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Case Reports
. 2021 Nov 10;13(1):117-120.
doi: 10.4103/tjo.tjo_35_21. eCollection 2023 Jan-Mar.

Unusual presentation of a vascularized iris lesion in an infant

Sasikala Elizabeth Anilkumar  1 , Parag K Shah  2 , V R Saravanan  3 , Radhakrishnan Shanthi  4 , Narendran Kalpana  1
Affiliations
Case Reports

Unusual presentation of a vascularized iris lesion in an infant

Sasikala Elizabeth Anilkumar et al. Taiwan J Ophthalmol. .

Abstract

We report an unusual presentation of a 10-month-old girl with left eye (LE) redness and watering. Evaluation showed an iris vascular lesion and lens opacity in her LE. Child underwent USG B-scan and ultrasound biomicroscopy, by which an extensive mass lesion arising from iris and ciliary body with absent calcification was revealed. Following extensive evaluation, child underwent cataract extraction and trans-scleral total excision of the mass lesion. Histopathology proved it as juvenile xanthogranuloma (JXG) with vascular proliferation. JXG is a rare benign self-limiting dermatologic disorder affecting mainly infants and small children. Ocular lesions are the most common extracutaneous manifestation. Cataract in JXG is less frequently reported. This case is reported due to its rarity and as it presented solely as an intraocular lesion with combined diffuse infiltration into ciliary body and cataract which is unusual. Early recognition and systematic approach helped in sight saving and organ salvaging.

Keywords: Cataract; diffuse infiltration of iris and ciliary body; histiocytosis; juvenile xanthogranuloma; vascular iris lesion in children.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Clinical photograph of the left eye showing localized pink globular mass with vascularization on iris at 4 o'clock with few nodules on the surface of iris denoted by white arrow head
Figure 2
Figure 2
(a-c) Ultrasound biomicroscopy showing a large heterogenous mass lesion involving ciliary body with pockets of intralesional cyst, involving anterior chamber, angle, and iris with pupillary membrane denoted by black arrow heads
Figure 3
Figure 3
(a-d) Histopathological examination with hematoxylin and eosin staining of the mass lesion obtained from surgical excision. (a) A ×10 magnification demonstrates fragments of pigmented tissue of ciliary body and sclera with minimal hyperplasia of ciliary muscles and pigment epithelium. (b) Touton giant cells (black arrow) with histiocytic infiltration of the iris stroma (×10). (c) A ×40 magnification demonstrates diffuse infiltration of chronic inflammatory cells and xanthomatous histiocytes. (d) Showing stromal infiltration of mixed inflammatory cells eosinophils, histiocytes with spindling (black arrow), and interspersed lymphocytes (×40)
Figure 4
Figure 4
(a-c) Postoperative images. (a) Clinical photograph of the left eye showing healed corneal surgical scar inferiorly, iridectomy, and aphakia. (b) Postoperative fundus picture showing a normal optic disc, macula, and a fibrovascular retinal fold extending anteriorly away from the disc with attached retina. (c) Postoperative ultrasound biomicroscopy showing surgically resolved juvenile xanthogranuloma with no recurrence of lesion
See this image and copyright information in PMC

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