Nasal hemophilic pseudotumor: favorable response to radiotherapy.

Hemophilic pseudotumors are rare but dangerous complications of Hemophilia. We hereby report a 3-year-old boy with Hemophilia B, presenting with nasal pseudotumor, showing favorable response to radiotherapy after unsuccessful treatment with factor IX replacement therapy. The diagnosis and treatment of this rare condition is also reviewed.

Primary malignant melanoma of the nose: a rare cause of epistaxis in the elderly.

We report the case of a primary nasal malignant melanoma in an elderly woman, illustrating the dilemmas involved in clinical decision making with non-specific symptoms, and the treatment of cancer in elderly patients. We also discuss the incidence, clinical presentation, diagnosis, principles of management and outcome.

Jugulotympanic paraganglioma (glomus tumour) presenting with recurrent epistaxis.

A case is presented where a left jugulotympanic paraganglioma (JTP) extended to the nasopharynx and the patient presented with recurrent epistaxis. Although initial biopsy of an aural polyp had been suggestive of the diagnosis several years previously, the diagnosis was not confirmed until the patient presented with recurrent epistaxis and severe anaemia. To the best of our knowledge, this is the first case reported of such a presentation of JTP.

External beam radiotherapy for severe epistaxis from Osler-Weber-Rendu disease.

Hereditary Hemorrhagic Telangiectasia (HHT) is a genetic disorder of blood vessels, which affects about 1 in 10,000 people. The disorder is also sometimes referred to as Osler-Weber-Rendu Disease after the doctors who studied HHT. The disorder affects males and females from all racial and ethnic groups, and the most commonly affected organs are the nose, lungs, gastrointestinal tract, and brain--in that order. Radiation therapy should be considered for intractable bleeding from the upper airway or alimentary tract.

[Rendu-Osler disease. Follow-up of 6 patients treated by embolization. Nasal telecobaltotherapy in a refractory case]. / Enfermedad de Rendu-Osler. Seguimiento de seis casos tratados mediante embolización. Telecobaltoterapia nasal en un caso refractario.

Patients with Rendu-Osler-Weber disease can present severe nose bleeding. The treatment of 6 patients with this condition is reported. Supraselective embolization was the treatment of choice. One patient showed no improvement with embolization and suffered massive nosebleed requiring blood transfusion. After a review of the literature, the nasal cavity was irradiated.s.

Management of epistaxis in Rendu-Osler disease: is brachytherapy effective?

PURPOSE: This paper reviews the results of intranasal brachytherapy for epistaxis in 43 patients with Rendu-Osler disease treated between 1971-1991 at Henri Mondor Hospital. METHODS AND MATERIALS: 2-3 intranasal catheters were afterloaded with 192Ir sources. Computer dosimetry was performed and then the dose was prescribed to an isodose thought to cover the nasal mucosa. The dose rate ranged from 0.16 Gy/h-0.63 Gy/h with a median of 0.34 Gy/h. Dose at one application ranged from 15-35 Gy with a median of 30 Gy. The severity of epistaxis was graded 1 to 5. RESULTS: The time to recurrence of significant epistaxis ranged from 6-178 months with a median of 24 months. The dose prescribed did not correlate with control rate. The only brachytherapy complication was septal perforation in 4 patients; in one this was a result of repeated nasal coagulation. CONCLUSION: We suggest that intranasal brachytherapy is a useful modality in the management of epistaxis in Rendu-Osler disease.

[Rendu-Osler disease treated by endonasal radiotherapy (evaluation of 62 cases)]. / Maladie de Rendu-Osler traitée par plésiocuriethérapie endo-nasale. (Bilan de 62 cas)

Two groups of patients were treated by plesiocurietherapy. The first group of 17 patients received only one exposure to radium (between 1949 and 1960). The second group received one or more doses of irridium 192 (between 1960 and 1973). The average period for stabilization of the disease was 13,2 months with radium and 20,4 months with irridium. Unfortunately, this does not give a permanent cure and, for irridium 192, the percentage of successes is only 30 p. 100 after 5 years. Logically, radiotherapy with irridium 192 should only be used when other methods have failed. The progressive accumulation of radio-lesions may, in fact, make the use of other techniques impossible.