|Year : 2006 | Volume
| Issue : 1 | Page : 45-46
Bilateral spontaneous hyphaema in juvenile xanthogranuloma
P Vijayalakshmi1, Shashikant Shetty1, Jitendra Jethani1, TB Uma Devi2
1 Aravind Eye Care System, 1, Anna Nagar, Madurai, India
2 Thanjavur Medical College, Thanjavur, India
Pediatric Ophthalmology and Strabismus Department, Aravind Eye Care System, 1, Anna Nagar, Madurai 625020, Tamil Nadu
Source of Support: None, Conflict of Interest: None
This report describes a rare occurrence of bilateral, spontaneous, nontraumatic hyphema in a 6 weeks old infant, associated with a small, multiple skin lesions. The diagnosis of juvenile xanthogranuloma was confirmed by histopathological examination of the cutaneous lesions. The hyphaema cleared gradually in 2 weeks time with conservative management.
Keywords: Hyphaema, juvenile xanthogranuloma, Touton giant cells
|How to cite this article:|
Vijayalakshmi P, Shetty S, Jethani J, Uma Devi T B. Bilateral spontaneous hyphaema in juvenile xanthogranuloma. Indian J Ophthalmol 2006;54:45-6
|How to cite this URL:|
Vijayalakshmi P, Shetty S, Jethani J, Uma Devi T B. Bilateral spontaneous hyphaema in juvenile xanthogranuloma. Indian J Ophthalmol [serial online] 2006 [cited 2020 Jul 10];54:45-6. Available from: http://www.ijo.in/text.asp?2006/54/1/45/21616
Juvenile xanthogranuloma (JXG) is a rare benign cutaneous disorder that presents predominantly with skin lesions during infancy; more than 80% of the cases occurring during the first year of life. The ocular lesions are typically unilateral, although in few cases bilateral lesions have been described with variable presentation., Blank and co-workers reported the association between the skin lesions and ocular findings in JXG. We describe a rare manifestation of bilateral hyphaema in an infant who was later diagnosed as JXG. To our knowledge, this probably is only the fifth case of an infant presenting with bilateral spontaneous hyphaema owing to JXG.
| Case Report|| |
A male child aged 44 days presented to us with the complaints of redness and watering in right eye of 10-days duration. The child was a product of full-term normal delivery born to a second-degree consanguineous marriage. Neonatal period was uneventful and there was no history suggestive of trauma. The clinical findings in the right eye included mild corneal edema, fresh blood in anterior chamber spread over the iris surface with fibrin membrane covering the pupillary area, and segmentary heterochromia of the iris with no obvious neovascularization. The lens appeared normal and an ultrasonography showed normal posterior segment. Intraocular tension measured by pulse air tonometry was within normal limits. The left eye was normal at this time. The child was treated conservatively (0.3% tobramycin and 0.1% dexamethasone in combination four times a day with atropine eye ointment 1% twice a week) and the hyphaema cleared gradually. Fifteen days later, the child was examined under short anesthesia and the findings in the right eye included clear anterior segment with residual blood pigments over the lens and the back of the cornea. It also showed iris heterochromia ([Figure - 1], Image 1). The cornea measured 11 mm in diameter and the intraocular tension by tonopen was 17 mm. Fundus showed mild pallor of the optic disc with no evidence of any mass lesion or inflammation. In the left eye, the anterior and posterior segments were normal.
Two months later, the child presented with the complaints of redness in left eye. Clinical examination showed fresh hyphaema partly filling the chamber with no other significant ocular signs ([Figure - 1], Image 2). General examination showed multiple, discrete, yellowish, and vascularized skin nodules over face, scalp, and chest, few of them discharging blood and mucus ([Figure - 1], Images 3 and 4). The history revealed that these swellings started a month earlier than the involvement of the second eye. Histopathological examination of the cutaneous lesion revealed sheets of histiocytes and the diagnostic cell of JXG-the Touton giant cell ([Figure - 2], shown by the marker). In this cell, a central annulus of nuclei encloses a central eosinophilic cytoplasm, and the outer cytoplasm is pale and vacuolated. The background stroma is composed of lymphocytes, plasma cells, and occasional eosinophils mixed with the mononucleated polygonal and spindled histiocytes. Other investigations carried out were TORCH titers and HIV, which were negative. Hyphema in the left eye also cleared gradually with conservative management (0.3% tobramycin and 0.1% dexamethasone in combination four times a day, with 1% atropine eye ointment twice a week, along with 0.5% timolol maleate once a day).
| Discussion|| |
JXG is mainly a skin disorder characterized by a typically, raised, orange skin lesion, occurring either singly or in crops and regresses spontaneously with occasional involvement of the eyes. The most common ocular finding is diffuse or discrete iris nodules, which could be quite vascular and may bleed spontaneously, resulting in hyphema. Occasionally, the lesions may present in other areas such as ciliary body, anterior choroid, cornea, lids, and orbit. Our case presented primarily with hyphaema but iris neovascularisation could not be confirmed.
