Non surgical management of unilateral lacrimal gland swellin
A 10-year-old girl presented to us with painless progressive swelling of the upper and outer quadrant of oculus dexter (OD) of six weeks duration, along with painless swelling of the right cervical area of eight weeks duration, failing to respond to persistent antibiotic treatment. She also complained of diplopia on dextroelevation for the past four weeks. There was no history of systemic disease, surgery, trauma or associated visual or ocular complaints such as redness, discharge, lacrimation etc.

Her uncorrected visual acuity was 6/6, N6 with normal color vision and an intraocular pressure of 14 mm of Hg in both eyes. OD showed non-axial inferonasal proptosis of 4 mm with restriction of dextroelevation. There was no evidence of discoloration of the lid skin, vascular prominence or change in size or shape of swelling with posture changes or Valsalva maneuver. On palpation, OD revealed a well-defined, non-tender, firm orbital swelling of approximately 2.0 × 1.0 cm, beneath the anterior orbital rim in the superotemporal quadrant, with no definite palpable posterior margin and associated irregular anterior orbital margin. Anterior segment examination was normal, while fundus examination showed retinochoroidal folds in the superotemporal quadrant. Oculus sinister (OS) was normal.

On systemic examination, multiple massive, confluent, non-tender and rubbery enlarged anterior cervical lymph nodes were noted, the largest one being approximately 2.5 × 2.0 cm. No other organ involvement was observed.

Blood picture demonstrated microcytic hypochromic anemia with leukocytosis, elevated erythrocyte sedimentation rate (ESR) with hypergammaglobulinemia and marginally elevated serum Angiotensin Converting Enzyme (ACE) level. Rest of the investigations were normal. Fine needle aspiration cytology (FNAC) of the cervical lymph nodes was done, which showed non-specific lymphadenitis. Excisional biopsy of the cervical lymph was done and, based on which a confirmatory diagnosis of Rosai-Dorfman disease was made.

To assess the extent of disease, PET scan was also reported before and after treatment and. Computerized Tomography (CT) of the head and orbit demonstrated a homogenously enhancing soft tissue mass of the right orbit, approximately 22 mm cranio-caudally, 31 mm antero-posteriorly, 16 mm transversely in size, causing external compression over right globe with remodelling of anterior orbital rim of lateral wall and displacement of superior and lateral rectus.

After consulting the paediatric oncologist, medical management was started with intravenous dexamethasone for five days, followed by oral steroids and 6-mercaptopurine for eight weeks with excellent resolution of lacrimal gland swelling clinically. Over the next three years, there was no reported recurrence of the disease in the orbit or any focus of sinus histiocytosis elsewhere in the body.

Source: Indian Journal of Ophthalmology

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