title: Orbital presentation of a nasal midline destructive lesion in a young boy.
abstract: Midline Destructive Lesions (MDL) are well known to cause nasal problems. There is a long differential diagnosis of such lesions. However, in the pediatric population, the 2 main diseases to be aware of are Non-Hodgkin's T-cell lymphoma and granulomatosis with polyangiitis (previously known as Wegener's granulomatosis). The authors present the report of a 15-year-old boy who presented with epiphora, chemosis, and limitation of left abduction. CT scan of his orbits suggested a destructive lesion of the ethmoid sinuses. His laboratory investigations revealed a positive ANCA. The patient underwent endoscopic sinus surgery, and this was characteristic for granulomatosis with polyangiitis. He was treated with systemic steroids and then maintained on cyclophosphamide, which controlled his disease activity. This case highlights the need for ophthalmologists to have a high index of suspicion for MDL and concomitant orbital disease.
authors.affiliation: *Department of Ophthalmology, Wolverhampton & Midland Counties Eye Infirmary, and Departments of †Otolaryngology, and ‡Histopathology, New Cross Hospital, Wolverhampton, United Kingdom.
authors.forename: Kwesi Nyan
authors.initials: KN
authors.lastname: Amissah-Arthur
authors.forename: John
authors.initials: J
authors.lastname: Matthews
authors.forename: Anjali
authors.initials: A
authors.lastname: Bhatnagar
authors.forename: Soupramanien
authors.initials: S
authors.lastname: Sandramouli
mesh_terms: D000293:Adolescent; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D004724:Endoscopy; D005005:Ethmoid Sinus / Q000473:pathology; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000276:immunology / Q000601:surgery; D006801:Humans; D007766:Lacrimal Apparatus Diseases / Q000175:diagnosis / Q000276:immunology / Q000601:surgery; D008297:Male; D009915:Orbit / Q000000981:diagnostic imaging; D009916:Orbital Diseases / Q000175:diagnosis* / Q000276:immunology / Q000601:surgery; D010254:Paranasal Sinus Diseases / Q000175:diagnosis / Q000276:immunology / Q000601:surgery; D014057:Tomography, X-Ray Computed
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic