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 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 720-723

An unusual case of juvenile xanthogranuloma presenting as a subconjunctival nodule with review of literature

1 Department of Oculoplasty and Ocular Oncology Services, Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pediatric Ophthalmology Services, Chacha Nehru Bal Chikitsalya, Geeta Colony, New Delhi, India
3 Department of Ocular Pathology Services, Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

Date of Submission02-Dec-2020
Date of Acceptance25-May-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Meenakshi Wadhwani
Department of Pediatric Ophthalmology Services, Room No 228, Chacha Nehru Bal Chikitsalya, Geeta Colony, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_3587_20

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Juvenile xanthogranuloma (JXG) is a benign histiocytic disorder that belongs to the non-Langerhans cell group. Extracutaneous involvement in the eye usually involves the iris and presents as recurrent spontaneous episodes of hyphema or secondary glaucoma. A 3-year-old child presented with a subconjunctival mass in the left eye and concurrent scalp nodules. Ultrabiomicroscopic examination showed a moderately reflective episcleral mass with extension into cornea but no scleral involvement. Histopathology of the excised subconjunctival mass showed a dense infiltrate of polygonal/spindle mononuclear cells with moderate amounts of cytoplasm and scattered Touton giant cells, suggestive of JXG. Systemic evaluation did not reveal any other sites of involvement. Postoperatively, the patient was treated with topical steroids. At last follow-up 2.5 years, the patient is free on any recurrence.

Keywords: xanthogranuloma, juvenile, touton giant cells

How to cite this article:
Meel R, Wadhwani M, Kashyap S. An unusual case of juvenile xanthogranuloma presenting as a subconjunctival nodule with review of literature. Indian J Ophthalmol Case Rep 2021;1:720-3

How to cite this URL:
Meel R, Wadhwani M, Kashyap S. An unusual case of juvenile xanthogranuloma presenting as a subconjunctival nodule with review of literature. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2022 Jan 19];1:720-3. Available from: https://www.ijoreports.in/text.asp?2021/1/4/720/327680

Juvenile xanthogranuloma (JXG) is a cutaneous granulomatous disease that occurs primarily in infants but may occasionally affect older children and adults. It is characterized by orange red macules or papules that occur on face, neck, and upper trunk.[1] These skin lesions generally resolve spontaneously between 1 and 5 years of age. Histopathologically, they are characterized by the presence of touton giant cells; absence of Langerhans cell histiocytes helps to differentiate the benign lesion from the more serious histopathological disorder known as Langerhans' cell histiocytosis.[1],[2] Herein, we report a case of JXG presenting in a 3-year-old child as a subconjunctival mass and also describe the imaging features of the same on ultrasound biomicroscopy (UBM).

  Case Report Top

A 3-year-old male child presented with an ocular surface mass of 6 months duration. It was not associated with any ocular congestion or pain, and there were no other systemic complaints. On examination, it was 15 × 5.5 mm, smooth-surfaced, well-defined, pinkish mass located at superior limbus of the left eye with a 3 mm extension into the adjacent clear cornea [Figure 1]a and [Figure 1]b. Cardiff visual acuity was 6/9 in both the eyes. The anterior segment and fundus were normal on slit-lamp biomicroscopy and indirect ophthalmoscopy, respectively. Two yellowish red nodular lesions were also noted on the scalp [Figure 1]c. UBM of the ocular mass revealed a homogeneous mass with moderate echogenicity reaching a height of 5.5 mm and no scleral extension [Figure 1]d. Complete haemogram and peripheral blood smear were performed and found unremarkable. The subconjunctival nodule was excised after lifting and dissecting under the overlying conjunctiva and leaving the cornea untouched [Figure 1]e. On histopathological examination, the mass was composed of a dense infiltrate of polygonal or spindle mononuclear cells with moderate amounts of cytoplasm and scattered Touton giant cells suggestive of JXG [Figure 1]f. Systemic evaluation conducted by a pediatrician including computed tomographic scan head, ultrasound abdomen, and bone marrow aspirate was within normal limits. There was no associated central nervous system lesion. A diagnosis of JXG was made. Postoperatively, the child was treated with steroid eye drops and ointment. There was regression of residual corneal lesion with corneal opacification. At last follow-up of two-and-half years, the child was free of any ocular recurrence or systemic disease [Figure 1]g, [Figure 1]h.
Figure 1: Clinical photograph showing (a) and (b) pinkish mass at the superior limbus extending into cornea, (c) yellowish pink scalp lesions (arrow), (d) UBM of the subconjunctival nodule showing a homogeneous mass with moderate intensity echoes, (e) intraoperative photograph showing the lesion more clearly, (f) microphotograph showing low-power view showing numerous histiocytes along with few capillaries (H & E Stain, × 100) Touton giant cells (arrows) (H & E Stain X200), (g) and (h) Clinical photo at last follow-up, showing residual conjunctival discoloration with corneal opacity

