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Objective To investigate clinical features and anesthetic management of multiple endocrine neoplasia (MEN) associated with pheochromocytoma.Methods Medical records of patients who were diagnosed as multiple endocrine neoplasia a ssociated with pheochromocytoma in our hospital from April 1977 to April 2001 were reviewed retrospectively. The demographic data, clinical presentations, fami ly history, biochemical examinations, type of MEN, sequence of different surgica l procedures, anesthetic methods and hemodynamics during surgery were analyzed. Results Thirteen cases of MEN associated with pheochromocytoma were investigated, accounting for 6% (13/213) of the pheochromocytoma patients admitted into our hospital. Nine of the 13 patients presented as type Ⅱa MEN (Sipple syndrome), on e as type Ⅱb MEN, and three as mixed MEN. Four patients with typeⅡa MEN had a family history of similar disease. Five patients with other coexisting endocri ne disorders first underwent excision of the pheochromocytomas, although only tw o had hypertensive symptoms at the time of admittance. Seven patients without h istories of hypertension received surgical treatment for pheochromocytoma second ly. The excision of pheochromocytoma was performed under general anesthesia in 8 patients and epidural block in 4 patients. Marked hemodynamic fluctuation was recorded in 8 patients. No perioperative death was recorded. Conclusion Pheochromocytoma may be linked to other endocrine disorders during MEN, either as the main clinical presentation or most frequently as an occult tumor. Recognition of this feature of pheochromocytoma is of importance to the improvement of diagnosis and treatment both for pheochromocytoma and MEN.