1/8. Extensive sterile abscess in an invasive fibrous thyroiditis (Riedel's thyroiditis) caused by an occlusive vasculitis.Riedel's thyroiditis is a rare disease determined by an invasive fibrosclerotic transformation of the thyroid gland. It may be one manifestation of multifocal fibrosis with still unknown etiology. Because it mimics carcinoma, a biopsy must be performed to get the correct diagnosis. The condition is self-limiting when confined to the neck. prognosis depends on the extent of extracervical fibrosclerosis. We present a patient with a huge cervical and mediastinal, unilateral thyroid mass expanding to the aortic curve, which led to tracheal deviation and compression with symptoms of stridor and dyspnea. These symptoms continued under a course of high-dose steroids; thus an operation was necessary to relieve the airway obstruction and limit inflammation. Intraoperative and pathological findings showed an inflammatory infiltration of the adjacent neck muscles and a sterile abscess caused by an occlusive vasculitis. Therefore, hemithyroidectomy had to be performed instead of a local limited resection.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/8. A rare case of Riedel's thyroiditis, 6 years after retroperitoneal fibrosis: two diseases with one pathogenesis?We describe a 70-yr-old female patient in whom both a retroperitoneal fibrosis and 6 years later a Riedel's thyroiditis were diagnosed. Both diseases belong to the group of fibrotic diseases called "multifocal fibrosis". retroperitoneal fibrosis is now known to be an auto-allergic reaction to lipid components of the atherosclerotic process in the abdominal aorta, resulting in a fibrotic reaction around it. We hypothesize that similarly Riedel's thyroiditis is in fact a periarteritis of the carotid artery, resulting in cervical fibrosis, secondarily involving the thyroid. This hypothesis would account for the occurrence of these two rare diseases in one patient.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/8. Lymphocytic hypophysitis. The clinical spectrum of the disorder and evidence for an autoimmune pathogenesis.Lymphocytic hypophysitis, a rare disease hitherto restricted to women, usually presents with symptoms of hypopituitarism in relationship to pregnancy. Two patients who developed pituitary insufficiency from lymphocytic hypophysitis are described. In the first, visual deterioration due to chiasmal compression from hypophysitis arising in ectopic pituitary tissue responded to bromocriptine and corticosteroids. In the second, an insidious onset of hypopituitarism occurred over 5 years in an elderly male. Combined HLA and complement typing confirmed that both patients shared MHC class I, II and III alleles. These class II and III alleles have been described in association with Hashimoto's thyroiditis and insulin-dependent diabetes mellitus (IDDM), both of which may be associated with antipituitary antibodies. The features of these two cases extend the known clinical and pathological spectrum of this disease and, through identifying a common immunogenetic background, provide a possible link between the previous associations of this disorder and autoimmune thyroid disease and IDDM.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/8. Pyogenic thyroiditis due to branchial pouch sinus.Suppurative thyroiditis due to branchial pouch sinus is a rare disease, affecting children and young adults. It presents as an abscess in the lower lateral neck, usually on the left side. Suppurative thyroiditis can be suggested by ultrasound, radionuclide scintigraphy or CT scan, but the diagnosis can only be established by barium swallow showing the sinus from the apex of the pyriform sinus to the perithyroid region. Complete surgical excision of the sinus is essential. Two patients are presented and the role of the various imaging modalities in the diagnosis is discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/8. Recurrent suppurative thyroiditis due to fourth branchial pouch sinus.Acute suppurative thyroiditis is a rare disease in childhood. The etiology of this condition is not always obvious. The description of a pharyngeal pouch fistula extending from the pyriform sinus to the thyroid gland or perithyroid area, high-lighted the etiopathogenesis of some of the thyroiditis cases. Presented here are the clinical, radiological and pathological aspects of an internal pharyngeal fistula manifesting with recurrent suppurative thyroiditis, until it was completely removed. The clinical features of caudal branchial pouch remnants and their role in the development of acute suppurative thyroiditis is reviewed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/8. Acute suppurative thyroiditis.Acute suppurative thyroiditis is a rare disease which may be confused with other, more common thyroid disorders. Gram-positive organisms such as streptococcus, staphylococcus, and Pneumococcus are most frequently responsible for this condition. Appropriate antibiotics should be selected to control local and systemic signs of infection. Incision and drainage of fluctuant areas are indicated. Thyroid function almost always returns to normal with eradication of infection.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/8. Extracervical fibrosclerosis causing obstruction of a ventriculo-peritoneal shunt in a patient with hydrocephalus and invasive fibrous thyroiditis (Riedel's struma).The association of invasive fibrous thyroiditis (IFT) with foci of extracervical fibrosclerosis is well recognized. Affected sites include the retroperitoneum, the mediastinum, the biliary tract, and the orbit. The development of subcutaneous fibrosclerosis, however, is extremely rare. We report a patient with known invasive fibrous thyroiditis and hypoparathyroidism who presented with localized subcutaneous fibrosclerosis of the anterior chest wall resulting in compression of his ventriculoperitoneal shunt. The aetiology of IFT has remained unclear. Several histological and serological features, including the presence of mononuclear cells within the fibrosclerotic process, the occurrence of microscopic vasculitis, and the detection of autoantibodies directed against thyroid-specific antigens in a large proportion of patients with IFT, currently support the notion of autoimmune mechanisms playing a role in the pathogenesis of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/8. Cerebral venous sinus thrombosis: a late sequel of invasive fibrous thyroiditis.Riedel's invasive fibrous thyroiditis (IFT) is a rare disease of unknown etiology characterized by a dense fibrosis involving the thyroid gland and its surrounding tissues. Clinically, patients present with a stony hard goiter frequently associated with compressive symptoms. Involvement of the surrounding neck structures by IFT can lead to various clinical sequelae. We report the case of a 55-year-old woman with known IFT who developed thrombosis in the right internal jugular vein that progressed to the right sigmoid, transverse, and superior sagittal sinuses. IFT could have predisposed to cerebral venous sinus thrombosis by causing venous stasis, vascular damage and possibly a hypercoagulable state. To our knowledge, this is the first report of cerebral venous sinus thrombosis that developed as a complication of IFT.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |