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One Minute Ophthalmology

Stalagmite iridopathy

Malik, Kunal; Yaghy, Antonio; Shields, Carol L

Author Information
Indian Journal of Ophthalmology: August 2021 - Volume 69 - Issue 8 - p 1993
doi: 10.4103/ijo.IJO_2619_20
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Case

An 18-month-old African-American boy with 1-month history of left upper eyelid swelling and mild conjunctival injection was noted to have an iris lesion in the left eye (OS). On examination, visual acuity was fix-and-follow in both eyes (OU). Intraocular pressures were normal OU. On anterior segment examination OS, the anterior chamber was deep, and there was no keratic precipitate, hyphema, posterior synechia, or ectropion uveae. There was, however, diffuse 360° infiltration on the surface of the iris most notably between 8 and 12 o’clock [Fig. 1a].

Figure 1
Figure 1:
An 18-month-old African-American boy with left upper eyelid swelling and mild conjunctival injection for 1 month was noted to have an iris lesion in the left eye. RetCam imaging showed (a) diffuse infiltration on the surface of the iris most notably between 8 and 12 o’clock. Fluorescein angiography revealed (b) multifocal pinpoint areas of iris neovascularization in the region of the iris infiltration. Anterior-segment optical coherence tomography showed (c) the infiltration to be on the surface of the iris (arrows) with (d) a distinctive “stalagmite-like” configuration as well as diffuse angle involvement

What is your next step?

  1. Perform anterior chamber tap and injection of antibiotics
  2. Provide intra-arterial chemotherapy
  3. Obtain ancillary imaging and perform fine-needle aspiration biopsy (FNAB)
  4. Observe and follow up in 3 months

Findings

Fluorescein angiography (FA) identified multifocal pinpoint areas of iris neovascularization in the region of the iris mass [Fig. 1b]. Ultrasound biomicroscopy confirmed diffuse infiltration on the surface of the iris (arrows) and documented absence of ciliary body or posterior segment involvement [Fig. 1c]. Anterior-segment optical coherence tomography (AS-OCT) depicted epi-iridic debris in a distinctive “stalagmite-like” configuration (arrows) at the site of the mass and diffuse angle involvement [Fig. 1d]. FNAB revealed atypical spindle cell proliferation that stained positive for CD68 (histiocyte marker) and negative for GFAP (astrocytic marker) and HMB-45 and Melan A (melanocyte markers). These findings were compatible with juvenile xanthogranuloma (JXG).

Diagnosis: JXG OS

Correct answer: C

Discussion

Juvenile xanthogranuloma is the most common non-Langerhans cell histiocytosis and is often self-limiting. Ocular disease is estimated in 0.35% of cases with the iris being the most commonly affected tissue.[1] Other sites of JXG include conjunctiva (19%), eyelid (6%), choroid (6%), and orbit (3%).[1] In the eye, JXG can appear nodular or diffuse, as in our case. Both forms pose a risk for iritis, hyphema, and secondary glaucoma.[1] Manjandavida et al. were the first to recognize the distinctive “stalagmite-like” epi-iridic configuration of diffuse JXG on AS-OCT and they surmised that this feature was nearly pathognomonic for this condition, not seen with iris malignancies such as iris melanoma or diffuse anterior retinoblastoma.[23] Others have since confirmed this AS-OCT feature in additional cases.[3] Management of iris JXG involves aggressive topical corticosteroids on an every 2 h basis or even periocular depot corticosteroids until resolution is documented, then a slow taper of topical corticosteroids over 3–4 months to prevent recurrence.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

1. Samara W, Khoo CT, Say EA, Saktanasate J, Eagle RC Jr, Shields JA, et al Juvenile xanthogranuloma involving the eye and ocular adnexa:Tumor control, visual outcomes, and globe salvage in 30 patients Ophthalmology. 2015;122:2130–8
2. Manjandavida FP, Arepalli S, Tarlan B, Shields CL. Optical coherence tomography characteristics of epi-iridic membrane in a child with recurrent hyphema and presumed juvenile xanthogranuloma J AAPOS. 2014;18:93–5
3. Matsunaga D, Williams BK Jr, Desai KB, Shields CL. Extensive “stalagmite“epi-iridic juvenile xanthogranuloma simulating diffuse melanoma in a 16-year-old girl J AAPOS. 2020;24:253–5
© 2021 Indian Journal of Ophthalmology | Published by Wolters Kluwer – Medknow