Multinodular goiter cystic kidney polydactyly
Overview
the thyroid is a small gland in the neck that takes up iodine from the body to produce hormones that help control the body's metabolism, and regulate how ... more about Daneman Davy Mancer syndrome.
Symptoms
* Goiter * Cystic kidney disease * Finger-like thumbs * Extra big toe * Extra finger
Diagnosis
Routine tests include a CBC, sedimentation rate, urinalysis, thyroid profile with a TSH immunoassay, chemistry panel, chest x-ray, and EKG. Thyroid antibodies may be tested if Hashimoto's thyroiditis is suspected. The most important study is a thyroid technetium-99m or iodine-123 uptake and scan. If the results of these are abnormal, then an endocrinologist or general surgeon should be consulted to assist in the interpretation. If the scan indicates a cold nodule, ultrasonography may be done to determine whether the nodule is cystic or solid. If it is cystic, generally it can be aspirated and followed. If it is solid, a biopsy or aspiration and biopsy should be undertaken. If there are malignant cells or at least suspicious cells for malignancy, surgery should be done. If the scan reveals a hot nodule and there is clinical and laboratory evidence of thyrotoxicosis, the patient should be treated with radioactive iodine or surgery. If the scan shows diffuse uptake of radioactive materials and there is clinical thyrotoxicosis, the patient also may be treated with radioactive iodine or surgery.
Treatment
Total or subtotal thyroidectomy, with modified node dissection (bilateral or unilateral) on the side of the primary cancer (papillary or follicular cancer) Total thyroidectomy and radical neck excision (for medullary, giant, or spindle cell cancer) Radiation (131I) with external radiation (for inoperable cancer and sometimes postoperatively in lieu of radical neck excision) or alone (for metastasis) Adjunctive thyroid suppression, with exogenous thyroid hormones suppressing TSH production, and simultaneous administration of an adrenergic blocking agent such as propranolol, increasing tolerance to surgery and radiation Chemotherapy for symptom-producing, widespread metastasis is limited, but doxorubicin is sometimes beneficial.
