Neuroblastoma (NB) is one of the most common solid tumors of infancy. It can present with a variety of symptoms related to size and/or metabolic activity of the tumor. While affected children almost invariably have elevated circulating catecholamine (CCM) levels, it is rare for stigmata of CCM excess to be the hallmark of the clinical presentation. We present a novel approach to periop preparation of a 16-day old full-term neonate who presented in cardiogenic shock in the setting of a 5x5 cm CCM secreting NB. While previously published cases of NB causing sympathetic surge have described using phenoxybenzamine for pre-op alpha blockade, we successfully managed this patient’s pre-op state with a labetalol infusion alone. To our knowledge this is the first case reported in which the CCM secretion caused overt heart failure in an infant.
The patient initially presented to an OSH ER with several days of lethargy and anorexia. In the ER, she was found to be hypothermic, hypogylemic , bradycardic, and subsequently went into full cardiac arrest. She was successfully resuscitated and transferred to our institution. An echocardiogram revealed biventricular hypertrophy and biventricular cardiomyopathy with reduced systolic function. An abdominal ultrasound, followed by MRI suggested the diagnosis of NB, which was confirmed with a . Urine VMA assay.
In the PICU she remained intubated, receiving inodilator support with milrinone. She developed ectopic atrial tachycardia with ventricular response rates as high as 210 bpm. A labetalol infusion was started in order to prepare her for surgical excision of her tumor. She remained on the infusion for several days. Once her response to alpha and beta blockade with labetalol was deemed adequate, she presented to the OR for surgical excision.
Anesthesia was induced with sufentanil, sevoflourane and vecuronium. Anesthesia was maintained with Precedex and sufentanil infusions. We discontinued labetalol infusion as we were achieving hemodynamic stability within our goals from our anesthetic agents, and milrinone was maintained for inotropy. The patient remained stable throughout the resection, with minimal blood loss and without evidence of CCM surges or withdrawal during tumor manipulation and resection.
The patient did well in the postop period. She was discharged on POD13. On 6 month follow-up, patient had improvement in her tachycardia induced cardiomyopathy. At 1 year follow-up, she was transitioned off all meds, remained asymptomatic and was demonstrating normal growth and developmental milestones.
