| Abstrakt: |
Introduction: PHACES syndrome is a rare neurocutaneous vascular disorder presenting in childhood, and can be associated with cardiovascular defects including aortic arch anomalies. There are several reports of postoperative spinal cord ischemia complicating aortic coarctation repairs in children. We describe what we believe is the first reported use of intraoperative cerebral and spinal cord neuromonitoring in pediatric cardiac surgery to mitigate the risk of neural ischemia during a high risk coarctation repair in a child with PHACES syndrome. Case report: A 22 months' old child presented with a long segment coarctation of the distal aortic arch and descending aorta and an abnormal branching pattern of the head and neck vessels. Cerebral and spinal cord blood supply were via the the left carotid artery, an isolated aberrant left verterbral artery, and an aberrant right subclavian artery giving rise to the right veretbral artery. Furthermore, the cord was supplied by multiple segmental radicular arteries arising from the cervical, intercostal, and lumbar branches from the descending thoracic and abdominal aorta. Intraoperative cerebral oximetry and neurophysiology monitoring were utilised to assess neural function during the surgical repair and to ensure early detection and immediate rectification of cerebral and/or cord ischemia during the surgical repair. We utilised near-infrared spectroscopy (NIRS) cerebral oximetry as well as neurophysiology monitoring of the upper and lower limbs using bilateral stimulating electrodes for somatosensory-evoked potentials (SEPs), motor-evoked potentials (MEPs) and electromyography (EMG), with scalp (cortical) recording electrodes. The first clamp was placed proximal to the left subclavian artery. The second clamp was placed proximal to the aberrant right sublavian artery. Due to the threatened cerebral and cord perfusion upon the application of the aortic cross clamps, two cardiopulmonary bypass cannulas were used successfully to serve as a Gott shunt, diverting blood from the ascending aorta to the distal thoracic aorta, bypassing the segment undergoing the repair and maintaining distal perfusion. Results: This technqiue allowed for uninterrupted cerebral and spinal cord perfusion via the left carotid, the aberrant left vertebral (pre-clamp) and the aberrant right sublavian arteries (post-clamp) respectively during the repair. Cerebral and cord perfusion were monitored and neural function confirmed as intact throughout the surgery by way of the above techniques. Careful attention should be paid to cerebral and spinal cord perfusion during surgery involving the aortic arch. This case presented a multitude of challenges in view of the complex arterial anatomy and required thinking 'outside of the box' and cross-disciplinary collaboration. Unlike cerebral perfusion, monitoring of spinal cord perfusion is not as straightforward. Multimodal intraoperative neurophysiology, commonly used in neurosurgery and vascular surgery, can play a critical role in early detection of impaired cerebral and cord perfusion, and guide decision-making in real time to modify management and mitigate the risk. To our knowledge, this is the first reported case of intraoperative neuromonitoring in pediatric cardiac surgery. We suggest this approach may be used by clinicians in cases which present similar anatomical and physiological challenges, with the aim of enhancing perioperative safety and quality of care. [ABSTRACT FROM AUTHOR] |
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