Proptosis ocular como una manifestación de la granulomatosis con poliangeítis (Wegener): Reporte de un caso y revisión de la literatura / Ocular proptosis as a manifestation of Wegener's granulomatosis: A case report

RESUMEN La granulomatosis con poliangeítis (Wegener) es considerada como una enfermedad granulomatosa sistémica, no infecciosa, caracterizada histológicamente por una vasculitis necrosante de pequeño vaso. El tracto respiratorio superior e inferior son los más frecuentemente afectados, en asociación a manifestaciones renales. Sin embargo, también se describe el compromiso aislado de un solo órgano, como es el caso del globo ocular y la órbita. Presentamos el caso de una mujer cuya principal manifestación consistió en un proceso inflamatorio de tejido periorbitario y proptosis del globo ocular izquierdo. La paciente fue valorada en manejo conjunto con el servicio de plástica ocular, otorrinolaringología y finalmente se derivó a reumatología para inicio de terapia inmunosupresora. Se realizó una revisión de la literatura, en búsqueda de reportes de caso y series de caso que permitieran comparar las presentaciones clínicas y desenlaces más frecuentes.

A B S T R A C T Granulomatosis with polyangiitis (Wegener) is considered as a systemic, non-infectious granulomatous disease, histologically noted for its small vessel necrotising vasculitis. The upper and lower respiratory tracts are the most frequently affected, in association with renal manifestations. However, the isolated involvement of a single organ, such as the eyeball and the orbit is also described. The case is presented of a woman whose main manifestation was an inflammatory process of periorbital tissue and proptosis of the left eyeball. The patient was evaluated jointly with eye plastic surgery, and the ear, nose and throat, and rheumatology departments. A literature review was carried out, looking for case reports and case series that allowed comparisons to made between the clinical manifestations and the most frequent outcomes.

Management of pseudo tumour in haemophilia patients

A hemophilic pseudotumor is one of the rare complications of hemophilia that results from repetitive bleeding, forming an expanding destructive encapsulated hematoma and necrosed tissue. It has a considerable amount of morbidity. These have become rare over the years with better treatment modalities like factor replacement. Presently, excision is the preferred treatment by many authors. There are instances where surgical excision is not feasible. In such situations, radiotherapy and arterial embolization should be considered either alone or as an adjunct to surgery. A 32 years old male patient, with severe hemophilia A diagnosed with an abdominal tumour 7 years ago during routine screening, that progressively grew to encompass the lower abdominal area. A 44 years old male patient with hemophilia A presented with slow growing swelling over the left gluteal region since, 10 years associated with ulceration and bleeding since, 3 days. The management of a patient with a haemophilic pseudo tumour is complex, with a high rate of potential complications. Surgical excision is the treatment of choice but can only be carried out by a multidisciplinary surgical team. The main postoperative complications are rebleed, infection, fistula and pathological fractures. Pelvic pseudo tumours can even become complicated by fistula formation to the large bowel and by obstruction of the ureters. Untreated pseudo tumours will ultimately destroy soft tissues, erode bone, and may produce neurovascular complications. The hemophilic pseudo tumour is a rare entity which is slow growing painless tumour, with few reports worldwide in the management of this rare complication.