Manejo perioperatorio de emergencia hipertensiva en paciente con debut de feocromocitoma.

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Bibliographic Details
Title:	Manejo perioperatorio de emergencia hipertensiva en paciente con debut de feocromocitoma.
Alternate Title:	Perioperative management of hypertensive emergency in a patient with new onset of pheochromocytoma.
Authors:	Tomarelli, R. Gianfranco¹ gianfrancotomarelli@gmail.com, Donoso, F. Alejandro¹, Ampuero, A. Camila¹, Morales, G. Ignacio², Morales, T. Dagoberto³
Source:	Andes Pediatrica. Feb2025, Vol. 96 Issue 2, p1-9. 9p.
Subjects:	PHEOCHROMOCYTOMA, HYPERTENSIVE crisis, PERIOPERATIVE care, AMBULATORY blood pressure monitoring, CRITICAL care medicine, PEDIATRICS, ADRENALECTOMY
Abstract (English):	Pheochromocytoma is a rare neuroendocrine tumor in pediatrics. It usually appears in the context of a genetic syndrome and is a potentially curable cause of secondary arterial hypertension. Perioperative management of blood pressure (BP), as well as other clinical aspects, is a challenge for the health team. Objective: To emphasize therapeutic particularities specific to Intensive Care and perioperative care of patients with pheochromocytoma. Clinical Case: An 8-year-old schoolboy with a one-year history of headache associated with precordalgia and hyperhidrosis. He was hospitalized for hypertensive emergency secondary to a confirmed diagnosis of right pheochromocytoma. Six weeks later, a successful laparoscopic adrenalectomy was performed after alpha adrenergic blockade with doxazosin associated with propranolol and calcium antagonists. Von Hippel-Lindau disease (type 2C) was confirmed, heterozygous variant c.482G>A in the VHL gene. The patient had an uneventful evolution until the time of follow-up, one year after surgery. Conclusion: BP control is crucial prior to surgery, with a1 adrenergic blockers and calcium channel antagonists being the fundamental therapeutic measures. b-blockers are reserved for the management of secondary tachycardia. Intraoperatively, it is essential to be prepared for variations in heart rate and BP both during anesthetic induction and tumor manipulation. [ABSTRACT FROM AUTHOR]
Abstract (Spanish):	El feocromocitoma es un tumor neuroendocrino infrecuente en pediatría. En pediatria habitualmente aparece en el contexto de un síndrome genético y constituye una causa potencialmente curable de hipertensión arterial secundaria. El manejo perioperatorio de la presión arterial (PA), como de otros aspectos clínicos, son un desafío para el equipo de salud. Objetivo: Enfatizar particularidades terapéuticas propias de Cuidados Intensivos y perioperatorios del paciente con feocromocitoma. Caso Clínico: Escolar de 8 años con historia de un año de evolución de cefalea asociado a precordalgia e hiperhidrosis. Fue hospitalizado por emergencia hipertensiva secundaria a diagnóstico confirmado de feocromocitoma derecho. Luego de seis semanas se realizó en forma exitosa suprarrenalectomía laparoscópica previo bloqueo a adrenérgico con doxazosina asociado a propranolol, y antagonistas del calcio. Se confirmó enfermedad de Von Hippel-Lindau (tipo 2C) variante heterocigota c.482G>A en el gen VHL. Presentó una evolución sin complicaciones hasta el momento de su seguimiento, un año después de la cirugía. Conclusión: El control de la PA es crucial previo a la cirugía, siendo los bloqueadores a1 adrenérgicos y los antagonistas de los canales de calcio las medidas terapéuticas fundamentales. Los b-bloqueadores se reservan para el manejo de la taquicardia secundaria. En el intraoperatorio es esencial estar preparado para las variaciones del ritmo cardíaco y PA tanto en la inducción anestésica como en la manipulación tumoral. [ABSTRACT FROM AUTHOR]
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Database:	MedicLatina

Description
Abstract:	Pheochromocytoma is a rare neuroendocrine tumor in pediatrics. It usually appears in the context of a genetic syndrome and is a potentially curable cause of secondary arterial hypertension. Perioperative management of blood pressure (BP), as well as other clinical aspects, is a challenge for the health team. Objective: To emphasize therapeutic particularities specific to Intensive Care and perioperative care of patients with pheochromocytoma. Clinical Case: An 8-year-old schoolboy with a one-year history of headache associated with precordalgia and hyperhidrosis. He was hospitalized for hypertensive emergency secondary to a confirmed diagnosis of right pheochromocytoma. Six weeks later, a successful laparoscopic adrenalectomy was performed after alpha adrenergic blockade with doxazosin associated with propranolol and calcium antagonists. Von Hippel-Lindau disease (type 2C) was confirmed, heterozygous variant c.482G>A in the VHL gene. The patient had an uneventful evolution until the time of follow-up, one year after surgery. Conclusion: BP control is crucial prior to surgery, with a1 adrenergic blockers and calcium channel antagonists being the fundamental therapeutic measures. b-blockers are reserved for the management of secondary tachycardia. Intraoperatively, it is essential to be prepared for variations in heart rate and BP both during anesthetic induction and tumor manipulation. [ABSTRACT FROM AUTHOR]
ISSN:	24526045
DOI:	10.32641/andespediatr.v96i2.5458