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  Discussion Top

Mc Donaugh, a dermatologist, first reported the skin lesions of JXG as nevo xantho endothelioma in 1909.[2] Subsequent studies found that nearly 10% of cases of JXG have associated ophthalmic presentation. In eye and adnexa, lesions of JXG may affect iris, ciliary body, optic nerve, eyelids, and sometimes orbit.[3],[4],[5],[6],[7],[8],[9] Unlike dermal lesions that usually resolve spontaneously by the age of 5 years, ocular lesions can cause significant morbidity and typically need surgical intervention. Iris is the most common site reported to be affected and iris lesions tend to bleed spontaneously and recurrently causing uncontrolled secondary glaucoma and a painful blind eye that often requires enucleation. Eyelid and ocular surface lesions require surgical excision.

JXG affecting ocular surface is uncommon. On a review of literature, we found 20 cases of pediatric JXG reported to present with a subconjunctival mass. Of these, 15 (75%) cases occurred in children less than 5 years of age and 5 (25%) cases had associated skin lesions also [Table 1].[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18] UBM features of ocular surface JXG lesion have been reported rarely. In previous reports by Lau et al.[17] and Niu et al.,[18] authors described a moderately reflective mass with extension into sclera and corneal stroma. Surgical excision was done with lamellar sclerectomy and lamellar keratectomy, respectively, in these cases. In the current case, UBM showed a moderately reflective episcleral mass with no scleral extension. Intraoperatively, this finding was confirmed and the mass could be dissected of the sclera without need of lamellar scelerctomy. Thus, UBM can be useful for assessing the extent of JXG mass into the contiguous tissues. Ocular surface JXG can manifest as an episcleral mass with/without scleral invasion and a preoperative UBM can help in evaluating the need for lamellar scleralectomy.
Table 1: Clinical findings, treatment, and outcome details in cases of JXG presenting with ocular surface mass reported in literature

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The most common modality of treatment reported in a limbal JXG is surgical excision.[3],[4],[5],[6],[7],[8],[9],[10],[11],[12] Although the exact pathology of JXG is unknown, the presence of histiocytes possibly shows a relationship with inflammation. Topical steroids are useful in treatment of iris lesions. However, steroids alone are insufficient in treatment of ocular surface lesions as reported by various studies and are mostly used as an adjunctive treatment. A complete excision is usually advocated as it prevents its extension to deep corneal stroma. Some authors prefer a lamellar corneal grafting to take care of corneal component.[6],[8],[12],[18]

In the current case, we excised the subconjunctival mass leaving the corneal part untouched, which showed regression with topical steroids. However, it resulted in a residual corneal opacity. A lamellar corneal excision with corneal grafting may have avoided this.

  Conclusion Top

To conclude, JXG may infrequently present as an ocular surface mass in children. JXG must be kept in the differential diagnosis especially if there are associated skin lesions or a history of the same is present. Complete surgical excision remains the treatment of choice. Preoperative UBM may be used to plan complete surgical resection.

