Abstract
Consider Wegener's granulomatosis in a child with non-resolving lid swelling.
Case report
A 9-year-old child was referred by her general practioner (GP) to eye casualty clinic with a six-week history of unexplained painless swelling of her right upper lid. Her GP, having treated first as an allergy with oral antihistamine, was now concerned that this was a case of preseptal cellulits progressing to orbital cellulitis and commenced a course of Erythromycin and Fucithalmic ointment and referred for review.
A detailed history had revealed four months previously she had developed right upper lid swelling in the context of a mild upper respiratory tract infection. However this was short-lived and resolved without any therapeutic intervention. She also frequently suffered from recurrent ear infections, which required eventual grommet insertion, and nose bleeds.
On examination she was found to have right painless upper lid swelling with no associated proptosis (Figure 1). Her ocular movements were full with no diplopia. Best corrected vision was 6/9 both eyes, her conjunctiva was injected in the right eye, anterior segment examination was normal with pressures of 14 both eyes. Pupils were reactive and equal with, no evidence of a relative afferent pupillary defect. Colour vision was also full. Posterior segment examination was normal no evidence of disc swelling or pallor.
Child showing right upper lid swelling at presentation
The antibiotics were continued with a provisional diagnosis of dacroadenitis. In view of the chronicity of the symptoms an orbital computed tomography scan and bloods were ordered.
Her CT scan displayed diffuse homogenous soft tissue swelling within the right lacrimal gland suggesting an inflammatory process, extraocular eye muscles were normal (Figure 2).
Diffuse swelling of the right lacrimal gland
Her chest X-ray was normal and urine analysis was also normal.
Her blood tests was positive for cANCA with a titre of 1:20 and pANCA with a titre of 1:40, ESR was normal at 11 the rest of her blood tests including full blood count, U&Es, LFTs, autoantibody screen and sACE were also normal. She was referred on to a paediatric rheumatologist to confirm the diagnosis of Wegener's granulomatosis and assist on further management.
A biopsy of the lacrimal failed to show the classical triad of granulomatous inflammation with giant cell, necrosis and vasculitis although there was evidence of chronic inflammation with perivascular infiltrates. Further examination of the child revealed saddling of the nose and a congenital sinus in the tragus of the left ear. Systemic examination failed to reveal any renal or pulmonary involvement. In view of the positive ANCA and clinical examination, a diagnosis of Wegener's granulomatosis was made. She was commenced initially with intravenous methylprednisolone for three days then on a tapering dose of oral steroids. The lid swelling has reduced and she is currently in remission.
Discussion
Wegner's granulomatosis is a necrotizing granulomatous vasculitis. It has an unknown aetiology and most commonly presents with respiratory or renal symptoms. The condition is often overlooked among children and misinterpreted as infectious disease as in the case of this child.
Ocular involvement in Wegener's granulomatosis among children as initial presentation has been reported. Levi et al. reported a series of six children, which is the largest to date that presented with initial ocular symptoms prior to their diagnosis. 1 Ocular manifestations included scleritis, bilateral papailloedema, idiopathic orbital psudotoumour and proptosis secondary to orbital inflammation. Lacrimal enlargement has been reported in children aged 5 and 9 years, both overseas. 2,3 Diagnosis of Wegener's granulomatosis is dependent upon appropriate clinical findings with pathological correlation. The ANCA test remains a useful diagnostic marker. Antibodies against proteinase 3 antibodies (c-ANCA) have sensitivity and specificity for active Wegener's granulomatosis of 91% and 99%, respectively. 4 Traditionally pathological triad of granulomatous inflammation, necrosis and vasculitis is only demonstrated in only 54% of orbital biopsies. 5 In our case both pANCA and cANCA was positive, the biopsy did show evidence of chronic inflammation and perivascular infiltrates which has been reported in isolation of cases of Wegener's granulomatosis. 5 This combined with characteristic clinical features of a flattened nasal bridge and ear deformities strengthened the diagnosis of Wegener's granulomatosis in our patient.
From this case we would like to highlight that recurrent unilateral upper lid swelling in a child with no response to antibiotics or antihistamines may be the first presentation of Wegener's granulomatosis.
Appropriate diagnosis, investigation and treatment are important to prevent progressive systemic manifestations of this condition, which may rarely result in mortality.
Treatment with systemic corticosteroids remains the first line of treatment, though steroid sparing agents are often employed due to the frequent nature of relapses.
The management of these patents should be under the care of physicians specializing in immunological disease.
DECLARATIONS
Competing interests
None declared
Funding
None
Ethical approval
Written informed consent to publication was obtained from the patient or next of kin
Guarantor
MD
Contributorship
All authors contributed equally
Acknowledgements
None
Reviewer
Simon Kelly