Hydrocephalus-induced neurogenic stunned myocardium and cardiac arrest in a child: completely reversed with CSF diversion

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Neurogenic stunned myocardium (NSM) is a potentially fatal cause of sudden cardiogenic dysfunction due to an acute neurological event, most commonly aneurysmal subarachnoid hemorrhage in adults. Only two pediatric cases of hydrocephalus-induced NSM have been reported. Here the authors report a third case in a 14-year-old boy who presented with severe headache, decreased level of consciousness, and shock in the context of acute hydrocephalus secondary to fourth ventricular outlet obstruction 3 years after standard-risk medulloblastoma treatment. He was initially stabilized with the insertion of an external ventricular drain and vasopressor treatment. He had a profoundly reduced cardiac contractility and became asystolic for 1 minute, requiring cardiopulmonary resuscitation when vasopressors were inadvertently discontinued. Over 1 week, his ventricles decreased in size and his cardiac function returned to normal. All other causes of heart failure were ruled out, and his impressive response to CSF diversion clarified the diagnosis of NSM secondary to hydrocephalus. He was unable to be weaned from his drain during his time in the hospital, so he underwent an endoscopic third ventriculostomy and has remained well with normal cardiac function at more than 6 months’ follow-up. This case highlights the importance of prompt CSF diversion and cardiac support for acute hydrocephalus presenting with heart failure in the pediatric population.

ABBREVIATIONS EKG = echocardiography; ETV = endoscopic third ventriculostomy; EVD = external ventricular drain; ICP = intracranial pressure; NSM = neurogenic stunned myocardium; SAH = subarachnoid hemorrhage; VP = ventriculoperitoneal.

Article Information

Correspondence Roy W. R. Dudley: Montreal Children’s Hospital, McGill University, Montréal, QC, Canada. roy.dudley@mcgill.ca.

INCLUDE WHEN CITING Published online April 19, 2019; DOI: 10.3171/2019.2.PEDS18711.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.

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    Chest radiograph taken at presentation, showing diffuse pulmonary edema with a normal cardiac silhouette and no pleural effusion or lobar consolidation.

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    A and B: Axial noncontrast CT images taken at presentation, demonstrating dilated lateral ventricles and fourth ventricle with prominent transependymal edema. C: Sagittal noncontrast CT image taken at presentation, demonstrating tonsillar herniation and compression of the medulla against the clivus. D: Sagittal T1-weighted MR image obtained 3 months before presentation, demonstrating no tonsillar herniation, stable ventricle size compared to its appearance on a previous scan, and no tumor recurrence.

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    Axial T2 FLAIR MR images (A and B) acquired on postadmission day 1, demonstrating dilatation of the lateral and fourth ventricle with transependymal edema affecting the frontal and occipital horns and the walls and floor of the fourth ventricle. Sagittal gadolinium-enhanced T1 MR image (C) demonstrating absence of tonsillar herniation post-CSF diversion on postadmission day 1. Sagittal T2 MR images demonstrating a flow artifact through the floor of the third ventricle after ETV (E), suggesting a patent ventriculostomy, which was not seen before ETV (D). Axial T2 FLAIR MR image (F) taken 3 months postoperatively, showing a significant decrease in ventricular size and resolved transependymal edema.

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