Case ReportFirst Case of Autonomic Dysreflexia Following Elective Lower Thoracic Spinal Cord Transection in a Spina Bifida Adult
Introduction
Autonomic dysreflexia (AD) is an acute syndrome of excessive, uncontrolled sympathetic output with potentially serious consequences in patients who have spinal cord injury (SCI). Typically; the syndrome is characterized by lesions at or above the level of sympathetic splanchnic outflow (T6 level).1
AD is the result of an exaggerated autonomic response to pain or other triggers below the level of the SCI, resulting in an increase in blood pressure along with other compensatory symptoms. The most common symptoms of AD are sweating, pounding headache, tingling sensation on the face and neck, blotchy skin around the neck, and piloerection (goose bumps). Not all the symptoms always appear at once, and their severity may vary. AD is considered a potentially life-threatening condition and a medical emergency requiring immediate attention; untreated and extreme cases may lead to stroke and/or death.2, 3
Few procedures are as inherently displeasing to neurosurgeons as the deliberate transection of the spinal cord. Nevertheless, in certain highly selective cases, the surgical division of distally nonfunctional spinal cord elements as a measure to protect proximal functional segments can prove effective.3 Currently, there are limited data on various aspects of the procedure, including postoperative complications, despite its being considered the most common standard for the treatment of symptomatic repetitively tethered cord in spina bifida patients with paraplegia.
Although AD is discussed in the literature as a possible complication of surgical spinal cord transection, it has yet to be reported in the literature. To the best of our knowledge, this case will be the first report describing AD as a possible complication following therapeutic spinal cord transection.1 We hope this case can serve to supplement the present data and aid in the differential diagnosis and surgical and medical decision making of AD resulting from intentional spinal cord transection in spina bifida patients.
Section snippets
Case Description
A 51-year-old male with history of complex spina bifida presented with an active cerebrospinal fluid (CSF) leakage from the lumbar wound and severe intractable back pain. He was born with a complex and large myelomeningocele that was left unrepaired with an initial intention of management via palliative care. Over the years, a layer of abnormal epithelium formed over the placode, and he did relatively well for 50 years without intervention until developing the aforementioned symptoms and sought
Discussion
AD is a potentially serious consequence in patients who have SCI with lesions at or above the level of sympathetic outflow of the most rostral of the thoracic splanchnic nerves4 (Figure 2). The most common symptoms of AD are related to the interruption of descending inhibition of sympathetic motor activity and the consequent development of hyperresponsiveness of peripheral receptors. Such symptoms and signs include sweating; pounding headaches; tingling sensations of the face and neck; blotchy,
Conclusion
Elective transection of the spinal cord is an extreme procedure reserved for a highly selective set of indications. Appropriately, there are limited data on the topic. To the best of our knowledge, this is the first case of AD following elective transection of the spinal cord at the thoracolumbar junction. The case highlights AD as a potential major side effect from such an operation, as well as its successful management. If unrecognized and untreated, AD can cause significant distress and
Acknowledgments
We would like to thank Cyrus Kafai, M.S., and Alex Pham, B.S., for their support writing this manuscript.
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Conflict of interest statement: The authors have no financial interest to declare in relation to the content of this article. Material support was provided by Ochsner Medical Center.
Juanita Garces and Mansour Mathkour are the first authors and contributed equally.