, eccentric pupil, iris membranes attachment to lens causing deprivational amblyopia, and not reported to cause such an advanced degree of buphthalmos with angle closure and flat anterior chamber at this age.

  Conclusion Top

We have presented a unique case of unilateral buphthalmos with angle-closure glaucoma secondary to uveitis in a newborn

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Chang BM, Liebmann JM, Ritch R. Angle closure in younger patients. Trans Am Ophthalmol Soc 2002;100:201-12; discussion 212-4.  Back to cited text no. 1
Robb RM. Fibrous congenital iris membranes with pupillary distortion. Trans Am Ophthalmol Soc 2001;99:45-51.  Back to cited text no. 2
Mets MB, Chhabra MS. Eye manifestations of intrauterine infections and their impact on childhood blindness. Surv Ophthalmol 2008;53:95-111.  Back to cited text no. 3
Saxena RB, Gadhvi KM. Neonatal uveitis. Indian J Ophthalmol 1971;19:80-1.  Back to cited text no. 4
[PUBMED]  [Full text]  
Pollock IP. Tran of the New Orleans Acad. of Ophth. P. 49. C. V. Mosby Co., Sant (cited in 4).  Back to cited text no. 5
Ritch R, Chang BM, Liebmann JM. Angle-closure in younger patients. Ophthalmology 2003;110:1880-9.  Back to cited text no. 6
Cibis GW, Waeltermann JM, Hurst E, Tripathi RC, Richardson W. Congenital pupillary-iris-lens membrane with goniodysgenesis (A new entity). Ophthalmology 1986;93:847-52.  Back to cited text no. 7


  [Figure 1], [Figure 2]


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