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Year : 2020  |  Volume : 32  |  Issue : 3  |  Page : 302-304

A challenging case of refractory childhood glaucoma associated with bilateral congenital ectropion uveae

Glaucoma Services, Sankara Eye Hospital, Bengaluru, Karnataka, India

Date of Submission09-May-2020
Date of Decision03-Jun-2020
Date of Acceptance06-Jun-2020
Date of Web Publication23-Dec-2020

Correspondence Address:
Dr. Meena Menon
Sankara Eye Hospital, Bengaluru, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/kjo.kjo_54_20

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Congenital ectropion uveae (CEU) is a rare disorder, resulting from the proliferation of iris pigment epithelium on the anterior surface of the iris. We report the case of a 3-year-old boy, who was recently diagnosed with glaucoma, on treatment with two topical antiglaucoma medications and referred to us for further evaluation and management. His visual acuity, at presentation, was 6/7.5 (Cardiff) in both his eyes. Slit-lamp examination of both his eyes revealed the presence of ectropion uveae, with intraocular pressure (IOP) of 32 mm Hg. Gonioscopy in both eyes showed anterior insertion of iris. On Fundoscopy, both eyes showed glaucomatous disc changes. He had no systemic associations. Owing to inadequate IOP control, despite optimal medical therapy, he underwent trabeculectomy with intra-operative application of Mitomycin-C. However, the IOP reduction post-trabeculectomy was inadequate, and he subsequently required a bleb needling, which also failed. Hence, he underwent Ahmed glaucoma valve implantation in his right eye, with a favorable final outcome. IOP in the right eye was brought under control following multiple surgical interventions and additional medical treatment. CEU in children can be associated with refractory childhood glaucoma. It is important to evaluate for glaucoma in CEU cases to prevent blindness, especially in young patients and improve their overall prognosis.

Keywords: Ahmed glaucoma valve, bilateral congenital ectropion uveae, refractory childhood glaucoma

How to cite this article:
Harshavardhan V K, Menon M. A challenging case of refractory childhood glaucoma associated with bilateral congenital ectropion uveae. Kerala J Ophthalmol 2020;32:302-4

How to cite this URL:
Harshavardhan V K, Menon M. A challenging case of refractory childhood glaucoma associated with bilateral congenital ectropion uveae. Kerala J Ophthalmol [serial online] 2020 [cited 2022 Dec 8];32:302-4. Available from: http://www.kjophthal.com/text.asp?2020/32/3/302/304557

  Introduction Top

Congenital ectropion uveae (CEU) is a rare, nonprogressive neural crest cells' disorder resulting from proliferation of iris pigment epithelium on the anterior surface of the iris.[1],[2] Usually unilateral, the condition is frequently associated with ipsilateral glaucoma due to iridotrabecular dysgenesis.[1],[2],[3],[4] CEU is reported with various systemic associations of which neurofibromatosis is the most common disorder.[1] Filtering surgery often becomes the effective treatment modality for necessary reduction of intraocular pressure (IOP).[3],[4],[5] This report describes a rare and challenging case of bilateral CEU presenting in early childhood with unilateral refractory glaucoma without any associated systemic disease.

  Case Report Top

A 3-year-old male child was referred for glaucoma management. He was on topical Dorzolamide and Timolol fixed drug combination eye drops for elevated IOP in both eyes.

On examination, visual acuity was 6/7.5 both eyes (Cardiff). There were few prominent nerves noticed in otherwise clear cornea with normal cell morphology in both eyes. Anterior chamber (AC) was normal in depth in both eyes. Iris showed a loss of normal pattern with ectropion uveae present in the entire 360° around pupillary border in the right eye and around 270°–300° in the left eye sparing inferonasal quadrant. This ectropion uveae extended to the mid periphery of the iris and was separated from an otherwise smooth and cryptless, atrophic rest of the iris by a definite demarcation border. IOP measured 32 mm Hg in both eyes. Both pupils were circular with right pupil more dilated than the left pupil. Pupillary reaction was sluggish in both eyes [Figure 1] and [Figure 2]. Gonioscopy revealed anterior insertion of iris over trabecular meshwork in both eyes. On fundoscopy, the cup-disc ratio was 0.8:1 with neuroretinal rim thinning superiorly and inferiorly in the right eye. In left eye, cup-disc ratio was 0.6:1 with vertically oval cup with early neuroretinal rim thinning inferiorly. Other fundus details were within normal limits in both eyes. There were no signs of any systemic association.
Figure 1: Slit-lamp photograph of the right eye

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Figure 2: Slit-lamp photograph of the left eye

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Initially, the patient was advised to commence additional topical Travoprost eyedrops in both eyes along with Dorzolamide and Timolol eyedrops. The IOP was controlled in the left eye. However, IOP was still high in the right eye (28 mm Hg) for which he underwent Trabeculectomy surgery with intraoperative application of 0.4 mg/mL Mitomycin C for 2 minutes. The supero-nasal triangular trabeculectomy flap was closed with single apical suture [Figure 3]. Postoperative medications included tapering doses of topical Prednisolone acetate 1% for 5 weeks, topical Moxifloxacin 0.5%, and Homatropine hydrobromide for 2 weeks each.
Figure 3: Filtering bleb in the right eye

