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Year : 2020  |  Volume : 68  |  Issue : 5  |  Page : 910-911

Developmental glaucoma in a patient of ectrodactyly-ectodermal dysplasia

1 Department of Ophthalmology, Maharaja Agrasen Medical College, Agroha (Hisar), Haryana, India
2 Department of Dentistry, Maharaja Agrasen Medical College, Agroha (Hisar), Haryana, India

Date of Submission05-Aug-2019
Date of Acceptance06-Nov-2019
Date of Web Publication20-Apr-2020

Correspondence Address:
Dr. Parveen Rewri
Department of Ophthalmology, Maharaja Agrasen Medical College, Agroha (Hisar) - 125 047, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1413_19

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Keywords: Developmental glaucoma, ectrodactyly-ectodermal dysplasia, glaucoma valve, keratoplasty

How to cite this article:
Rewri P, Ali W, Grover A, Yadav V. Developmental glaucoma in a patient of ectrodactyly-ectodermal dysplasia. Indian J Ophthalmol 2020;68:910-1

How to cite this URL:
Rewri P, Ali W, Grover A, Yadav V. Developmental glaucoma in a patient of ectrodactyly-ectodermal dysplasia. Indian J Ophthalmol [serial online] 2020 [cited 2020 May 26];68:910-1. Available from: http://www.ijo.in/text.asp?2020/68/5/910/282933

A 23-year-old Indian female, already on intraocular pressure (IOP) lowering eye drops was referred for control of IOP. Systemic involvement was marked for coarse facial features [Figure 1]a, dental and skeletal abnormalities [Figure 1]c, [Figure 1]d, [Figure 1]e, [Figure 1]f, [Figure 1]g suggestive of ectrodactyly-ectodermal dysplasia (EED). The right eye was in exotropia with no perception of light. The left eye had generalized nebular corneal opacification [Figure 1]b, wide-angle recess with numerous iris processes on gonioscopy, and visual acuity of 20/800. Applanation IOP was 28 mm Hg in the right eye and 46 mm Hg in left eye. Pachymetry and axial lengths were 523 μm and 24.32 mm, respectively in the left eye. Hazy view of fundus revealed glaucomatous optic disc changes.
Figure 1: (a) Coarse facial features. (b) Dense corneal opacity in the right eye and the left eye with hazy cornea. (c) Oligodontia. (d) Orthopantomograph showing oligodontia with underdeveloped alveolar bone. (e and f) Ectrodactyly of hands. (g) Ectrodactyly and syndactyly of feet

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After lowering IOP with oral acetazolamide, Ahmed glaucoma valve FP7 (New World Medical, Inc CA) was implanted in superotemporal quadrant. The tube was placed in in the anterior chamber. At 4 months, full-thickness penetrating keratoplasty (PK) was done. In the early postoperative period vision was 20/1000 and IOP varied between 6 to 9 mm Hg [Figure 2]a. At 7 months follow-up after PK, best-corrected visual acuity (BCVA) was 20/80 [Figure 2]b. The corneal graft was clear but cataractous changes were noticed [Figure 2]c. The IOP was 16 mm Hg, and the tube was well placed in the anterior chamber [Figure 2]d with diffuse functional bleb [Figure 2]e. The left eye fundus examination revealed average-sized disc with vertical cup-to-disc ratio of 0.75 and inferior rim thinning [Figure 2]f. At 18-month follow-up, graft was clear, BCVA was 20/50, and IOP was 16 mmHg.
Figure 2: (a) Some edema of the graft 1-week post-keratoplasty (b) Clear graft at 7-month follow-up (c) Clinically significant cataract. (d) Ahmed glaucoma valve FP7 tube in situ (white arrow) in the anterior chamber. (e) Bleb (red arrow). (f) Fundus photo depicting glaucomatous cupping in the left eye

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  Discussion Top

The reported ocular involvement in EED includes telecanthus, blepharophimosis, entropion,  Meibomian gland More Details absence, atresia of lacrimal outflow tract components, ocular surface and tear film disorders, and keratopathy.[1],[2] Infantile glaucoma has been reported with ectodermal dysplasia.[3] The absence of iris anomalies and presence of ectrodactyly differentiate this case from  Axenfeld-Rieger syndrome More Details.[4]

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

Baum JL, Bull MJ. Ocular manifestations of the ectrodactyly, ectodermal dysplasia, cleft lip-palate syndrome. Am J Ophthalmol 1974;78:211-6.  Back to cited text no. 1
Keklikci U, Yavuz I, Tunik S, Ulku ZB, Akdeniz S. Ophthalmic manifestations in patients with ectodermal dysplasia syndromes. Adv Clin Exp Med 2014;23:605-10.  Back to cited text no. 2
Willson II FM, Grayson M, Pieroni D. Corneal changes in ectodermal dysplasia. Case report, histopathology, and differential diagnosis. Am J Ophthalmol 1973;75:17-27.  Back to cited text no. 3
Callea M, Vinciguerra A, Willoughby CE, Deroma L, Clarich G. Infantile bilateral glaucoma in a child with ectodermal dysplasia. Ophthalmic Genet 2013;34:58-60.  Back to cited text no. 4


  [Figure 1], [Figure 2]


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