Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1016/j.bjane.2021.02.045
Brazilian Journal of Anesthesiology
Case Report

Anesthetic management of a patient with type 1 neurofibromatosis and an occult pheochromocytoma: a case report

Manejo anestésico de paciente com neurofibromatose tipo 1 e feocromocitoma oculto: relato de caso

Polyxeni Theodosopoulou, K. Nastos, A. Paraskeva

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Abstract

Neurofibromatosis type 1 is a complex genetic disorder affecting multiple organ systems. Cardiovascular manifestations include hypertension, often associated with concomitant pheochromocytoma. We present a hypertensive crisis during induction of anesthesia in a patient with neurofibromatosis type 1, scheduled for abdominal myomectomy, which revealed an undiagnosed pheochromocytoma. The case highlights the importance of assessing all patients with neurofibromatosis type 1 for pheochromocytoma, because if it is left undiagnosed, it can be disastrous in the setting of anesthesia and surgery.

Keywords

Anesthetic management,  Pheochromocytoma,  NF-1,  Hypertensive crisis

Resumo

A neurofibromatose tipo 1 é uma doença genética complexa que afeta vários sistemas orgânicos. As manifestações cardiovasculares incluem hipertensão, frequentemente associada a feocromocitoma concomitante. Apresentamos crise hipertensiva durante indução anestésica em paciente com neurofibromatose tipo 1, agendada para miomectomia abdominal, que revelou feocromocitoma não diagnosticado. O caso destaca a importância de avaliar todos os pacientes com neurofibromatose tipo 1 para feocromocitoma, pois se não for diagnosticado pode ser desastroso no contexto da anestesia e da cirurgia.

Palavras-chave

Manejo anestésico; Feocromocitoma; NF-1; Crise de hipertensão

References

1. Hirsch NP, Murphy A, Radcliffe JJ. Neurofibromatosis: clinical presentations and anaesthetic implications. BJA Br J Anaesth. 2001;86:555–64.

2. Guerrero-Domínguez R, López-Herrera-Rodríguez D, AcostaMartínez J, Jiménez I. Anaesthetic implications in Von Recklinghausen disease: A case report. Colomb J Anesthesiol. 2015;43:107–10.

3. Sonntagbauer M, Koch A, Strouhal U, Zacharowski K, Weber CF. Catecholamine crisis during induction of general anesthesia. Anaesthesist. 2018;67:209–15.

4. Holldack HJ. Induction of Anesthesia Triggers Hypertensive Crisis in a Patient With Undiagnosed Pheochromocytoma: Could Rocuronium be to Blame? J Cardiothorac Vasc Anesth. 2007;21:858–62.

5. Barancik M. Inadvertent diagnosis of pheochromocytoma after endoscopic premedication. Dig Dis Sci. 1989;34:136–8.

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