1/7. Surgical management of localized amyloidosis.OBJECTIVE: To demonstrate the role of two-dimensional reconstruction images on computed tomography (CT) in the treatment planning for laryngeal amyloidosis. To discuss the treatment for isolated laryngeal amyloidosis and compare the role of endoscopic versus an open surgical approach to management. STUDY DESIGN: Retrospective review. methods: The medical records from 1984 to the present with the diagnosis of localized respiratory tract amyloidosis at Geisinger Medical Center were reviewed. RESULTS: Five previously unpublished cases of localized laryngeal amyloidosis were identified with the supraglottic region the major site of involvement. hoarseness and airway compromise were the presenting symptoms. CT two-dimensional reconstruction imaging was used to evaluate two cases with extensive laryngeal involvement that required an external surgical approach to relieve symptoms. CONCLUSIONS: Localized laryngeal amyloidosis is a rare disease that requires surgical management when symptomatic. CT two-dimensional reconstruction can be helpful in detailing the extent of disease and planning surgery. A lateral external supraglottic approach has been found to be successful in treating patients with large supraglottic masses.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/7. Laryngopyocele: three new clinical cases and review of the literature.Laryngopyocele is a fairly rare disease. It is a complication of laryngocele. Its clinical picture is often alarming; thus it needs fast differential diagnosis. Computed tomography allows early diagnosis. Surgical treatment permits a complete recovery. An association between laryngocele and carcinoma of the larynx has been reported by some authors. Three new cases of laryngopyocele are presented and discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/7. Lipoid proteinosis of the larynx.Lipoid proteinosis is a rare disease that presents with hyaline deposits in many tissues. It involves predominantly the skin and upper aerodigestive tract, presenting with small yellowish papules and hoarseness. It may involve the central nervous system and cause intracerebral calcifications. Laryngeal lesions may resemble singer's nodule or chronic laryngitis. The pathogenesis of the disease is not clear although several studies suggest a defective collagen production and/or lysosomal storage disease. In this article two cases with skin and larynx involvement are reported.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/7. head and neck amyloidosis: a clinicopathologic study of 15 cases.amyloidosis refers to the idiopathic, extracellular deposition of fibrillar proteins, termed amyloid, in tissues. Although amyloidosis is a rare disease, the head and neck region has been reported as a frequent site of amyloid deposits, accounting for approximately 19% of reported amyloid cases in one review. Fifteen cases of head and neck amyloid, excluding the brain, with clinical follow-up were identified in the Surgical pathology files from 1985 to 2005 at Emory University Hospital. The histopathology, histochemistry, and patient follow-up were reviewed. Nine men and six women with an age range of 18-76 years (mean 55.7 years) were identified. The initial clinical presentation was dependent on the site of amyloid deposits. The clinical types of amyloidosis included localized amyloid deposits in the larynx and tongue, plasma cell dyscrasia associated AL amyloidosis, and hemodialysis-associated amyloidosis. Secondary amyloidosis developed in one patient with carcinoid tumor.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/7. Laryngeal amyloidosis.Laryngeal amyloidosis is a rare disease. Surgery has been the mainstay of treatment either endoscopically or by an external neck approach. In more recent years, surgery with the carbon dioxide laser has been advocated for this condition. Four cases of laryngeal amyloidosis are presented. The application and the advantages of the carbon dioxide laser for the treatment of laryngeal amyloidosis are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/7. amyloidosis of the larynx.amyloidosis of the larynx is a rare disease, accounting for less than 1% of all benign laryngeal 'tumours'. Three cases of this type of lesion are reported--one of the vocal cord, one of the false vocal cord and one of the subglottis and trachea. In 2 of the patients the amyloidosis was localized, while the third was later found also to have an epipharyngeal solitary plasmacytoma with amyloid deposits and in addition amyloidosis of the nasal cavity. However, the amyloidosis in this patient may still be regarded as being localized, as the clinical examination and laboratory tests afforded no evidence of generalized disease. amyloidosis of the larynx may be manifested as a localized tumour or as a diffuse infiltration. The symptoms and signs will, of course, depend on the site of the amyloid deposit. When the vocal cords are involved hoarseness may result, and this was the most prominent sign in the present cases. The treatment of laryngeal amyloidosis is primarily by endoscopic excision. Amyloid substance has specific staining properties. The congo red reaction with a green birefringence in polarized light and Phorwhite BBU using fluorescence microscopy are regarded as the most reliable staining reactions. Electron microscopy has revealed a typical fibrillar structure of amyloid.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/7. hydralazine-induced relapsing polychondritis-like syndrome. Report of a case with severe chronic laryngeal complications.Relapsing polychondritis is a rare disease of uncertain etiology, characterized by inflammation of cartilage. cartilage of the respiratory tract is engaged in more than half of all cases. hydralazine-induced systemic lupus erythematosis (SLE) is a well known disease. Females who are slow acetylators and are HLA-DR4 positive when treated with hydralazine are at serious risk of contracting SLE syndrome. We describe here a woman, treated for 10 years with hydralazine, who was a slow acetylator and was HLA-DR4 positive and who presented with a relapsing polychondritis and ultimately required a permanent tracheostoma. To our knowledge, this is the first published case of relapsing polychondritis induced by hydralazine.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |