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A healthy 8-month-old boy was referred with a 3-week history of colour change of the left iris. For 3 days the eye had also been inflamed. On examination, left vision was reduced (based on asymmetric resentment to cover of each eye), the left upper and lower lids were slightly edematous, the conjunctiva was injected, and there were 1+ cells in the anterior chamber and a dull red reflex. A raised ochre-yellow vascular lesion was evident on the temporal aspect of the left iris (Fig. 1A). The left intraocular pressure (IOP) was 15 mm Hg (iCare Tonometer; iCare, Raleigh, NC). Ocular ultrasound demonstrated a 2.3 mm diameter anechoic iris lesion with no ocular calcification and no posterior segment pathology. Complete blood count and general chemistry laboratory values were all normal.
Fig. 1(A) At presentation, an ochre-yellow vascular lesion occupied the temporal aspect of the left iris. There is associated lid edema and conjunctival injection. (B) After 48 hours of topical prednisolone acetate, there is decreased left eyelid edema, conjunctival injection, and anterior uveitis.
Clinical examination was most suggestive of juvenile xanthogranuloma (JXG). Systemic examination revealed no cutaneous JXG lesions or evidence of neurofibromatosis type I. The left eye was treated with topical prednisolone acetate drops every hour while awake and dexamethasone ointment at bedtime. The right eye was patched for 2 hours daily to reverse the left-sided amblyopia. Two days later, the eyelid edema had decreased, and the conjunctival injection and anterior uveitis had almost resolved (Fig. 1B). The patient was seen every few days at first and then every week for the first month, and progressive shrinkage of the iris lesion was documented photographically. By 4 months, the lesion was significantly smaller (Fig. 2A). Topical prednisolone was tapered to 6 times daily for 2 weeks, 4 times daily for 1 week, twice daily for 1 month, and then once daily. Treatment was discontinued 8 months after presentation, and by 9 months of follow-up, the lesion had completely disappeared (Fig. 2B). IOP remained normal throughout, and no hyphema occurred. Visual acuities were equal from the second month, so patching was stopped.
Fig. 2(A) Follow-up at 4 months shows that the width and height of the lesion have decreased. (B) At 9 months, the patient had stopped all treatment 1 month earlier. The lesion has completely disappeared.
JXG is an uncommon but prognostically favorable non–Langerhans cell histiocytic disorder. Histiocytes and multinucleated Touton giant cells are characteristic histopathologic features. JXG primarily affects infants and young children and is more common in neurofibromatosis type I.
Typically the skin of the head and neck is involved by rapidly developing fawn-coloured lesions that resolve spontaneously after some months, leaving no scar. JXG can involve many organs, including the liver, spleen, lungs, and the central nervous system (CNS). The eyes may be affected by deposits in the eyelids, conjunctiva, iris, optic nerve, retina, choroid, and orbit.
Yet in the presence of cutaneous JXG, a screening ocular examination is likely to be negative unless there are visible ocular features or visual concerns because the overall incidence of ocular involvement is estimated to be about 0.24%.
Iris JXG is highly vascular and may be complicated by hyphema (spontaneous or traumatic/surgical), secondary elevation of IOP, and corneal edema. The differential diagnosis of intraocular JXG includes amelanotic melanoma, leukemic deposits, retinoblastoma, iris leiomyoma, tuberculous granuloma, hemangioma, lymphatic venous malformation (lymphangioma), and metastasis. A diagnosis of iris JXG may be determined on clinical appearance alone. Ultrasound, anterior segment optical coherence tomography, and fluorescein angiography may be helpful to differentiate it from mimicking lesions. Significant risks of intraocular hemorrhage complicated by glaucoma and potential corneal staining accompany any surgical manipulation such as biopsy. When in doubt, anterior chamber aspiration with histopathology of the aspirate is preferable to biopsy, yet this may also be liable to complication by hyphema.
Treatments for iris JXG have included corticosteroids (topical, subtenon, and systemic administration), radiation therapy, methotrexate, intravitreal bevacizumab, and surgical excision.
Treatment and complete resolution of iris JXG exclusively with topical corticosteroids has not been described previously. Our patient's classic presentation in infancy with an ochre-yellow vascular iris lesion, brisk anterior chamber reaction, and negative hematologic and ultrasound features allowed us to overlook the important guideline that tissue diagnosis is mandatory for any iris lesion prior to treatment. General anesthesia was avoided, and noninvasive topical treatment with close follow-up was used successfully for this young infant.
Footnotes and Disclosure
The authors have no proprietary or commercial interest in any materials discussed in this article.
References
Dehner LP.
Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations.
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