Anesthetic management in a child with moya-moya disease and sickle cell anemia: case report / Tratamiento anestésico en niño con enfermedad de moya-moya y anemia falciforme: caso clínico

Objective. The objective of this case report is to describe the anesthetic management in a child with moya-moya disease and sickle cell anemia provided in a tertiary hospital. Case. A 6 year-old patient, diagnosed with moya-moya disease and sickle cell anemia, both conditions associated with a greater incidence of intracranial ischemic events, with a history of two strokes of the ischemic subtype, was submitted to general anesthesia for the execution of multiple cranial burr holes in order to produce the neovascularization in poorly perfused regions. There were no complications in the perioperative period and the child was discharged from the hospital on the second postoperative day. Conclusion. Although scarcely described in the medical literature, the anesthetic management in a patient with moya-moya disease must ensure the maintenance of cerebral blood flow, normocapnia and the appropriate mean arterial pressure. In a patient with sickle cell disease, an adequate tissue perfusion, adequate oxygenation and hydration and strict pain control are to be primarily expected. The anesthesiologist is expected to know the physiopathology of both conditions to provide the best outcome for these patients (AU)

Objetivo. El objetivo de este caso clínico es el de describir el tratamiento anestésico en un niño con la enfermedad de moya-moya y anemia falciforme en un hospital terciario. Caso. Se trataba de un paciente de 6 años de edad, diagnosticado de enfermedad de moya-moya y anemia falciforme. Ambas condiciones se asocian a una elevada incidencia de episodios de isquemia cerebral. El paciente presentaba como antecedentes médicos 2 accidentes cerebrovasculares isquémicos. Se procedió a la administración de la anestesia para la realización de múltiples orificios craneales, y así favorecer la neovascularización en regiones cerebrales previamente hipoperfundidas. Conclusión. Aunque escasamente descrito, el procedimiento anestésico aplicado en el paciente con enfermedad de moya-moya debe asegurar la estabilidad del flujo de sangre en el cerebro, normocapnia y el apropiado control de la presión arterial. En un paciente con anemia falciforme, la adecuada perfusión tisular, oxigenación e hidratación y el estricto control del dolor son los primeros objetivos a conseguir. El anestesiólogo debe conocer la fisiopatología de ambas condiciones para proveer el mejor cuidado de este tipo de pacientes (AU)

Anesthetic management in a child with moya-moya disease and sickle cell anemia: Case report.

OBJECTIVE: The objective of this case report is to describe the anesthetic management in a child with moya-moya disease and sickle cell anemia provided in a tertiary hospital. CASE: A 6 year-old patient, diagnosed with moya-moya disease and sickle cell anemia, both conditions associated with a greater incidence of intracranial ischemic events, with a history of two strokes of the ischemic subtype, was submitted to general anesthesia for the execution of multiple cranial burr holes in order to produce the neovascularization in poorly perfused regions. There were no complications in the perioperative period and the child was discharged from the hospital on the second postoperative day. CONCLUSION: Although scarcely described in the medical literature, the anesthetic management in a patient with moya-moya disease must ensure the maintenance of cerebral blood flow, normocapnia and the appropriate mean arterial pressure. In a patient with sickle cell disease, an adequate tissue perfusion, adequate oxygenation and hydration and strict pain control are to be primarily expected. The anesthesiologist is expected to know the physiopathology of both conditions to provide the best outcome for these patients.