Angiomiolipoma hemorrágico espontáneo asociado a esclerosis tuberosa que conduce a nefrectomía urgente – Reporte de un caso y revisión de la literatura
DOI:
https://doi.org/10.48193/revistamexicanadeurologa.v80i4.617Palabras clave:
Hemorragia, riñón, neoplasias, angiomiolipomaResumen
Descripción: Acudió una mujer de 34 años a urgencias con dolor abdominal moderado en hipocondrio derecho (duración 3h), acompañado de hematuria y distensión abdominal progresiva y dolorosa. La tomografía reveló una tumoración sólida heterogénea de 14 cm, en riñón derecho, con hemorragia en su interior. Por datos de bajo gasto hemodinámico se transfundieron hemoderivados y mantuvo en vigilancia, persistiendo con descenso de hemoglobina hasta 7.2 gr/dl, por lo que se sometió a angioembolización selectiva urgente, tras lo cual remitió temporalmente el sangrado, recurriendo con hematuria y choque hipovolémico, que derivó en tratamiento quirúrgico definitivo mediante nefrectomía total. Su recuperación transcurrió sin incidentes. El examen histopatológico reveló un angiomiolipoma renal con hemorragia extensa.
Relevancia: El síndrome de Wünderlich (Angiomiolipoma hemorrágico espontáneo) es una entidad rara cuya complicación más grave e indeseable es la hemorragia refractaria, que puede ser mortal.
Implicaciones clínicas: Es importante la intervención temprana en pacientes con angiomiolipoma hemorrágico para prevenir potenciales complicaciones como hemorragia masiva y poner en riesgo la vida del paciente.
Conclusiones: En caso de angiomiolipoma con hemorragia intratumoral y síntomas del síndrome de Wünderlich, la nefrectomía total resulta buena opción para preservar la vida del paciente, después del manejo conservador y/o angioembolización selectiva.
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