Orbital Wegener's granulomatosis as the initial presentation in a 9-year-old child

Abstract

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. 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). Her chest X-ray was normal and urine analysis was also normal. Her blood tests was positive for cANCAwith a titre of 1:20 and pANCAwith 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.