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Abstract
We report a patient with Graves’ disease who remained persistently hyperthyroid after
a total thyroidectomy and also developed
de novo Graves’ ophthalmopathy 5 months after surgery. She was subsequently found to have
a mature cystic teratoma containing struma ovarii after undergoing a total hysterectomy
and salpingo-oophorectomy for an incidental ovarian lesion.
Learning points:
It is important to investigate for other causes of primary hyperthyroidism when thyrotoxicosis
persists after total thyroidectomy.
TSH receptor antibody may persist after total thyroidectomy and may potentially contribute
to the development of
de novo Graves’ ophthalmopathy.
Graves' ophthalmopathy is clinically relevant in approximately 50% of patients with Graves' disease, severe forms affecting 3%-5% of patients. Two age peaks of incidence are observed in the fifth and seventh decades of life, with slight differences between women and men. The disease is more frequent in women than in men, although the female-to-male ratio is only 1:4 in severe forms of eye disease. The natural history of Graves' ophthalmopathy is incompletely defined, but in many instances, especially in mild forms, the disease may remit or improve spontaneously. The onset of the ophthalmopathy is in most cases concomitant with the onset of hyperthyroidism, but eye disease may precede or follow hyperthyroidism. Cigarette smoking plays an important role in the occurrence of the ophthalmopathy, and is also associated with a higher degree of disease severity and a lower effectiveness of its medical treatment. Primary prevention (i.e., avoidance of the occurrence of the ophthalmopathy) is presently not feasible, but smoking withdrawal in relatives of patients with Graves' disease might be important. In terms of secondary prevention (i.e., avoidance of progression of subclinical eye disease into overt and severe ophthalmopathy) in addition to refraining from smoking, early and accurate control of thyroid dysfunction (both hyperthyroidism and hypothyroidism), as well as early diagnosis and treatment of mild eye disease are important. As to the role that management of hyperthyroidism may play in the course of Graves' ophthalmopathy, while antithyroid drugs and thyroidectomy are not disease-modifying treatments, radioiodine therapy causes a progression of the ophthalmopathy in approximately 15% of patients, especially high-risk patients, who smoke, have severe hyperthyroidism or uncontrolled hypothyroidism, high levels of thyrotropin (TSH)-receptor antibody, or preexisting eye disease. However, the risk of radioiodine-associated progression of the opthalmopathy can be eliminated by concomitant treatment with middle-dose glucocorticoids. In terms of tertiary prevention (i.e., avoidance of deterioration and complications of overt disease) early immunosuppressive treatment or orbital decompression, as appropriate, are essential tools. Smoking withdrawal may increase the effectiveness of immunosuppressive treatment.
To evaluate the clinical characteristics of struma ovarii. Twenty-five cases of struma ovarii were reviewed retrospectively from June 1994 to April 2007. The presenting clinical, radiologic, and pathologic features of the patients were reviewed. The mean age of the patients in this study was 45.3 years. The majority was of premenopausal status. Sixteen patients had clinical symptoms such as low abdominal pain, palpable abdominal mass and vaginal bleeding. Although one patient had an abnormal thyroid function test, the laboratory findings normalized after operative treatment. CA-125 levels were elevated in 6 cases. Diagnosis by preoperative imaging studies were 8 dermoid cysts, while only 3 cases were diagnosed as struma ovarii. There were 4 cases of malignant struma ovarii, and no patients with recurrent disease. Struma ovarii is a rare tumor. The presented clinical, laboratory and radiological findings of patients are very diverse. The diagnosis was confirmed by pathologic findings. The treatment of benign struma ovarii is surgical resection only. The cases of malignant struma ovarii may need adjuvant treatment, but recurrence is uncommon.
Graves' ophthalmopathy is an inflammatory autoimmune disorder of the orbit. The close clinical and temporal relationships between Graves' hyperthyroidism and ophthalmopathy have long suggested that both conditions derive from a single systemic process and share the thyrotropin receptor as a common autoantigen. This receptor is expressed not only in thyroid follicular cells, but also in orbital fibroblasts with higher levels measured in orbital cells from ophthalmopathy patients than in cells from normal individuals. Recent studies from several laboratories have shown that thyrotropin receptor activation in orbital fibroblasts enhances hyaluronic acid synthesis and adipogenesis, both cellular functions that appear to be upregulated in the diseased orbit. The phosphoinositide 3-kinase/Akt signaling cascade, along with other effector pathways including adenylyl cyclase/cAMP, appears to mediate these processes. Future therapies for this condition may involve inhibition of thyrotropin receptor signaling in orbital fibroblasts.
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