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The IOP was still uncontrolled in the right eye for which he underwent bleb needling along with subconjunctival injection of 0.1 mL of 5-fluorouracil, 7 months posttrabeculectomy. This procedure failed to bring the IOP down to satisfactory level. The patient further underwent Ahmed glaucoma valve (AGV-FP8 model New World Medical, Rancho Cucamonga, CA, USA) implantation with tube entry into anterior chamber, 3 months after bleb needling procedure [Figure 4]. The procedureinvolved corneal traction suture placement, securing the implant with in supero-temporal quadrant using two 9-0 Nylon sutures and covering the tube with donor scleral patch graft. Postoperative medications included tapering doses of topical Prednisolone acetate 1% for 5 weeks, topical Moxifloxacin 0.5%, and Homatropine hydrobromide for 2 weeks each. At the last follow up, 1 year after AGV implantation, IOP in the right eye was under control with additional medical treatment.
Figure 4: Ahmed glaucoma valve implantation in the right eye with tube entry into anterior chamber. Distal end of tube is seen superotemparally

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  Discussion Top

CEU is a disorder resulting from proliferation of iris pigment epithelium on the anterior surface of iris. CEU can present either in early childhood or in adolescence.[1],[2],[6] CEU is unilateral in most of the cases, and there are only few bilateral cases described in the literature.[4],[5] Ritch et al.[1] found glaucoma in seven out of eight cases of CEU and Dowling et al.[2] in eight of nine cases, highlighting the frequent association of glaucoma with CEU. It is important to evaluate all the patients with CEU for glaucoma. CEU is also associated with prominent corneal nerves.[5],[6] The most common association is neurofibromatosis, though other ocular and systemic associations such as facial hemihypertropy, Prader-Willi syndrome, chromosomal abnormalities, and Rieger's syndrome have been described.[1]

In this case, there was a bilateral CEU, prominent corneal nerves and glaucoma, one eye severely affected than the other. The severely affected eye was particularly refractory to treat. The greater extent of CEU may also be the indicator of poor prognosis. There was no systemic association found.

Glaucoma in CEU may be in part due to developmental abnormalities of the angle structures as well as progressive angle closure secondary to abnormal endothelial cell membranes.[7] Most CEU cases only temporarily respond to medical management often necessitating surgical treatment.[2],[3],[4],[5] Wang et al.[7] performed glaucoma drainage device surgery in their series of cases and noted early encapsulation of the plates which often required additional glaucoma surgery (cycloablation or trabeculectomy with mitomycin C) within 4 months of tube implantation.

In this case, the better eye was treated satisfactorily with medical management alone, whereas the severely affected eye was associated with relatively refractory glaucoma which required multiple intraocular surgeries. Target IOP was achieved in the right eye after multiple surgeries along with additional medical treatment and is maintaining the same at the last follow-up, 1 year after the last surgery.

  Conclusion Top

CEU is an ocular abnormality, often missed especially when it is in milder form. However, its frequent association with glaucoma calls for a careful examination and early surgical intervention if required, to preserve good vision. This rare form of refractory glaucoma may require multiple surgeries and warrants close monitoring of intraocular pressures.

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

Ritch R, Forbes M, Hetherington J Jr., Harrison R, Podos SM. Congenital ectropion uveae with glaucoma. Ophthalmology 1984;91:326-31.  Back to cited text no. 1
Dowling JL Jr., Albert DM, Nelson LB, Walton DS. Primary glaucoma associated with iridotrabecular dysgenesis and ectropion uveae. Ophthalmology 1985;92:912-21.  Back to cited text no. 2
Bansal A, Luck J. Primary iris pigment epithelial hyperplasia and glaucoma. Br J Ophthalmol 2002;86:352-3.  Back to cited text no. 3
Hertzberg R. Congenital ectropion uveae and glaucoma. Aust N Z J Ophthalmol 1985;13:45-8.  Back to cited text no. 4
Sethi H, Pal N, Dada T. Bilateral juvenile glaucoma with iridotrabecular dysgenesis, congenital ectropion uveae, and thickened corneal nerves. Eye 2005;19:1347-9.  Back to cited text no. 5
Mandal AK. Late-onset unilateral primary developmental glaucoma associated with iridotrabecular dysgenesis, congenital ectropion uveae and thickened corneal nerves: A new neural crest syndrome? Ophthalmic Surg Lasers 1999;30:567-70.  Back to cited text no. 6
Wang GM, Thuente D, Bohnsack BL. Angle closure glaucoma in congenital ectropion uvea. Am J Ophthalmol Case Rep 2018;10:215-20.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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