title: Anesthetic Management of Clinically Silent Familial Pheochromocytoma with MEN 2A: A Report of Four Cases.
abstract: Familial pheochromocytomas are commonly associated with multiple endocrine neoplasia type 2 (MEN 2) syndrome. Majority of the patients present with normal clinical and biochemical parameters in the preoperative period, the incidence of hypertension being only 50 %. Even though patients may be clinically asymptomatic, surveillance and proper preoperative evaluation is important, as surgery for associated tumors may precipitate a hypertensive crisis and result in severe complications. A family of 19 members, of which 12 were positive for MEN 2A syndrome, presented to our hospital. Seven of the 12 patients had pheochromocytoma and medullary thyroid carcinoma (MTC), while the other 5 had only raised plasma calcitonin levels. Two of the 7 patients presented with bilateral pheochromocytoma and underwent an open adrenalectomy. The other 5 patients had a left-sided adrenal tumor and underwent left laparoscopic adrenalectomy under combined general and epidural anesthesia. We present our experience with four of these five cases. We here state that how paucity of literature on perioperative preparation of clinically and biochemically silent pheochromocytomas led to serious intraoperative complications in one of four cases.
authors.affiliation: Department of Anesthesia, V.M.M.C. and Safdarjung Hospital, A7/14 Mianwali Nagar Paschim Vihar, New Delhi, 110087 India.
authors.forename: Shipra
authors.initials: S
authors.lastname: Aggarwal
authors.affiliation: Department of Anesthesia and Intensive Care, V.M.M.C. and Safdarjung Hospital, New Delhi, 110029 India.
authors.forename: Vandana
authors.initials: V
authors.lastname: Talwar
authors.affiliation: Department of Anesthesia, V.M.M.C. and Safdarjung Hospital, A7/14 Mianwali Nagar Paschim Vihar, New Delhi, 110087 India.
authors.forename: Pooja
authors.initials: P
authors.lastname: Virmani
authors.affiliation: Department of Anesthesia, V.M.M.C. and Safdarjung Hospital, A7/14 Mianwali Nagar Paschim Vihar, New Delhi, 110087 India.
authors.forename: Suniti
authors.initials: S
authors.lastname: Kale
keywords: Familial pheochromocytoma; MEN; Multiple endocrine neoplasia; Plasma metanephrines; VMA; Vanillylmandelic acid