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 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 812-813

Detached Schwalbe's line in Axenfeld anomaly

Department of Glaucoma, Shroff Charity Eye Hospital, Daryaganj, New Delhi, India

Date of Submission17-Jan-2021
Date of Acceptance28-May-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Darshana Daga
Shroff Charity Eye Hospital, 5027 Kedarnath Lane, Daryaganj, New Delhi - 110 002
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_147_21

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How to cite this article:
Daga D, Dubey S, Garg P. Detached Schwalbe's line in Axenfeld anomaly. Indian J Ophthalmol Case Rep 2021;1:812-3

How to cite this URL:
Daga D, Dubey S, Garg P. Detached Schwalbe's line in Axenfeld anomaly. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 19];1:812-3. Available from: https://www.ijoreports.in/text.asp?2021/1/4/812/327627

A 14-year-old boy presented with best-corrected visual acuity of Oculus Dexter (OD) 6/6p, Oculus Sinister (OS) 6/9, Goldmann applanation tonometry of Oculus Uterque (OU) 14 mm Hg. On examination, the cornea was clear, anterior chamber depth was slightly shallow. Iris had patchy atrophy with a white cord-like structure adhered to the iris [Figure 1], pupil was irregular but reacting to light, the lens was clear. On gonioscopy, angles were open with multiple peripheral anterior synechiae and iris processes [Figure 2]. It also showed a white cord-like structure taking origin from prominent Schwalbe's line, likely a detached Schwalbe's line [Figure 3]. On posterior segment examination, a medium-sized disc with a cup-disc ratio of 0.4:1 with a healthy neuroretinal rim was seen. A diagnosis of anterior segment dysgenesis with  Axenfeld anomaly More Details was made and he was started on 0.5% of timolol maleate twice a day, and was kept on a regular follow-up for monitoring Intra-ocular Pressure (IOP) and disc changes for glaucoma. On follow-up, an IOP in low teens was maintained with 0.5% timolol maleate with no disc changes.
Figure 1: Detached Schwalbe's line (red arrow): white cord-like adhered to iris, with irregular pupil and loss of iris pattern

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Figure 2: Gonioscopic image showing iris processes and anterior insertion of the iris

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Figure 3: Gonioscopic image of white cord-like structure adhered to iris (blue arrow) taking origin from Schwalbe's line

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  Discussion Top

In the Axenfeld anomaly, an anteriorly displaced Schwalbe's line (SL) is seen as a white line in the peripheral cornea near the limbus. A detached SL, which may be an unusual presentation in Axenfeld anomaly was reported similarly by Parikh et al.[1] and Espana et al.[2] Iris and angle structures have a common origin by neural crest cells, which may have impaired migration and differentiation during embryonic development due to the genetic mutation of PITX2 and FOXC1 genes. A band of iris tissue is attached to the thickened SL. The iris seems to be pulled toward the peripheral anterior synechiae, possibly due to the contraction of endothelial cells that migrate across the peripheral anterior synechiae onto the exposed iris stroma. The SL that is attached to the iris is also probably pulled and gets detached.[3],[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

Parikh RS, Parikh SR, Debashish B, Harsha BL, Thomas R. Unusual presentation in Axenfeld-Rieger syndrome. Indian J Ophthalmol Case Rep2011;59:312-4.  Back to cited text no. 1
Espana EM, Mora R, Liebmann J, Ritch R. Bilateral prominent Schwalbe ring in the anterior chamber in a patient with Axenfeld-Rieger syndrome and megalocornea. Cornea 2007;26:379-81.  Back to cited text no. 2
Shields MB. Axenfeld-Rieger syndrome: A theory of mechanism and distinctions from the iridocorneal endothelial syndrome. Trans Am Ophthalmol Soc 1983;81:736-84.  Back to cited text no. 3
Stamper RL, Lieberman MF, Drake MV. Becker-Shaffer's Diagnosis and Therapy of the Glaucomas. 8th ed. Mosby Elsevier; 2019.  Back to cited text no. 4


  [Figure 1], [Figure 2], [Figure 3]


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