Zimmerman identified five presenting clinical patterns of intraocular involvement in infants and young children with JXG in his case series of 53 patients, which include an asymptomatic localized or diffuse iris tumor, unilateral glaucoma, spontaneous hyphaema, red eye with signs of uveitis, and congenital or acquired iris heterochromia. The other positive finding in our patient noted was iris heterochromia apart from hyphaema. The heterochromia was segmental and was present only in one eye. Blank and co-workers first noted the association between the skin lesions and ocular findings in JXG. The skin lesions in JXG can precede, follow, or occur simultaneously with the ocular involvement. Our case, when first presented with unilateral hyphaema, did not have any skin lesion, these only appeared in the interim period between the episodes of hyphaema in the two eyes. This suggests that in suspected cases, the parents should be instructed to be alert about the possibility of occurrence of cutaneous lesions.
JXG is an uncommon non-Langerhans-cell histiocytic inflammatory skin disorder. The systemic histiocystosis (i.e, Hand-Schuller-Christian disease, Letterer-Siwe disease, and eosinophilic granuloma) can be differentiated from JXG both clinically and histologically. Clinically, JXG patients show a lack of systemic signs and symptoms; histopathologically, there is a lack of intracytoplasmic organelles (Langerhans' granules) in biopsy specimens from JXG lesions. The histopathological findings are those of reactive granuloma containing foreign body giant cells and Touton giant cells, as well as histiocytes, lymphocytes, and eosinophils. Typically, JXG lesions are distinguished by the lack of staining for S-100 protein. These also show positivity for macrophage markers, such as CD68 and HAM 56, but lack in Birbeck granules on electron microscopy. Due to lack of facilities, these tests could not be performed on our patient.
The hyphema cleared completely with conservative management, with no trace of glaucoma or any other complications in our patient. Several modalities of treatment have been recommended for JXG skin lesions, ranging from local excision to the use of local and systemic corticosteroids and irradiation. Our patient had local excision for the swellings that were larger than 10 mm in size, especially for the purpose of biopsy.
Hyphaema in early childhood may be seen in retinoblastoma, persistent hyperplastic primary vitreous, retinopathy of prematurity, vascularized pupillary membrane, iris hemangioma, fetal distress syndrome, medulloepithelioma, leukemia, and other blood dyscrasias and metastatic tumors. In our case routine blood investigations, detailed examination under magnification, fundus evaluation, and ocular ultrasonogram were performed towards differentiating the above apart from the confirmatory biopsy of the skin nodule. Our clinical findings confirm that the diagnosis of JXG should be considered as a differential diagnosis of discrete iris lesions with or without uveitis, in cases of "spontaneous" hyphaema and heterochromia during infancy and early childhood.
| Acknowledgments|| |
This research work was supported by ORBIS International.
| References|| |
Sonoda T, Hashimoto H, Enjoji M. Juvenile xanthogranuloma: clinico pathologic analysis and immunohistochemical study of 57 patients. Cancer
Karcioglu ZA, Mullancy PB. Diagnosis and management of iris juvenile xanthigranuloma. J Paediatr Ophthalmol Strabis
Zimmerman LE. Ocular lesions of juvenile xanthogranuloma, nevoxanthoendothelioma. Trans Am Acad Ophthalmol Otolarngal
Blank H, Eglick PG, Beerman H. Nevoxantho-endothelioma with ocular involvement. Pediatrics
Chaudhry IA, Al-Jishi Z, Shamsi FA, Riley F. Juvenile xanthogranuloma of the corneoscleral limbus: Case report and review of literature. Surv Ophthalmol
Howard GM. Spontaneous hyphaema in infancy and childhood. Arch Ophthalmol 1962;68:615-20.
Casteels I, Olivder J, Malone M, Taylor D. Early treatment of juvenile xanthogranuloma of the iris with subconjunctival steroids. Br J Ophthalmol
[Figure - 1], [Figure - 2]