Sir,

Crohn's disease is an idiopathic inflammatory disease of the upper and lower intestinal tract characterized by transmural inflammatory ulcers of the digestive system featuring noncaseating granulomas. Extraintestinal manifestations are present in 25–35% of patient with Crohn's disease. Ocular manifestations occur in less than 10% of cases and include episcleritis, scleritis, uveitis, retinal vasculitis, optic neuropathy, and idiopathic orbital inflammatory disease.1, 2, 3

We report the case of a 10-year-old female who presented with bilateral dacroadenitis as one of the initial manifestations of previously unrecognized Crohn's disease.

Case report

A previously healthy 10-year-old Caucasian female was referred to the eye department with a 1-week history of bilateral upper eyelid swelling associated with moderate discomfort. The patient had a 3-weeks history of fever, abdominal pain, diarrhoea, aphthous oral lesions, and arthritis.

Examination disclosed uncorrected visual acuity of 20/20 OU, and bilateral upper eye lid swelling, more prominent temporally, conferring an ‘S’-shaped contour to the margin (Figure 1). The upper eyelids were tender, and erythematous with a light purple hue. Bilateral mild lacrimal gland swelling was present. There was no evidence of proptosis or ocular motility abnormalities. Examination of the anterior segment, retina, and optic nerve was unremarkable. A diagnosis of bilateral dacroadenitis was made.

Figure 1
figure 1

Bilateral upper eyelid swelling, more prominent temporally, conferring an ‘S’-shaped contour to the margin. The skin was erythematous and had a light purple hue. Lacrimal gland swelling was present bilaterally.

Full size image

Laboratory investigation revealed leukocytosis, anaemia, and an ESR of 70 mm/h. Autoantibody screen, and serology for Epstein–Barr virus were negative. Colonoscopy disclosed multiple deep ulcers throughout the colon. Histopathologic examination of biopsied specimen demonstrated granulomatous inflammation involving the terminal ileum and colon, confirming the diagnosis of Crohn's disease.

The patient was treated with intravenous methyl prednisolone (1.6 mg/kg/day) for 3 days followed by oral therapy. Her symptoms responded promptly to the treatment with complete resolution of dacroadenitis within 2 weeks.

Comment

Crohn's disease is an immune-based systemic disorder that predominantly involves the gastrointestinal tract. Extraintestinal manifestations often correlate with the disease activity and may precede, occur concurrently, or follow the initial gastrointestinal presentation of the disease. Ocular complications occur in approximately 10% of patients with Crohn's disease and include conjunctivitis, keratitis, episcleritis, scleritis, uveitis, retinal haemorrhage and oedema, retinal vasculitis, neuroretinitis, optic neuropathy, orbital myositis, and neuro-ophthalmologic abnormalities.1, 2, 3, 4, 5 Dacroadenitis is rarely associated with Crohn's disease.4, 5 The presence of one or more extraintestinal complications significantly increases the chance of ocular involvement. Patients with Crohn's disease affecting the colon or ileocolon are more likely to have ocular inflammation than those with small bowel involvement. Medical or surgical treatment of gastrointestinal inflammation frequently results in the improvement of the ophthalmic complications.

Orbital myositis and idiopathic orbital inflammatory disease may occur in association with Crohn's disease. Our patient did not exhibit any symptoms or signs suggestive of these conditions and it appears that the lacrimal gland was the primary orbital tissue that was clinically affected. Other causes of dacroadenitis were ruled out by clinical and laboratory findings in our patient.

In summary, we report a case of bilateral dacroadenitis that presented concurrently with the first manifestations of Crohn's disease. The patient received systemic corticosteroid therapy with rapid resolution of dacroadenitis, and the inflammatory bowel disease.