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

McDonaugh JER. Spontaneous disappearance of endotheliomata (nevoxanthoma). Br J Dermatol 1909;21:254.  Back to cited text no. 1
Lever WF, Schaumburg-Lever G. Histopathology of the Skin. 6th ed. Philadelphia: JB Lippincott Co; 1983. p. 398-9.  Back to cited text no. 2
Cogan DG, Kuwabara T, Parke D. Epibulbar nevoxanthoendothelioma. Arch Ophthalmol 1958;59:717-21.  Back to cited text no. 3
Zimmerman LE. Ocular lesions of juvenile xanthogranuloma. Am J Ophthalmol 1965;60:1011-35.  Back to cited text no. 4
Nordentoft B, Andersen SR. Juvenile xanthogranuloma ofthe cornea and conjunctiva. Acta Ophthalmol (Copenh) 1967;45:720-6.  Back to cited text no. 5
Collum LM, Power WJ, Mullaney J, Farrell M. Limbal xanthogranuloma. J Pediatr Ophthalmol Strabismus 1991;28:157-9.  Back to cited text no. 6
Lewis JR, Drummond GT, Mielke BW, Hassard DT, Astle WF. Juvenile xanthogranuloma of the corneoscleral limbus. Can J Ophthalmol 1990;25:351-4.  Back to cited text no. 7
Kaufmann JG, Driebe WT Jr, Hamed LM, Margo CE. Juvenile xanthogranuloma of the corneoscleral limbus. Ophthalmic Surg 1993;24:428-30.  Back to cited text no. 8
Ashmore ED, Wilson MW, Morris WR, Hill DA, Rodriguez-Galindo C, Haik BG. Corneal juvenile xanthogranuloma in a 4-month-old child. Arch Ophthalmol 2003;121:117-8.  Back to cited text no. 9
Park S-H, Rah S-H, Kim Y-H. Juvenile xanthogranuloma as anisolated corneoscleral limbal mass: A case report. Korean J Ophthalmol 2003;17:63-6.  Back to cited text no. 10
Chaudhary IA, Jishi ZA, Shamsi FA, Riley F. Juvenile xanthogranuloma of the corneo scleral limbus: Case report and review of literature. Surv Ophthalmol 2004;49:608-14.  Back to cited text no. 11
Lim-I-Linn Z, Li L. Juvenile xanthogranuloma of the corneoscleral limbus. Cornea 2005;24:745-7.  Back to cited text no. 12
Nishi T, Nawa Y, Nitta N, Hara Y. A case of juvenile limbal xanthogranuloma. Jpn J Ophthalmol 2007;51:301-2.  Back to cited text no. 13
Mocan MC, Bozkurt B, Orhan D, Kuzey G, Irkec M. Juvenile xanthogranuloma of the corneal limbus: Report of two cases and review of the literature. Cornea 2008;27:739-42.  Back to cited text no. 14
De Keyser C, Maudgal P, Legius E, Van Ginderdeuren R, Casteels I. Juvenile xanthogranuloma of the corneoscleral limbus: Report of two cases. Ophthalmic Genet 2010;32:54-6.  Back to cited text no. 15
Longmuir S, Dumitrescu A, Kwon Y, Boldt HC, Hong S. Juvenile xanthogranulomatosis with bilateral and multifocal ocular lesions of the iris, cornealscleral limbus, and choroid. J AAPOS 2011;15:598-600.  Back to cited text no. 16
Lau HHW, Yip WWK, Lee A, Lai C, Fan DSP. Three different ophthalmic presentations of juvenile xanthogranuloma. Hong Kong Med J 2014;20:261-3.  Back to cited text no. 17
Niu L, Zhang C, Meng F, Cai R, Bi Y, Wang Y, et al. Ocular juvenile xanthogranuloma. Optom Vis Sci 2015;92:e126-33.  Back to cited text no. 18


  [Figure 1]

  [Table 1]


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