Anaesthetic Management for Laparascopic Adrenalectomy for Pheochromocytoma as Part of Vonn Hippel-Lindau Syndrome in Child

Abstract

G Ivanova, MP Atanasova, G Todorov, K Grozdev, MN Khayat, A Elenkova, R Ivanova, S Zacharieva

Pheochromocytoma is a tumor that originates from either chromaffincells in adrenal medulla or in other paraganglia tissues (paragangliomas) of the sympathetic nervous system. It might be familial and associated with hereditary tumors such as Von Hippel-Lindau syndrome and multiple endocrine neoplasia type II. The symptoms are variable related to the level of secreted catecholamine. The most common are hypertension, tachycardia, headache and abdominal pain. The most effective treatment is surgical resection as in recent years, laparoscopic surgery has been more preferred. The perioperative management is quite challenging especially in view of hemodynamic fluctuations. Although there is a considerable amount of information on the management of the adult with phaeochromocytoma, much less has been written concerning children with the disease. We present a case of 9-year-old hypertensive boy with left sided pheochromocytoma, scheduled for excision of tumour. He had presented with complaints of pain in abdomen, excessive sweating, headache and raised blood pressure, as well as family anamnesis for von Hippel-Lindau syndrome (father with confirmed diagnosis). Diagnosis was confirmed by CT scan (abdomen), raised 24-hour urinary catecholamine levels and genetics tests. Preoperative blood pressure was controlled with prazosin (ά-adrenergic blocker). The anaesthetic technique used was general anaesthesia. Child was later discharged on oral antihypertensive.

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